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Il Giornale di Chirurgia

Istiocitoma fibroso maligno, un raro tumore della laringe: descrizione di un caso clinico

Articolo, 224 - 225
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Malignant fibrous istiocytoma (MFH) is the most frequent soft tissue sarcoma in adults. It principally occurs as a mass of the extremities, abdominal cavity, or retroperitoneum. Lanryngeal presentation of MFH is uncommon. Only sporadic laryngeal cases had been reported in Literature. It is prevalent in males. The neoplasm is characterized by spindle-shaped atypical cells arranged in a diffuse storiform pattern. Mitoses are prominent, numerous, and often atypical. Immunohistochemically, neoplastic cells are strongly positive for a1-antichymotrypsin but are negative for cytokeratins and s-100 protein. In the histopathologic point of view the MFH can be distinguished into several types; the storiform-pleomorphic type is more commonly observedin larynx. By this time, among risk factors it seems to be verified the role of ionizing radiations as reported in several works that can be found in Literature. The MFH shows a high tendency to local recurrence and to metastasizing. The most frequent seat of metastasis is the lung, but cases of bony, cerebral and neck lymph nodes metastasis were described too. The treatment of choice is the complete exeresis of the mass within a safety margin. MFH is scarcely responsive to radiotherapy, so the chemiotherapy seems the treatment adjuvant to be preferred.
Prognosis is very bad. We report a case of glottic MFH on a 46 years old man. Laser surgery (chordectomy) with associate chemiotherapy was used to treat this tumor. About two years after surgery the patient is well, with no evidence of disease.

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