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Biomedical subjects

F M Enzinger

Publications and source records attributed to F M Enzinger.

At least 55 records · Page 3Linked to original sources
PubMed1983

Liposarcoma occurring in children. An analysis of 17 cases and review of the literature.

Liposarcoma, one of the most common malignant mesenchymal neoplasms affecting adults, has rarely been documented to arise in children. Therefore, in this retrospective study, the clinicopathologic features of 17 liposarcomas retrieved from the files of the Armed Forces Institute of Pathology (AFIP) which occurred in patients between the ages of eight months and 15 years were analyzed. In contrast to the slight male prevalence verified in most studies of adult liposarcoma, this group of 17 cases disclosed a distinct female majority (65%). The tumor had a predilection for the lower extremity (41%), particularly the thigh; the remaining examples, which included cases from the neck, back, axilla, buttock, and forearm, reflected a seemingly random anatomic distribution. The retroperitoneum, a very common primary location for adult liposarcoma, accounted for only one example in the group. The histologic variants of liposarcoma coincided with the types delineated in most generally accepted classifications employed for liposarcoma in adults, but their distribution varied considerably from that found in most extensive series of adult liposarcomas. Specifically, there were 13 myxoid liposarcomas, two well-differentiated liposarcomas, and one each of the round cell and mixed variants. Follow-up information, obtained for 15 cases, revealed local recurrence in three patients, one of whom eventually died as a result of direct tumor extension into the pleural cavity.

Adolescent↗
PubMed1983

Malignant melanoma of soft parts. A reassessment of clear cell sarcoma.

A retrospective study of 141 cases of clear cell sarcoma of tendons and aponeuroses revealed that the tumor occurred predominantly in young patients between 15 and 35 years of age, was slightly more common in female than in male patients, and originated chiefly from tendons, aponeuroses, and fascial structures of the extremities with a predilection for the feet and knees. The tumor presented as an insidiously growing mass causing pain or tenderness in slightly more than half of the patients. The preoperative duration of symptoms varied greatly, and in 29 cases the time interval between discovery of the tumor and operation exceeded 5 years. Microscopically, the tumors varied little in appearance and were composed of short fascicles of fusiform cells with a clear to granular eosinophilic cytoplasm, vesicular nuclei with prominent nucleoli, and occasional multinucleated giant cells. Melanin was demonstrated in 72% of the 92 tumors in which the Fontana or Warthin-Starry preparation for melanin was performed. S-100 protein, a neuroectodermal marker, was positive in 13 of 19 cases. Follow-up information was available in 115 patients. Thirty-four (29.6%) were alive and well with no evidence of recurrence. Twenty-four (20.9%) patients were alive with one or two recurrences, three (2.6%) were alive following pulmonary lobectomy for metastatic sarcoma, and one was alive with metastasis in the thoracic spine. Fifty-three of the 115 patients with follow-up information had died, 50 from metastatic tumor and three from miscellaneous causes. The prevailing sites of metastasis were the lung and the regional lymph nodes. The exact histogenesis remains obscure, but the presence of intracellular melanin in two-thirds of the cases supports origin from migrated neural crest cells with the capacity for producing melanin. For this reason the term, malignant melanoma of soft parts, seems preferable over the purely descriptive term of clear cell sarcoma.

Adolescent↗
PubMed1983

Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors.

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.

Humans↗
PubMed1982

Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma.

Epithelioid hemangioendothelioma is a unique tumor of adult life which is characterized by an "epithelioid" or "histiocytoid" endothelial cell. Forty-one cases of this rare tumor have been recognized at the Armed Forces Institute of Pathology. They may occur in either superficial or deep soft tissue, and in 26 cases appeared to arise from a vessel, usually a medium-sized or large vein. They are composed of rounded or slightly spindled eosinophilic endothelial cells with rounded nuclei and prominent cytoplasmic vacuolization. The latter feature probably represents primitive lumen formation by a single cell. The cells grown in small nests or cords and only focally line well-formed vascular channels. The pattern of solid growth and the epithelioid appearance of the endothelium frequently leads to the mistaken diagnosis of metastatic carcinoma. The tumor can be distinguished from a carcinoma by the lack of pleomorphism and mitotic activity in most instances and by the presence of focal vascular channels. Ultrastructural study in four cases confirmed the endothelial nature of the tumor in demonstrating cells surrounded by basal lamina, dotted with surface pinocytotic vesicles, and occasionally containing Weibel-Palade bodies. Follow-up information in 31 cases indicated that 20 patients were alive and well following therapy; three developed local recurrences and six metastases. It is suggested the term epithelioid hemangioendothelioma be used to designate these biologically "borderline" neoplasms. The significance of the epithelioid endothelial cell is not entirely clear. Since it may be observed in both benign and malignant vascular lesions, its presence alone does not define a clinicopathologic entity.

Adolescent↗
PubMed1982

Orofacial synovial sarcoma: a clinicopathologic study of 11 new cases and review of the literature.

Synovial sarcoma arises most commonly in the lower extremity, particularly in the region of the lower thigh and knee. Yet the occurrence of thus mesenchymal neoplasm in the head and neck area has been convincingly documented, albeit confined almost exclusively to cervical and parapharyngeal sites. Therefore, in view of its rarity in the head and neck, we analyzed a group of 11 synovial sarcomas arising in the orofacial region. The series comprised nine men (82%) and two women (18%). In common with synovial sarcoma at more conventional sites, this is a disease of young adults: ages ranged from 16-49 years (median, 34 years). Topographically, two subsets were delineated, a more common facial group with eight cases (four cheek, two parotid region, one infraorbital, one submental), all arising as gradually enlarging, usually nontender, solitary tumors; and three intraoral ones (two tonsillar, one lingual), two of which were polypoid and one was an exophytic tonsillar mass which presented with hemoptysis and stridor. Follow-up data, obtained for nine patients (range, 1.3-15.0 years), disclosed three (33%) tumor-related deaths, all belonging to the facial group. Treatment, difficult to significantly correlate with survival in this small series, varied from surgical excision alone to a multimodality approach including both irradiation and chemotherapy. Histologically, all the neoplasms revealed characteristic biphasic features, predominantly fibrosarcomatous in one, but, more typically, showing epithelial clefts and/or pseudoacinar spaces in the others. Differential diagnosis, depending on the proportion of the biphasic components, ranged from spindle cell mesenchymal neoplasms to various adenocarcinomas, including those arising in mixed tumors of salivary gland.

Adolescent↗
PubMed1982

Calcifying synovial sarcoma.

Analysis of the clinicopathological features and the available follow-up data in 32 patients with extensively calcified synovial sarcoma revealed an age incidence and anatomic distribution similar to that of ordinary synovial sarcomas without or with minimal calcification, but indicated a more favorable survival. The median age of 32 patient in this series was 26 years; the predominant location of the tumor was the soft tissues of the lower extremities; its average size was 4.6 cm (range, 1.5-20 cm). All of the tumors showed a focal biphasic cellular pattern and exhibited roentgenographic or microscopic evidence of extensive calcification and/or osseous metaplasia. Of the 26 patients with follow-up information (average 8.9 years) 17 were alive and well and six had died of their diseases, two after 7 and 19 years. Three additional patients were living with no signs of disease 1 1/2, 2 1/2, and three years after diagnosis, respectively. The five-year survival rate of 82.6 percent of this series is considerably better than the reported five-year survival rates of synovial sarcoma which range from 25-51%.

Adolescent↗
PubMed1982

Neuromuscular hamartoma--a benign "triton tumor" composed of mature neural and striated muscle elements.

Two infants with benign, hamartomatous tumors composed of well-differentiated and intimately admixed neural and striated muscle components are presented. Their histogenesis is discussed in light of embryologic concepts of the derivation of mesenchymal tissues from neuroectoderm and in reference to previous reports of benign and malignant tumors of the nervous system with striated muscle components.

Axilla↗
PubMed1981

Infantile myofibromatosis.

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."

Age Factors↗
PubMed1981

Pleomorphic lipoma: a benign tumor simulating liposarcoma. A clinicopathologic analysis of 48 cases.

A clinicopathologic study of 48 cases of pleomorphic lipoma from the files of the Armed Forces Institute of Pathology reveals that this tumor occurs principally in males (83%) in the fifth to seventh decades (mean 57 years) and shows a predilection for the posterior neck, shoulder, and back. Typically, the lesion appears as a painless, circumscribed subcutaneous mass that, on gross examination, resembles an ordinary lipoma. However, microscopically, in contrast to the uniform appearance of the mature adipose tissue cells of an ordinary lipoma, this neoplasm is characterized by an intimate admixture of variably-sized fat cells, and bizarre, pleomorphic, multinucleated giant cells. Many of the latter cells show a distinctive floret-like arrangement of the nuclei and are associated with interlacing bundles of dense birefringent collagen. Despite this pleomorphic picture, which not infrequently leads to a misdiagnosis of liposarcoma, follow-up data obtained for 34 patients (median follow-up period of three years) establish the invariably benign clinical behavior of this unusual tumor.

Adult↗
PubMed1981

Intravascular fasciitis: a report of 17 cases.

Seventeen cases of intravascular fasciitis were reported from the files of the AFIP (14 cases) and the Pathology Department, Thomas Jefferson University Hospital (three cases). It is characterized by the histologic features of nodular fasciitis but with intraluminal, intramural, and extramural involvement of small to medium-sized veins and arteries and multinodular or serpentine growth along the course of affected blood vessels. A painless, slowly growing mass was most common. Fourteen patients (82%) were under age 30 (range 1/2-57 years); the sex ratio was about equal. Seven cases were located in the upper extremity (three hand, two wrist or forearm, two shoulder), three on the ankle or lower leg, five on the head and neck, and two in the chest wall and breast. Most cases were small with a mean diameter of 1.5 cm, but two were elongated masses measuring 4-5 cm in length. At surgery, four cases showed intimate association with large veins or arteries. This type of fasciitis is rare and has been found in only one of 14 consecutive cases of modular fasciitis operated at this university hospital between 1969 and 1978. Six of 15 consultation cases were originally confused with sarcoma. Intravascular fasciitis is benign. Seven patients were free of disease from 2.5 to 20 years (mean 9.5 years); two patients had local recurrences.

Adolescent↗
PubMed1980

Cranial fasciitis of childhood.

Nine cases of fibroblastic lesions occurring in the cranium of young children were reviewed. The age of the patients at the time of initial treatment ranged from three weeks to six years (median 18 months), with the lesions being congenital in two cases. There was 2:1 male predominance. The size of the lesions averaged 2.5 cm in greatest dimension with the largest being 9.0 cm. All cases presented as rapidly growing masses with a preoperative duration of only two months. The lesions presented as soft-tissue masses deep in the scalp with involvement of the underlying cranium in all eight of the cases in which roentgenograms or operative reports were available for review. Characteristically, there was erosion of only the outer table of the skull, although in three cases the lesion extended through the inner table to attach to the underlying dura mater. It was not possible to detect the exact site or origin, although origin from one of the deep fascial layers of the scalp or the underlying periosteum seems most likely. Microscopically, the lesion appeared to be a proliferation of loosely arranged fibroblasts which most closely resembled nodular fasciitis. Mitotic figures as well as foci of osseous metaplasia were present. Treatment consisted of excision of the mass with local resection or curettage of the affected underlying bone in some cases. Followup revealed a benign clinical course with no recurrent or aggressive behavior.

Child↗
PubMed1979

Malignant Schwannoma associated with von Recklinghausen's neurofibromatosis.

A series of 46 malignant schwannomas occurring in soft parts of patients having von Recklinghausen's neurofibromatosis was analyzed. The diagnosis of malignant schwannoma was based upon the occurrence of malignant spindled cells closely resembling Schwann cells in the neoplasm and the close association or origin of the malignant schwannoma in a neurofibroma (27 tumors), or a large peripheral nerve (31 tumors). Additional histologic features useful in making the diagnosis of malignant schwannoma included the arrangement of the spindled tumor cells in a whorled pattern about thin-walled, gaping blood vessels, perivascular cellular proliferation and the presence of prominent myxoid stroma containing abundant hyaluronidase-sensitive acid mucopolysaccharides. Nuclear palisading was present in only one case. Eight tumors containing both neoplastic Schwann cells and rhabdomyoblasts and five containing both neoplastic Schwann cells and rhabdomyoblasts (malignant "Triton" tumors) and five containing foci of malignant cartilage cells were included in the series. The neoplasms occurred principally in adults (median age, 34 years) and were most common in the lower extremity (18 cases) and retroperitoneum (11 cases). A mass with or without pain was the most common presenting symptom (28 cases). The median size of excised tumors was 11 cm. The malignant schwannomas were highly malignant neoplasms, causing the death of 39 patients within five years and two patients within 6--10 years after diagnosis. Only four patients were alive and free of tumor 5--15 years after diagnosis.

Adolescent↗
PubMed1979

Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm.

This article describes 41 examples of an unusual fibrohistiocytic sarcoma which occurred primarily in the extremities of young individuals between the ages of 5 and 25 years (median 13 years). It manifested as a nodular subcutaneous growth that seldom caused tenderness or pain, and clinically was often mistaken for a hematoma or a hemangioma. Grossly, the tumor presented as a circumscribed, multinodular or multicystic, hemorrhagic mass that ranged in size from 0.7 to 10 cm (median 2.5 cm). On microscopic examination, it consisted principally of 1) solid arrays or nests of fibroblast- and histiocyte-like cells, not infrequently containing varying amounts of intracellular hemosiderin or lipid, 2) focal areas of hemorrhage or hemorrhagic cyst-like spaces, sometimes occupying the major portion of the tumor, and 3) aggregates of chronic inflammatory cells, chiefly lymphocytes and plasmacytes, a feature that caused confusion with a lymph node metastasis in several cases. Follow-up information, available in 24 patients, revealed a variable clinical course. Twenty-one patients were alive, 11 with recurrence (including one with 9 recurrences in a 21-year period) one with recurrence and metastasis and one with metastasis. Three patients had died of metastasis 1, 3, and 13 years respectively, after the initial surgical therapy. The exact histogenesis is still obscure. Most likely it is a tumor of fibroblast- and histiocyte-like cells, akin to malignant fibrous histiocytoma, but different in its age incidence, microscopic appearance and behavior.

Adolescent↗
PubMed1978

Chondroma of soft parts.

The clinical and pathological features of 104 cases of chondroma of soft parts are presented. The tumor occurred predominately in the third and fourth decades, was slightly more common in male (61%) than in female patients, and affected chiefly the soft tissues of the hand (64%) and feet (20%). The presenting symptom was usually a slowly and insidiously growing mass, occasionally causing tenderness or pain. Nearly always the tumor was well demarcated and lobulated and measured between 1 and 2 cm in greatest diameter. Microscopically, most of the tumors were composed of adult-type hyaline cartilage, undergoing calcification in a large percentage of cases. In addition, histologic variants with giant cell proliferation and chondroblastic activity could be distinguished. Despite the slight cellular pleomorphism and the plump appearance of many cartilage cells in the chondroblastic variants, there was no evidence that these tumors behaved differently from the tumors composed predominately of adult-type hyaline cartilage. Of the 56 patients with follow-up information (median follow-up period, 5.7 years) 44 were alive and well with no evidence of recurrence in the follow-up period. In 10 patients the tumor had recurred once. Multiple recurrences or metastatic lesions were no observed. Two patients died of unrelated cause. Complete local excision appears to be the treatment of choice.

Adolescent↗
PubMed1978

Malignant fibrous histiocytoma: an analysis of 200 cases.

The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow-up information are presented. This tumor occurred principally as a mass on an extremity (lower extremity 49%, upper extremity 19%) or in the abdominal cavity or retroperitoneum (16%) of adults (peak incidence 61-70 years of age). It typically involved deep fascia (19%) or skeletal muscle (59%) and only rarely was confined to the subcutis without fascial involvement (7%). The MFH had variable morphologic features and frequently showed transitions from areas having a highly ordered storiform pattern to less differentiated areas having a pleomorphic appearance. The rate of local recurrence of the tumor was 44%, and of metastasis, 42%. Metastasis was most frequently to the lung (82%) and lymph nodes (32%). Factors that influenced the rate of metastasis included depth, size, and inflammatory component of the tumor. Tumors that were small, superficially located, or had a prominent inflammatory component metastasized less frequently than larger, more deeply located tumors. In our experience the MFH is the most common soft tissue sarcoma of late adult life, and many tumors previously diagnosed as pleomorphic variants of liposarcoma, fibrosarcoma, or rhabdomyosarcoma are probably examples of MFH. Although the histogenesis of this neoplasm remains controversial, we feel it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.

Adolescent↗
PubMed1978

Silica reaction simulating fibrous histiocytoma.

An unusual foreign body reaction to silica (quartz), resembling fibrous histiocytoma, is described in 7 patients (age range 22 to 71 years). These lesions occurred in the inguinal region (4 cases) or the abdominal wall (3 cases), and in 5 instances were associated with a history of injection at the same site 10 to 41 years previously. Most commonly the injection consisted of the administration of a sclerosing agent for the repair of a hernia. Microscopically these lesions contained broad sheets of histiocytes separated by bands or concentrically lamellated nodules of collagen. Intra- and extra-cellular birefringent crystals, identified by x-ray diffraction as silica (quartz), were present in all cases and served to distinguish this process from a true neoplasm. Although the injection therapy for hernia is now obsolete, it was formerly practiced in the United States with a variety of sclerosing agents including silica (quartz). Since these lesions are often confused with a benign or malignant fibrous histiocytoma it is recommended that fibro-histiocytic lesions associated with a previous hernia or injection at the same site be examined for the presence of silica.

Adult↗
PubMed1977

Myxoid variant of malignant fibrous histiocytoma.

It has been generally recognized that malignant fibrous histiocytoma (MFH) may assume a highly myxoid, hypocellular appearance. Eighty cases of malignant fibrous histiocytoma having varying degrees of myxoid change were reviewed. These tumors typically arose on the extremities (leg, 61%; arm, 21%) of adults (peak age incidence, 60-69 years). They were usually attached to fascia (31%) or involved skeletal muscle (51%) and had a mucoid or translucent appearance. The myxoid areas consisted of widely spaced spindled and pleomorphic cells embedded in a matrix of acid mucopolysaccharides. The cellular areas were indistinguishable from those of the typical pleomorphic MFH. The rate of local recurrence of these tumors was 61%, and of metastasis, 23%, but metastasis was less likely when the tumor was small, superficially located, or had a prominent myxoid component. In fact, the degree of myxoid change was inversely related to the rate of metastasis. Therefore, because of the more favorable prognosis of the myxoid variant, it seems appropriate to separate it from the usual nonmyxoid form of MFH. The myxoid variant must also be clearly distinguished from benign myxoid lesions such as myxoma or nodular fasciitis, with which it is often confused.

Adult↗