Subcutaneous nodules from a newborn boy with "multiple fibromatosis" involving the head, neck, trunk, and all four extremities were studied by light microscopy, transmission electron microscopy, and immunofluorescent techniques. Light microscopy suggested a hamartomatous process with fibroblastic adipose, vasoformative and apparent smooth muscle components. The principal cell population combined ultrastructural characteristics of both fibroblasts and smooth muscle cells. Immunofluorescent studies revealed binding of human anti-smooth muscle antibody to the cytoplasm of the spindle cell population of the subdermal nodules but not to fibroblasts of the overlying un-involved skin. The ultrastructural and immunofluorescent studies revealed the previously underscribed fact that fibrous hamartoma of infancy is principally a proliferation of myofibroblasts. At age 8 months, there was complete spontaneous regression of all subcutaneous nodules not previously altered by excisional biopsy. The authors conclude that myofibroblasts are fibrocontractile cells, which play a role in shrinkage and eventual disappearance of these subdermal hamartomas.
Cystic lymphangiomas (cystic hygromas) are comparatively rare tumors. Seventy-five percent of these lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region, pelvis, or groin. Rarest are retroperitoneal cystic lymphangiomas. Beahrs et al 1 reported nine cases of such tumors diagnosed at the Mayo Clinic from 1911 to 1947. Rauch 2 found 22 cases of retroperitoneal lymphangiomas reported before 1959. Kafka and Novak 3 added 10 cases and reported one of their own. Recently, Singh et al 4 described another case, bringing the total number of reported cases of retroperitoneal cystic lymphangiomas to 34. We report two cases with unusual features: one patient had preoperative angiograms, the second patient had an acute condition within the abdomen.
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