"Unicorn in a Field of Horses": A Rare Case of Carcinosarcomatous Transformation in a Uterine Leiomyoma.

Malignant transformation in leiomyoma is rare, with a few documented cases of sarcomatous transformation in leiomyomas. However, carcinosarcomatous transformation in leiomyomas is extremely infrequent. A 45-year-old female presented with a mass sensation per abdomen for the last five months. An abdominal ultrasound revealed multiple uterine fibroids. Contrast-enhanced magnetic resonance imaging revealed a large multiloculated abdominopelvic mass arising from the fundus of the uterus, along with multiple smaller subserosal fibroids. A total abdominal hysterectomy with bilateral salpingectomy was performed. Grossly, the uterus was bosselated with multiple subserosal fibroids. The larger degenerated fibroid showed a smooth outer surface; however, the cut surface was predominantly cystic and filled with necrotic material. Microscopically and immunohistochemically, the larger mass showed a high-grade biphasic tumor comprising carcinomatous and sarcomatous components with the peripheral solid areas showing compressed smooth muscle bundles representing the residual leiomyomatous areas. A final diagnosis of carcinosarcomatous transformation in leiomyoma was rendered. The index report highlights the significance of systematic gross and microscopic examination of all the uterine masses in patients with multiple leiomyomata.

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.

OBJECTIVE: Juvenile granulosa cell tumor(JGCT) is an uncommon ovarian sex-cord stromal tumor, with diverse clinical, radiological and histopathologic features. The present study describes the clinicopathological and histomorphological spectrum of JGCTs, and highlights the key differentiating features from its mimics. METHODS: A retrospective analysis of all cases reported as JGCTs during 2011-19 (8 years) was performed with detailed evaluation of clinical, histopathologic data and follow-up details. RESULTS: Of a total 115 GCTs reported during the study period, 15(13%) were reported as JGCTs. The mean age at presentation was 17 years. Abdominal pain and distension were the most common clinical presentations. Five patients were pre-menarchal with 3 exhibiting precocious puberty. Majority of tumors were unilateral(left>right), solid-cystic, ranging in size from 4 to 20 cm. Microscopically, macrofollicular architecture was most frequent (n = 12;80%). The tumor cells depicted variable nuclear pleomorphism, small distinct nucleoli and moderate-abundant pale eosinophilic-clear/vacuolated cytoplasm. Mitotic activity ranged from 1 to 10/10HPFs. Uncommon histopathologic features included microcystic and tubulo-cystic architecture, myxoid degeneration, bizarre tumor giant cells, hob-nailing of the tumor cells, intracytoplasmic hyaline globules, multifocal calcification and thick hyalinized blood vessels. Majority(n = 12;80%) presented in stage I. Surgical treatment included unilateral salpingo-oophorectomy without any adjuvant chemotherapy, bilateral salpingo-oophorectomy (BSO) and total abdominal hysterectomy with BSO with adjuvant BEP chemotherapy (Bleomycin, etoposide, cisplatin). CONCLUSIONS: JGCT is a rare ovarian tumor affecting young women and children with diverse histopathologic features. Despite an aggressive histopathology, these tumors have a good outcome, when diagnosed at an early stage.

Bilateral Adnexal Masses in a Young Female: Rare Presentation of Hepatocellular Carcinoma With Review of the Literature.

Ovaries are a common niche for metastasis. Metastatic malignancies account for 5-30% of all ovarian malignancies. Hepatocellular carcinoma (HCC) is one of the rare malignancies to metastasize to the ovaries. Of all the variants of HCC, fibrolamellar HCC (FLHCC) variant is extremely uncommon and accounts for around 1% of all HCC cases. FLHCC metastasizing to ovaries, at presentation, is an exceptional occurrence. We present a case of a young female who presented with bilateral adnexal masses and was diagnosed as metastatic FLHCC on histopathological examination and confirmed by immunohistochemistry. In addition, a thorough literature review highlighting the previously reported cases is also presented.

'Size does matter': Prophylactic gonadectomy in a case of Swyer syndrome.

Swyer syndrome also known as pure or complete gonadal dysgenesis is a very rare disorder of sex development wherein the individuals are phenotypically females with 46, XY genotype and preserved mullerian structures. These individuals characteristically have dysgenetic streak gonads which carry an increased risk of malignant transformation. Prophylactic gonadectomy is highly recommended as soon as a clinical diagnosis is established to diminish the chances of tumor development. We present a case of complete gonadal dysgenesis with bilateral small gonads with a dysgerminoma arising in a background of gonadoblastoma in one gonad and immature teratoma in the other. The present case, besides adding a rare case to the literature, highlights the importance of detailed pre-operative assessment of gonadal size and prompt prophylactic gonadectomy in cases with gonadal dysgenesis.