Primary Retroperitoneal Extraovarian Granulosa Cell Tumor: A Case Report

Abstract

Abbreviations and AcronymsEMA = epithelial membrane antigen GCT = granulosa cell tumor PgR = progesterone receptors SMA = smooth muscle actin ABSTRACT A primary extragonadal granulosa cell tumor (GCT) is very rare. It is believed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. We encountered one such tumor in a 45-year-old female who presented with a large intraabdominal mass. She had an abdominal hysterectomy with unilateral salpingooophorectomy 10 years previously for dysfunctional uterine bleeding. She underwent exploratory laparotomy and excision of the mass. The histologic features failed to differentiate between a carcinoid and granulosa celltumor. Immunohistochemistry showed that the neoplastic cells expressed calretinin, smooth muscle actin, melan-A, inhibin-alpha, and progesterone receptors and were negative for desmin, CD10, cytokeratin, epithelial membrane antigen, synaptophysin, or chromogranin-A. An English literature search revealed only 4 similar cases, primarily arising from the retroperitoneum. Immunohistochemistry helps to differentiate GCTs from other tumors.

An unusual presentation of a granulosa cell tumor in broad ligament: morphological features and close differential

“…Extraovarian granulosa cell tumor (e GCTs) are very uncommon tumors. Amongst all the cases published so far, most of the eGCTs have been reported in various parts in the retroperitoneum [2][3][4][5][6][7][8], followed by broad ligament [9][10][11], fallopian tube [12,13], mesentery [14] and even adrenals [15].…”

“…Like GCTs, they also show positivity for Inhibin, Calretinin, pan CK, CAM 5.2, and are negative for EMA. But FATWOs are always positive for CK 7 and generally negative for FOXL2 which comes to the rescue of a pathologist in differentiating it from an eGCT [3,4].…”

“…It is noteworthy that a diagnosis of eGCT should be carried out only after excluding any previous history of GCT of the ovary [1]. Amongst all the cases published so far, most of the eGCTs have been reported in various parts of the retroperitoneum [2][3][4][5][6][7][8], followed by broad ligament [9][10][11], fallopian tube [12,13], mesentery [14] and even adrenals [15]. Interestingly, eGCTs share a significant overlap topographically, morphologically, and immunohistochemically with another very rare tumor, Female Adnexal Tumor of probable Wolffian Origin (FATWO), which comes across as its closest differential [9,10], especially amongst the broad ligament tumors.…”

Primary granulosa cell tumor of retroperitoneal origin: A rare presentation with emphasis on cytomorphology

“…The primary extraovarian GCT is an extremely rare tumor. [ 3 ] To have primarily originated retroperitoneal GCT is even rarer. [ 1 ] In medical literature in English from 1938 till today, only 12 cases have been reported.…”

“…[ 1 ] In medical literature in English from 1938 till today, only 12 cases have been reported. [ 3 ] Although, the histology of ovarian adult-type granulosa cell tumors (AGCTs) is well-documented, it is rarely encountered in cytological specimens and the identification of tumor cells is very difficult. Here, we describe a case of extraovarian AGCT with emphasis on cytologic features.…”