Ovary-nontumor

Last revised 20 April 2009

Last major update February 2009

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Table of Contents

Primary references, embryology, normal anatomy, normal histology

 

Gonadal dysgenesis: general, pure, mixed, female pseudohermaphroditism, testicular feminization, true hermaphroditism, Turner’s syndrome

 

Inflammatory disorders: abscess, autoimmune oophoritis, eosinophilic perifolliculitis, giant cell arteritis, gonorrhea, granulomatous inflammation, microsporidia, pelvic inflammatory disease

 

Non-neoplastic cysts/other: DD of small cystic follicles, calcification, cholelithiasis, corpus luteum cyst, developmental cyst, ectopic decidual reaction, endometriosis, endosalpingiosis, epidermoid cyst, follicular cyst, germinal inclusion cyst, heterotopic ovarian splenoma, hyperreactio luteinalis, hyperthecosis, hypothyroidism, large solitary luteinized follicular cyst of pregnancy and puerperium, massive edema, ovarian pregnancy, polycystic ovary disease, postoperative carbon pigment granuloma, pregnancy luteoma, rete ovarii cyst, splenic gonadal fusion, stromal hyperplasia, supernumerary ovaries, torsion, uterus-like mass

 

See also Ovary-tumor chapter

 

Primary references - Ovary-nontumor chapter

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AJCC Cancer Staging Manual (6th Ed)

American Journal of Clinical Pathology, January 1975 to February 2009

American Journal of Surgical Pathology, March 1977 to February 2009

Archives of Pathology and Laboratory Medicine [always free full text and no registration], January 1976 to January 2009

Biomed Central [always free full text and no registration], 27 May 1999 to 3 February 2009

Human Pathology, December 1971 to February 2009

Modern Pathology [free full text and no registration after 1 year], March 1988 to February 2009

Rosai, J:  Ackerman’s Surgical Pathology (9th Ed), Mosby, 2004

Scully: Tumors of the Ovary, Maldeveloped Gonads, Fallopian Tube, and Broad Ligament (AFIP Atlas of Tumor Pathology, Series 3, Vol 23); 1999

Tavassoli: Tumours of the Breast and Female Genital Organs (WHO, 2003)

Websites: PathoPic

Virtual slides - USCAP, vSlides

Journal search terms: each disease entity listed - last searched January 2009

 

Images needed - click here

 

Please refer to these primary references for more detailed discussions and photographs

 

Embryology of Ovary - Ovary-nontumor chapter

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First, germ cells migrate from yolk sac to posterior body wall at level of T10 to enter Genital Ridge

Then germ cells differentiate into oogonia, then primary oocytes, then arrest until puberty

Then ovary descends to pelvis along gubernaculum (attached inferiorly to inguinal region); gubernaculum becomes part of uterine wall at entry of fallopian tube, and persists in adults as ovarian and round ligaments

Future ovarian surface epithelium (OSE) forms part of coelomic epithelium, the mesoderm derived epithelial lining of intraembryonic coelom; overlies presumptive gonadal area and gives rise to gonadal blastema

At 10 weeks to 5 months, OSE becomes multistratified and papillary on well defined basement membrane, but reverts to single layer by term

OSE is negative for CA125, unlike other coelomic derivatives

Gonadal development is influenced by both male and female promoting signals (Mol Endocrinol 2008;22:1)

Diagrams: fetal development

References: Wikipedia

 

Normal anatomy - Ovary-nontumor chapter

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Paired ovoids close to lateral pelvic wall, behind broad ligament, anterior to rectum

Connected to broad ligament by mesovarium (double fold of peritoneum), to uterine cornu by utero-ovarian ligament, to lateral pelvic side wall by infundibulopelvic (suspensory) ligament

Premenopausal ovaries are each 4 cm long and weigh 5-8 g
Neonates often have cysts, which spontaneous resolve (
J Pediatr Endocrinol Metab 2007;20:397)

Lymphatic drainage to para-aortic, internal iliac, external iliac, common iliac, sacral, obturator, pelvic, retroperitoneal, inguinal nodes image (AFIP)

Function first described by Reinier de Graaf (Arch Pathol Lab Med 2000;124:1115)

Ovulation: induces cyclic rupture and regenerative repair of the ovarian coelomic epithelium

Gross images: drawing #1#2#3#4sagittalovary about to release an egg 

References: Wikipedia

 

Normal histology- Ovary-nontumor chapter

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Covered by surface epithelium (modified mesothelium, also called coelomic or germinal epithelium, Reprod Biol Endocrinol 2006 Aug 21;4:42), which is one layer of flat to cuboidal mesothelial-type cells, which appear to actively participate in ovulatory rupture and repair process; are closely related to mullerian duct lining epithelium

Follicles: primordial, maturing (primary, secondary, tertiary, graafian), atretic; corpora lutea, corpora albicantia; 400K primordial follicles containing primary oocytes are present at birth in ovarian stroma (100K at gestational age 15 weeks, 680K at 8 months, Fertil Steril 2007;88:675); follicular decay appears to advance with increasing age (Hum Reprod 2008;23:699)

primordial follicle: travels from yolk sac endoderm to ovary, develops into oogonia and oocytes, arrests at prophase of mitosis; neo-oogenesis may occur in adults (Endocrine 2005;26:301)

maturing follicle: oocyte with granulosa layer; lacks reticulum; contains Call-Exner bodies (rosette-like formations with central filamentous / eosinophilic material consisting of excess basal lamina), theca cells (within follicle are luteinized and produce sex hormones; external to follicle are very cellular); immunoreactive for vimentin, keratin, desmoplakin

Corpus luteum: 2 cm, round, yellow, lobulated structure with cystic center; has luteinized granulosa and theca cells; in pregnancy is larger, bright yellow with prominent central cavity, hyaline droplets and calcification

Corpus albicans: remnant of corpus luteum

 

Hilus (hilar) cells: in ovarian medulla, round to polygonal, epithelial appearing, presumed vestigial remnant of gonad from its "ambisexual" phase; produce steroids; resemble Leydig cells of testis; may produce masculinizing tumors (hilar cell tumors); closely associated with large hilar veins and lymphatics and may protrude within their lumina; also associated with nerves; may contain Reinke’s crystalloids, lipid, lipochrome pigment; resemble steroid cells by EM with microtubular smooth endoplasmic reticulum, mitochondria with tubular cristae

Hilar cell hyperplasia: associated with hCG administration, pregnancy, choriocarcinoma

AFIP micro images: hilus cell hyperplasia #1-large nodular masses of hilus cells are present, one surrounding the rete ovarii#2 in post-menopausal woman-the hilus cells have bizarre shapes and abundant eosinophilic cytoplasm, some nuclei are enlarged and hyperchromaticnodular hilar cell proliferation

Hilar mesonephric rests: case report of association with nonteratoid prostatic differentiation in 70 year old woman (Am J Surg Pathol 1999;23:232)

 

Normal histology- Ovary-nontumor chapter (continued)

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Rete ovarii: counterpart of rete testis; clefts, tubules, cysts, papillae lined by epithelium, surrounded by spindle cell stroma

Stroma: in whorls/storiform pattern, resembles fibroblasts, surrounded by dense reticulin network; stroma contains luteinized stromal cells, decidual cells, smooth muscle, fat, neuroendocrine cells, endometrial stroma-like cells

Walthard cell nests: urothelial-type epithelium, variable mucin, cystic/solid, in mesovarium, mesosalpinx, ovarian hilus

Ovary at birth and puberty: prominent cystic follicles present

Post-menopausal ovary: thick walled medullary and hilar vessels, also granulomas and hyaline scars

Drawings: #1#2#3follicle maturation #1#2

Gross images: corpus luteum #1#2-hemorrhagic#3-cystic#4-with follicular cysts

Micro images: primordial and antral folliclesprimordial follicles #1#2tertiary follicleruptured follicle #1#2atretic follicle #1#2serosahiluscorpus luteum-hemorrhagic

AFIP images: normal - low columnar cells are separated from cortical stroma by basement membraneovarian stroma is composed of spindle cells with scant cytoplasm in a storiform patternluteinized stromal cells have moderate eosinophilic cytoplasm, round nucleus and prominent nucleolus

adrenocortical rest in mesosalpinx - top image shows fibrous capsule and zonation into glomerulosa, fasciculata, and reticularis layers; bottom image shows cells of zona glomerulosa and zona fasciculata with spongy (lipid- rich) cytoplasm

corpus luteum of menstruation - larger cells are granulosa lutein cells, smaller cells with clear (lipid- rich) cytoplasm are theca lutein cellsinvoluting corpus luteum has granulosa lutein cells filled with (lipid) vacuoles and containing pyknotic nuclei

corpus luteum of pregnancy - central hyaline bodies are presentfoci of calcification in involuting corpus luteum

follicles - 4 primordial follicles (left), 2 primary follicles (center) and a secondary follicle (right) are presentsecondary follicle-stratified granulosa cells surround the oocyte enveloped by its zona pellucida, theca interna layer is not yet apparentgraffian follicle-cumulus oophorus, containing the oocyte and its zona pellucida, projects into the antrumgranulosa cells of proliferating follicle exhibit marked mitotic activity

follicles - mature - #1-layers from top to bottom are antrum, a layer of granulosa cells with a single Call- Exner body, a layer of luteinized theca interna cells, an ill- defined theca externa, and the ovarian stroma#2-reticulin stain shows abundant reticulin in theca interna layer but no reticulin in granulosa layer#3-theca externa has numerous mitotic figures

 

Normal histology- Ovary-nontumor chapter (continued)

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hilus cells - darkly staining hilus cells are adjacent to a small nerve fiber (top)rounded hyaline bodies are present at the top, Reinke crystals are seen below with 2 parallel strands of a nerve

mesothelial hyperplasia - present between outer surface of an ovarian serous borderline tumor (left) and vascular adhesion (right))mesothelial cells have abundant pale eosinophilic cytoplasm, small focus of calcification is evidentnumerous small tubular structures and cords of mesothelial cells are growing in a parallel array

miscellaneous - surface epithelial stromal proliferationvacuolar change within surface epithelial inclusion glandsartifact-granulosa cells in blood vessel lumen #1#2infarcted appendix epiploica attached to ovarian surface, with focal calcification

pregnancy related changes - granulosa cell proliferation #1-an atretic follicle is filled with a proliferation of granulosa cells growing in a pattern resembling a microfollicular granulosa cell tumor#2-the granulosa cell layer of a cystic atretic follicle is replaced by solid tubules resembling a Sertoli cell tumor. the tubules are surrounded by a layer of theca lutein cellsectopic decidua #1-one cell is vacuolated, somewhat resembling a signet- ring cell#2-large nodules were present on the ovarian and peritoneal surfaces, the cells have abundant eosinophilic cytoplasm and central, pale, round nuclei

post-menopausal - atrophic ovary has scant cortical stroma, most of medulla is filled by corpora albicantiacortical granuloma consists of loose cluster of spindle cells, epithelioid cells and lymphocytes

rete ovarii - network of branching tubules in cellular stroma, which may resemble ovarian stromapolypoid projections with fibrous cores occupy the lumen of a cystically dilated portion; epithelium varies from flat to cuboidal to columnar

surface epithelial inclusion glands - arise from invaginations of surface epithelium into cortical stromalined predominantly by columnar cells

Walthard nest - composed of transitional cells that abut the ovarian stroma

Virtual slides: normal ovary-infant

Videos: #1corpus albicanscorpus luteum

Positive stains: keratin, EMA, calretinin (granulosa and theca cells), actin/desmin (stroma cells), CD56, WT1, ER-beta, PR, Ber-EP4, CA-125, vimentin, desmoplakin

Negative stains: CD10

 

 

Gonadal dysgenesis

Gonadal dysgenesis-general - Ovary-nontumor chapter

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A disorder of sex development, defined as a congenital condition with atypical development of chromosomal, gonadal or anatomic sex

Disorders of sex development are either female pseudohermaphroditism (46XX with 2 ovaries), male pseudohermaphroditism (46XY with two testes), true hermaphroditism (ovotestis present) or gonadal dysgenesis (either pure with normal 46XX or 46XY chromosomes and bilateral streak gonads, or mixed with streak gonad)

 

Pure (complete) gonadal dysgenesis - Ovary-nontumor chapter

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Either 46XX or 46XY

 

Pure gonadal dysgenesis - 46XX - Ovary-nontumor chapter

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Phenotypic female, 46XX, but no functional ovaries are present to induce puberty (may have streak ovaries)

May be familial (Am J Med Sci 1980;280:157)

Occasionally due to mutation in FSH receptor (Cell 1995;82:959)

Perrault syndrome: with sensorineural deafness

Laboratory: low serum estrogen and progesterone (since no functional ovaries), high serum FSH and LH

Treatment: estrogen and progesterone therapy

References: OMIM 233300, Wikipedia

 

Pure gonadal dysgenesis - 46XY - Ovary-nontumor chapter

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Also called Swyer syndrome

Phenotypic female, hypoplastic (streak) gonads without germ cells,

Causes: mutation in SRY, the sex-determining region of the Y chromosome in 10-15%

Presents with primary amenorrhea (delayed puberty), since no functional gonads are present to induce puberty

May develop pubic hair through androgens produced from adrenal gland

Absence of Mullerian inhibiting factor (various causes) leads to absence of testosterone, which causes Wolffian duct regression and failure of male internal reproductive tracts to develop; absence of Mullerian inhibiting factor also allows Mullerian ducts to differentiate into oviducts and uterus

High risk of gonadoblastoma or germ cell tumor from gonads (Zhonghua Fu Chan Ke Za Zhi 2008;43:442); dysgerminoma may develop by age 10 years (BJOG 2008;115:737)

Laboratory: low serum estrogen and progesterone, high serum FSH and LH

Case reports: successful pregnancy using donor oocytes and oocyte transfer (Fertil Steril 2008;90:2015.e1)

Treatment: early excision of gonads (J Gynecol Obstet Biol Reprod (Paris) 2008 Jan 31 [Epub ahead of print]), estrogen and progesterone therapy

Diagrams: normal embryologic development

References: OMIM 306100, Gene Reviews

 

Mixed gonadal dysgenesis - Ovary-nontumor chapter

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A type of asymmetrical gonadal dysgenesis (usually testis plus streak gonad) due to chromosomal abnormality

Often mosaicism: 45X / 46XY, 45X / 46XX or 45X / 47XXY; may also have 46XX but missing part of one X chromosome

Patients with 45X mosaicism are often considered to have a variant of Turner’s syndrome

Phenotype may be ambiguous, male, female or intersex

Either (a) testis plus contralateral streak gonad, (b) testis and contralateral gonadal agenesis, (c) hypoplastic gonads with tubules in one gonad or (d) streak gonad with contralateral tumor

Usually also bilateral fallopian tubes, chromatin negative Barr bodies; occasionally vas deferens

Streak testis: streak tissue identified at periphery of differentiated testis

Streak gonad: ovarian-type stroma without differentiated gonadal structures

External genitalia: female with clitoromegaly, male with hypospadias or normal male

Phenotypic females may develop virilization at puberty, often complete; no breast development except with tumors

High risk for gonadoblastoma (30%) if Y chromosome material is present, which may obliterate testicular elements and cause incorrect diagnosis

Higher risk for other medical problems, including deficient immunoglobulin levels, aberrant bony development of inner ear structures, cardiovascular and renal anomalies

Laboratory: elevated FSH

Case reports: short stature in phenotypic male with 45X / 46XY mosaicism (Nat Clin Pract Endocrinol Metab 2008;4:524)

Treatment: excise gonads early to prevent tumors

Micro: prepubertal patients show normal immature testis; at/after puberty, tubules exhibit mild hypospermatogenesis to total sclerosis; streak gonad has ovarian stroma without primordial ovarian follicles; streak ovary is streak gonad with primordial follicles and primitive sex cord-like structures with or without germ cell components within the ovarian-type stroma, mimicking gonadoblastoma, granulosa cell tumor or Sertoli cell tumors

Micro images: mixed gonadal dysgenesis #1#2#3 (AFIP)-streak tissue and rete (top) are separated by tunica albuginea from testis (bottom)

Molecular: FISH may be helpful (Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2008;25:570); no mutations of SRY (Eur J Obstet Gynecol Reprod Biol 2004;115:55)

DD: true hermaphroditism (ovary has numerous primordial follicles containing primary oocytes, nature of internal or external genitalia is not relevant, Mod Path 2002;15:1013)

 

Female pseudohermaphroditism - Ovary-nontumor chapter

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Female genotype (46XX), female internal phenotype (two ovaries) with variable virilization

Two types: associated with congenital adrenal hyperplasia or not

 

Female pseudohermaphroditism associated with congenital adrenal hyperplasia - Ovary-nontumor chapter

46XX, Barr bodies are chromatin positive, autosomal recessive

Ovary with female ducts, variable virilized external genitalia, amenorrhea at puberty with virilization, markedly elevated 17 ketosteroids

Usually due to 21-hydroxylase or 11beta-hydroxylase deficiency; also other rare mutations (Arq Bras Endocrinol Metabol 2005;49:126), partial 17alpha-hydroxylase/17,20-lyase deficiency (Gynecol Endocrinol 2008;24:362)

Diagrams: steroid pathways #1#2

 

Female pseudohermaphroditism of nonadrenal cause - Ovary-nontumor chapter

46XX, Barr bodies are chromatin positive

Ovary with female ducts and variable virilized external genitalia; normal puberty

May be due to maternal exposure to progestins or androgens

17-keratosteroids and estrogen levels are normal

Case reports: with luteoma of pregnancy (Hum Reprod 2002;17:821)

References: eMedicine

 

Testicular feminization - Ovary-nontumor chapter

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Most common type of male pseudohermaphroditism

Also called androgen insensitivity syndrome

Male genotype (46XY), female phenotype due to end order defect (androgen insensitivity)

Patients present with amenorrhea or sterility; vagina but no uterus; also bilateral cryptorchid testes with nodular masses of immature tubules resembling Sertoli-Leydig tumor

9% develop tumors in cryptorchid testis, which should be removed after puberty

Case reports: 36 year old with abdominal seminoma (J Obstet Gynaecol Res 2004;30:109)

Gross images - AFIP: multiple hamartomas are present in a background of brown parenchyma.  Nodule at each upper (medial) pole is a mass of smooth muscle; the small white nodule at the lower (lateral) pole of the left testis was composed largely of fibromatous tissue and resembles an ovarian fibroma.  The remaining hamartomas contained Sertoli and Leydig cells and stroma; a small adnexal cyst is present at the lower (lateral) pole of the left testis

Micro images (AFIP): ovarian type stroma with a storiform pattern containing a few lutein-like cells envelops two small solid tubules containing Sertoli cellstubules containing immature Sertoli cells are separated by cells resembling Leydig cellstestis is composed almost entirely of stroma resembling ovarian stromahamartomas are composed of Sertoli and Leydig cells and lie in the upper left, testicular parenchyma is at lower right

References: Wikipedia, eMedicine

 

True hermaphroditism - Ovary-nontumor chapter

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Rare; ovarian and testicular tissue in same patient; either bilateral or unilateral ovatestes OR testis opposite ovary

All have uterus, most have fallopian tubes, some have vas deferens

Usually ambiguous genitalia (J Pediatr Endocrinol Metab 2001;14:421)

At puberty, 80% have gynecomastia, 50% menstruate

80% have chromatin positive Barr bodies

60% are 46XX only in blood cells with Y chromosome present in most of rest of cells

Hormonal levels are normal

Important to assign gender early since (a) usually no other developmental anomalies and (b) will have normal sexual and reproductive lives if remove gonad inappropriate for gender assignment

May be associated with transexualism (Urologiia 2008;2:14)

Case reports: due to tetragametic chimerism (fertilization of two ova by two sperm, followed by the fusion of the zygotes and the development of an organism with intermingled cell lines, Urology 2009;73:293), 16 year old phenotypic male with gynecomastia and pubertal arrest (Horm Res 2007;68:261)

Treatment: conservative gonadal surgery with long term followup (J Urol 2007;177:726)

Micro: ovary usually normal (prepubertal ovary has primordial follicles with primary oocytes with a few primary or antral follicles); prepubertal testis has immature seminiferous tubules lined by immature Sertoli cells and primitive germ cells, usually without spermatogonia

Micro images: ovatestis

DD: mixed gonadal dysgenesis (ovarian tissue, if present, lacks primordial follicles; internal/external genitalia are not relevant for distinguishing these two conditions)

 

Turner’s syndrome - Ovary-nontumor chapter

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Also called Ullrich-Turner syndrome

1/2500 live female births; increased frequency in abortuses or women with short stature

Have female ducts and external genitalia, but usually no pubertal development, 50% of chromosomes lack Barr bodies (the inactivated X chromosome)

Patients are 45X (also called 45X0) in some or all cells

Associated with nongonadal neoplasms including atypical polypoid adenomyoma of uterus, endometrial adenocarcinoma, leukemia and soft tissue tumors; also other medical disorders

Laboratory: markedly elevated serum FSH, reduced serum estrogen

Case reports: mother and daughter with nonmosaic Turner's syndrome (Fertil Steril 2004;82:923)

Treatment: growth hormone (to increase stature), estrogens; also search for hidden Y chromosome mosaicism (J Pediatr Endocrinol Metab 2006;19:1113); followup medical care for various conditions in adulthood (Endocr Rev 2002;23:120)

Clinical diagrams: classic clinical features

Clinical images: short webbed neck, cubitus valgus [elbows are turned in] and lymphedema

Micro: streak gonad with fibrous tissue resembling ovarian stroma

References: Wikipedia

 

 

Inflammatory disorders

Abscess - Ovary-nontumor chapter

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Nonspecific inflammation is usually due to tubal and endometrial involvement

Gross: large, loculated cyst with pus or secretion

Gross images (AFIP): tubo-ovarian abscess complicating colonic diverticulitis - the left ovary and tube have been transformed into a multicystic mass with a yellow liningovarian abscess associated with Crohn’s disease shows replacement of ovarian tissue by yellow soft tissue

Micro: cyst wall often contains ovarian stroma

Micro images (AFIP): xanthogranuloma - the ovarian stroma is replaced by collagenous tissue containing numerous chronic inflammatory cells 

 

Autoimmune oophoritis - Ovary-nontumor chapter

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Causes 10% of premature ovarian failure, defined as amenorrhea before age 40 years with elevated serum gonadotropins

Associated with Addison’s disease and hypothyroidism (Endocr Rev 1997;18:107)

In teenagers, associated with autoimmune polyendocrine syndrome type I or II (Wikipedia, Ann N Y Acad Sci 2008;1135:118)

Diagnosis: ovarian failure plus serum adrenal cortical or steroid cell antibodies (StCA), or serum antibodies to adrenal and ovarian steroidogenic enzymes; higher serum levels of total inhibin and inhibin B than other ovarian failure cases (J Clin Endocrinol Metab 2008;93:1263)

Laboratory: low estradiol, high FSH, normal/high inhibin A and B

Treatment: hormone replacement therapy; steroids are not standard treatment

Gross: cystic ovaries

Micro: lymphocytes and plasma cells infiltrate developing follicles and corpora lutea but not primordial follicles; preservation of granulosa cells

Micro images: lymphocytic oophoritis

References: Fertil Steril 2005;84:958, Johns Hopkins

 

Eosinophilic perifolliculitis - Ovary-nontumor chapter

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May cause autoimmune oophoritis

Case reports: 45 year old woman with ovarian cyst (J Reprod Med 2006;51:141), two middle aged women with cystic ovaries (Int J Gynecol Pathol 1993;12:360)

Gross: cystic ovaries

Micro: eosinophils, lymphocytes, macrophages and plasma cells

 

Giant cell arteritis - Ovary-nontumor chapter

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Part of generalized condition or isolated; usually elderly women

Common presentation is elderly woman with apparent ovarian or breast tumor and constitutional symptoms and elevated sedimentation rate (Medicine (Baltimore) 2000;79:349)

Micro images: epithelioid granulomas (arrows) of ovarian hilar arteriespresent in mesenteric vessels as incidental finding in colonic adenocarcinoma

References: Am J Surg Pathol 1986;10:696

 

Gonorrhea - Ovary-nontumor chapter

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Begins in Bartholin’s or other vestibular or periurethral glands; spreads to cervix, fallopian tubes, ovaries

A type of pelvic inflammatory disease (see below)

Micro: acute suppurative reaction, tubo-ovarian abscess

 

Granulomatous inflammation - Ovary-nontumor chapter

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Causes: actinomyces (associated with IUDs, Am J Clin Pathol 1977;68:622), Crohn’s disease (extension from bowel), Enterobius vermicularis, foreign materials (talc or starch, keratin from ruptured teratoma), post-surgical (including carbon, Hum Pathol 1996;27:1008), schistosomiasis, tuberculosis (hematogenous spread to ovary, also involves fallopian tube and uterus, Arch Gynecol Obstet 2008;278:359), xanthogranulomatous oophoritis (solid mass in long standing cases of inflammation with foamy macrophages, Indian J Pathol Microbiol 2003;46:80); rarely sarcoidosis

Case reports: due to microfibrillar collagen hemostat (Avitene) used to control bleeding (Arch Pathol Lab Med 1995;119:1161), due to malakoplakia (Obstet Gynecol 1987;69:537)

Micro: central suppuration and surrounding giant cells in Crohn’s diseasetuberculosis

AFIP images: actinomycosis - the ovary is replaced by tissue that was soft and yellow, a characteristic sulfur granule surrounded by polymorphonuclear leukocytes is on the righthistiocytes have abundant bubbly gray-pink cytoplasm and resemble signet-ring cells #1#2-histiocytes are mucicarmine+

 

Microsporidia - Ovary-nontumor chapter

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Case reports: disseminated microsporidiosis due to Encephalitozoon cuniculi involving ovary of Italian woman with AIDS (Mod Path 2002;15:577)

Micro images: gram stain

 

Pelvic inflammatory disease (PID) - Ovary-nontumor chapter

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Also called tubo-ovarian abscess

Pelvic pain, adnexal tenderness, fever, vaginal discharge

Due to gonorrhea, chlamydia, enteric bacteria or puerperal infections (from end of third stage of labor until uterus completely involutes at 3-6 weeks); usually polymicrobial

Usually begins in endometrium and is associated with tubal involvement, tubo-ovarian abscess or cyst

Complications are infertility, peritonitis, bacteremia or intestinal obstruction due to adhesions

Gross images: abscess #1#2

References: eMedicine, Wikipedia

 

 

Non-neoplastic cysts / other

DD of small, cystic follicles - Ovary-nontumor chapter

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Cystadenoma / cystadenocarcinoma, cystic corpus luteum, cystic granulosa cell tumor, endometriosis, epidermoid tumor, rete ovarii cyst, surface inclusion cyst

 

Calcification - Ovary-nontumor chapter

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Usually more important to radiologists than pathologists

Often in normal ovaries by ultrasound, but may be associated with neoplasms (Radiology 1996;198:415)

Most calcifications are found in stroma (Mod Pathol 2003;16:219)

Asymptomatic children may present with calcified abdominal mass (Radiology 1981;141:83)

Case reports: 50 year old with bilateral, multifocal ovarian stromal calcification (Arch Pathol Lab Med 1992;116:204), ossification adjacent to endometriosis (Clin Exp Obstet Gynecol 2007;34:113), heavily calcified hemangioma (Br J Radiol 2008;81:e269)

 

Cholelithiasis - Ovary-nontumor chapter

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Gallstones may be lost during laparoscopic cholecystectomy and adhere to ovary

Case reports: surgery 2-3 years previous (Arch Pathol Lab Med 2001;125:579, Arch Pathol Lab Med 1997;121:155), surgery > 7 years previous (J Reprod Med 2007;52:968)

Gross images: gallstone adherent to ovary

 

Corpus luteum cyst - Ovary-nontumor chapter

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Usually occurs during reproductive years, at end of menstrual cycle or during pregnancy

Note: normal corpus luteum is cystic

Case reports: ruptured corpus luteum cyst of pregnancy causing massive hemoperitoneum (J Pediatr Adolesc Gynecol 2007;20:97), spontaneous rupture of hemorrhagic corpus luteum cyst torsion (Kaohsiung J Med Sci 2003;19:75)

Gross: single, usually 2-6 cm, convoluted yellow lining; fluid may be bloody

Gross images: corpus luteum #1#2-hemorrhagic#3#4 

AFIP images: cyst is full of blood, probe at bottom right indicates point of rupturewall is thick and yellow and lining is smooth

Micro: inner lining of connective tissue, outer layer of large luteinized granulosa cells; pregnant patients have hyaline bodies and calcific foci within granulosa cells; when rupture, may be difficult to distinguish from endometriosis or ruptured ectopic pregnancy

Micro images: corpus luteum-hemorrhagic #1#2

AFIP images: layer of luteinized granulosa cells with abundant cytoplasm overlying small theca cells

 

Developmental cyst - Ovary-nontumor chapter

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From mesonephric (wolffian) or paramesonephric (mullerian) remnants

Common in ovarian hilus

Case reports: with serous borderline tumor (Histopathology 1983;7:601)

Mesonephric (wolffian) origin: cuboidal, nonciliated epithelium, on well developed basement membrane; has smooth muscle coat

Paramesonephric (mullerian) origin: resembling fallopian tube; taller, often ciliated epithelium with larger nuclei, inconspicuous basement membrane; has smooth muscle coat

 

Ectopic decidual reaction - Ovary-nontumor chapter

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Definition: response of stromal cells to high levels (systemic or local) of estrogen or progesterone from functioning corpus luteum

May occur as early as week 9 of gestation

Found in almost all ovaries at term (Am J Anat 1978;152:7)

May be associated with trophoblastic disease, pelvic radiation, other causes of increased estrogen / progesterone

Gross: tan nodules on ovarian surface, may be hemorrhagic

Micro: decidua usually within superficial cortical stroma or adhesions; resembles decidua elsewhere; some cells may resemble signet ring cells due to cytoplasmic vacuoles (Mod Pathol 2005;18 Suppl 2:S113)

Negative stains: keratin

 

Endometriosis - Ovary-nontumor chapter

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Ovary is most common site

Defined as endometrial glands and stroma outside the uterus

Usually associated with infertility (although mechanism is unclear, Fertil Steril 2008;90:247), remains active during reproductive years

Pain, occasionally massive ascites or perforation

Associated with clear cell or endometrioid carcinoma (Singapore Med J 1997;38:289), particularly if atypia (Hum Pathol 1988;19:1080)

Rarely presents as polypoid endometriosis which is usually also associated with nonpolypoid endometriosis, no atypia and no hypercellularity (Am J Surg Pathol 2004;28:285)

Case reports: with ovarian ossification (Clin Exp Obstet Gynecol 2007;34:113)

Gross: small, raised blue spots on ovarian surface with fibrous adhesions; chocolate cysts are due to repeated hemorrhage; rarely have granulomatous nodules attached to peritoneum or free within peritoneal cavity (Am J Surg Pathol 1988;12:390)

Gross images: chocolate cysthemorrhagic nodulesintraoperative image #1#2 with adhesions#3

AFIP images: the external surfaces of ovarian wedges show red, blue, and brown areas, some associated with fibrotic puckering #1#2cyst #1 has outer surface with many foci of red-brown discoloration and adhesions, contains chocolate- colored fluidcyst #2 has predominantly pale and smooth lining plus several foci of dark brown discoloration consistent with endometriosis

Micro: need 2 of 3 features of endometrial glands, endometrial stroma or hemorrhage; stromal cells have naked nuclei, are surrounded by reticulin and spiral arterioles; smooth muscle stroma is common; repeated hemorrhage may destroy stromal tissue; specimen may be composed of necrotic pseudoxanthomatous nodules; may see endometrial glandular atypia (atypical endometriosis), hyperplasia, carcinoma (endometrioid most common); rarely Liesegang rings (acellular, ringlike structures that may form near inflamed or necrotic tissue, Int J Gynecol Pathol 1998;17:358), stromal elastosis

 

Endometriosis - Ovary-nontumor chapter (continued)

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Micro images: endometrial glands and stroma in ovaryH&E and CD10smooth muscle actin+ wall of endometriotic cyst

AFIP images: endometrioid glands with variable size are within cellular endometrial-type stroma surrounded by denser ovarian stromathe wall of an endometriotic cyst contains pseudoxanthoma cells filled with hemofuscin pigmentdeeper portion of the wall of an endometriotic cyst is fibrotic, the subepithelial layer is composed of hemorrhagic cellular stromaLiesegang rings are scattered in debrispolyp composed mainly of cystic glands projects into the lumen of an endometriotic cysttwo pseudoxanthomatous necrotic nodules are seen at the top and bottom, an endometriotic gland is seen in the upper nodulea pseudoxanthomatous nodule is centrally necrotic with a surrounding granulomatous reactionpatient taking norethynodrel with mestranol has marked decidual change of stromaduring pregnancy, a gland is lined by cells with hyperchromatic, smudgy nuclei resembling Arias-Stella reaction; the adjacent stroma shows decidual change

Cytology images: cyst of endometriosis #1#2

Virtual slides: endometriotic cyst

Videos: #1

Molecular: Trisomy 1 and 7 and monosomy 9 and 17 are more common in ovarian versus nonovarian endometriosis (Mod Pathol 2006;19:1615)

Positive stains: CD10 (highlights endometrial cells, Arch Pathol Lab Med 2003;127:1003)

 

Endosalpingiosis - Ovary-nontumor chapter

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Arises from secondary mullerian system (pelvic and lower abdominal mesothelium)

Associated with ovarian serous tumors, usually borderline

May be difficult to distinguish from implants

Case reports: cystic mass involving ovary and full thickness of uterine fundus (Ultrasound Obstet Gynecol 2008;32:106), with psammoma bodies (J Reprod Med 1991;36:675)

Micro: benign glands and tubules lined by tubal type cells; calcifications or psammoma bodies are common (Mod Pathol 2003;16:219); smooth contours; variable papillae beneath the peritoneal surface

Micro images: site unknown;  omentum: CD34 and calretinin

AFIP images: glands lined by ciliated epithelium lie in fibrous stromaciliated, secretory and intercalated cells line the cystic space

DD: noninvasive or invasive ovarian implants

 

Epidermoid cyst - Ovary-nontumor chapter

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Also called epidermoid inclusion cyst

Very rare; often an incidental finding (Am J Obstet Gynecol 1976;124:523)

May be associated with monodermoid teratoma or Walthard nests

No skin adnexae or other tissues (or would be a mature cystic teratoma)

May be associated with squamous cell carcinoma (Clin Exp Obstet Gynecol 2005;32:265)

Case reports: with ovarian endometrioid carcinoma (Zentralbl Gynakol 2002;124:443)

Micro: smooth inner lining of stratified squamous epithelium with no evidence of hair or a Rokitansky tubercle (Int J Gynecol Pathol 1996;15:69); also focally cystic nests within cyst wall containing mucin and keratin debris (Am J Clin Pathol 1980;73:272)

 

Follicular cyst - Ovary-nontumor chapter

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Ovarian follicle matures during proliferative phase of menstrual cycle, then LH surge causes release of mature oocyte at mid-cycle, then follicle transforms to a corpus luteum; if no fertilization, corpus luteum typically atrophies

If no LH surge or no ovulation, follicle grows and becomes a cyst, which usually disappears within 2-3 menstrual cysts but may persist

Follicular cyst is common in reproductive years, usually asymptomatic or forms an adnexal mass

Pedicle may twist and cause hemorrhagic infarction

Associated with precocious puberty in children; also endometrial hyperplasia and metrorrhagia (cyst fluid may contain estrogens)

See also hyperreactio luteinalis, large solitary luteinized follicular cyst of pregnancy and puerperium

Case reports: associated with tamoxifen (Gynecol Obstet Invest 1993;35:62)

Treatment: excision if symptomatic, LHRH agonists, watchful waiting (Arch Pediatr 1994;1:903)

Gross: up to 10 cm, glistening membrane, thin walled, unilocular or multilocular, clear serous fluid, no solid component

Drawings: follicular cyst

Gross images: follicular cystfollicular cysts and corpus luteumlarge follicular cyst #1#2#3a unilocular cyst has a smooth lining

Micro: outer layer of theca interna cells, usually luteinized, may be surrounded by reticulum; variable inner layer of granulosa cells, luteinized after puberty

Cytology: may be highly cellular with granulosa cells, resembling granulosa cell tumor (Acta Cytol 2003;47:1059); may have atypical cells (Diagn Cytopathol 1991;7:48)

Micro images: epithelial liningtheca lutein cystinhibin stain

AFIP images: cyst is lined by luteinized granulosa cells overlying luteinized theca cells

Virtual slides: #1#2-with rupture

References: eMedicine

 

Germinal inclusion cyst - Ovary-nontumor chapter

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Definition: invaginations of surface epithelium with loss of connection from surface

Also called inclusion cyst, serous inclusion cyst

Common in older women; small, multiple, no clinical significance

Probably not a risk factor for ovarian cancer (Am J Epidemiol 1993;138:90)

May be related to ovulation (Gynecol Oncol 2005;96:496)

Micro: cuboidal, columnar or flat epithelium; tubal metaplasia common; psammoma bodies occasionally seen

 

Heterotopic ovarian splenoma - Ovary-nontumor chapter

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Definition: form of heterotopic splenic hamartoma consisting of red pulp tissue without associated white pulp in otherwise normal ovary

Very rare

Case reports: Arch Pathol Lab Med 2001;125:1483

Micro: interanastomosing vascular channels of splenic sinusoidal red pulp; sinuses lined by cuboidal to flattened cells with ovoid and grooved bland-looking nuclei

Micro images: splenic hamartoma

Positive stains: von Willebrand factor, CD8 (splenic lining cells)

EM: Weibel-Palade bodies, lysosomes

EM images: Weibel-Palade body

 

Hyperreactio luteinalis - Ovary-nontumor chapter

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Definition: ovarian enlargement due to multiple luteinized follicle cysts secondary to hCG stimulation OR hypersensitivity to hCG

Rare; associated with hydatidiform mole, choriocarcinoma, fetal hydrops due to Rh sensitization, multiple gestations

Rarely seen in uncomplicated single pregnancy; only rarely recurs (J Clin Pathol 2005;58:439)

Usually no symptoms, may have pain from hemorrhage; rarely ascites

Virilization in 15% of mothers but not female infants in cases without gestational trophoblastic disease

Case reports: associated with HELLP syndrome (Fertil Steril 2008;90:2008 e13), with hirsutism (Gynecol Endocrinol 2007;23:248), with marked virilization and hyperglycemia (Am J Perinatol 2006;23:85)

Treatment: cysts involute within 6 months; operate only to remove infarcted tissue or to control hemorrhage

Gross: multiple, bilateral, thin-walled cysts, filled with clear or hemorrhagic fluid

AFIP images: both ovaries are enlarged by multiple thin-walled cysts

Micro: follicular cysts with luteinization of theca interna or granulosa cells, edema of theca layer and stroma

AFIP images: two cysts are lined by luteinized granulosa cells overlying luteinized theca cellsthere is prominent edema in the luteinized theca cell layerthe ovarian stroma is markedly edematous

References: Arch Pathol Lab Med 1989;113:921

 

Hyperthecosis - Ovary-nontumor chapter

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Definition: hyperplastic stroma with clusters of luteinized stromal cells

Similar symptoms to polycystic ovarian syndrome but higher testosterone levels and no/fewer cysts

Typically patients are obese with striking virilization and insulin resistance; either pre- or post-menopausal

Luteinized stromal cells produce androgens

Case reports: associated with endometrial adenocarcinoma and pseudosarcomatous changes in endometrial stroma (Am J Surg Pathol 1984;939), due to adrenal tumor (Arq Bras Endocrinol Metabol 2008;52:1184)

Treatment: possibly excision, leuprolide if poor surgical candidate (Obstet Gynecol 2002;99:893)

Gross: uniform enlargement of both ovaries; solid, yellow-white cut surface; may have superficial cysts

AFIP images: both ovaries are enlarged, the cut surface of the left ovary is composed of firm white tissue simulating a neoplasm

Micro: scattered nests of luteinized theca cells in ovarian stroma

Micro images: stromal cells are enlarged and pale staining

AFIP images: numerous nests of vacuolated luteinized cells are present in the ovarian stromathe luteinized cells have eosinophilic, nonvacuolated cytoplasm and regular, round nuclei with prominent nucleolia large nodule of luteinized cells is present within the ovarian stroma (nodular hyperthecosis)the lutein cells contain abundant lipid (Oil red-O stain)

 

Hypothyroidism and ovarian cysts - Ovary-nontumor chapter

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Van Wyk and Grumbach syndrome: juvenile hypothyroidism, precocious puberty and ovarian enlargement - regresses with thyroid medication (Pediatr Radiol 2008;38:538)

Case reports: 9 year old girl, 22 year old woman with multiple ovarian cysts and hypothyroidism (Gynecol Endocrinol 2008;24:586)

Gross images: various images

Micro images: various images

Virtual slides: multicystic ovary associated with hypothyroidism and juvenile granulosa cell tumor

 

Large solitary luteinized follicular cyst of pregnancy and puerperium - Ovary-nontumor chapter

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Definition: rare solitary follicular cyst that occurs during pregnancy and puerperium, may be related to hCG

Palpable adnexal mass or seen at C-section

No clinical endocrine disturbance

Treatment: conservative, may need to excise if symptomatic (Obstet Gynecol 2005;105:1218)

Gross: large, solitary, unilocular cyst, resembles follicular cyst, but markedly larger (mean 25 cm)

Gross images: 25 cm unilocular cyst

Micro: cyst lined by 1 or more layers of luteinized cells with clear to pink cytoplasm, no theca/granulosa distinction; focal nuclear atypia (enlarged, pleomorphic and hyperchromatic nuclei) is probably degenerative; also nests of luteinized cells within fibrous tissue of cyst wall (Pathol Res Pract 2006;202:471)

Micro images: luteinized cyst wall (fig c)

References: Am J Surg Pathol 1980;4:431, Arch Pathol Lab Med 1986;110:928

 

Massive edema of ovary - Ovary-nontumor chapter

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Definition: tumor like enlargement of ovary due to edema fluid

First described in 1969 (Obstet Gynecol 1969;34:564)

Pain, abdominal mass, menstrual irregularities, virilization, precocious puberty, Meig’s syndrome (with ascites and pleural effusion, eMedicine)

May be due to partial torsion of mesovarium leading to interference of venous / lymphatic drainage

Usually young women; right sided involvement is more common

Gross: marked ovarian enlargement, watery cut surface, no necrosis

Micro: marked edema of stroma surrounding follicles and clusters of luteinized cells; stroma around vessels and in superficial cortical zone is normal; variable stromal luteinization

Micro images: loose stroma with occasional inflammatory cells #1#2

AFIP images: clusters of lutein cells lie in the edematous stroma

DD: fibroma (circumscribed, tumor cells replace ovarian architecture), Krukenberg tumor (usually bilateral, signet ring cells present)

References: Am J Surg Pathol 1979;3:11, Arch Pathol Lab Med 1979;103:42

 

Ovarian pregnancy - Ovary-nontumor chapter

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1-3% of ectopic pregnancies; often conceptus dies or involutes spontaneously

Resembles tubal pregnancy due to similar symptoms (abdominal pain, amenorrhea, abnormal vaginal bleeding) and risk factors (pelvic inflammatory disease, prior pelvic surgery, IUD [Eur J Obstet Gynecol Reprod Biol 2004;114:92], use of progesterone only minipill)

Due to retention of ovum in ovarian operculum and continued entrapment within ruptured ovarian follicle; sperm fertilizes entrapped ovum, and implantation occurs within ovary

Difficult to diagnose clinically or even intraoperatively

Patients have excellent prognosis for future fertility; recurrence is virtually nonexistent

Spiegelberg criteria: (1) intact ipsilateral tube, clearly separate from the ovary; (2) gestational sac within or replacing the ovary; (3) sac connected to the uterus by the ovarian ligament; and (4) histologically proven ovarian tissue located in the sac wall (Arch Gynaekol 1878;13:73)

Case reports: 23 year old woman (Indian J Pathol Microbiol 2008;51:37), 31 year old woman (Arch Pathol Lab Med 2003;127:1635), patient with intact IUD (Arch Pathol Lab Med 1981;105:112)

Treatment: methotrexate, wedge resection if unsuccessful (MedGenMed 2006;8:35)

Gross: enlarged gray-tan ovaries; may clinically resemble tumor (Ann Afr Med 2007;6:36)

Gross images: intraoperativehemorrhagic cavityovary is replaced by a hemorrhagic mass containing a small, pale yellow fetus (AFIP)ovary containing gestational sac 

Micro: evidence of pregnancy (chorionic villi, trophoblastic tissue), ovarian tissue in wall of gestational sac

Micro images: corpus luteum, chorionic villi and trophoblastic tissuechorionic villi surrounded by corpus luteum

 

Polycystic ovary disease (PCO) - Ovary-nontumor chapter

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Formerly called Stein-Leventhal syndrome

Affects 5-10% of women in US; most common cause of anovulatory infertility

Two different diagnostic criteria:

NIH: androgen excess, oligoovulation and exclusion of other entities that cause polycystic ovaries

ESHRE/ASRM in Rotterdam: 2 of 3 present - (a) oligoovulation or anovulation, (b) excess androgen activity, (c) polycystic ovaries present (by ultrasound) but no other endocrine disorders

Occurs during teenage and childbearing years

Associated with endometrial hyperplasia, well differentiated adenocarcinoma; also acne, obesity, hirsutism, insulin resistance and diabetes

Endometrium may show metaplastic changes resembling adenoacanthoma or adenocarcinoma (Am J Surg Pathol 1982;6:223)

Treatment (for infertility): clomiphene citrate or FSH, weight loss; also “ovarian drilling”-puncture of small follicles with electrocautery (Hum Reprod 2002;17:2851); formerly did wedge resections

Gross: large ovaries (2x normal), numerous subcortical cysts (“cysts” may be immature follicles)

Gross images: numerous small peripheral follicles

AFIP images: the enlarged ovary is pearly white, multiple cysts are visible beneath the surface

Micro: multiple cystic follicles covered by a dense fibrous capsule; luteinization of the theca interna (hyperthecosis), few corpora lutea or corpora albicantia since anovulatory, atretic follicles simulate corporate albicantia

AFIP images: the outer cortex is collagenized with several follicle cysts arrayed beneath ita prominent band of luteinized theca cells surrounds the cavity of an atretic follicle (follicular hyperthecosis)

Virtual slides: polycystic ovary disease

Videos: #1

References: Wikipedia, eMedicine #1#2

 

Postoperative carbon pigment granuloma - Ovary-nontumor chapter

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At sites of prior laser or fulguration surgery (Hum Pathol 1996;27:1008)

 

Pregnancy luteoma - Ovary-nontumor chapter

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Definition: single or multiple nodules of luteinized cells with abundant eosinophilic cytoplasm, detected during pregnancy

Rare (100 cases described), probably hyperplasia due to hCG, not neoplasia

80% in multiparous women, 80% in blacks

Usually an incidental finding at cesarean section or post-partum tubal ligation

25% are associated with virilization in latter half of pregnancy, 2/3 of female infants of virilized mothers are virilized (note: usually placenta aromatizes androgens to estrogens); testosterone levels are 70x normal, elevated even in non-virilized patients

Case reports: with rupture and bleeding (Singapore Med J 2008;49:e78), with female pseudohermaphroditism (Hum Reprod 2002;17:821)

Treatment: none - tumors are benign and regress weeks after delivery (infarct like necrosis leads to scar)

Gross: soft, fleshy, circumscribed, yellow/orange nodules, hemorrhagic, may be very large; 1/3 bilateral, 1/2 multiple, may see separate corpus luteum

Gross images: bilaterally enlarged ovaries

Micro: sharply circumscribed, rounded masses of polygonal cells with abundant pink cytoplasm containing little lipid (theca-lutein cells), round nuclei, variably prominent nucleoli, mild nuclear atypia; 2-3 MF/10 HPF, scant stroma; may have colloid filled spaces

Micro images: #1#2#3#4

AFIP images: several follicle-like spaces are presentthe cells have abundant eosinophilic cytoplasm and regular, round nuclei with prominent nucleolitwo mitotic figures are visible

Positive stains: alpha-inhibin, cytokeratin, vimentin, CD99

EM: smooth ER, dispersed Golgi, tubular cristae in mitochondria (associated with steroid hormone producing cells)

DD: large solitary luteinized follicular cyst of pregnancy and puerperium, hyperreactio luteinalis, corpus luteum of pregnancy (central cavity, convoluted border, cells have hyaline or calcified bodies); also granulosa tumor, thecoma, steroid cell tumor (not usually in pregnant women, usually unilateral, more non-luteinized foci, more lipid, more nuclear atypia), melanoma (pigment present, S100+, HMB45+, alpha-inhibin negative)

 

Rete ovarii cyst - Ovary-nontumor chapter

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Also called adenoma of rete ovarii

Rare; mean age 59 years

Case reports: 11 year old girl (J Pediatr Surg 2005;40:e17),

Gross: mean 9 cm, usually hilar

Micro: tubulopapillary proliferations of columnar cells with clear cytoplasm; stroma has extensive polygonal, Leydig-like cells associated with simple endometrial hyperplasia; rete and hilar mesonephric remnants found in vicinity of the lesion

Micro images: a small cyst in the hilus is lined by cuboidal epithelial cells, Wolffian remnants are visible below

Positive stains: CAM 5.2, vimentin, EMA, progesterone receptor

References: Int J Gynecol Pathol 1988;7:330, Hum Pathol 1997;28:1428

 

Splenic-gonadal fusion - Ovary-nontumor chapter

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Definition: congenital anomaly due to fusion of splenic and gonadal anlage during embryonic development

Extremely rare (90 cases)

More common in males; often associated with skeletal anomalies or cryptorchidism

Either continuous or discontinuous

Continuous: cord-like structure connects the spleen and the gonadal-mesonephric structures

Discontinuous: no such connection exists

Case reports: adult woman-continuous (Pathol Int 1995;45:871), adult woman-discontinuous (Hum Pathol 1989;20:486)

Gross images: a nodule of brown red splenic tissue abuts the ovary (AFIP)

 

Stromal hyperplasia - Ovary-nontumor chapter

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Definition: tumor like proliferation of ovarian stroma, without luteinization

Less frequently estrogenic or androgenic than stromal hyperthecosis

Patients may be obese, have hypertension or abnormal glucose tolerance

Gross: ill defined white-yellow nodules in ovary; may lead to enlarged ovary

AFIP images: ill-defined, pale yellow tissue occupies the center of the ovary

Micro: medullary or occasionally cortical clusters of densely cellular ovarian stroma with scant collagen; may entirely replace ovarian architecture; no luteinized stromal cells; no mitotic figures

AFIP images: the ovarian medulla and cortex of an elderly woman are replaced by cellular stromaconfluent nodules of hyperplastic stroma occupy the medulla

DD: stromal hyperthecosis (luteinized cells), low grade endometrial stromal sarcoma (spiral arterioles and mitotic figures present)

 

Supernumerary ovaries - Ovary-nontumor chapter

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Also called ectopic ovary, accessory ovary

Very rare, usually < 1 cm

Supernumerary: occurs widely situated from normal ovary, formed from a separate anlage

Accessory: due to splitting of embryonic gonad, usually attached to the normal gonad

May actually be ovarian implant or due to torsion

Case reports: stillborn (Arch Pathol Lab Med 1991;115:233), in labia of 5 month old (J Belge Radiol 1998;81:288), in omentum with dermoid cyst (Arch Gynecol Obstet 1995;256:111), intrarenal supernumerary ovary with completely duplicated pelvis and ureter (Int Urogynecol J Pelvic Floor Dysfunct 2007;18:1243), on broad ligament (J Reprod Med 2000;45:435), on sigmoid colon (J Obstet Gynaecol Res 2006;32:613), cases with fibroma and endometriosis (Pathol Res Pract 2001;197:847), ovarian remnant at laparoscopic port site (J Minim Invasive Gynecol 2008;15:505), ovary possibly placed in inguinal canal at prior surgery (Fertil Steril 2006;85:1822e9),

 

Torsion - Ovary-nontumor chapter

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Definition: partial or complete rotation of ovarian vascular pedicle, causing obstruction to venous outflow and arterial inflow

Rare; associated with cysts, neoplasms and in vitro fertilization; in children, ovary is often normal (Arch Pediatr Adolesc Med 2005;159:532)

Presents with abdominal pain, patients need emergency ultrasound and laparoscopy (J Pediatr Adolesc Gynecol 2008;21:201)

Case reports: normal ovary in third trimester (J Med Case Reports 2008 Dec 8;2:378)

Treatment: unwinding (J Obstet Gynaecol Res 2008;34:683); even necrotic appearing ovaries can often be saved (Clin Obstet Gynecol 2006;49:459)

Gross images: torsion #1#2#3due to large ovarian fibromadue to large serous cystadenomaenlarged right ovary with edematous tube (fig 2)

Micro images: corpus luteum cyst torsion

References: eMedicine #1#2

 

Uterus like mass of ovary - Ovary-nontumor chapter

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Case reports: 18 year old (Arch Pathol Lab Med 1985;109:361), 31 year old with endometriosis (Arch Pathol Lab Med 1981;105:508), three women in India ages 38-43 years with breast cancer (Am J Surg Pathol 1998;22:333); resembling adenomyoma (Arch Pathol Lab Med 2005;129:1041)

May be due to endomyometriosis-like metaplasia or congenital malformation of urogenital system (Int J Gynecol Pathol 2005;24:382)

Gross: resembles uterus, no anatomic abnormalities

Gross images: gross and microscopic images

Micro: resembles uterus but residual ovarian stroma

 

End of Ovary-nontumor chapter

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