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Published on: May 20, 2014
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): About three case reports
J Benichou1, J Varinot2, R Bossi-Croci3
1Department of Obstetrics and Gynecology, Tenon University Hospital, Sorbonne University, 4, rue de la Chine, 75020 Paris, France.
Background:
Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is an extremely rare and aggressive ovarian cancer affecting young women, typically associated with poor survival. We present three cases of SCCOHT and review the current literature regarding clinical features, diagnosis, management, and survival.
Cases:
Three patients aged 16, 29, and 39 years were treated for ovarian masses. None presented with hypercalcemia. In case 1, MRI findings mimicked a mucinous cystadenocarcinoma, while the other two cases appeared as benign ovarian tumors. Following initial surgery and histological diagnosis of SCCOHT, all patients received comprehensive radical surgery, multi-agent chemotherapy, radiotherapy, and autologous stem cell transplantation. Two patients remain recurrence-free at 5 and 20 years, while the third has a 10-month follow-up. Genetic testing was performed in two patients, revealing no constitutional mutations in SMARCA4 or related genes.
Conclusions:
These cases highlight the difficulty of establishing a preoperative diagnosis of SCCOHT. Unlike previous reports, our data suggest that intensive multimodal therapy can result in favourable outcomes for stage I SCCOHT. Further studies are needed to define optimal treatment strategies.
