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

Abstract

Background: Though Granulosa cell tumors (GCTs) are the most common ovarian sex cord stromal tumors, they are very rare in extra ovarian sites with a handful of cases reported in retroperitoneum, broad ligament, fallopian tube, mesentery and even in the adrenals. Female Adnexal Tumor of probable Wolffian Origin (FATWO), which is also a very rare tumor comes up as its closest differential because of a significant overlap of topographic, morphologic, and immunohistochemical (IHC) features. Case Summary: Present case is of a post-menopausal female who complained of abdominal swelling and post-menopausal bleeding. On CT, she was shown to have a broad ligament mass (fibroid) which was excised. On histopathological reporting the tumor showed mainly the lesser known morphological variants of GCT that lead to difficulty in reaching at the exact diagnosis and a differential of an extraovarian GCT [eGCT] vs. FATWO was given which on IHC finally turned out to be an eGCT. Conclusion: Broad ligament is the site of two very rare tumors of the female genital tract, i.e. eGCT and FATWO which are close mimickers of each other not only on topography, but also on morphology and immunohistochemistry. Hence distinguishing between these two entities may be quite challenging for a pathologist, but aid of certain features can be taken to arrive at the diagnosis many a times.

Primary retroperitoneal extraovarian granulosa cell tumor

“…Studies using subunit specific ELISA show inhibin B to be the major form secreted in GCT, and that inhibin B is more accurate than inhibin A in detecting GCT. 14 Studies have further confirmed that inhibin B is a sensitive and specific marker for GCTs. 16,17 Thus, inhibin B levels can be helpful in the early detection and recurrence of GCTs.…”

“…13 Serum inhibin levels are elevated in GCT thus, inhibin can be used as a marker for GCT. 14 Lappohn et al 15 was the first to demonstrate the value of inhibin as a marker for both primary and recurrent disease and showed that a rise in inhibin level preceded clinical recurrence as early as 20 months. Studies using subunit specific ELISA show inhibin B to be the major form secreted in GCT, and that inhibin B is more accurate than inhibin A in detecting GCT.…”