Rare Rheumatology News

Disease Profile

Ovarian small cell carcinoma

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Small cell carcinoma of the ovary


Rare Cancers


Ovarian small cell carcinoma is a rare form of ovarian cancer that typically occurs in young women. It is typically an aggressive cancer that can spread (metastasize) to other parts of the body. There are two types of ovarian small cell carcinoma: hypercalcemic and pulmonary.[1] Approximately 2/3 of individuals with ovarian small cell carcinoma have the hypercalcemic type.[2] Symptoms are fairly nonspecific and are similar to other types of ovarian cancer.[3] Symptoms may include: bloating, pelvic or abdominal pain, and appetite changes.[4] Treatment may involve surgery to remove the tumor/tumors if the cancer is detected at an early stage, chemotherapy, and radiation therapy.[1]


There are no standard recommendations guiding the treatment of ovarian small cell carcinoma. Some authors suggest that treatment should be similar to that of other small cell carcinomas. This might include surgery to remove the tumor or tumors if appropriate, chemotherapy, and radiation therapy. Many different chemotherapy drugs have been utilized with varying rates of success including but not limited to: carboplatin, paclitaxelvinblastinecyclophosphamide, bleomycindoxorubicin, and etoposide.[5]


Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

        In-Depth Information

        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Ovarian small cell carcinoma. Click on the link to view a sample search on this topic.


          1. K. Münstedt, R. Estel, T. Dreyer, A. Kurata, A. Benz. Small Cell Ovarian Carcinomas – Characterisation of Two Rare Tumor Entities. Geburtshilfe Frauenheilkd. July 2013; 73(7):698-704. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3858994/.
          2. Lisa F Gamwell, Karen Gambaro, Maria Merziotis, Colleen Crane, Suzanna L Arcand, Valerie Bourada, Christopher Davis, Jeremy A Squire, David G Huntsman, Patricia N Tonin, Barbara C Vanderhyden. Small cell ovarian carcinoma: genomic stability and responsiveness to therapeutics. Orphanet J Rare Dis. 2013; 8(33):https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3635907/.
          3. About Ovarian Cancer. Minnesota Ovarian Cancer Alliance. https://mnovarian.org/about-ovarian-cancer/. Accessed 6/28/2016.
          4. Signs and symptoms of ovarian cancer. American Cancer Society. 02/04/2016; https://www.cancer.org/cancer/ovariancancer/detailedguide/ovarian-cancer-signs-and-symptoms.
          5. Jian-jun Wang, Qi Liu, Nan Wu, Xiao-die Zhou, Hai Wang, Xuan Wang, Pin Tu, Xiao-jun Zhou, Qun-li Shi. Ovarian small-cell carcinoma hypercalcemic type successfully treated: a case report and literature review. Onco Targets Ther. 2016; 9:1409-1414. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4795582/.

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