Search PubMed⌕ Search

Biomedical subjects

F M Enzinger

Publications and source records attributed to F M Enzinger.

At least 19 recordsLinked to original sources
PubMed1995

Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma.

We report 25 cases of a peculiar sclerosing epithelioid variant of fibrosarcoma (SEF) simulating an infiltrating carcinoma. The tumors occurred primarily in the deep musculature and were frequently associated with the adjacent fascia or periosteum. The patients' ages were 14 to 87 years (median, 45). Fourteen were male and 11 female. The tumors were located in the lower extremities and limb girdles (12 cases), trunk (9), upper limb girdles (2), and neck (2). They measured 2 to 14.5 cm in greatest dimension (median size, 7 cm) and were gray to white and firm. Histologically, the lesions were characterized by a proliferation of rather uniform, small, slightly angulated, round to ovoid epithelioid cells with sparse, often clear cytoplasm arranged in distinct nests and cords. In all cases there was prominent hyaline sclerosis, sometimes reminiscent of osteoid or cartilage and foci of conventional fibrosarcoma. Occasional myxoid zones with cyst formation and foci of hyaline cartilage, calcification, and metaplastic bone were also seen. Mitotic figures were generally scarce. Vimentin was detected in 13 of 14 cases, epithelial membrane antigen in seven, S100 protein in four, and neuron-specific enolase in two. Cytokeratins were detected with AE1/AE3 and CAM 5.2 in two cases. Leukocyte common antigen, CD68 antigen, HMB45, desmin, and alpha-smooth muscle actin were negative in all cases. In 13 of 14 cases, 75% or more of the cells stained for proliferating cell nuclear antigen (PCNA). Ki67 immunostaining with MIB 1 showed low proliferative activity in all cases, averaging 5% of tumor cells or less. In all cases, p53 was detected by immunohistochemical methods; bcl-2, an antiapoptosis marker, was detected in more than 90% of the cells in 11 of 12 cases. Ultrastructurally, both the epithelioid and spindled tumor cells had features of fibroblasts. Follow-up in 16 cases ranging from 13 months to 17 years 3 months (median, 11 years 4 months) revealed persistent disease or local recurrences in 53% of patients and metastases in 43%. The metastases were to the lungs (4 cases), skeleton (3), chest wall/pleura (3), pericardium (1), and brain (1). Four patients died of disease, four were alive with disease, two were known to be alive but disease status unknown, and six had no evidence of further disease at last follow-up. The data suggest that SEF is a relatively low-grade fibrosarcoma; yet it is fully malignant despite the presence of histologically benign-appearing foci. The proliferation markers PCNA and Ki67 did not correlate with prognosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1994

Plexiform malignant peripheral nerve sheath tumor of infancy and childhood.

We present nine cases of an atypical cellular peripheral nerve sheath tumor, designated plexiform malignant peripheral nerve sheath tumor (MPNST) of infancy and childhood, occurring in five boys and four girls aged 50 days to 13 years (median, 1.5 years). The tumors were located in the lower extremities (five cases), upper extremities (three cases), and the orbital region (one case), and they ranged from 1.5 to 8 cm in size (median, 3 cm). Two lesions were congenital, and another was associated with a history of von Recklinghausen's disease. Follow-up, available in six cases, ranged from 6 months to 15 years (median, 51 months); four patients had local recurrences within 8 to 31 months after excision of the initial lesion, and one with an orbital tumor died of locally invasive disease within 6 months. Histologically, the initial lesions were characterized by a predominantly superficial location within the dermis and subcutis, with occasional extension into deeper soft tissues, had infiltrative or sharply demarcated margins, and resembled entangled or intertwined hypercellular nerve trunks, resulting in a plexiform appearance at low magnification. The nuclei were oval to serpentine with a vesicular chromatin pattern and small basophilic nucleoli; mitoses were seen in all cases and averaged from 1 to 18/10 high-power fields (hpf) (median, 4/10 hpf). Neither necrosis nor vascular invasion was seen. Features diagnostic of plexiform or cellular schwannoma, such as nuclear pleomorphism, Antoni B areas, thick-walled hyalinized blood vessels, and secondary degenerative features were lacking. Other than a prominent plexiform architecture, the lesions were indistinguishable from MPNST occurring in other sites in infants and children. Although none metastasized, the histologic similarity of plexiform MPNST to other childhood MPNST, its relatively high propensity for local recurrences, and its potential to behave in a locally aggressive manner indicate that it is best regarded as low-grade malignant. Overall, the behavior of plexiform MPNST is better than other MPNST in children, probably because of its relatively small size, peripheral and superficial location in most instances, absence of necrosis or vascular invasion, occurrence in young patients, and infrequent association with von Recklinghausen's disease rather than a function of its prominent plexiform architecture. Distinction of plexiform MPNST from cellular and plexiform schwannoma, plexiform neurofibroma, and hamartomatous lesions of childhood is important. Complete excision should be ensured to prevent local recurrences and potential metastases.

Adolescent↗
PubMed1993

Chondroid lipoma. A unique tumor simulating liposarcoma and myxoid chondrosarcoma.

We report 20 cases of a peculiar fatty tumor that occurred in 16 female and four male patients who were 14-70 years old (median, 36 years). Most lesions were situated in the subcutis, superficial muscular fascia, or skeletal muscle of the limbs and limb girdles (15), trunk (3), and the head and neck (2). They were 1.5-11 cm in size (median, 4 cm) and usually described as yellow (13 of 15) and encapsulated (13 of 15). Microscopically they were well circumscribed and consisted of nests, strands, and sheets of eosinophilic and vacuolated cells, which contained glycogen and fat droplets, resembling brown fat cells, lipoblasts and chondroblasts. In all cases there was a variable background of mature adipose tissue associated with a prominent, partially fibrinous to hyalinized myxoid matrix that contained acid mucopolysaccharides usually resistant to hyaluronidase digestion. Several cases had foci of serous atrophy, perivascular fibrosis, and small thrombi; two were focally calcified. The lesions stained for S100 protein (11 of 12), vimentin (10 of 11), and CD68 antigen with KP1 (9 of 11); focal staining for keratin was also seen (4 of 11), but none stained for epithelial membrane antigen or actin or with HMB45. Follow-up in 12 cases (median, 9.5 years) revealed no local recurrences or metastases. Despite its deep location and atypical cellular features, the lesion's nonaggressive behavior suggests it is benign and neither a myxoid liposarcoma nor a myxoid chondrosarcoma, with which it is most frequently confused. The presence of glycogen in vacuolated fat cells is similar to brown fat, and the presence of sulfated stromal mucins supports focal chondroid differentiation. Although the pathogenesis remains uncertain, a lipoma with hibernomatous features, myxoid change, chondroid metaplasia, and secondary degenerative features is favored over a lipogranulomatous process.

Adolescent↗
PubMed1992

Juxta-articular myxoma: a clinical and pathologic study of 65 cases.

We describe 65 cases of juxta-articular myxoma (JAM) that occurred in the vicinity of large joints, possessed histologic features of a myxoma, and were frequently associated with cystic changes that resembled a ganglion cyst. The vast majority of cases (57, 88%) occurred in the region of the knee; a minority involved the shoulder (three cases), elbow (three), ankle (one), and hip (one) regions. Patients' ages ranged from 16 to 83 years (median, 43 years; mean, 44 years) and nearly three fourths of the patients (72%) were male. Thirty-seven lesions presented as a swelling or mass, 21 were associated with pain or tenderness, and sizes ranged from 0.6 to 12 cm (median, 3.5 cm; mean, 3.8 cm). Duration of symptoms was highly variable, spanning from 1 week to 18 years. Fourteen JAMs were intimately associated with the meniscus and five of these had a concomitant tear; in five other cases JAM was an incidental finding at the time of total knee or hip arthroplasty for severe osteoarthritis. Of 29 cases with follow-up, 10 (34%) recurred: five recurred once, two recurred twice, two recurred three times, and one recurred four times. While the majority of JAMs were correctly diagnosed as benign, a sarcoma was seriously considered or diagnosed in 15 (23%) cases.

Adolescent↗
PubMed1992

Proliferative fasciitis and myositis of childhood.

Eleven cases of proliferative fasciitis and myositis in children, ages 2.5 months to 13 years, are presented. Eight lesions averaging 2.3 cm in size occurred in the extremities, two in the head and neck region and one on the chest wall. Like proliferative fasciitis and myositis in adults, these lesions consisted of admixtures of large polygonal to spindled, ganglion-cell-like fibroblasts with vesicular nuclei and prominent inclusion-like nucleoli. Seven of 11 lesions were initially diagnosed as sarcomas, most commonly rhabdomyosarcoma. Four patients were treated by wide excision (three with regional lymphadenectomy), three received chemotherapy, and one was given radiation therapy. There were some histologic differences from adult-type proliferative fasciitis and myositis. The childhood lesions were generally well circumscribed, lobulated, extremely cellular with less collagen production, and often associated with acute inflammation and microscopic foci of necrosis. Immunohistochemical comparison with adult proliferative fasciitis and myositis showed similar immuneprofiles; the ganglion-like cells stained for vimentin and actin and focally with KP1, suggesting myofibroblastic and histiocytic features. None of the lesions stained for keratin, desmin, or S-100 protein. Ultrastructural examination of two cases revealed cells with a constellation of fibroblastic, myofibroblastic, and histiocytic features. Follow-up of seven patients, averaging 58 months from diagnosis, confirmed that all are alive and well. Recognition of this cellular variant of proliferative fasciitis and myositis is important to prevent misdiagnosis as a sarcoma and unnecessary, excessive therapy.

Actins↗
PubMed1992

Malignant peripheral nerve sheath tumors (malignant schwannomas) in children.

We studied the clinical and pathologic features of 78 malignant peripheral nerve sheath tumors in children less than or equal to 15 years of age. There were 42 boys and 36 girls, with a median age of 10 years. The majority of the tumors (42, or 54%) were central or axial in location; the rest were peripheral. Sixteen patients (21%) had a history of von Recklinghausen's disease. Fourteen (18%) had a malignant peripheral nerve sheath tumor arising in a nerve trunk or a neurofibroma and were unassociated with von Recklinghausen's disease. Patients typically presented with a painful mass of variable duration. Tumors ranged from 2 to 33 cm (median, 7.5 cm) and demonstrated a wide histologic spectrum that included spindled, epithelioid, and primitive neuroepithelial-like cells as well as heterologous elements (11). Immunohistochemical staining revealed S-100 protein in 28 of 50 cases (56%) as well as vimentin (13 of 21 cases, or 62%), Leu 7 (22 of 49 cases, or 45%), actin (eight of 20 cases, or 40%), and keratin (seven of 27 cases, or 26%). Survival status was known for 57 patients (73%). Kaplan-Meier estimates revealed a median survival of 45 months. Half of the patients had local recurrences at 12 months, and half had metastases at 24 months, most commonly to lungs, followed by lymph nodes, liver, bone, soft tissue, and brain. Age greater than or equal to 7 years, male sex, presence of von Recklinghausen's disease, central location, larger tumor size, and tumors with greater than or equal to 25% necrosis were found to be potentially significant adverse prognostic indicators by univariate analysis. Multivariate analysis revealed that larger tumor size, age greater than or equal to 7 years, tumor necrosis greater than or equal to 25%, and von Recklinghausen's disease to be independent adverse prognostic factors.

Adolescent↗
PubMed1992

Atypical decubital fibroplasia. A distinctive fibroblastic pseudotumor occurring in debilitated patients.

We report 28 cases of atypical decubital fibroplasia, a distinctive pseudosarcomatous fibroblastic proliferation occurring primarily but not exclusively in physically debilitated or immobilized patients. The subjects included 16 women and 12 men ranging in age from 15 to 95 years. Peak incidence was in the 8th and 9th decades of life. Anatomic locations included the soft tissues overlying the shoulder (eight cases), posterior chest wall (five cases), sacrum (five cases), greater trochanter (four cases), buttock (two cases), thigh (two cases), and arm (two cases). Symptoms were due to a painless mass of 3 weeks' to 6 months' duration. Most lesions were ill-defined, focally myxoid masses that ranged from 1 to 8 cm. Histologically, they were situated in the deep subcutis and secondarily involved adjacent skeletal muscle (11 cases) and tendon (three cases). Extensive epidermal ulceration was typically absent. Microscopically, the lesions had a lobular configuration. They were characterized by zones of fibrinoid necrosis and a prominent myxoid stroma rimmed by ingrowing, ectatic, thin-walled vascular channels. All cases contained atypical, enlarged, degenerated fibroblasts with abundant basophilic cytoplasm, large hyperchromatic, smudged nuclei, and prominent nucleoli; these features resulted in a superficial resemblance to proliferative fasciitis. The enlarged, atypical fibroblasts stained diffusely and strongly for vimentin (15 of 15 cases) and focally for muscle-specific actin (10 of 15 cases), keratin (one of 15 cases), CD68 (10 of 15 cases), and CD34 (five of nine cases) antigens; none of the cases stained for desmin. A malignant diagnosis was considered in 43% of cases. Follow-up in 21 patients ranged from 2 to 78 months (median, 12 months). Two lesions recurred once, one recurred twice, and none metastasized; no deaths were attributable to the lesions. The clinical, histologic, and immunohistochemical features of atypical decubital fibroplasia indicate it is a unique type of pressure sore displaying degenerative and regenerative features distinct from decubitus ulcer. Its recognition by pathologists and clinicians in elderly and debilitated patients is important to avoid misdiagnosis as a sarcoma and to prevent or minimize the occurrence of decubital fibroplasia in progressively aging patient populations.

Adolescent↗
PubMed1991

Myolipoma of soft tissue.

Nine cases of a previously undescribed benign soft tissue tumor are reported. They were composed of variable amounts of benign smooth muscle and mature adipose tissue. Patient ages ranged from 28 to 73 years. One was located in the subcutaneous adipose tissue, one in the rectus sheath of the anterior abdominal wall, two within the abdominal cavity and attached to the abdominal wall, two in the inguinal region, and three in the retroperitoneum. Sizes varied between 3.5 and 26 cm and averaged 16 cm in greatest dimension. Two of the retroperitoneal tumors were incidental findings during other operative procedures. The remaining seven cases were clinically palpable masses. Eight of the nine lesions were originally diagnosed as benign, and another (retroperitoneal) was diagnosed as well-differentiated liposarcoma. Five of the tumors were at least partially encapsulated. In three of the cases, a nonlipomatous component was grossly recognized. Although the benign nature of this lesion is usually recognized in superficial locations, deeply situated tumors are more likely to be confused with a well-differentiated liposarcoma.

Adipose Tissue↗
PubMed1991

Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor.

We report 38 cases of inflammatory fibrosarcoma occurring in 23 females and 15 males, 2 months to 74 years of age (median, 8.5 years; mean, 15 years) with symptoms of abdominal pain (17 cases), anemia (21 cases), fever (14 cases), mass (16 cases), and gastrointestinal obstruction (7 cases). Primary tumor sites included mesentery and retroperitoneum (31 cases), omentum (two cases), mediastinum (two cases), liver (one case), diaphragm (one case), and abdominal wall (one case). Sizes ranged from 2.4 cm to 20 cm (mean, 9.6 cm). Follow-up data in 27 cases revealed local recurrences in 10 patients, with multiple local recurrences in three and histologically proven distant metastases to lung (two cases) and brain (one case). Five patients died from their disease (median, 20 months). All tumors, including metastases, consisted of fibroblasts, myofibroblasts, and plasma cells, with variable degrees of fibrosis and calcification. Immunostains indicate myofibroblastic differentiation; 18 of 20 (90%) stained for actin, 15 of 18 (83%) for vimentin, and 10 of 13 (77%) for keratin (primarily in a submesothelial location). Ultrastructural studies also disclosed myofibroblastic features. The locally aggressive, recurrent nature of these neoplasms, as well as the occurrence of metastases and tumor deaths, indicate that they are potentially malignant neoplasms that we believe are better classified as inflammatory fibrosarcomas, not as cellular inflammatory pseudotumors.

Adolescent↗
PubMed1991

Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature.

Angiosarcoma arising in the central or peripheral nervous system has rarely been reported. Eight patients with primary angiosarcoma of the central nervous system are described here; these included five males and three females ranging in age from 2 weeks to 72 years (mean 38 years). Of the eight neoplasms, six were located in the cerebral hemispheres and one was in the meninges; the site was unknown in the other. All patients underwent surgical resection. Five of the eight patients died, four within 4 months after surgery and one after 30 months. Two of the remaining three patients were 17 and 27 years old at the time of diagnosis and were alive at follow-up review 39 and 102 months after surgery, respectively. One patient was lost to follow-up monitoring. Microscopically, all eight tumors demonstrated a well-differentiated pattern with irregular vascular channels and intraluminal papillae; in addition, four showed poorly differentiated solid areas. Immunohistochemical staining of neoplastic cells to factor VIII-related antigen and Ulex europaeus agglutinin I was performed in five tumors and was focally positive in four. No correlation could be shown between the histological features and the growth and biological behavior of the tumors.

Adolescent↗
PubMed1989

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans.

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).

Adolescent↗
PubMed1989

Pathologic analysis of advanced adult soft tissue sarcomas, bone sarcomas, and mesotheliomas. The Eastern Cooperative Oncology Group (ECOG) experience.

A total of 488 tumors entered in the Eastern Cooperative Oncology Group (ECOG) Study EST 3377 were evaluated histologically by a panel of pathologists from member institutions for quality control purposes. The overall agreement rate between the eligible submitting diagnosis and the pathology review panel's diagnosis was 74% (312/424). In 10% (44/424), the case was excluded because it was deemed to be nonsarcoma. In the other 16%, the disagreement concerned the type of sarcoma. The histologic type with the lowest agreement rate was rhabdomyosarcoma (17%), followed by sarcoma not otherwise specified (NOS) (27%), angiosarcoma (33%), and fibrosarcoma (48%). These figures reflect the significant degree of difficulty in the diagnosis of these tumor types. The treatment response rate of soft tissue sarcomas in the randomized study of Adriamycin (Adria Laboratories, Columbus, OH) regimens was slightly higher for those with lower grade sarcomas, i.e., 25% (four of 16) response rate for Grade 1 lesions; 22% (17/77) for Grade 2, and 21% (35/170) for Grade 3. When adjusted for type of sarcoma, there was no noticeable difference between Grade 1-2 versus 3 in response rate. A statistically significant difference in the percentage of complete responders was noted between Group A tumors (synovial sarcoma, hemangiopericytoma, sarcoma NOS, and Ewing's; 12.2%) versus Group B tumors (all other types--mostly spindle cell sarcomas; 3.5%) (P = 0.02).

Adolescent↗
PubMed1989

Ossifying fibromyxoid tumor of soft parts. A clinicopathological analysis of 59 cases.

We describe 59 cases of a microscopically unique neoplasm that has not been previously reported. The tumor almost exclusively affected adults (range 14-79 years) and had a male predominance (38 men and 21 women). It presented in most cases as a small, painless, well-circumscribed mass (median, 4 cm) in subcutis or muscle. It occurred chiefly in the upper and lower extremities (40 cases) and less frequently in the trunk (11 cases) and the head and neck region (eight cases). Microscopically, the tumor was partly lobulated and composed of small, round cells that had vesicular nuclei and indistinct cytoplasm. Typically, the cells were arranged in a cord- or nestlike pattern within a myxoid matrix that frequently showed transitions toward hyaline fibrosis and focal osteoid formation. In about two-thirds of the cases, the cells contained immunoreactive S-100 protein. An additional typical feature, seen in 48 (81%) of the 59 cases, was the presence of an incomplete shell of mature bone in the capsular region of the tumor. Follow-up information, available in 41 cases, revealed that 11 patients (27%) experienced one or more recurrences. One patient with three recurrences developed a second tumor in the opposite thigh, presumably a metastasis. None of the patients died of the tumor, but three died of causes unrelated to the disease. Although the histogenesis is uncertain, cartilaginous or neural origin seem to be most likely. Until this issue is resolved, we prefer the descriptive and less committal designation of "ossifying fibromyxoid tumor of soft parts."

Adolescent↗
PubMed1989

Malignant lymphoma. A study of 75 cases presenting in soft tissue.

We report 75 cases of malignant lymphoma presenting in soft tissue taken from the files of the Armed Forces Institute of Pathology. All histologic subtypes with the exception of lymphoblastic lymphoma were represented. Our findings indicate that virtually any soft tissue site may be involved; there is no sex predilection; and size is not helpful in predicting survival. Among the 55 patients for which race was known, there were no black patients. Thirty-three patients with extensive evaluations at the time of diagnosis had no evidence of disseminated disease, but eight of these exhibited widespread disease within 3 months of diagnosis, and seven of the eight died of disease (median survival, 4 months). The remaining 25 patients had much better outcomes; 18 of 19 with intermediate and high-grade lymphomas were alive and well at a median of 74 months after diagnosis. Some tumors exhibited a propensity for involvement of remote soft tissue sites.

Humans↗
PubMed1988

Postradiation soft tissue sarcomas. An analysis of 53 cases.

The clinicopathologic features of 53 cases of postradiation soft tissue sarcoma (PRS) were correlated with the physical characteristics of the administered radiation. All but three patients received radiation for malignant processes. Of the secondary sarcomas, malignant fibrous histiocytoma (MFH) accounted for 36 cases (68%), followed by seven extraskeletal osteosarcomas (13%), six fibrosarcomas (11%), two malignant Schwannomas (4%), one extraskeletal chondrosarcoma, and one angiosarcoma. The sex incidence, age of the patient at time of diagnosis, and location of the PRS correlated only with the clinical characteristics of the initial treated condition. The latency period (mean 10 years) showed an indefinite relationship to patient survival but no definite relationship to the patient's age at the time of the initial radiation. There was no difference between patients treated with megavoltage radiation (39 patients) and with orthovoltage radiation (seven patients) in the type of sarcoma, location, or survival, although the orthovoltage group received a lower mean radiation dose (3880 rads) than the megavoltage group (4446 rads). Megavoltage radiation, however, produced deeper tissue radiation changes and was associated with a shorter latency period. Most PRS were poorly differentiated, produced abundant collagen, and had a dismal prognosis.

Adult↗
PubMed1988

Malignant fibrous histiocytoma associated with hyperlipoproteinemia.

A unique case of malignant fibrous histiocytoma arising within the thigh of a patient with Type II A (hypercholesterolemic) hyperlipoproteinemia is described. The tumor, which appeared grossly as a benign tuberous xanthoma, demonstrated pleomorphic spindled and multinucleated cells focally in the distinct storiform pattern of a malignant fibrous histiocytoma and areas composed of bland xanthoma cells associated with cholesterol deposits. Foci of xanthoma cells with mildly atypical nuclei admixed with highly pleomorphic cells were also noted. These unusual histologic features--as well as the uniform staining of all the tumor cells with anti-alpha-1-antichymotrypsin and the absence of anti-alpha-1-antitrypsin and of anti-lysozyme staining--support the conclusion that all elements of this malignant tumor were derived from a common mesenchymal precursor. The exaggerated histiocytic capability of the tumor cells in the form of xanthomatous change was probably in response to the hyperlipoproteinemic microenvironment.

Aged↗
PubMed1988

Plexiform fibrohistiocytic tumor presenting in children and young adults. An analysis of 65 cases.

We report 65 cases of a hitherto undescribed neoplasm that occurs chiefly in children and young adults, and has morphologic features reminiscent of both a fibrous histiocytoma and fibromatosis. The median age of the 65 patients was 14.5 years; two-thirds (67.7%) of the patients were younger than 20 years. The lesion was more common in female patients (46 cases) than in male patients (19 cases). It usually presented as a slow-growing, poorly demarcated dermal or subcutaneous mass that rarely exceeded 3 cm in greatest diameter. Its most common location was the upper extremity (63.1%), especially the regions of shoulder and forearm. Under the microscope, the lesions were characterized by a multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells. Differential diagnosis chiefly includes cutaneous fibrous histiocytoma, plexiform neurofibroma, fibromatosis, and benign and malignant giant cell tumor. Twenty of the 32 cases (62.5%) with follow-up information were alive and well after local excision, but the tumor recurred in 12 cases (37.5%). In two patients with recurrence, the disease metastasized to regional lymph nodes 9 and 36 months after the initial excision, respectively. Metastasis to the lung or other organs was not observed. We were unable to demonstrate a close correlation between biologic behavior and any specific clinical or morphologic parameter.

Adolescent↗