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Published on: May 20, 2014
Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases
D Callegaro-Filho1, D M Gershenson2, A M Nick2
1Department of Medical Oncology, Hospital Israelita Albert Einstein, São Paulo, Brazil; Division of Gynecologic Oncology, Universidade Federal de São Paulo, São Paulo, Brazil.
Objective:
Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare disease with a poor prognosis. SCCOHT has recently been shown to be associated with SMARCA4 gene mutations as well as molecular and genetic similarities to malignant rhabdoid tumors (MRT). The objective of our study is to describe the clinical characteristics, treatment modalities and outcomes of 47 patients with SCCOHT.
Methods:
We performed a retrospective analysis of 47 patients with SCCOHT evaluated at MD Anderson Cancer Center between 1990 and 2014. Medical records were reviewed for demographic information, pathologic findings, treatment regimens and outcomes.
Results:
Median age at diagnosis was 30 years (range 5-46). All patients underwent surgery with unilateral salpingo-oophorectomy (USO) performed in 26 patients (55%), and hysterectomy with bilateral salpingooophorectomy (BSO) in 21 patients (45%). Sixteen patients (34.0%) had stage I disease, six (12.8%) stage II, 23 (48.9%) stage III, and two patients (4.3%) had stage IV disease. Information on adjuvant treatment was available for 43 patients: 83.3% received chemotherapy alone, 9.5% chemotherapy followed by radiotherapy, 2.4% chemoradiation, and 4.8% did not receive any adjuvant therapy. Median follow-up was 13.2 months (range, 0.1 to 210.7) with a median overall survival of 14.9 months. Multi-agent chemotherapy and radiotherapy were associated with a better prognosis.
Conclusion:
Our findings suggest that aggressive therapy including multi-agent chemotherapy and possibly radiotherapy may extend survival. Further study is needed to improve outcomes in these patients including the adoption of systemic therapies used in MRT as well as the development of novel agents targeting specific mutations.
Insights
Aggressive treatment, including multi-agent chemotherapy and radiotherapy, may improve survival for patients with rare Small Cell Carcinoma of the Ovary-Hypercalcemic type (SCCOHT). Further research into novel agents and MRT-based therapies is crucial for better outcomes.
Area of Science:
- Gynecologic Oncology
- Rare Cancers
- Molecular Pathology
Background:
- Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare ovarian malignancy with a poor prognosis.
- SCCOHT shares molecular and genetic similarities with malignant rhabdoid tumors (MRT) and is associated with SMARCA4 gene mutations.
Purpose of the Study:
- To describe the clinical characteristics, treatment modalities, and outcomes of 47 patients diagnosed with SCCOHT.
- To evaluate the impact of different treatment strategies on patient survival.
Main Methods:
- Retrospective analysis of 47 SCCOHT patients treated at MD Anderson Cancer Center from 1990 to 2014.
- Review of medical records for demographic data, pathological findings, treatment regimens, and patient outcomes.
Main Results:
- The median age at diagnosis was 30 years. Most patients (55%) underwent unilateral salpingo-oophorectomy.
- Stage III disease was most common (48.9%). Multi-agent chemotherapy and radiotherapy were associated with improved prognosis.
- Median overall survival was 14.9 months, with aggressive therapies potentially extending survival.
Conclusions:
- Aggressive therapeutic approaches, including multi-agent chemotherapy and potentially radiotherapy, may enhance survival in SCCOHT patients.
- Further investigation is warranted to optimize treatment strategies, considering systemic therapies used for MRT and novel targeted agents.
