Lumbar extradural infiltrating angiolipoma: a case report and review of 17 previously reported cases with infiltrating spinal angiolipomas.

BACKGROUND CONTEXT: Spinal extradural angiolipomas are rare benign tumors, and most of them are noninfiltrating tumors located in the extradural space. However, there are 17 cases with extradural infiltrating spinal angiolipomas extending into the vertebral bodies or posterior vertebral arches in literature. These 17 tumors are mostly located at the thoracic region, and anterior or in the anterolateral extradural space, and they generally infiltrate only one vertebra. Only two of them are located at the lumbar region, four are mainly located in the posterior extradural space, and three infiltrate more than one vertebra. PURPOSE: To present an exceptional case with infiltrating extradural spinal angiolipoma involving two lumbar segments and mainly located in the posterior extradural space. STUDY DESIGN: A case report. METHODS: A 41-year-old woman with infiltrating spinal angiolipoma was treated by incomplete surgical removal of the tumor. RESULTS: There were no complaints nor recurrence after 18 months follow-up. CONCLUSIONS: Outcome after surgery for spinal angiolipomas is very good overall even in the cases with infiltrating tumors. Although complete removal is certainly preferred, outcomes remained favorable despite incomplete resections.

A solitary calvarial lytic lesion with typical histopathological findings of juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare systemic disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and osteolytic lesions on long bones and the skull. It has recently been reported that the disease is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG-2). To date, fewer than 60 cases have been published in the literature. Partial disease expression is common, but no cases featuring a solitary calvarial lesion have been reported. The authors discuss this 4-year-old boy with a solitary calvarial osteolytic lesion whose histopathological examination exhibited findings characteristic of JHF. Mutational analysis, however, revealed that there were no mutations in the CMG-2 gene. Two years after surgery, he was free of any complaints as well as gingival hyperplasia, joint contractures, and new skull or skin lesions. This patient's condition may represent clinical or genetic heterogeneity associated with JHF. Whether solitary lesions mimicking JHF can arise from somatic mutation of the CMG-2 gene remains to be proven.