Abstract
Background: Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare but highly undifferentiated, aggressive malignancy that primarily affects young women. Most SCCOHT patients present with advanced disease and have a very poor prognosis. Although several therapeutic regimens have been proposed, there is no consensus on the optimal treatment strategy. Case: Herein, we describe three cases of advanced-stage SCCOHT aged from16 to 36 treated with cytoreductive surgery and chemotherapy with or without immune checkpoint blockade treatment. With different strategy, the patients showed utterly different outcomes. Conclusion: The cases highlight the importance of prompt diagnosis as well as early, aggressive, and combined modality treatment of SCCOHT. We believe that improved therapies could result in more young patients surviving SCCOHT.
Highlights
Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare, highly undifferentiated, aggressive ovarian malignancy of unknown cell origin, which usually occurs in young women
Several therapeutic regimens have been proposed for SCCOHT; to date, there is no consensus on the optimal treatment strategy
SCCOHT patients should be encouraged to participate in clinical trials if available and consider off-label immune checkpoint blockade treatment based on drug availability
Summary
Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare, highly undifferentiated, aggressive ovarian malignancy of unknown cell origin, which usually occurs in young women. It was first described by Dickersin et al [1] in 1982 as paraneoplastic hypercalcemia. Several therapeutic regimens have been proposed for SCCOHT; to date, there is no consensus on the optimal treatment strategy. With the development of high-throughput sequencing technology, molecular characteristics of SCCOHT have been gradually revealed, and new molecular targets have been proposed. We describe three SCCOHT cases with different clinical stages, different treatment approaches, and different outcomes and present an overview of the clinical and pathological features, molecular features, multimodality treatment or therapy, molecular targeting and immunotherapy, genetic counseling, and prevention of SCCOHT
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