Ovary-nontumor
Last revised 20 April 2009
Last major update February 2009
Copyright (c) 2002-2008, PathologyOutlines.com, Inc.
Printer Friendly Versions (HTML, PDF)
Bold and underlined topics are hypertext links
References in green are to journals with free full text-no registration
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
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
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
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
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; #4; sagittal; ovary about to release an egg
References: Wikipedia
Normal histology- Ovary-nontumor chapter
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 hyperchromatic; nodular 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)
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; #3; follicle maturation #1; #2
Gross images: corpus luteum #1; #2-hemorrhagic; #3-cystic; #4-with follicular cysts
Micro images: primordial and antral follicles; primordial follicles #1; #2; tertiary follicle; ruptured follicle #1; #2; atretic follicle #1; #2; serosa; hilus; corpus luteum-hemorrhagic
AFIP images: normal - low columnar cells are separated from cortical stroma by basement membrane; ovarian stroma is composed of spindle cells with scant cytoplasm in a storiform pattern; luteinized 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 cells; involuting corpus luteum has granulosa lutein cells filled with (lipid) vacuoles and containing pyknotic nuclei
corpus luteum of pregnancy - central hyaline bodies are present; foci of calcification in involuting corpus luteum
follicles - 4 primordial follicles (left), 2 primary follicles (center) and a secondary follicle (right) are present; secondary follicle-stratified granulosa cells surround the oocyte enveloped by its zona pellucida, theca interna layer is not yet apparent; graffian follicle-cumulus oophorus, containing the oocyte and its zona pellucida, projects into the antrum; granulosa 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)
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 evident; numerous small tubular structures and cords of mesothelial cells are growing in a parallel array
miscellaneous - surface epithelial stromal proliferation; vacuolar change within surface epithelial inclusion glands; artifact-granulosa cells in blood vessel lumen #1; #2; infarcted 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 cells; ectopic 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 albicantia; cortical 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 stroma; polypoid 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 stroma; lined predominantly by columnar cells
Walthard nest - composed of transitional cells that abut the ovarian stroma
Virtual slides: normal ovary-infant
Videos: #1; corpus albicans; corpus 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
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
Either 46XX or 46XY
Pure gonadal dysgenesis - 46XX - Ovary-nontumor chapter
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
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
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
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
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)
Micro images (AFIP): ovarian type stroma with a storiform pattern containing a few lutein-like cells envelops two small solid tubules containing Sertoli cells; tubules containing immature Sertoli cells are separated by cells resembling Leydig cells; testis is composed almost entirely of stroma resembling ovarian stroma; hamartomas 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
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
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
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 lining; ovarian 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
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
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
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 arteries; present in mesenteric vessels as incidental finding in colonic adenocarcinoma
References: Am J Surg Pathol 1986;10:696
Gonorrhea - Ovary-nontumor chapter
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
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 disease; tuberculosis
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 right; histiocytes have abundant bubbly gray-pink cytoplasm and resemble signet-ring cells #1; #2-histiocytes are mucicarmine+
Microsporidia - Ovary-nontumor chapter
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
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
DD of small, cystic follicles - Ovary-nontumor chapter
Cystadenoma / cystadenocarcinoma, cystic corpus luteum, cystic granulosa cell tumor, endometriosis, epidermoid tumor, rete ovarii cyst, surface inclusion cyst
Calcification - Ovary-nontumor chapter
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
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
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 rupture; wall 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
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
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
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 cyst; hemorrhagic nodules; intraoperative 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; #2; cyst #1 has outer surface with many foci of red-brown discoloration and adhesions, contains chocolate- colored fluid; cyst #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)
Micro images: endometrial glands and stroma in ovary; H&E and CD10; smooth muscle actin+ wall of endometriotic cyst
AFIP images: endometrioid glands with variable size are within cellular endometrial-type stroma surrounded by denser ovarian stroma; the wall of an endometriotic cyst contains pseudoxanthoma cells filled with hemofuscin pigment; deeper portion of the wall of an endometriotic cyst is fibrotic, the subepithelial layer is composed of hemorrhagic cellular stroma; Liesegang rings are scattered in debris; polyp composed mainly of cystic glands projects into the lumen of an endometriotic cyst; two pseudoxanthomatous necrotic nodules are seen at the top and bottom, an endometriotic gland is seen in the upper nodule; a pseudoxanthomatous nodule is centrally necrotic with a surrounding granulomatous reaction; patient taking norethynodrel with mestranol has marked decidual change of stroma; during 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
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 stroma; ciliated, secretory and intercalated cells line the cystic space
DD: noninvasive or invasive ovarian implants
Epidermoid cyst - Ovary-nontumor chapter
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
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 cyst; follicular cysts and corpus luteum; large follicular cyst #1; #2; #3; a 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 lining; theca lutein cyst; inhibin 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
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
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
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 cells; there is prominent edema in the luteinized theca cell layer; the ovarian stroma is markedly edematous
References: Arch Pathol Lab Med 1989;113:921
Hyperthecosis - Ovary-nontumor chapter
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 stroma; the luteinized cells have eosinophilic, nonvacuolated cytoplasm and regular, round nuclei with prominent nucleoli; a 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
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
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
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
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: intraoperative; hemorrhagic cavity; ovary 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 tissue; chorionic villi surrounded by corpus luteum
Polycystic ovary disease (PCO) - Ovary-nontumor chapter
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 it; a 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
At sites of prior laser or fulguration surgery (Hum Pathol 1996;27:1008)
Pregnancy luteoma - Ovary-nontumor chapter
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
AFIP images: several follicle-like spaces are present; the cells have abundant eosinophilic cytoplasm and regular, round nuclei with prominent nucleoli; two 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
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
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
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 stroma; confluent 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
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
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; #3; due to large ovarian fibroma; due to large serous cystadenoma; enlarged 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
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
See also Ovary-tumor chapter