ABSTRACT
Alveolar soft-part sarcoma is a clinically and morphologically distinct soft-tissue sarcoma of adolescent and young adult patients. Though immunohistochemical stains implicate a myogenic origin, the histogenesis of this tumor has not yet been established. Its high vascular nature leads to dissemination of the tumor cells into the bloodstream and metastasis. It comprises less than 1% of all soft-tissue sarcomas and less than 0.1% of sarcomas of the head and neck, preferably involving the orbit (48%) and tongue (25%). Lingual involvement is very rare and only about 31 cases have been reported in English literature. Their deceivingly indolent clinical courses often lead to misdiagnosis and delayed treatment. The reported case indicates its asymptomatic nature and microscopic similarity to granular cell tumor, which is the common benign tumor of the tongue.
Subject(s)
Adult , Histocytochemistry , Humans , Immunohistochemistry , Male , Sarcoma, Alveolar Soft Part/diagnosis , Sarcoma, Alveolar Soft Part/pathology , Tongue/pathology , Tongue Neoplasms/diagnosis , Tongue Neoplasms/pathologyABSTRACT
We report two cases of alveolar soft part sarcoma associated with bone involvement. The first case concerned a 61-year old female patient with a left sus-clavicular mass and the second a 67-year old male patient with a tumor of the left hand. The bone involvement, observed on skeletal radiography, was histologically confirmed. Surgery was the treatment in both cases, followed by radiotherapy in one case. Alveolar soft part sarcoma is a rare soft tissue malignant tumor with a local slowly growing and pejorative prognosis. If metastasis of lung, brain and bone are often present at diagnosis, the local bone involvement is not well documented
Subject(s)
Humans , Male , Female , Sarcoma/surgery , Sarcoma, Alveolar Soft Part/pathologySubject(s)
Adult , Breast Neoplasms/pathology , Female , Humans , Sarcoma, Alveolar Soft Part/pathologyABSTRACT
Os autores descrevem, em homem de 25 anos, um caso de sarcoma alveolar de partes moles (SAPM), localizado na coxa direita e com metástases pulmonares. Seis anos antes do aparecimento da neoplasia, o paciente sofreu grave traumatismo, na mesma sede onde se desenvolveu o tumor. Realçam a pouca freqnência da neoplasia, discutem as dificuldades do diagnóstico diferencial, os aspectos histogenéticos, a eventual relaçäo entre o traumatismo e o aparecimento do SAPM e expöem as razöes da conduta adotada.