PubMed · 14397222
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R CLEMENT. 1955. Polyiphyseal dystrophy.. https://pubmed.ncbi.nlm.nih.gov/14397222/
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Hemangiomas are vascular tumors, and are distinct from vascular malformations. The term "hemangioma" is frequently misapplied by various medical specialists. This article briefly summarizes the main types of hemangiomas: infantile, rapidly involuting, and noninvoluting types. More complex conditions are also reviewed, such as hemangiomas of the liver and PHACES. The discussion then focuses on common vascular misnomers and proper descriptive terminology for hemangiomas: solitary (small, large, or diffuse) and multifocal. It is concluded that the term "segmental hemangioma" is imprecise, ill-advised, misguided, and should be abandoned.
A case of Maffucci's syndrome in an 18-month old boy is presented, in which the vascular malformation consists of lymphangioma. This is a rare variant of Maffucci's syndrome. So far only six other cases have been reported in which lymphangiomas featured. We believe that the case described here is only the second case to be reported for which a lymphangioma is the only vascular malformation in the syndrome. The literature of the other six cases was reviewed and various features identified enabling this particular disease to be classified as a distinct variant of Maffucci's syndrome.
Maffucci syndrome is a rare dysembryoplasia associating cartilage tumors similar to enchondromas in Ollier's disease and vessel tumors. We report the case of a 50-year-old woman with Maffucci syndrome involving the lower legs who developed a variety of vascular proliferations. This patient had capillary hemangiomas, cavernous angiomas, and lymphangiomas facing the cartilage tumefactions on the thigh. Regular clinical surveillance is required for such patients due to the risk of degeneration of the enchondromas and the angiomas, but also due to the possible occurrence of other malignant tumors besides the cartilage and vessel tumors.