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Microscopic pathology image showing Granular Cell Tumor of the Skin. H&E stain.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Malignant Granular Cell Myoblastoma
  • Malignant Granular Cell Neoplasm
  • Malignant Granular Cell Tumor of Skin

What is Malignant Granular Cell Tumor? (Definition/Background Information)

  • Malignant Granular Cell Tumor is very rare variant of Granular Cell Tumor (GCT); less than 1 in 50 GCTs, has a tendency of becoming malignant
  • Generally, a Malignant Granular Cell Tumor would have higher amounts of abnormal cells (called cellular atypia) and an increased proliferation/growth
  • About 1 in 10 individuals with a benign Granular Cell Tumor may have multiple GCT in the body. However, having multiple benign GCTs do not indicate that the individual has a Malignant Granular Cell Tumor

Who gets Malignant Granular Cell Tumor? (Age and Sex Distribution)

  • Malignant Granular Cell Tumor can affect an individual at any age, but is usually seen between the ages of 30 and 50 years. It is rarely observed in children

What are the Risk Factors for Malignant Granular Cell Tumor? (Predisposing Factors)

No documented risk factors have been reported for Malignant Granular Cell Tumor, however:

  • Though Granular Cell Tumors do not usually run within families, rare cases of increased incidence within families, have been reported

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Malignant Granular Cell Tumor? (Etiology)

  • The exact cause of Malignant Granular Cell Tumor is unknown
  • Researchers have documented certain genetic changes within the tumor. However, cases where these specific genetic mutations have been observed are rare. Thus, studies regarding genetic changes are limited
    • Malignant GCT is not associated with any known congenital syndrome

What are the Signs and Symptoms of Malignant Granular Cell Tumor?

The signs and symptoms of Malignant Granular Cell Tumor are:

  • The malignant variant of Granular Cell Tumor is more commonly found in the arms and legs, unlike the benign Granular Cell Tumor, which is more common in the head and neck region
  • A Malignant GCT is particularly common in the thigh region
  • Unlike benign GCT, the malignant variety tumor grows more rapidly
  • A Malignant Granular Cell Tumor may show the following features: Size greater than 3 cm (along the larger dimension), ulceration of overlying skin, and a rapid increase in size
  • About 1 in 10 individuals with a benign Granular Cell Tumor may have multiple GCT in the body. However, having multiple benign GCTs do not indicate that the individual has a Malignant Granular Cell Tumor

How is Malignant Granular Cell Tumor Diagnosed?

A Malignant Granular Cell Tumor is a rapidly-growing tumor. If the tumor occurs in non-skin areas then the diagnosis is usually more difficult to make since the tumor is not readily visible to the eye. Malignant Granular Cell Tumor is diagnosed using the following methods:

  • Complete physical examination and a thorough medical history
  • A biopsy of the nodule:

The biopsy is sent to a laboratory where the pathologist examines the nodule under a microscope to arrive at a conclusive diagnosis. A pathologist would be able to distinguish a benign Granular Cell Tumor from a Malignant Granular Cell Tumor. Normally, a malignant tumor would have a higher amount of abnormal cells and increased proliferation (growth).

Clinical correlation is very important to arrive at a diagnosis of Malignant Granular Cell Tumor. A Malignant GCT may show the following features: Size greater than 3 cm (along the larger dimension), ulceration of overlying skin, and a rapid increase in size

  • Radiological imaging studies are also helpful in making a determination, if the tumor is malignant, or not
  • Benign Granular Cell Tumors are also found within the muscles, tendons, ligaments, soft tissues, and rarely within lymph nodes. Presence of GCT within the lymph nodes is a difficult problem for the doctors. This is because it is difficult to tell whether the presence of such tumors in the lymph nodes are due to a metastasis from a different body site, or if the GCT originated within the lymph nodes itself (primary site)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Malignant Granular Cell Tumor?

Complications due to a Malignant Granular Cell Tumor could include:

  • The skin over the malignant tumor could break causing ulceration, infecting the skin
  • The tumor can spread into the surrounding areas through infiltration, and also spread to distant regions of the body (metastasis). Extensive metastasis may result in a fatality

How is Malignant Granular Cell Tumor Treated?

The treatment measures for Malignant Granular Cell Tumor include:

  • Complete surgical resection of the tumor is usually curative, if the tumor is confined to one site. Occasionally, a surgical treatment may not be effective, if the tumor is at an inaccessible location, for the surgical procedure
  • If the tumor has spread to other parts of the body then a combination of surgery, chemotherapy, and radiation therapy may be considered by the healthcare provider
  • Sometimes, it is difficult to distinguish between a benign GCT and a malignant GCT. Hence, a complete removal of the tumor is recommended

How can Malignant Granular Cell Tumor be Prevented?

  • The cause of Malignant Granular Cell Tumor is unknown; hence, there are no known methods to prevent the tumor occurrence
  • Early diagnosis with close monitoring, and treatment of the tumor is important

What is the Prognosis of Malignant Granular Cell Tumor? (Outcomes/Resolutions)

  • The prognosis of Malignant Granular Cell Tumor depends upon the stage of the tumor (i.e. how much has the tumor spread in the body)
  • Lower stage tumors have a better prognosis than higher stage tumors

Additional and Relevant Useful Information for Malignant Granular Cell Tumor:

  • The Malignant Granular Cell Tumors should be clinically and histologically distinguished from other tumors. These tumors include rhabdomyoma, hibernoma, fibroxanthoma, neurofibroma and schwannoma.  A pathologist will help eliminate other tumor types
  • Sometimes the pathologist may perform special studies, which include immunohistochemical stains and histochemical stains. The common immuno stains used are S100 protein, Neuron Specific Enolase (NSE), calretinin, peripheral myelin protein, Myelin Basic Protein (MBP), alpha-1 antitrypsin protein, CD68, and vimentin. These immuno stains are usually positive in Malignant Granular Cell Tumors. The Malignant GCTs are negative for neural filaments and GFAP immunostains
  • The tumors are positive for PAS histochemical stains when examined by the pathologist under a microscope. Even though the tumors are positive for PAS stain, the tumors do not contain glycogen

What are some Useful Resources for Additional Information?

American Cancer Society (ACS)
1599 Clifton Road, NE Atlanta, GA 30329-4251
Toll-Free: (800) 227-2345
TTY: (866) 228-4327
Website: http://www.cancer.org

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A03
31 Center Drive, MSC 8322 Bethesda, MD 20892-2580
Phone: (301) 435-3848
Toll-Free: (800) 422-6237
TTY: (800) 332-8615
Email: cancergovstaff@mail.nih.gov
Website: http://www.cancer.gov

References and Information Sources used for the Article:

http://www.ncbi.nlm.nih.gov/pubmed/9669341 (accessed on 06/07/2013)

Helpful Peer-Reviewed Medical Articles:

Berg, J. C., Tewari, K. S., Del Rosario, R., & Berman, M. L. (2003). Unusual presentation of a malignant granular cell tumor of the pelvis: case report and literature review. Gynecol Oncol, 90(1), 224-230.

Isogai, K., Yamamoto, E., Yamaoka, S., Tarasawa, I., Murakami, R., Morimo, I., & Endo, T. (2005). [A case report of the malignant granular cell tumor coexisting with squamous cell carcinnoma of the esophagus]. Nihon Shokakibyo Gakkai Zasshi, 102(8), 1025-1029.

Nasser, H., Danforth, R. D., Jr., Sunbuli, M., & Dimitrijevic, O. (2010). Malignant granular cell tumor: case report with a novel karyotype and review of the literature. Ann Diagn Pathol, 14(4), 273-278. doi: 10.1016/j.anndiagpath.2009.08.004

Wang, J., Zhu, X. Z., & Zhang, R. Y. (2004). [Malignant granular cell tumor: a clinicopathologic analysis of 10 cases with review of literature]. Zhonghua Bing Li Xue Za Zhi, 33(6), 497-502.

Uzoaru, I., Firfer, B., Ray, V., Hubbard-Shepard, M., & Rhee, H. (1992). Malignant granular cell tumor. Archives of pathology & laboratory medicine, 116(2), 206-208.

Klima, M., & Peters, J. (1987). Malignant granular cell tumor. Archives of pathology & laboratory medicine, 111(11), 1070-1073.

Gokaslan, S. T., Terzakis, J. A., & Santagada, E. A. (1994). Malignant granular cell tumor. Journal of cutaneous pathology, 21(3), 263-270.

Urabe, A., Imayama, S., Yasumoto, S., Nakayama, J., & Hori, Y. (1991). Malignant granular cell tumor. The Journal of dermatology, 18(3), 161-166.

Donhuijsen, K., Samtleben, W., Leder, L. D., & Schmidt, C. G. (1979). Malignant granular cell tumor. Journal of cancer research and clinical oncology, 95(1), 93-98.

Jardines, L., Cheung, L., LiVolsi, V., Hendrickson, S., & Brooks, J. J. (1994). Malignant granular cell tumors: report of a case and review of the literature. Surgery, 116(1), 49-54.