One hundred and sixty-five cases of malignant schwannoma were reviewed. Sixty-five (40%) of the patients had evidence of disseminated neurofibromatosis. Patients with neurofibromatosis were younger, had malignant schwannomas that were centrally rather than peripherally located, and had a shorter five-year survival (23%) than patients with solitary malignant schwannomas (47%). Histologically, tumors developing in patients with neurofibromatosis had a collagenous appearance, while tumors in patients without neurofibromatosis were undifferentiated and highly cellular. The clinical course of both groups of patients tended to be that of multiple local recurrences, although local recurrence had a more ominous prognosis in patients with neurofibromatosis. Chemotherapy responses in all these patients were extremely poor; however, the results of adjuvant therapy after surgery appeared encouraging. Fourteen patients (8.5%) had a malignant schwannoma in an area of prior radiation therapy and died of disease a median of 14 months after diagnosis. Malignant schwannoma should be considered in the differential diagnosis of tumors developing in areas previously treated with radiation.
The clinical records and histologic material of 48 patients with extraosseous osteogenic sarcoma were reviewed. Most patients developed their tumors in the fifth or sixth decades of life. Five patients (10%) developed neoplasms in an area of prior radiation therapy, a median of 15 years after their exposure. Six patients (13%) related a history of trauma to the area where their extraosseous osteogenic sarcoma developed. The course of most patients was that of multiple local recurrences (69%) followed by pulmonary metastases (80%) and death (76%). Amputation or wide resection followed by irradiation appeared to be the most effective types of therapy, with median survivals greater than 60 months for patients receiving these treatments, compared to 28 months for patients initially treated with resection alone. Chemotherapy was not effective for patients with advanced disease; however, adjuvant chemotherapy after surgery may have been of value. Four of five patients who received adjuvant chemotherapy after surgery are alive and disease‐free; the only two survivors after development of pulmonary metastases received adjuvant chemotherapy after surgical resection of visible pulmonary metastases.
One‐hundred‐twenty‐three cases of malignant pleural mesothelioma were reviewed. Exposure to asbestos or to other industrial dusts or chemicals was an important etiologic factor with 24% of patients relating such a history. A history of prior irradiation or previous lung disease was also occasionally noted. Diagnosis was most often made by exploratory thoracotomy, with pleural biopsy or cytology rarely helpful. Except for nine patients, tumor was confined to the chest at the time of diagnosis, but in 33 of the remaining 114 patients, spread to the abdomen or distant metastasis was seen during the course of disease. Surgery and radiotherapy were ineffective in preventing local recurrence. There were only three major responses to chemotherapy in 111 trials. Median survival was 12 months, and only seven patients (5.6%) lived more than five years. Patients with epithelial mesothelioma and Stage I disease had the most favorable prognosis.
Forty‐four cases of lymphangiosarcoma treated at Memorial Sloan‐Kettering Cancer Center were reviewed. With the exception of four patients with primary lymphangiosarcoma of the scalp, all patients had had chronic lymphedema of the involved limb for many years, usually following mastectomy for breast carcinoma, but occasionally due to other causes. Although chronic lymphedema could be implicated in the etiology of lymphangiosarcoma in all patients with neoplasms of an extremity, a significant number of patients did not have a history of radiation therapy at the site where their tumor developed. Histologically, although there were no differences in the lymphangio‐sarcomas between any of the groups of patients, the morphology of the tumors was influenced in the individual patient by the size and anatomic site of the sarcoma. Early amputation seemed to give the best chance of long‐term survival, with responses to wide resection, chemotherapy, and radiation therapy generally only of short duration. Amputation after local recurrence was ineffective in preventing pulmonary metastases and death. At present, early amputation appears to be the treatment of choice for patients with lymphangiosarcoma.
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