Ovary

• Ovarian stromal hyperplasia is a nonneoplastic functional disorder resulting in nodular or diffuse proliferation of ovarian stroma
• Hyperthecosis is characterized by proliferation of ovarian stroma along with luteinization of stromal cells (J Turk Ger Gynecol Assoc 2013;14:182)

• ICD-11: 5A80.Y - other specified ovarian dysfunction

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• Diffuse or nodular proliferation of ovarian stroma in cortex, medulla or both
• Stromal proliferation of spindle cells with scant intervening collagen
• May be diffuse or present as a nodule with coalescent nodules (Endocrinol Diabetes Metab Case Rep 2021;2021:20)
• Entrapped follicular derivatives may be present
• In hyperthecosis, single cells, nests or nodules of polygonal luteinized theca cells with eosinophilic or clear cytoplasm < 1 cm are present

Contributed by Hafza Asma Adnan, M.B.B.S. and AFIP

• Fibroma:
  • Mostly unilateral mass
  • Usually well circumscribed and composed of variable cellular spindle to oval cells with scant cytoplasm, arranged as intersecting fascicles with intervening collagen; sometimes hyaline plaques are also present
  • Cellular fibroma is densely cellular with little intervening collagen that may be mitotically active
• Steroid cell tumor, NOS:
  • This tumor is comprised of expansile diffuse proliferation of large polygonal cells with eosinophilic, pale or vacuolated cytoplasm
  • Steroid cells nodules > 1 cm arising in a background of stromal hyperthecosis are now best diagnosed as steroid cell tumor, NOS, as the previous diagnostic category of stromal luteoma is no longer recognized as a distinct entity
• Luteinized thecoma associated with sclerosing peritonitis:
  • Extremely rare lesion, patient presents with abdominal swelling, ascites and bowel obstruction
  • Almost always bilateral, ovaries have tan to red sectioned surface
  • Microscopically, ovaries are hypercellular and show microcystic appearance due to edema
  • Cells are predominantly spindled with minority component of small luteinized cells
  • SF1 and FOXL2 are positive in spindle cells while calretinin and inhibin are positive in luteinized cells
• Normal ovary:
  • Normal appearance of cortex and medulla
  • Cortex is the hypercellular outer zone having majority of follicles in premenopausal women; medulla is the hypocellular central area containing blood vessels and diffuse or nodular population of spindle cells
  • Young women have thick cortex and contain follicles in various stages of development, while this is atrophied in old age and mainly contain corpus albicans
  • Diagnosis of stromal hyperplasia and hyperthecosis is a subjective estimation of cortex and medullary cellularity with respect to age
• Sex cord stromal tumor, NOS:
  • Both sex cord and stromal components are present
  • Stromal component highlighted by reticulin staining
• Pregnancy luteoma:
  • Associated with pregnancy
  • Comprised of oval to round uniform cells with abundant eosinophilic cytoplasm
• Massive ovarian edema and fibromatosis:
  • Prominent edema of stroma sparing cortex
  • Clusters of luteinized cells may be present
• Krukenberg tumor:
  • Isolated atypical cells with signet ring cell morphology in ovarian stroma
  • AE1 / AE3 positive
• Low grade endometrial stromal sarcoma:
  • Oval to spindle cells with minimal atypia and associated arterioles
  • CD10 positive

A specimen for bilateral oophorectomy is received with enlarged ovaries. One ovary measures 44 x 30 x 25 mm while the second measures 35 x 30 x 25 mm. Sectioning shows homogeneous solid areas with a single 3 mm cyst. Microscopy shows a diffuse population of spindle cells with no pleomorphism, mitosis or necrosis. What is the likely diagnosis?

1. Adult granulosa cell tumor
2. Fibroma
3. Metastatic tumor
4. Sex cord stromal tumor, NOS
5. Stromal hyperplasia

E. Stromal hyperplasia. There is a homogeneous population of spindle cells with no atypia. Answer A is incorrect because adult granulosa cell tumor will show atypia with variable patterns and nuclear grooving. Answer B is incorrect because no hyalinized areas with intersecting fascicles are present. Answer C is incorrect because no epithelial elements are present in this tumor. Answer D is incorrect because no sex cord-like areas or biphasic cell populations are identified.

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Reference: Stromal hyperplasia and hyperthecosis

A 57 year old woman presents with postmenopausal bleeding. Endometrial biopsy showed FIGO grade 1 endometrioid type endometrial cancer and hysterectomy revealed stage IA endometrioid type endometrial carcinoma with enlarged bilateral ovaries. Microscopic findings are shown in the image above. What is the diagnosis?

1. Fibroma
2. Leydig cell tumor
3. Metastatic endometrioid type endometrial cancer
4. Sex cord stromal tumor
5. Stromal hyperplasia and hyperthecosis

E. Stromal hyperplasia and hyperthecosis. Clusters of luteinized cells are present in stromal hyperplasia. Answer A is incorrect because no hyalinized areas with intersecting fascicles are present. Answer B is incorrect because no Leydig cell population is identified and the cells do not contain Reinke crystals. Answer C is incorrect because no epithelial elements are identified. Answer D is incorrect because no tumor-like mass or epithelial elements are identified.

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Reference: Stromal hyperplasia and hyperthecosis