Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Patient Characteristics, Treatment, and Outcome—A Systematic Review

Abstract

Background: Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare aggressive ovarian malignancy mainly affecting children, adolescents, and young adults. Since the discovery of mutations in the SMARCA4 gene in 2014, SCCOHT has become the subject of extensive investigation. However, international uniform treatment guidelines for SCCOHT are lacking and the outcome remains poor. The aim of this systematic review is to generate an overview of all reported patients with SCCOHT from 1990 onwards, describing the clinical presentation, genetic characteristics, treatment, and outcome. Methods: A systematic search was performed in the databases Embase, Medline, Web of Science, and Cochrane for studies that focus on SCCOHT. Patient characteristics and treatment data were extracted from the included studies. Survival was estimated using Kaplan–Meier’s methodology. To assess the difference between survival, the log-rank test was used. To quantify the effect of the FIGO stage, the Cox proportional hazard regression model was estimated. The chi-squared test was used to study the association between the FIGO stage and the surgical procedures. Results: Sixty-seven studies describing a total of 306 patients were included. The median patient age was 25 years (range 1–60 years). The patients mostly presented with non-specific symptoms such as abdominal pain and sometimes showed hypercalcemia and elevated CA-125. A great diversity in the diagnostic work-up and therapeutic approaches was reported. The chemotherapy regimens were very diverse, all containing a platinum-based (cisplatin or carboplatin) backbone. Survival was strongly associated with the FIGO stage at diagnosis. Conclusions: SCCOHT is a rare and aggressive ovarian cancer, with a poor prognosis, and information on adequate treatment for this cancer is lacking. The testing of mutations in SMARCA4 is crucial for an accurate diagnosis and may lead to new treatment options. Harmonization and international collaboration to obtain high-quality data on diagnostic investigations, treatment, and outcome are warranted to be able to develop international treatment guidelines to improve the survival chances of young women with SCCOHT.

A Rare Case of Advanced-Stage Small Cell Carcinoma of the Ovary Identified During the Third Trimester of Pregnancy

“…SCCOHT is a rare malignancy that predominantly arises in young females and is associated with a poor prognosis. A review of the literature demonstrates only a few cases of SCCOHT in pregnancy [ 2 , 8 ]. The clinical presentation of SCCOHT is typically non-specific with the most frequently mentioned symptoms being localized abdominal pain and abdominal distension [ 2 ], which are symptoms easily masked in pregnancy.…”

“…A review of the literature demonstrates only a few cases of SCCOHT in pregnancy [ 2 , 8 ]. The clinical presentation of SCCOHT is typically non-specific with the most frequently mentioned symptoms being localized abdominal pain and abdominal distension [ 2 ], which are symptoms easily masked in pregnancy. Definitive preoperative diagnosis of SCCOHT is generally challenging without a tissue biopsy [ 2 , 9 ], especially when compounded by pregnancy.…”

“…The clinical presentation of SCCOHT is typically non-specific with the most frequently mentioned symptoms being localized abdominal pain and abdominal distension [ 2 ], which are symptoms easily masked in pregnancy. Definitive preoperative diagnosis of SCCOHT is generally challenging without a tissue biopsy [ 2 , 9 ], especially when compounded by pregnancy. Histopathological examination remains the gold standard for diagnosis of SCCOHT [ 1 , 2 , 9 ].…”

“…Definitive preoperative diagnosis of SCCOHT is generally challenging without a tissue biopsy [ 2 , 9 ], especially when compounded by pregnancy. Histopathological examination remains the gold standard for diagnosis of SCCOHT [ 1 , 2 , 9 ]. In this case report, suspected ovarian malignancy was identified in a pregnant female at 27 years old, who had no known significant medical history or risk factors.…”

“…The majority of women diagnosed with SCCOHT are women who are less than 50 years old [ 1 ]. The likelihood of survival is strongly associated with the stage of the disease at the time of diagnosis rather than the treatment modalities used [ 2 , 3 ]. The limited data available describes a one-year survival rate of only 50%, with an overall five-year survival rate of approximately 10% [ 3 ].…”