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

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.
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PMID:Spindle cell rhabdomyosarcoma (so-called) in adults: report of two cases with emphasis on differential diagnosis. 953 74

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 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

Divergent differentiation occurs in a subset of malignant peripheral nerve sheath tumors (MPNST), but differentiation toward epithelial elements is unusual. MPNST with both heterologous rhabdomyosarcomatous differentiation (malignant Triton tumor), and glandular epithelial differentiation is exceedingly rare, with only 11 cases reported since 1973, and only 1 with histologically proven metastasis. We describe a case occurring in the mediastinum (between the ascending aorta and the main pulmonary artery) of a 40-year-old man with neurofibromatosis type-1. Despite treatment, the tumor metastasized to the right frontal lobe 16 months after diagnosis. Histologically, the primary neoplasm comprised cellular fascicles of atypical spindle cells, with discrete foci of well-formed or more primitive glandular epithelial structures, while the metastases comprised spindle cell sarcoma, including rhabdomyosarcoma, without epithelial elements. We review and discuss the literature on these little characterized neoplasms, including histology and clinical features, adding to the spectrum of documented cases of divergent epithelial differentiation in Triton tumor.
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PMID:Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous and Glandular Elements: Rare Epithelial Differentiation in a Triton Tumor. 2591 65