The clinical and pathologic features of 61 cases of congenital and infantile myofibromatosis are presented. The tumor affected almost exclusively infants and young children; 88% of cases occurred before the age of two years, and 60% were noted at birth or shortly thereafter. Solitary (45 cases) and multicentric (16 cases) forms of the tumor could be distinguished; the solitary form was more common in males (69%) and affected chiefly the soft tissues of the head-neck region and the trunk. The multicentric form, on the other hand, predominated in females (63%) and was found not only in soft tissues but also in bones and viscera. Fourteen of the 16 cases with multicentric lesions were present at birth, and four of them had evidence of visceral involvement. Among the 16 cases were two sets of siblings, both brother and sister. Grossly, nearly all of the tumors were well demarcated; they measured from 0.5 to 7 cm in greatest diameter. Microscopically, they formed well-circumscribed nodules consisting of short bundles of plump, spindle-shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. Necrosis or a hemangiopericytoma-like pattern was often found in the center of the tumor nodules. Intravascular growth was encountered in several instances, but this feature did not seem to affect the clinical behavior. Of the 43 patients with follow-up data (mean-follow up 5.1 years), 36 were alive and well. Of the solitary lesions, three recurred. Of the multicentric lesions, five of the 15 patients showed evidence of spontaneous regression of both soft tissue and bone lesions. Four died, three of extensive involvement of multiple viscera, especially the lung, and one of "crib-death."

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The clinical and pathological features of 138 cases of fibroma of tendon sheath are presented. The tumor predominated between the 3rd and 5th decades; the youngest patient was 5 months of age, the oldest 70 years (median 31 years). The tumor was more common in males (75%) than in females; it affected chiefly the tendons and tendon sheaths of the fingers (49%), hands (21%), and wrist (12%). The main presenting symptom was an insidiously growing mass causing mild tenderness or pain in about one-third of the patients. The preoperative duration of symptoms ranged from l week to 6 years with a median of 5 months. The tumor was generally well circumscribed, and often lobulated. It measured between 1 and 2 cm in greatest diameter (median 1.8 cm), and in all but one of the cases was attached to tendon and/or tendon sheath. Microscopically, most were composed of dense fibrocollagenous tissue bearing dilated or slit-like vascular channels some of which resembled tenosynovial spaces. Although cellular variants focally mimicking fibrous histiocytoma and nodular fasciitis could be distinguished, there was always a transition from cellular toward the typical fibrocollagenous areas. Of the 54 patients with follow-up information (median follow-up period of 4.5 years) 40 or 74% were well with no evidence of recurrence in the follow-up period. In 10 patients, the tumor had recurred once and in 3 it had recurred twice. All of the recurrences were in the fingers or the hands. The overall recurrence rate was 24% which is comparable to that of giant cell tumor of tendon sheath.One patient died of unrelated cause. Local excision appears to be the treatment of choice for these tumors, including the lesions that recur. ENIGN GIANT CELL TUMORS of tendon sheath B are relatively common and readily recognized by most pathologists. There is, however, a group of morphologically different and less well-known benign tumors that are well-circumscribed and composed of fibroblasts embedded in a dense "hyaline" fibrous stroma containing a rather prominent network of dilated but slit-like vascular channels. Geschickter and Copelands referred to these tumors as an entity but since their description in 1936 no additional cases have been added to the literature. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the view of the Department of the Army or the Department of Defense.Accepted for publication December 11, 1978.This study was aimed at establishing morphological criteria for the diagnosis of this entity and for determining its incidence, anatomical location and clinical behavior on the basis of long-term follow-up information. Preliminary data on this study have been presented elsewhere.2 MATERIALS AND METHODSClinical records and microscopic sections of 138 cases selected from the files of the Armed Forces Institute of Pathology (AFIP) were reviewed and analyzed. These had been accessioned over a period of 42 years (1935-1977) and coded under a number of di...