The malignant giant cell tumor of soft parts is an extraskeletal malignant neoplasm somewhat resembling a giant cell tumor of bone and consisting of multinucleated giant cells (occasionally containing asteroid bodies), monon‐nuclear histiocytes, and fibroblasts arranged in a multinodular pattern. Thirty‐two cases of this tumor in the AFIP files were reviewed and subdivided into a superficial and a deep group; the superficial tumors (12 cases) were small and occurred in the region of the subcutis and superficial fascia, with a predilection for the leg. They recurred frequently after excision, but only two metastasized and terminated in death. Six of the 12 patients with superficial tumors survived 5 or more years following local excision. The deep tumors were large and involved skeletal muscle, deep fascia, and tendons. They affected the thigh chiefly. Fourteen of the 20 patients developed pulmonary or widespread metastasis and died; eight of the 14 had been treated by amputation of the extremity. Four of the 20 patients with deeply located malignant giant cell tumor of soft parts survived 5 years.
Nineteen cases of bronchopulmonary leiomyosarcoma and 13 cases of bronchopulmonary fibrosarcoma in the files of the Armed Forces Institute of Pathology, as well as 48 cases of each of these tumors previously reported, were reviewed in an attempt to clarify their histopathologic criteria and biologic behavior. The number of mitoses, the size, and the endobronchial or intrapulmonary location of the tumors seemed to be the best bases for predicting their biologic behavior. The small endobronchial tumors confined to the bronchial wall had a benign behavior for the most part. On the other hand, the intrapulmonary tumors varied in their degree of malignancy, depending upon their number of mitoses and size. Contrary to earlier belief, many of the latter tumors were found to be of a relatively high grade of malignancy because of their large size, local extension, or metastasis.
PCR procedures amplifying portions of the 16S rRNA and NADH oxidase genes of Brachyspira aalborgi and Serpulina pilosicoli were applied to DNA extracted from paraffin-embedded human colonic or rectal tissues from 30 Norwegian, Australian, and U.S. patients, 16 of whom had histologic evidence of intestinal spirochetosis (IS). B. aalborgi-specific sequences were identified by PCR in 10 of the IS patients (62.5%) but none of the others, while S. pilosicoli sequences were not detected in tissues from any patient. Direct sequencing of products from three of the positive samples provided further confirmation of the presence ofB. aalborgi. B. aalborgi may be a more common cause of intestinal spirochetosis than has been previously thought.
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.
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