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Ovary-nontumor
Table of Contents Primary references, embryology, normal anatomy, normal histology
Reviewers: Mohiedean Ghofrani, M.D., Sanjay Logani, M.D. (see Reviewers page)
Revised: 28 December 2011, last major update August 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.
Gonadal dysgenesis: general, pure, -46XX, -46XY, mixed, female pseudohermaphroditism, -associated with CAH, -non adrenal, testicular feminization, true hermaphroditism, Turner’s syndrome
Inflammatory disorders-noninfectious: autoimmune oophoritis, eosinophilic perifolliculitis, giant cell arteritis
Infectious disorders: abscess, gonorrhea, granulomatous inflammation, microsporidia, pelvic inflammatory disease
Non-neoplastic cysts/other: DD of small cystic follicles, calcification, cholelithiasis, corpus luteum cyst, developmental cysts, 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
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AJCC Cancer Staging Manual (7th ed)
American Journal of Clinical Pathology
American Journal of Surgical Pathology
Archives of Pathology and Laboratory Medicine
Human Pathology
Modern Pathology
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, Webpathology.com
Virtual slides - USCAP, vSlides
Please refer to these primary references for more detailed discussions
Non-neoplastic cysts/other
Hypothyroidism and ovarian cysts - Ovary-nontumor chapter
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; 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
