Benign triton tumor Q87.-

Last updated on: 03.08.2021

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History
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Markel and Enzinger (1982)

Definition
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Benign Triton tumor (BTT), also known as neuromuscular hamartoma, is a rare hamartoma occurring in a tumor form and consisting of mature neural as well as well-differentiated striated muscle elements (see also Triton tumor, malignant).

Occurrence/Epidemiology
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Mostly early childhood onset. Also in adolescents (Amita K et al. 2013). Occurrence in adulthood is an exceptional rarity (Bae DH et al. 2014).

Localization
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The tumor formations primarily affect the major central cranial nerves and cause respective deficits (visual and auditory disturbances; facialis palsies- Bae DH et al. 2014; Gersdorff MC et al.1996; Corrales CE et al. 2010). Furthermore, they can occur as intracranial or as tumor masses of the spinal cord (Chapon F et al. 1991). In peripheral localization (peripheral nerves are affected), they may appear as asymptomatic cutaneous or subcutaneous nodules or nodules or as circumscribed swellings (see also case report).

Histology
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Mixture of normally matured nerve trunks as well as striated and smooth muscles.

Note(s)
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The name Triton tumor originated from studies of Triton salamanders in which supernumerary extremities grew with bone and muscle after implantation of sciatic nerves in the back.

Case report(s)
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Reported was the case of a 14-year-old boy who presented with an asymptomatic swelling of the chin over a period of 18 months which gradually increased and involved the left side of the lower lip. Clinically, a diagnosis of neurofibroma was made. An excisional biopsy confirmed the diagnosis of a "benign triton tumor" (Amita K et al. 2013).

Literature
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  1. Amita K et al (2013) Benign triton tumor: a rare entity in head and neck region. Korean J Pathol 47:74-76.
  2. Bae DH et al (2014) Adulthood benign triton tumor developed in the orbit. J Korean Neurosurg Soc 56:146-148.
  3. Chapon F et al. (1991) Lipome spinal associé à un hamartome neuromusculaire. A propos d'un cas. Ann Pathol11:345-348.
  4. Corrales CE et al. (2010) Facial and vestibulocochlear nerve avulsion at the fundus of the internal auditory canal in a child without a temporal bone fracture. Otol Neurotol. 31:1508-1510.
  5. Gersdorff MC et al.(1996) Neuromuscular hamartoma of the internal auditory canal. Eur Arch Otorhinolaryngol 253:440-442.
  6. Markel SF et al. (1982) Neuromuscular hamartoma--a benign "triton tumor" composed of mature neural and striated muscle elements. Cancer 49:140-144.

Outgoing links (1)

Malignant triton-tumor;

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 03.08.2021