Advanced small-cell ovarian carcinoma, hypercalcemic type: a challenging therapeutic entity

Bitton, Rafael Caparica; Mak, Milena Perez; Takahashi, Tiago Kenji; Aguiar, Fernando Nalesso; Abdo, Elias; Diz, Maria Del Pilar Estevez

doi:10.4322/acr.2014.028

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…Multivariate analyses have demonstrated that tumor stage at diagnosis is the most significant determinant of prognosis ( Witkowski et al, 2016 ). Reports demonstrate a five-year overall survival of 33–55 % in patients with stage I disease ( Witkowski et al, 2016 , Young et al, 1994 ) and as low as 10 % in advanced stage patients ( Bitton et al, 2014 , Harrison et al, 2006 ). Witkowski et al evaluated determinants of survival and found that after tumor stage, treatment modality was the second most significant factor impacting survival.…”

Section: Discussionmentioning

confidence: 99%

“…Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is a rare gynecologic malignancy with most literature limited to case report documentation ( Bitton et al, 2014 , Tewari et al, 1997 ). SCCOHT tends to present in early adulthood ( Witkowski et al, 2016 , Callegaro-Filho et al, 2016 ) though pediatric cases have been reported.…”

Section: Introductionmentioning

confidence: 99%

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Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A review and novel case with dual germline SMARCA4 and BRCA2 mutations

Sanders¹,

Wolsky²,

Doughty³

et al. 2022

Gynecologic Oncology Reports

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A review and novel case with dual germline SMARCA4 and BRCA2 mutations

Sanders¹,

Wolsky²,

Doughty³

et al. 2022

Gynecologic Oncology Reports

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“…193 One of the most common gynecological cancers in RTPS2 is small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), which is reported to have demonstrated five-year OS rates of 33%-55% in patients with stage I disease, 194,195 and ≤10% in advanced stage patients. 196,197 Since ≥40% of women with SCCOHT may have a SMARCA4 GPV, RTPS2 may be suggested when bilateral SCCOHT is detected at a young age. 198,199 As SCCOHT affects females aged 5-46 years, abdominalpelvic ultrasound is the recommended method of surveillance for individuals with RTPS2, particularly among children and teenagers.…”

Section: Smarca4mentioning

confidence: 99%

Landscape of somatic mutated genes and inherited susceptibility genes in gynecological cancer

Watanabe,

Soeda,

Okoshi

et al. 2023

J of Obstet and Gynaecol

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Traditionally, gynecological cancers have been classified based on histology. Since remarkable advancements in next‐generation sequencing technology have enabled the exploration of somatic mutations in various cancer types, comprehensive sequencing efforts have revealed the genomic landscapes of some common forms of human cancer. The genomic features of various gynecological malignancies have been reported by several studies of large‐scale genomic cohorts, including The Cancer Genome Atlas. Although recent comprehensive genomic profiling tests, which can detect hundreds of genetic mutations at a time from cancer tissues or blood samples, have been increasingly used as diagnostic clinical biomarkers and in therapeutic management decisions, germline pathogenic variants associated with hereditary cancers can also be detected using this test. Gynecological cancers are closely related to genetic factors, with approximately 5% of endometrial cancer cases and 20% of ovarian cancer cases being caused by germline pathogenic variants. Hereditary breast and ovarian cancer syndrome and Lynch syndrome are the two major cancer susceptibility syndromes among gynecological cancers. In addition, several other hereditary syndromes have been reported to be associated with gynecological cancers. In this review, we highlight the genes for somatic mutation and germline pathogenic variants commonly seen in gynecological cancers. We first describe the relationship between clinicopathological attributes and somatic mutated genes. Subsequently, we discuss the characteristics and clinical management of inherited cancer syndromes resulting from pathogenic germline variants in gynecological malignancies.

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

Wens,

Hulsker,

Fiocco

et al. 2023

Cancers

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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.

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Advanced small-cell ovarian carcinoma, hypercalcemic type: a challenging therapeutic entity

Cited by 3 publications

References 15 publications

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A review and novel case with dual germline SMARCA4 and BRCA2 mutations

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A review and novel case with dual germline SMARCA4 and BRCA2 mutations

Landscape of somatic mutated genes and inherited susceptibility genes in gynecological cancer

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

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