Metastatic Granulosa Cell Tumor with Pattern of Sex Cord Tumor with Annular Tubules

Malignant Ovarian Sex Cord Tumor with Annular Tubules in a Patient with Peutz-Jeghers Syndrome: A Case Report

“…Both tumors in this case were unusually large for PJS-associated SCTAT, measuring 8.5 and 3.5 cm on the left and right sides, respectively. Although the 13 previously reported cases of malignant SCTAT also were relatively large, with diameters ranging from 11 to 22 cm, all proved to be unilateral (2,(5)(6)(7)(8)(9)(10)(11). In this regard, the finding in our case of large bilateral tumors associated with bilateral pelvic lymph node metastasis is also unique.…”

“…Malignant SCTAT seems to spread mainly via the lymphatics with typical sites of tumor metastasis being the pelvic, para-aortic, and supraclavicular lymph nodes. Other sites of tumor recurrence and metastasis include the retroperitoneum, parietal and visceral peritoneum, liver, kidney, and lung (2,(5)(6)(7)(8)(9)(10)(11). Unique to our case were the presence of tumor emboli in lymphovascular spaces of the left salpingeal mucosa and the identification of tumor cells in a cervicovaginal smear.…”

“…In the largest series of SCTAT to date, none of the 27 patients with both PJS and SCTAT exhibited tumor recurrence or metastasis in reported follow-up intervals ranging from 6 mo to 11 y (2). To date, all examples of malignant SCTAT reported in the literature have occurred in patients who did not have PJS (2,(5)(6)(7)(8)(9)(10)(11).…”

“…Stromal and vascular space invasion by individual tumor cells and tumor cell clusters has been reported in two cases of recurrent SCTAT, one of which also had infiltrated the capsule (6). The time of first recurrence in cases of malignant SCTAT has ranged from 3 mo to 20 y, and six patients reportedly have died of disease (2,(5)(6)(7)(8)(9)(10)(11). Malignant SCTAT seems to spread mainly via the lymphatics with typical sites of tumor metastasis being the pelvic, para-aortic, and supraclavicular lymph nodes.…”

Diagnostic Pathology of Ovarian Tumors

“…In contrast to SCTAT, gonadoblastomas typically occur in the setting of gonadal dysgenesis, contain tubules with a dual cell population (germ cells and Sertoli cells), and in some cases, may be associated with a second germ cell tumor, typically a dysgerminoma [269]. Sertoli cell tumor [238] and granulosa cell tumor [270] are also considered in the differential diagnosis, as both may show areas that overlap with a typical SCTAT. However, the presence of more conventional areas in these tumors will help to establish the correct diagnosis.…”