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1/334. radium-induced malignant tumors of the mastoid and paranasal sinuses.

    In the records of 5,058 persons with therapeutic or occupational exposure to radium, 21 patients with carcinoma of the mastoid and 11 with malignant tumors of the paranasal sinuses were identified. Tumor induction times were 21-50 years for mastoid tumors (median, 33) and 19-52 years for paranasal sinus tumors (median, 34). Dosimetric data are given for the patients whose body burdens of radium have been measured. We found a high proportion of mucoepidermoid carcinoma, comprising 38% of the mastoid and 36% of the paranasal sinus tumors. Three patients had antecedent bone sarcoma at 20, 11, and 5 years, respectively, and a bone sarcoma was discovered at autopsy in a fourth patient. Radiographic changes in the mastoid and paranasal sinuses were similar to those seen in nonradium malignant tumors. More than 800 known persons exposed to radium before 1930 and another group of unknown size who received radium water or injections of radium from physicians are still alive and at risk of developing malignant tumors of the mastoid and paranasal sinuses.
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ranking = 1
keywords = sarcoma
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2/334. Microcystic adnexal carcinoma arising in the setting of previous radiation therapy.

    While there are several reports of microcystic adnexal carcinoma developing in patients within sites of previous therapeutic irradiation, this relationship is not well described in the dermatologic literature. We report a case of a 42-year-old man with a remote history of therapeutic irradiation following surgical resection of periorbital rhabdomyosarcoma. Subsequently, he developed multiple basal cell carcinomas and a microcystic adnexal carcinoma within the field of irradiation. The histologic features were those of a classic microcystic adnexal carcinoma, with well differentiated nests and cords of keratinocytes displaying follicular and ductular differentiation infiltrating diffusely into the reticular dermis. Dense fibrosis was present surrounding the neoplastic keratinocytes. Nuclear atypia and mitotic figures were not identified. A carcinoembryonic antigen (CEA) stain demonstrated glandular differentiation. It is important for dermatologists to be aware of the apparent relationship between the rare microcystic adnexal carcinoma with its innocuous scar-like clinical appearance and prior local radiation therapy.
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ranking = 0.5
keywords = sarcoma
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3/334. Intra-abdominal angiosarcomatosis after radiotherapy.

    BACKGROUND: We report a case of a 61-year-old Japanese woman who developed intra-abdominal angiosarcomatosis 20 years after receiving radiotherapy for squamous cell carcinoma of the cervix. methods AND RESULTS: The surgically resected portion of the ileum showed diffuse proliferating angiosarcoma, with irregular channels lined by atypical vascular endothelial cells. Immunohistochemical studies showed that the tumour cells were positive for factor viii-related antigen and ulex europaeus agglutinin 1. At autopsy, the tumour had disseminated to the peritoneum and invaded into the right thoracic cavity. CONCLUSIONS: These findings were compatible with radiation-induced angiosarcomatosis.
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ranking = 3.5
keywords = sarcoma
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4/334. temporal bone tumours in patients irradiated for nasopharyngeal neoplasm.

    radiation-associated tumours are rare complications of radiotherapy. This study seeks to highlight and discuss the clinically challenging problem of radiation-associated tumours (rats) in the temporal bones of seven patients previously irradiated for nasopharyngeal neoplasm. Seven patients (six males and one female) with radiation-associated temporal bone tumours are presented (five squamous cell carcinomas, one osteogenic sarcoma and one chondrosarcoma). The initial nasopharyngeal disease for which radiotherapy was indicated was nasopharyngeal carcinoma (six patients) and nasopharyngeal lymphoma (one patient). The latency period between radiotherapy and presentation of temporal bone tumours ranged from five years to 30 years with a mean of 12.9 years. All the patients underwent surgical tumour resection. Three patients had post-operative radiotherapy and one patient underwent pre- and post-operative chemotherapy. Two patients died from the disease within three months of treatment with one patient surviving 36 months at the time of writing. One patient died from an unrelated medical condition three months after surgery. With refinement in radiotherapy techniques and the resultant increase in patient survival, there may be more patients with radiation-associated tumours in the future. It remains imperative for clinicians to be vigilant when patients previously irradiated for nasopharyngeal carcinoma present with otological symptoms as the key to the successful management of this condition lies in the early detection and expedient treatment of this difficult disease.
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ranking = 1
keywords = sarcoma
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5/334. meningioma after radiotherapy for Hodgkin's disease.

    The most common second primary tumors after treatment of childhood Hodgkin's disease are leukemia, lymphoma, breast cancer, soft tissue sarcoma, and thyroid cancer. Although intracranial meningioma has been reported after radiotherapy to the scalp for benign conditions and for intracranial primary brain tumors, this appears to be an extremely rare sequelae of treatment for Hodgkin's disease. The authors describe a 15-year-old boy who underwent radiotherapy for Hodgkin's disease and in whom a meningioma developed in the posterior fossa 27 years later.
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ranking = 0.5
keywords = sarcoma
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6/334. Multifocal osteosarcoma as second tumor after childhood retinoblastoma.

    We present a case of multifocal osteosarcoma (MFOS) arising 11.5 years after successful treatment of bilateral retinoblastoma. The clinical, imaging and pathological findings at onset, after therapy, and during follow-up are described. Fluorescent in situ hybridization did not reveal a deletion of the RB-1 retinoblastoma gene, although the presence of an inactivating mutation invisible to this method cannot be ruled out. The MFOS may have been a second multifocal tumor associated with the original retinoblastoma or a post-irradiation sarcoma with extensive metastases.
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ranking = 3
keywords = sarcoma
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7/334. Postradiation neural sheath sarcoma of the brachial plexus: a case report.

    Sarcoma arising in the brachial plexus is a rare complication following radiation therapy, with only 7 patients reported in the literature. The absolute number of patients is likely to increase with the growth of radiation therapy to treat breast cancer. Because of its rarity, early diagnosis requires a high degree of suspicion. We present a patient with neural sheath sarcoma of the brachial plexus arising 18 years after external beam radiation for breast cancer. The interval between presentation and definitive therapy was prolonged by workup and treatment of carpal tunnel syndrome and cervical nerve root compression. The English language literature regarding postradiation sarcoma is reviewed and the clinical, radiological, and electrophysiological workup of postradiation brachial plexopathy is discussed.
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ranking = 3
keywords = sarcoma
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8/334. Cytogenetic study of eight new cases of radiation-induced solid tumors.

    radiation-induced tumors were selected according to the criteria defined by Cahan (1948) for sarcomas. Cell cultures and/or xenografts in nude mice were performed with biopsies obtained from second primary tumors. Karyotypes of eight tumors were established after R-banding. After comparison with literature data on 15 other cases, two distinct cytogenetic patterns could be distinguished. One was characterized by polyclonal karyotypes, of which a large proportion were simple and carriers of balanced translocations. Another one was characterized by monoclonal chromosome alterations observed in highly aneuploid and complex karyotypes, in which many deletions were observed. These two different patterns could be related to the modality of metaphase harvesting. Polyclonal karyotypes were preferentially observed after long-term cultures, and monoclonal karyotypes after short-term cultures or xenografts. The following scheme of radiation oncogenesis is proposed: a) induction of recessive gene mutations including that of tumor suppressor genes; b) accumulation of genomic alterations in the irradiated tissue with aging, including deletions or mutations of normal alleles from mutated tumor suppressor genes; and c) loss of tumor suppressor gene function and initiation of a multistage tumor development and progression. Polyclonal abnormalities are assumed to exist in noncancerous cells which acquired radiation-induced chromosome aberrations.
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ranking = 0.5
keywords = sarcoma
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9/334. Benign lymphangiomatous papules of the skin following radiotherapy: a report of five new cases and review of the literature.

    AIMS: The aim of this report is to summarize the clinicopathological findings of five cases of a pseudosarcomatous vascular proliferation of the skin at the site of radiotherapy following surgery for carcinoma of the breast and carcinoma of the endometrium. To our knowledge, only five cases of this presumably rare pseudomalignant proliferation have been reported previously. methods AND RESULTS: All patients were females ranging in age from 44 to 70 years. The lesions appeared as solitary or multiple papules or vesicles localized to the field of radiation. Microscopically, they were composed of vascular spaces that exhibited atypical features without qualifying for a diagnosis of angiosarcoma. CONCLUSIONS: Our cases provide additional evidence supporting the benign nature of this atypical vascular proliferation, not recurring, never developing metastases and being cured readily by local excision. Clinical, histopathological and ultrastructural findings suggest a lymphatic origin. Whether these lesions represent a neoplastic or a reactive condition secondary to radiotherapy is unclear. The name 'benign lymphangiomatous papules of the skin following radiotherapy' is proposed.
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ranking = 1
keywords = sarcoma
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10/334. Fine structure of a radiation-induced osteogenic sarcoma.

    An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.
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ranking = 2.5
keywords = sarcoma
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