Congenital Generalized Fibromatosis (Renal and Skeletal) with Complete Spontaneous Regression

Abstract

A case of congenital generalized fibromatosis with involvement of the kidney and skeleton has been observed over an 18 month period. A total of 31 tumors in bone and one renal tumor was noted. These tumors were first found when the patient was one month old. The tumor in the kidney was surgically removed, and 2 skeletal tumors were biopsied. Complete spontaneous regression of the tumors in the bones was noted 16 months later.

Case Details

Personal Characteristics

The patient was the fourth child of the family. The prenatal period was uneventful, and he was born through a normal delivery on december 10, 1960. The birth weight was 5 pounds, 4 1/2 ounces.

Clinical Characteristics

A total of 31 tumors in bone and one renal tumor was noted. These tumors were first found when the patient was one month old. The tumor in the kidney was surgically removed, and 2 skeletal tumors were biopsied.

Remission Characteristics

Complete spontaneous regression of the tumors in the bones was noted 16 months later.

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

The tumor in the kidney was surgically removed, and 2 skeletal tumors were biopsied.

Additional Notes

The patient was discharged from the hospital on April 17, 1962, and closely followed at the Pediatric Clinic. He was noted to be growing satisfactorily. In August, 1962, a radiologic study showed that the cranial defect where the parietal fibroma had been removed was still present, but the small adjacent lesion, which had been left intact, had disappeared. There was no tumor palpable over the scalp. The multiple lesions of the long bones and pelvis had completely vanished. Recalcification of the bone lesions was complete. The long bones showed normal cortices and trabeculae. A few faint streaks of dense calcification at the metaphyses of the femurs are the only indication of the previous sites of the bone lesions. The right diaphragm remains elevated as it has been prior to the pyloromyotomy and nephrectomy. Fluoroscopy with barium meal disclosed the stomach in normal position. The liver was not enlarged. A bone survey and physical examination of the other 3 children of the family revealed no indication of bone pathology or evidence of fibromatosis.