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Disease Profile

Granular cell tumor

Prevalence
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.

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US Estimated

Europe Estimated

Age of onset

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ICD-10

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Inheritance

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

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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.

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X-linked
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.

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

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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.

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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.

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Not applicable

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Other names (AKA)

Giant granulocellular Abrikosov's tumor; Malignant variant of Abrikosov's tumor; Abrikosoff's granulous cell tumor;

Categories

Rare Cancers

Summary

Granular cell tumors (GCTs) are soft tissue tumors that can occur anywhere in the body. They are thought to arise from the cells that surround and insulate the nerve cells in our body (Schwann cells). Most granular cell tumors are benign (non-cancerous), although some may be locally aggressive. Less than 2% are malignant (cancerous), but these are aggressive and often are associated with a poor prognosis.[1] GCTs affect females more often than males and usually present as a solitary painless mass. A few individuals have multiple GCTs.[2] In most cases, these tumors are located in the skin of the head and neck, especially inside the mouth (70%), the tongue being the most common site. They may also occur in the breast, heart, pituitary gland, stomach, esophagus, genitalia, and upper respiratory tract.[3][4] GCTs may be part of some genetic syndromes, such as LEOPARD syndrome and neurofibromatosis. Complete surgical removal of the tumor is usually curative, but in some cases the tumors may recur.[4]

Organizations

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 Providing General Support

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    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

    • The American Cancer Society provides more information on Granular cell tumor.
    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • 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 Granular cell tumor. Click on the link to view a sample search on this topic.

        References

        1. Osipov VO. Granular Cell Tumors. Medscape Reference. September 8, 2016; https://emedicine.medscape.com/article/282430-overview.
        2. Granular Cell Tumor. NCI Methathesaurus. https://ncim.nci.nih.gov/ncimbrowser/pages/concept_details.jsf?dictionary=NCI%20MetaThesaurus&code=C0085167&type=properties.
        3. Thumallapally N, Ibrahim U, Kesavan M, Chang Q, Opitz L, Dhar M & Andrawes S. Esophageal Granular Cell Tumor: A Case Report and Review of Literature. Cureus. September 14, 2016; 8(9):e782. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5065346/.
        4. Richmond AM, La Rosa FG & Said S. Granular cell tumor presenting in the scrotum of a pediatric patient: a case report and review of the literature. J Med Case Rep. June 4, 2016; 10(1):161. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4893259/.