Small cell and neuroendocrine ovarian carcinomas are rare and very aggressive malignant tumours requiring a multi-modal approach to treatment, including surgery, systemic therapy, and radiotherapy in select cases. Established histologic variants include small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT), small cell carcinoma of the ovary pulmonary type (SCCOPT), and non-small cell neuroendocrine carcinomas (NSCNECs). These tumours present with unilateral or bilateral adnexal masses and associated abdominopelvic symptoms. SCCOHT often presents in young patients (median age 24) and is associated with a pathognomonic germline or somatic SMARCA4 mutation, which results in loss of BRG1 staining by immunohistochemistry. Patients and appropriate family members should be referred to a genetic counsellor for germline testing of SMARCA4. Management involves surgical cytoreduction, multi-agent chemotherapy, and, in select cases of patients with a complete response, high-dose chemotherapy followed by autologous stem cell transplant. Radiotherapy can be considered in cases of persistent disease or for palliation of symptoms in the recurrent setting. Treatment of these tumours, once diagnosed, is emergent as the disease behaves aggressively. These tumours may be transiently chemosensitive; however, responses to chemotherapy in the setting of persistent or recurrent disease is limited. Patients and their families can be referred to the SCCOHT/SMARCA4 registry to contribute to ongoing translational research efforts and understanding of genetic penetrance. SCCOPT often presents in patients>40 years of age and is histologically comparable to small cell neuroendocrine carcinoma of the lung. Therefore, metastatic neuroendocrine tumours of a non-gynaecologic primary should be excluded through expert pathology review and comprehensive baseline imaging, which may include an FDG-PET scan. SCCOPT is not known to be associated with a specific molecular profile. While data is limited in this subtype, the same principles of primary cytoreductive and staging surgery apply to these patients, followed by adjuvant chemoradiation or chemotherapy alone. Unfortunately, prognosis remains poor for these rare tumours with high recurrence rates despite multimodal treatment. This underscores the importance of ongoing research efforts and registry participation to guide future directions and novel therapeutic approaches. This chapter reviews the epidemiology, clinical presentation, diagnostic workup, pathologic characteristics, current treatment paradigms, and future directions in the management of small cell and neuroendocrine ovarian carcinomas.

Small Cell and Neuroendocrine Tumours of the Ovary

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发表日期：

2026年

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Brooke Grant,
Cristina Mitric,
Al Covens,
Amit Oza

摘要

Small cell and neuroendocrine ovarian carcinomas are rare and very aggressive malignant tumours requiring a multi-modal approach to treatment, including surgery, systemic therapy, and radiotherapy in select cases. Established histologic variants include small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT), small cell carcinoma of the ovary pulmonary type (SCCOPT), and non-small cell neuroendocrine carcinomas (NSCNECs). These tumours present with unilateral or bilateral adnexal masses and associated abdominopelvic symptoms. SCCOHT often presents in young patients (median age 24) and is associated with a pathognomonic germline or somatic SMARCA4 mutation, which results in loss of BRG1 staining by immunohistochemistry. Patients and appropriate family members should be referred to a genetic counsellor for germline testing of SMARCA4. Management involves surgical cytoreduction, multi-agent chemotherapy, and, in select cases of patients with a complete response, high-dose chemotherapy followed by autologous stem cell transplant. Radiotherapy can be considered in cases of persistent disease or for palliation of symptoms in the recurrent setting. Treatment of these tumours, once diagnosed, is emergent as the disease behaves aggressively. These tumours may be transiently chemosensitive; however, responses to chemotherapy in the setting of persistent or recurrent disease is limited. Patients and their families can be referred to the SCCOHT/SMARCA4 registry to contribute to ongoing translational research efforts and understanding of genetic penetrance. SCCOPT often presents in patients>40 years of age and is histologically comparable to small cell neuroendocrine carcinoma of the lung. Therefore, metastatic neuroendocrine tumours of a non-gynaecologic primary should be excluded through expert pathology review and comprehensive baseline imaging, which may include an FDG-PET scan. SCCOPT is not known to be associated with a specific molecular profile. While data is limited in this subtype, the same principles of primary cytoreductive and staging surgery apply to these patients, followed by adjuvant chemoradiation or chemotherapy alone. Unfortunately, prognosis remains poor for these rare tumours with high recurrence rates despite multimodal treatment. This underscores the importance of ongoing research efforts and registry participation to guide future directions and novel therapeutic approaches. This chapter reviews the epidemiology, clinical presentation, diagnostic workup, pathologic characteristics, current treatment paradigms, and future directions in the management of small cell and neuroendocrine ovarian carcinomas.