Meenakshi Kamboj

Primary pure angiosarcoma of the ovary: A case report

Abstract Ovarian angiosarcomas are rare tumors, with an incidence of one in 1,000,000 malignant ovarian tumors, which may present in a background of carcinosarcoma or teratoma. However, primary pure ovarian angiosarcoma is even rarer with a few cases published in the literature. Here, we present a case of a perimenopausal woman who presented with a 16 cm adnexal mass. She underwent bilateral salpingo-oophorectomy with omentectomy, which on histopathological examination revealed an ovarian angiosarcoma with tumor deposits in the pelvic peritoneum. No other component was demonstrable within the tumor, in light of which it was reported as a primary pure ovarian angiosarcoma. She received adjuvant paclitaxel- and carboplatin-based chemotherapy after discussion in a multispecialty tumor board. This case highlights the gross and microscopic findings of primary ovarian angiosarcomas and their distinction from their differential diagnosis.

Fumarate hydratase–deficient renal cell carcinoma: an oncology care institutional experience

Renal cell carcinoma (RCC) accounts for 2% of all cancer cases worldwide, and majority are sporadic. The latest World Health Organization (WHO) classification of renal cell tumors (fifth edition, 2022) has molecularly defined renal tumor entities, which includes fumarate hydratase (FH)–deficient RCC. FH‐deficient RCC is an aggressive carcinoma caused by pathogenic alterations in FH gene, seen in 15% of patients with hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC) syndrome. These tumors occur more frequently at a younger age and present at an advanced stage, carrying a dismal prognosis. We report a series of 10 cases of FH‐deficient RCC. The mean age was 49.8 years, and all cases presented in advanced stages (III and IV). Morphologically, the cases had varied architectural patterns with characteristic eosinophilic macronucleoli and perinucleolar halo. On immunohistochemistry (IHC), all showed diffuse nucleo‐cytoplasmic expression of S‐(2‐succino)‐cysteine (2‐SC), with loss of FH in seven cases. FH‐deficient RCCs are aggressive neoplasms and can be diagnosed using specific IHC markers (FH and 2‐SC). These patients should undergo germline testing for FH gene mutation, genetic counseling, and surveillance of family members.

Isolated nodal gliomatosis in ovarian immature teratoma