Author(s): Fialho, M. Pinto ; Gradil, A. ; Ferreira, C. ; Lérias, S.
Date: 2024
Persistent ID: http://hdl.handle.net/10400.21/21673
Origin: Repositório Científico do Instituto Politécnico de Lisboa
Subject(s): Ovarian cancer; Sebaceous carcinoma; Cystic teratoma; Case report
Background & objectives: Sebaceous carcinoma originating within an ovarian mature cystic teratoma (MCT) is an extremely rare entity. To the best of our knowledge, less than 14 cases have been reported in the literature. Methods: We report a case of a sebaceous carcinoma arising within a mature teratoma. Results: An 83-year-old female presented with urinary retention symptoms. A pelvic MRI scan revealed a left ovary lesion consistent with MCT. The patient underwent a bilateral adnexectomy. Macroscopically an 18cm ovary cystic mass showed a partially solid appearance intermixed with soft yellow sebaceous material. Histological examination revealed an MCT with a lobular component containing sebocytes surrounded by basaloid cells with focal squamoid features, nuclear pleomorphism, increased mitosis, and associated necrosis. Basaloid cells were immunoreactive to p63 and CK5/6. Sebaceous cells were EMA+ and PRAME+. Mismatch repair protein nuclear expression was intact. A diagnosis of a sebaceous carcinoma arising within an MCT was made. The patient is free of disease at 17 months. Conclusion: Sebaceous carcinoma within an ovarian MCT is a rare entity. Although ocular sebaceous carcinomas are aggressive, the prognosis of extraocular sebaceous carcinoma is thought to be better since local recurrence or metastasis has not yet been described. Given the paucity of data, the management of these patients remains unclear, however identifying tumor microsatellite instability or germline DNA mutations facilitates further cancer screening and treatment (PD1/PD-L1 inhibitors).
