PubMed · 3517733
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R J White. 1986. Gorbachev's brain.. https://pubmed.ncbi.nlm.nih.gov/3517733/
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BACKGROUND: Indications for active treatment of hemangiomas are those lesions which, by virtue of their size and site, compromise vital structures. The treatment of choice is oral or intravenous corticosteroids, but interferon alpha may represent alternative therapy. CASE REPORT: A 15-day-old girl was admitted for congenital hypothyroidism. She had a large cervicofacial hemangioma extending to periorbital and laryngeal areas. The patient was given systemic prednisone (2 mg/kg/day) and L-thyroxin without success. An episode of acute respiratory distress a few weeks later required tracheostomy while prednisone dosage was increased to 4 mg/kg/day. This drug was not tolerated and the hemangioma was treated by particle embolization that was followed by a partial decrease in the volume of the hemangioma. A second episode of cardiorespiratory distress at 7 1/2 months of age required interferon alpha, 3 million units/m2/day, that was progressively effective. After 11 months of treatment, the hemangioma disappeared, without relapse 6 months later. CONCLUSION: Interferon alpha is an interesting alternative therapy of infantile hemangiomas when they are resistant to steroid treatment.
BACKGROUND: Intramuscular myxoma is a rare benign mesenchymal lesion. Only very rare cases of cutaneous localization of this tumor have been described, in particular related to the somatic soft tissues of the face. This unusual localization may clinically mimic nodular or cystic facial lesions having different origins. OBJECTIVE: The aim of our work was to well characterize the phenotype of the spindle cells characteristic of intramuscular mixoma. METHODS: Tissue samples were processed for morphological and ultrastructural studies. Moreover, immunohistochemical stainings were performed to characterize the expression of different nonmuscular and muscular cytoskeletal proteins. RESULTS: The tumor was composed of sparse spindle cells embedded in a prominent mucoid matrix. Besides the predominance of a fibroblast-like appearance, some neoplastic cells displayed immunohistochemical and ultrastructural features resembling either myofibroblasts or primitive mesenchymal cells, with a modulation of cell actin expression. CONCLUSION: The presence of multiple phenotypes of nonmuscular, mesenchymal pathway of differentiation can be considered a peculiar feature of intramuscular myxoma.
Our objective was to determine the appearance, cause, and outcome of fetal face masses diagnosed antenatally by ultrasonography. Over a 6 year period, 10 consecutive fetuses with facial masses were identified. Ultrasonographic findings, neonatal pathologic findings, and outcome data were correlated. Four (40%) of the 10 fetuses died, including one with a palatal teratoma associated with a Dandy-Walker malformation and three with intracranial teratomas--one of which was associated with hydrops fetalis. Among the survivors, one fetus had a dacryocystocele that was managed conservatively and one had drainage of a salivary gland cyst. The remaining four neonates had successful excision of their tumors in the neonatal period and survived; these infants had a nasal teratoma, a thyroid teratoma, a gingival granular cell tumor, and a scalp hemangioma. Four of the 10 pregnancies had associated polyhydramnios, three of which ended in stillbirth or neonatal death. In conclusion, 40% of the fetuses with antenatal diagnosis of fetal facial masses did not survive. If those with intracranial teratomas are removed from this group, one of seven (14%) fetuses with extracranial masses died. The intracranial teratomas were uniformly fatal. Polyhydramnios was associated with poor outcome.