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17 February 2021 - Case of the Month #500

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Thanks to Dr. Sakinah A. Thiryayi and Dr. Gulisa Turashvili, Mount Sinai Hospital, Toronto (Canada) for contributing this case and writing the discussion and to Dr. Ricardo R. Lastra, University of Chicago Medical Center, Chicago, Illinois (USA), for reviewing the discussion.




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Case of the Month #500

Clinical history:
A 52 year old woman was found to have an ovarian mass on computed tomography imaging and an elevated CA-125 of 234 U/mL (reference range: less than 46 U/mL). The mass was excised.

Histopathology images:


What is your diagnosis?

Click here for diagnosis, test question and discussion:



Diagnosis: Endometrioid adenocarcinoma with functioning stroma

Test question (answer at the end):
What clinical manifestations have most commonly been associated with functioning stroma in ovarian tumors?

A. Adrenergic manifestations
B. Androgenic manifestations
C. Estrogenic manifestations
D. Progestogenic manifestations


Stains:

Inhibin

p53




Discussion:

Ovarian neoplasms, in addition to sex cord stromal tumors (SCST) and steroid cell tumors, may have stroma with endocrine function clinically, biochemically or histologically. This finding has been described in benign and malignant (both primary and metastatic) tumors. The stromal cells may produce estrogens, androgens or, rarely, progestogens, with estrogen most commonly produced by mucinous and endometrioid tumors (Endocr Pathol 2000;11:1). Endometrioid adenocarcinoma with functioning stroma is not a distinct WHO diagnosis.

Estrogenic manifestations include endometrial thickening and abnormal vaginal bleeding, especially in postmenopausal women, as well as abnormal squamous cell maturation in cervicovaginal cytology samples. The functioning stromal cells may either be polygonal lutein-like cells with abundant eosinophilic cytoplasm and round nuclei or theca-like ovoid or plump spindled cells. Both morphological types are capable of converting androgens to estrogens, mostly by peripheral aromatization, and the cell morphology has not been found to correlate with the level of serum estrogen (Int J Gynecol Pathol 2013;32:556).

Endometrioid adenocarcinomas with functioning stroma may closely resemble SCSTs. Gross features of a homogenously yellow tumor coupled with microscopic findings of tubular nests and strands separated by intervening luteinized Leydig-like stromal cells may mislead the pathologist, particularly at intraoperative diagnosis, to consider a SCST (Diagn Pathol 2012;7:164). The presence of areas with conventional endometrioid morphology or confirmatory endometrioid features and negative sex cord stromal markers in the epithelial component should allow accurate diagnosis.

Another pitfall is the presence of stromal hypercellularity associated with stromal mitotic activity, which may raise the differential diagnosis of carcinosarcoma / MMMT. Carcinosarcoma predominantly occurs in postmenopausal women. It is composed of intermingled malignant epithelial (most commonly high grade serous carcinoma) and sarcomatous components and shows aberrant p53 immunohistochemical expression in both components in most cases (Int J Gynecol Pathol 2003;22:368). Thus, the absence of a high grade carcinomatous component, positive sex cord stromal markers in the stromal component and wild type expression pattern of p53 are helpful features in this differential diagnosis.

Test question answer:
C.
Estrogenic manifestations

Image 01 Image 02