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Benign Triton Tumor (BTT) is an extremely uncommon tumor involving the nerves and muscles (neuromuscular lesion).

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

  • BTT (Benign Triton Tumor)
  • Nerve Rhabdomyoma
  • Neuromuscular Hamartoma

What is Benign Triton Tumor? (Definition/Background Information)

  • Benign Triton Tumor (BTT) is an extremely uncommon tumor involving the nerves and muscles (neuromuscular lesion). It is also known as a Neuromuscular Hamartoma
  • Currently, no cause or risk factors have been identified for Benign Triton Tumor. The tumor is mostly seen in children below 2 years of age
  • The tumors may involve large nerves (such as the sciatic nerve or brachial plexus) causing associated neurological signs and symptoms including numbness, weak muscles, and dull reflexes
  • The treatment of choice is a complete surgical excision and removal. The healthcare provider also attempts to preserve the nerve function as much as possible during surgery. In some cases, a ‘wait and watch’ approach may be recommended for small-sized tumors
  • The prognosis of Benign Triton Tumor is generally good with suitable treatment; however, it also depends upon the tumor location, size, and behavior (whether aggressive or non-aggressive)

Who gets Benign Triton Tumor? (Age and Sex Distribution)

  • Benign Triton Tumor is a very rare abnormal malformation, which is mostly present in children (including infants), less than 2 years old. In some rare cases, older children and adults are also known to be affected
  • Both males and females are affected; some case study reports indicate a female predominance (2:5 male-female ratio)
  • Individuals of all racial and ethnic background may be affected

What are the Risk Factors for Benign Triton Tumor? (Predisposing Factors)

  • Currently, no risk factors have been identified for Benign Triton Tumor

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 Benign Triton Tumor? (Etiology)

  • The cause of development of Benign Triton Tumor is largely unknown. Research is currently being undertaken to identify the relevant causal factors
  • Some researchers believe that since BTT is a malformation, it may occur due to abnormalities during fetal growth and development

What are the Signs and Symptoms of Benign Triton Tumor?

The signs and symptoms of Benign Triton Tumor may include:

  • Presence of a large-mass that causes increasing pain; BTT is often present as a multi-nodular mass
  • The tumor mass is firm and well-circumscribed
  • Since, the nerves are involved, children are seen to have numbness, pin-prick sensation, muscle weakness and cramping, and loss of reflexes
  • Common tumor sites include the following:
    • Sciatic nerve (a major nerve originating in the lower back and running down the thighs/legs)
    • Brachial plexus (a large bundle of nerves in the neck and shoulders running into the arms and trunk)
    • Rarely, the cranial nerves (such as the trigeminal nerve) may be involved; in which case an intracranial mass may be seen
    • Small-sized tumors may be seen affecting smaller/peripheral nerves
  • A few cases have revealed an aggressive involvement of the nerves and muscles by the tumor, making surgery difficult to perform. On such cases, tumors involving the central/major nerves are known to be more aggressive than the ones involving the peripheral nerves

Small-sized tumors may remain asymptomatic and detected only incidentally, during a medical examination for some other health conditions.

How is Benign Triton Tumor Diagnosed?

A diagnosis of Benign Triton Tumor may involve the following:

  • A thorough medical history and complete physical examination
  • Neurological exam of the entire body
  • Radiological studies (CT, MRI scan) of the affected region
  • Nerve conduction studies of the affected nerve
  • Tissue biopsy of tumor specimen: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive 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 Benign Triton Tumor?

Benign Triton Tumor is a very uncommon tumor that can cause significant complications depending on its location and size. The following complications may be seen:

  • Severe involvement of large nerves causing associated neurological complications or deficits
  • Muscular atrophy
  • Headaches and migraine, if the cranial nerves are involved
  • Complications during or following surgery, due to damage to the nerves and muscles
  • Recurrence of BTT after surgery is reported to be extremely rare

How is Benign Triton Tumor Treated?

The treatment of Benign Triton Tumor may involve the following:

  • The healthcare provider may recommend a ‘wait and watch’ approach in cases where the tumors are small, the peripheral nerves is involved, and the tumor behavior is less aggressive (or is indolent)
  • Conservative measures and pain management may be undertaken in such cases
  • Complete surgical excision and tumor removal is considered:
    • When some of the major or large nerves are involved
    • Healthcare providers recommend the removal of aggressively behaving tumors
    • Since tumor recurrence risk is very low, surgery is advocated in many cases
    • Preservation of the nerve function is important and is considered to the maximum extent possible during surgery
  • Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
  • Long-term follow-up care with regular screening and check-ups are very important

How can Benign Triton Tumor be Prevented?

Currently, there are no known methods to prevent Benign Triton Tumor occurrence.

What is the Prognosis of Benign Triton Tumor? (Outcomes/Resolutions)

  • The prognosis for Benign Triton Tumor is generally good with appropriate treatment, since it is a benign tumor
  • However, the prognosis also depends upon the location and size of the tumor, the nerves that are involved, the aggressive nature of the tumor, and surgical accessibility

Additional and Relevant Useful Information for Benign Triton Tumor:

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/cancer/

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:

WHO Classification of Tumours of Soft Tissue and Bone; Edited by Christopher D.M. Fletcher, Julia A. Bridge, Pancras C. W. Hogendoorn, Fredrik Mertens; 4th Ed., IARC Press, Lyon, 2013

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4200363/ (accessed on 08/20/16)

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3589612/ (accessed on 08/20/16)

Helpful Peer-Reviewed Medical Articles:

Thakrar, R., Robson, C. D., Vargas, S. O., Meara, J. G., Rahbar, R., & Smith, E. R. (2014). Benign Triton Tumor: Multidisciplinary Approach to Diagnosis and Treatment. Pediatric and Developmental Pathology, 17(5), 400-405.

VandenHeuvel, K. A., Carpentieri, D. F., Chen, J., Fung, K. M., & Parham, D. M. (2014). Ectomesenchymoma with embryonal rhabdomyosarcoma and ganglioneuroma, arising in association with benign triton tumor of the tongue. Pediatric and Developmental Pathology, 17(3), 226-230.

Singh, M., Gupta, M., Kala, C., Gupta, P., & Gupta, D. (2016). Neuromuscular Hamartoma in A 22 Year Male: A Case Report. Indian Journal of Applied Research, 6(6).

Smith, R. E., Kebriaei, M. A., Gard, A. P., Mccomb, R. D., Bridge, J. A., & Lennarson, P. J. (2014). Intracranial malignant triton tumor in a patient with neurofibromatosis type 1: case report and review of the literature. Brain tumor pathology, 31(2), 149-154.

Kao, S. C. S., Parham, D. M., & Fuller, C. (2015). Malignant tumors of peripheral nerves. In Pediatric Malignancies: Pathology and Imaging (pp. 399-414). Springer New York.

Yasuda, M., Muto, Y., Kuremoto, T., Murakami, K., Onisihi, T., Koida, A., ... & Hisa, Y. (2016). A case of recurrent malignant triton tumor successfully treated with radiotherapy. Auris Nasus Larynx.

Castro, D. E., Raghuram, K., & Phillips, C. D. (2005). Benign triton tumor of the trigeminal nerve. American journal of neuroradiology, 26(4), 967-969.

Daley, T. D., Darling, M. R., & Wehrli, B. (2008). Benign Triton tumor of the tongue. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics, 105(6), 763-766.

Kondziolka, D., Nathoo, N., Flickinger, J. C., Niranjan, A., Maitz, A. H., & Lunsford, L. D. (2003). Long-term results after radiosurgery for benign intracranial tumors. Neurosurgery, 53(4), 815-822.