Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant schwannoma with rhabdomyoblastic differentiation (Triton tumor) is a very rare variant of the malignant tumors arising in the peripheral nerve sheath. In 70% of the cases, the neoplasm is associated with Von Recklinghausen's neurofibromatosis; in the remaining 30% it is the sole morbid finding. Its biological behaviour is, in any case, more aggressive than that of simple malignant schwannoma; its course is rapid and its prognosis is dismal. Local recurrences are frequent, and distant metastases, preferentially situated in the lung and brain, are present in about 50% of the cases. Post-operative radiotherapy is always indicated. We report a case of a 64-year-old man who presented a right paralatero-nasal Triton tumor. Following exeresis, a cycle of high-dose radiotherapy was prescribed; the 10-year follow-up revealed no sign of recurrence or distant metastases.
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PMID:[Paralateral-nasal malignant schwannoma with rhabdomyoblastic differentiation (Triton tumor). Report of a case]. 1237 46

Malignant Triton Tumor (MTT) is a rare, malignant periphere nerve sheath tumor with rhabdomyoblastic differentiation. One third of described MTT's were located at the head and neck region. One third of these are associated with neurofibromatosis type 1. MTT most often appears in the third decade. MTT's are very aggressive tumors with early metastases and the overall survival is poor (26%). Therefore, early diagnosis and correct treatment is of utmost importance. We report a case of MTT of the left supraclavicular region in a 41-year-old man. We present the pathological findings, both light and immunohistochemically.
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PMID:Malignant triton tumor (MTT) of the neck. 1618 34

Malignant Triton tumor is a malignant peripheral nerve sheat tumor with rhabdomioblastic differentiation. These tumors are frequently associated with Neurofibromatosis type 1, sporadic cases being exteremly rare. Retroperitoneal localization have the most unfavorable prognois due to delayed diagnosis but also due to relation to adjacant organs. Preoperative diagnosis is inaccurate, but core needle biopsy gives more promising results. Aggressive surgical management remains the most effective modality since adjuvant forms of treatment like irradiation or chemotherapy do not have reproducible results. We present a 60-year-old female patient in whom a retroperitoneal presacral mass was postoperatively diagnosed as Triton tumor. At time of diagnosis, no visible metastases were present. The posterior pelvic exenteresis was performed. Intended chemotherapy was never started since multiple pulmonary, hepatic and splenic metastases were diagnosed only a month after surgery, with rapid lethal outcome. This case demonstrates the bad prognosis of malignant retroperitoneal tumors. Diagnostic tools such as refined biopsy techniques or cytogenetic analysis might help in differentiating patients who will benefit from radical surgery.
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PMID:Retroperitoneal "Triton" tumor. 1861 1