JUVENILE APONEUROTIC FIBROMA (CALCIFYING FIBROMA) A Distinctive Tumor Arising in the Palms and Soles of Young Child?-en LOUISA E. KEASBEY, M.D. HE PAST DECADE has seen a goodly reorienta- T tion regarding the spindle-cell tumors of the soft parts. The tumors stemming from fat, muscle, and Schwann cells have been more clearly distinguished; the niyxomas and mixed mesodermal tumors-mesenchyinomas- have been given more distinctive outlines; and new entities, such as hemangiopericytoinas, have been proposed. But a feeling of baffle- ment, a vagueness rather than precise defini- tion, still attaches to the large group of tu- mors of fibroblastic origin. The fibroblast is a highly versatile cell, re- lated at one extreme to the protean reticulum cell and, at the other, to such stable citizens as osteo- and chondroblasts. Fibroblasts are the cells abundantly mobilized in the reparative and defense processes of the body, not only in granulation- and scar-tissue formation, but also in desmoplasias, or so-called productive fibroses, accompanying invasive neoplastic growths. Hence proliferating fibroblasts not only take an active part in the formation of the palpable “tumors” produced by invading cancers, but they also appear at times to proliferate in a confusingly intimate fashion as a part of other sarcomatous neoplasms, for example, in con- junction with lipoblasts and primitive muscle cells in lipo- and myosarcomas. A perusal of the recent literature will show that this vagueness of boundary lines that per- tains to the great group of fibroblastic tumors is being attacked in a highly practical fashion by a series of careful studies of definite limited neoplasms of fibroblastic genesis, such as the desmoids and dermatofibrosarcomas protu- berans. When various species have been care- fully and clearly described, it is always much easier to outline a lucid concept of the genus. The purpose of this paper, with this back- From the Emery Tumor Group, the Department of Pathology, University of Southern California, and the Los Angeles County Hospital, Los Angeles, California. Case 3 (Table 1) from the Emery Tumor Group. Case 4 (Table 1) was kindly contributed by Dr. Edward M. Butt from the Pathology Service at St. Luke’s Hospi- tal, Pasadena, California. Received for publication, August 18, 1952. ground, is to describe, on the basis of a limited number oi cases, what appears to be a microscopically distinctive type of fibroma occurring in the palms and soles of small chil- dren. It seems of distinct clinical importance to emphasize this entity, since it appears to be a benign tumor, of self-limited growth impetus, that nevertheless imparts a histological impres- sion of cellularity and invasiveness and shows a disturbing propensity to recur postoperatively after too limited an excision. Amputation was considered in three of the four cases studied, although follow-up studies have shown appar- ent tumor arrest in all four cases after con- servative surgery. Clinically, this tumor, for which the name “calcifying juvenile aponeurotic fibroma” is proposed, occurred in three girls and one boy, who were first seen by us at the ages of 2y& 6, 7, and 9 years. In all these cases, however, the tumor was recurrent, and, from the history, appears to have arisen between the ages of 2 and 9 years. Two arose in the thenar space, one in the hypothenar area, and one apparently in the mid-line on the sole of the foot, in the fat pad over the calcaneous. It presented as a painless, poorly circumscribed, rather fixed, firm mass, never adherent to the skin and never associated with any evidence of con- tractures. Roentgenograms showed a relatively radio-opaque soft-tissue mass, with no associ- ted lesion of the bone, and on close inspection the fine stippling of the focal calcification to which these tumors are prone could be vis- ualized. T h e clinical diagnoses offered when these tumors were first seen were lipoma, ten- don-sheath tumor, angioma, synovioma and abscess (in two cases the tumor had at first been lanced, although no local redness, warmth, or tenderness was evident). These diagnoses are doubtless colored by a consideration of what tumors occur with any frequency in the palms and soles, but they likewise impart an impres- sion as to the clinical aspects with which these tumors presented. Essentially, the tumors have appeared as a rather nondescript symptomless mass, firm but not hard, poorly delimited rather 338
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