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• SCCOHT is an extremely rare and aggressive ovarian cancer affecting predominantly young women. • A stage IV SCCOHT case achieved long-term remission (>5 years) after intensive multimodal therapy. • High-dose chemotherapy with autologous stem cell rescue contributed to durable complete response. • Survivorship care must address relapse surveillance, late toxicities, fertility, endocrine, and psychosocial needs. Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is an extremely rare and aggressive ovarian malignancy that predominantly affects young women. Prognosis is stage-dependent, with a 5-year overall survival (OS) of approximately 20% in advanced stages (II–IV). Treatment typically involves a multimodal approach. Case Presentation. We present the case of a 24-year-old woman diagnosed with stage IV SCCOHT presenting multiple pulmonary nodules and enlarged retroperitoneal lymph nodes. Following standard treatment protocols, including surgery, chemotherapy and high-dose chemotherapy with autologous stem cell rescue, she achieved a complete response and has remained in remission for over five years despite initial pulmonary metastases. Given the rarity of SCCOHT, management was guided by recommendations and through the expertise of a national reference center. This case highlights that, while durable remission is possible, intensive multimodal therapy is required, and SCCOHT survivors face significant long-term challenges. A comprehensive, multidisciplinary survivorship approach is essential to optimize long-term outcomes and quality of life, in accordance with current guidelines.
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Castanet et al. (2025) studied this question.
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