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Biomedical subjects

F M Enzinger

Publications and source records attributed to F M Enzinger.

At least 73 records · Page 4Linked to original sources

Infantile fibrosarcoma.

The pathologic features and the behavior of 53 cases of infantile fibrosarcoma are presented. The tumor nearly always occurred during the first 2 years of life; 41 of the 53 cases occurred during the first, six during the second, and three each during the third and fourth year of life, respectively. Twenty of the 53 cases were present at birth. The tumor was more common in boys (60%) and affected chiefly the distal portions of the lower and upper extremities (72%). Most of the tumors grew rapidly and reached a large size within a few weeks or months. They were generally poorly circumscribed and infiltrated variously subcutaneous fat, muscle, fascia and tendons. Microscopically, they were composed of immature-appearing spindle-shaped cells and were marked by their high cellularity and prominent mitotic activity. Despite these features, follow-up information revealed a favorable clinical course, particularly as compared with the adult form of fibrosarcoma. Of the 48 patients with follow-up data, 31 were alive and well with no evidence of recurrence, eight were alive with recurrence, and one was alive following lobectomy for metastatic tumor. Of the living patients, 12 were treated with amputation, nine with radical or wide local excision, and 15 with simple excision. In two cases surgery was followed by chemotherapy, and in one, by radiotherapy. Eight of the 48 patients with follow-up had died, four of metastatic tumor (8.3%) and four of miscellaneous causes. Wide local excision appears to be the treatment of choice unless the size of the tumor and its anatomic location require amputation. Since late recurrent and metastatic lesions were encountered, long-term followup is necessary before one can safely assume that the patient has been cured.

Child, Preschool↗

Hemangiopericytoma. An analysis of 106 cases.

A series of 106 cases of hemangiopericytoma was analyzed. The neoplasms occurred principally in adults (median age, 45 years), were deep seated, and were most common in the thigh (27 cases) and the pelvic retroperitoneum (26 cases). A painless mass was the first symptom in 96 of the patients. The median size of the excised tumors was 6.5 cm. Surgical removal of the tumor was often complicated by hemorrhage because of marked dilatation of the vascular bed in the vicinity of the neoplasm, probably as the result of rapid exchange of blood from the arterial to the venous circulation within the tumor. Microscopically, benign and malignant forms could be distinguished. The latter were characterized by increased cellularity, prominent mitotic activity, and foci of necrosis or hemorrhage. Follow-up information was obtained in 93 cases. Seventy-one of the 93 patients were living (two with recurrence and four with metastasis), and 22 had died (13 as the result of recurrence or metastasis in more than two-thirds of the related causes). Recurrence preceded metastasis in more than two-thirds of the patients with evidence of metastasis. The 10 year survival rate was 70 per cent. The morphologic differences from other mesenchymal tumors showing a hemangiopericytoma-like vascular pattern are discussed, and the close resemblance of hemangiopericytoma to richly vascular forms of fibrous histiocytomas and synovial sarcoma is emphasized. Congenital or infantile hemangiopericytoma is described as a separate entity having a distinctive microscopic pattern and behavior.

Adolescent↗

Intravascular papillary endothelial hyperplasia.

Papillary endothelial hyperplasia is a peculiar benign intravascular process that bears a remarkable resemblance to a hemangiosarcoma. In 44 cases of this lesion studied from the files of the Armed Forces Institute of Pathology, the process manifested as a small tumor-like lesion that occurred most frequently in the subcutis of the fingers (14 cases), the head and neck region (ten), and the trunk (seven). Microscopically, the tuft-like or papillary proliferation of endothelial cells was nearly always intimately associated with a thrombus and seemed to represent a peculiar variant of an organizing process. Features that aided in recognition and differential diagnosis from a hemangio-sarcoma included the intraluminal location of the lesion, the absence of tissue necrosis, and the intimate association of the proliferated tuft-like structures with thrombotic material. Follow-up information obtained in 31 cases indicated a benign clinical course despite the sarcoma-like microscopic appearance of this condition.

Adolescent↗

Synovial sarcoma of the neck: a followup study of 24 cases.

This article reviews the clinicopathologic features and the biological behavior of 24 cases of synovial sarcoma that took origin from the cervical prevertebral connective tissue space and manifested as a retropharyngeal tumor or as a palpable mass in the anterior or posterior cervical triangle. The age of the 24 patients ranged from 10 to 51 years, with a median of 19 years. Ten patients were women and 14 men. Hoarseness or difficulty in breathing or swallowing were the first symptoms in eight patients. The tumors were solitary and ranged from 2 to 10 cm in greatest dimension. Microscopically, all of the cases showed the characteristic biphasic cellular pattern of a synovial sarcoma, with epithelioid and fibrosarcoma-like areas in varying proportions. Synovioblastic origin of the neoplasm was confirmed by the results of histochemical staining procedures and, in 1 case, by the examination with the electron microscope. Of the 21 cases followup information, 12 had died (10 with pulmonary metastasis) and 9 were alive and free of symptoms. Prompt and complete surgical removal is required to prevent complications from recurrent tumor growth or metastasis.

Adolescent↗

Extraskeletal neoplasm resembling Ewing's sarcoma.

This article reviews the pathologic features and the behavior of 39 small, round, or oval cell sarcomas occurring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone. The tumors affected chiefly young adults (median age 20 years) and most commonly involved the soft tissues of the lower extremity and the paravertebral region. Microscopically, they consisted of solidly packed small, round, or ovoid cells of great uniformity, arranged in sheets or lobules separated by strands of fibrous connective tissue. The nucleus of the tumor cells contained finely divided chromatin, a distinct nuclear membrane, and frequently a minute nucleolus. The scanty ill-defined cytoplasm contained varying amounts of glycogen. Sometimes the histologic picture was dominated by a "peritheliomatous" pattern, or by large areas of necrosis or hemorrhage. Followup data ranging from 1 month to 14 years were available in 35 of the 39 cases (93%). Of these, 13 were alive and 22 had died. In the majority of the fatal cases, the clinical course was rapid; metastatic lesions developed within a few months after the primary tumor was excised. The lungs and the skeleton were the two most common sites of metastasis. Cure may be achieved by wide local excision of the tumor at an early stage of the disease, combined with radiation therapy and chemotherapy.

Adolescent↗

Proliferative fasciitis.

The clinical and pathologic features of 53 cases of proliferative fasciitis, a pseudosarcomatous process involving fascia and interlobular fibrous septa of the subcutaneous fat, were reviewed. In the reviewed material, the lesion affected adults exclusively, with a high incidence in patients older than 40 years (median 54 years). It involved most commonly the upper and lower extremities, and microscopically was characterized by a diffuse infiltrative fibroblastic growth intimately associated with multifocal proliferation of large basophilic cells, closely resembling ganglion cells--a microscopic picture akin to proliferative myositis. In 16 of the 53 cases, the lesion had been initially confused with sarcoma because of its rapid growth and its bizarre histologic features. Although the term "proliferative fasciitis" has been used in the past as a symptom for nodular fasciitis, it is suggested that it be limited to the lesion under discussion, in order to emphasize its close relationship to proliferative myositis. Followup information revealed a benign clinical course. Therefore, despite its rapid growth and bizarre microscopic appearance, the lesion is adequately treated by local excision, and there is no indication for radical surgery.

Adult↗

Spindle cell lipoma.

Spindle cell lipoma is a specific type of lipoma that is easily mistaken for a liposarcoma. An analysis of 114 cases of this tumor from the files of the Armed Forces Institute of Pathology reveals that is occurs chiefly in male patients between 45 and 70 years of age and affects the regions of the shoulder and posterior neck almost exclusively. Microscopically, it consists of an intricate mixture of lipocytes and uniform spindle cells within a matrix of mucinous material traversed by a varying number of birefringent collagen fibers. Ultrastructural studies in one case support the fibroblastic nature of the spindle cells. Followup information obtained in 63 patients with this lesion revealed a uniformly favorable clinical course, indicating that local excision is the treatment of choice, and that there is no need or reason for radical surgical procedures.

Adult↗