Myofibroblastic Contraction In Spontaneous Regression Of Multiple Congenital Mesenchymal Hamartomas
Cancer 40(5): Nov 1977; 2343-2352
View Original Source →Abstract
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 uninvolved skin. The ultrastructural and immunofluorescent studies revealed the previously undescribed 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.
Case Details
Personal Characteristics
5-day-old white male infant
Clinical Characteristics
Multiple (24) subcutaneous nodules, nodules varied from 0.5 to 6 centimeters in greatest dimension, uniformly elevated, indurated, freely moveable, and apparently nontender, no discoloration of the overlying skin, erosion defects in the midshaft of the right humerus, distal right femur, tibia, fibula, and right fifth rib
Remission Characteristics
At age 8 months, there was complete spontaneous regression of all subcutaneous nodules not previously altered by excisional biopsy. At age 1 year, all of the nodules had disappeared with the single exception of a 1 centimeter lesion over the left greater trochanter which was excised. At age 15 months, there was no evidence of persistent or recurrent disease.
Treatment & Mechanisms
Proposed Remission Mechanisms
Myofibroblasts are fibrocontractile cells, which play a role in shrinkage and eventual disappearance of these subdermal hamartomas
Clinical Treatment
Excisional biopsy of a 1 centimeter nodule on the left shoulder was performed. A 1 centimeter lesion over the left greater trochanter was excised.
Additional Notes
The infant’s mother enjoyed excellent health during her pregnancy and related no history of infection, trauma, or drug ingestion. There was no family history of skin tumors.