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Cellular Neurothekeoma is a benign tumor affecting the covering of the nerves, called the nerve sheath.

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

  • Cellular Neuromyxoma
  • Cellular NTK

What is Cellular Neurothekeoma? (Definition/Background Information)

  • Cellular Neurothekeoma is a benign tumor affecting the covering of the nerves, called the nerve sheath
  • These are rare tumors that occur as nodules under the skin and can occur anywhere in the body
  • Cellular Neurothekeoma treatment usually involves complete surgical removal of the tumor

Who gets Cellular Neurothekeoma? (Age and Sex Distribution)

  • Both males and females are affected by Cellular Neurothekeoma, although it is more common in females than males
  • It commonly occurs in young individuals (in their mid-20s)
  • There is no racial or geographical restriction to Cellular Neurothekeoma

What are the Risk Factors for Cellular Neurothekeoma? (Predisposing Factors)

Cellular Neurothekeoma is a rare tumor and no risk factors have been identified so far.

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 Cellular Neurothekeoma? (Etiology)

  • The exact cause of Cellular Neurothekeoma formation is presently unknown
  • Researchers have not found the cell of origin of this tumor type

What are the Signs and Symptoms of Cellular Neurothekeoma?

The signs and symptoms of Cellular Neurothekeoma include:

  • They appear as a painless skin mass or nodule; but, occasionally they may be painful to touch
  • The skin over the nodule is pink, firm, and usually intact; no ulceration is identified
  • The mass is usually between 0.5 to 2 cm. Occasionally, multiple nodules are seen together
  • In a majority of the cases, these nodules do not cause any symptoms
  • The most common location of the nodules is the head and neck region. However, these tumors can occur anywhere on the body

How is Cellular Neurothekeoma Diagnosed?

Cellular Neurothekeoma is diagnosed as follows:

  • A thorough physical examination and a medical history
  • A biopsy of the mass/tumor: The mass is examined by a pathologist under the microscope to arrive at a conclusive diagnosis

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 Cellular Neurothekeoma?

  • Cellular Neurothekeoma are benign tumors and no complications are usually reported
  • Occasionally, if the tumors are present in the head and neck region, they may present a cosmetic issue

How is Cellular Neurothekeoma Treated?

  • The treatment of Cellular Neurothekeoma is complete surgical removal. Such a surgical procedure usually cures the condition
  • If the tumors are not completely removed, then they may recur after a period of time. Hence, it is important to completely remove them through surgery

How can Cellular Neurothekeoma be Prevented?

Currently, there are no methods to prevent Cellular Neurothekeoma occurrence.

What is the Prognosis of Cellular Neurothekeoma? (Outcomes/Resolutions)

The prognosis for Cellular Neurothekeoma is excellent, since it is a benign tumor.

Additional and Relevant Useful Information for Cellular Neurothekeoma:

  • A Cellular Neurothekeoma should be distinguished from a classical nerve sheath myxoma, which is also a benign tumor affecting the nerve sheath
  • The pathologist may use additional special techniques such as immunostains, for arriving at a diagnosis. Cellular Neurothekeoma is occasionally positive for S100 protein, CD34, smooth muscle actin, collagenous type IV, neuron specific enolase, and CD57. There is no specific staining pattern for Cellular Neurothekeoma that is diagnostic for the tumor

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/17325474 (accessed on May 26, 2013)

http://www.ncbi.nlm.nih.gov/pubmed/9639116 (accessed on May 26, 2013)

Helpful Peer-Reviewed Medical Articles:

Akhtar, S., Coldewey, J., & Timmons, M. J. (2004). Cellular neurothekeoma in the arm of a child. Br J Plast Surg, 57(6), 572-574. doi: 10.1016/j.bjps.2004.02.001

Benbenisty, K. M., Andea, A., Metcalf, J., & Cook, J. (2006). Atypical cellular neurothekeoma treated with Mohs micrographic surgery. Dermatol Surg, 32(4), 582-587; discussion 587. doi: 10.1111/j.1524-4725.2006.32120.x

Papadopoulos, E. J., Cohen, P. R., & Hebert, A. A. (2004). Neurothekeoma: report of a case in an infant and review of the literature. J Am Acad Dermatol, 50(1), 129-134. doi: 10.1016/S0190

Safadi, R. A., Hellstein, J. W., Diab, M. M., & Hammad, H. M. (2010). Nerve sheath myxoma (neurothekeoma) of the gingiva, a case report and review of the literature. Head Neck Pathol, 4(3), 242-245. doi: 10.1007/s12105-010-0183-5

Vij, M., Jaiswal, S., Agrawal, V., Jaiswal, A., & Behari, S. (2013). Nerve sheath myxoma (neurothekeoma) of cerebellopontine angle: case report of a rare tumor with brief review of literature. Turk Neurosurg, 23(1), 113-116. doi: 10.5137/1019-5149.JTN.4255-11.1

Hornick, J. L., & Fletcher, C. D. (2007). Cellular neurothekeoma: detailed characterization in a series of 133 cases. The American journal of surgical pathology, 31(3), 329-340.

Page, R. N., King, R., Mihm Jr, M. C., & Googe, P. B. (2004). Microphthalmia transcription factor and NKI/C3 expression in cellular neurothekeoma. Modern pathology, 17(2), 230.

Plaza, J. A., Torres-Cabala, C., Evans, H., Diwan, A. H., & Prieto, V. G. (2009). Immunohistochemical expression of S100A6 in cellular neurothekeoma: clinicopathologic and immunohistochemical analysis of 31 cases. The American Journal of Dermatopathology, 31(5), 419-422.