Low-Grade Malignant Triton Tumor of the Neck: A Case Report and Review of the Literature

Omar, Taissir A.; Raslan, Hanaa S.; Sheikh, Sahar El; Rizq, Moataz; Draz, Awatef

doi:10.1155/2014/674094

Cited by 5 publications

(5 citation statements)

References 30 publications

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“…Sporadic MPNSTs are rare, occurring in only 0.001% of the general population [3]. MTT is also associated with NF-1 in 69% of cases [16], and, otherwise, arises secondary to radiation therapy [17] or is sporadic [18]. The present patient had a sporadic MTT, since there was no history of NF-1 or radiation therapy.…”

Section: Discussionmentioning

confidence: 66%

Malignant triton tumor of the duodenum: report of a case

et al. 2018

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We report a case of malignant triton tumor of the duodenum, which is extremely rare. A submucosal malignant tumor was detected in the duodenum of a 49-year-old woman. The tumor was completely resected by performing pancreaticoduodenectomy. Pathological examination revealed that the lesion was a malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation, i.e., a malignant triton tumor. Long-term survival has been achieved with no recurrence at 8.5 years after surgery.

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Section: Discussionmentioning

confidence: 66%

Malignant triton tumor of the duodenum: report of a case

et al. 2018

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“…MPNSTs with elements of rhabdomyoblastic differentiation are termed as malignant triton tumors (MTTs). MTTs are referred to as a mosaic tumor due to their muscular and neurogenic components [ 12 ]. Masson was the first to report the presence of rhabdomyoblasts in this neurogenic tumor and described them as rhabdomyomas of the nerve [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…Nerve sheath differentiation is confirmed by S-100 protein and Leu-7 (CD57) positivity. Rhabdomyoblastic differentiation is confirmed by immunohistochemical positivity for desmin, actin, and myogenin [ 3 , 12 ]. Through these findings, the diagnosis of MTT can be positively made and the proper course of treatment can be pursued.…”

Section: Discussionmentioning

confidence: 99%

Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1

Basnet

Sebastian

Capo

2015

Case Reports in Oncological Medicine

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Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above.

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“…As expected, these cells (like the tumor cells of RMS) are positive for desmin and myogenin/MyoD1. Malignant Triton tumors are regarded to behave in an aggressive fashion, even more than usual MPNST, [22,23,30,31] though head and neck cases described as ''low-grade'' have been reported [32,33]. It has been suggested that tumors in the sinonasal tract have a more indolent course than those arising in other head and neck sites (however, at least some of those indolent ''malignant Triton tumors'' could in fact represent the newlydescribed ''low-grade sinonasal sarcoma with neural and myogenic features,'' a tumor that lacks myogenin/MyoD1 expression) [34,35].…”

Section: Malignant Triton Tumormentioning

confidence: 99%

Rhabdomyoblastic Differentiation in Head and Neck Malignancies Other Than Rhabdomyosarcoma

Bishop

Thompson

Cardesa

et al. 2015

Head and Neck Pathol

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Rhabdomyosarcoma is a relatively common soft tissue sarcoma that frequently affects children and adolescents and may involve the head and neck. Rhabdomyosarcoma is defined by skeletal muscle differentiation which can be suggested by routine histology and confirmed by immunohistochemistry for the skeletal muscle-specific markers myogenin or myoD1. At the same time, it must be remembered that when it comes to head and neck malignancies, skeletal muscle differentiation is not limited to rhabdomyosarcoma. A lack of awareness of this phenomenon could lead to misdiagnosis and, subsequently, inappropriate therapeutic interventions. This review focuses on malignant neoplasms of the head and neck other than rhabdomyosarcoma that may exhibit rhabdomyoblastic differentiation, with an emphasis on strategies to resolve the diagnostic dilemmas these tumors may present. Axiomatically, no primary central nervous system tumors will be discussed.

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Low-Grade Malignant Triton Tumor of the Neck: A Case Report and Review of the Literature

Cited by 5 publications

References 30 publications

Malignant triton tumor of the duodenum: report of a case

Malignant triton tumor of the duodenum: report of a case

Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1

Rhabdomyoblastic Differentiation in Head and Neck Malignancies Other Than Rhabdomyosarcoma

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