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1/8. carcinoma erysipelatoides-cutaneous lymphatic vessel spread of a poorly differentiated naso-pharyngeal carcinoma.

    carcinoma erysipelatoides, which is frequently called inflammatory carcinoma, is an uncommon form of skin metastasis. It originates most commonly from breast carcinoma and less often from endometrial or bronchogenic carcinoma. The naso-pharynx is an unusual source for carcinoma erysipelatoides. We present a case of carcinoma erysipelatoides due to poorly differentiated naso-pharyngeal carcinoma.
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2/8. lymphangioma circumscriptum of the vulva following surgical and radiological therapy of cervical cancer.

    BACKGROUND: lymphangioma circumscriptum of the vulva rarely develops after postoperative pelvic irradiation. GOAL: The goal was to describe two cases of lymphangioma circumscriptum and their treatment and present a brief review of the literature. STUDY: Two female patients, aged 75 years and 46 years, presented with persistent edema, papules, and vesicles of the labia majora, which had developed 15 and 9 years after hysterectomy, lymph node dissection, and subsequent irradiation of cervical cancer. The external diagnosis was genital warts. RESULTS: In both cases histology revealed lymphangioma circumscriptum of the vulva. Whereas the older woman's condition responded well to laser treatment, keloids developed in the second patient at the site of carbon dioxide laser vaporization. CONCLUSION: CO2 laser treatment recently has been recommended for vulvar lymphangioma circumscriptum and is effective in vaporizing the communicating vessels to deeper cisterns. To our knowledge this is the first description of keloid development after laser therapy for vulvar lymphangioma circumscriptum, and such an effect should be considered before CO2 laser surgery is applied for this particular entity.
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3/8. Radiation-induced meningiomas: experience at the Mount Sinai Hospital and review of the literature.

    From the records of The Mount Sinai Hospital, seven cases which met established criteria for radiation-induced meningiomas were identified. This represents the largest series of radiogenic meningiomas documented in north america and includes both intracranial and intraspinal tumors. The records and pathological specimens were reviewed and these data analyzed with other cases retrieved from the world literature. This study reveals that radiation-induced meningiomas can be categorized into three groups based on the amount of radiation administered: 1) low dose; 2) moderate dose and miscellaneous; and 3) high dose. The overwhelming majority of cases had received low-dose irradiation (800 rad) to the scalp for tinea capitis and the second largest group resulted from high-dose irradiation for primary brain tumors (greater than 2000 rad). The unique features distinguishing radiation-induced meningiomas from other meningiomas are reviewed. Although histologically atypical tumors were common in this series, overt malignancy was not encountered. The preoperative management of these lesions should include angiography to evaluate for large-vessel occlusive vasculopathy, a known association of meningiomas induced by high-dose irradiation. Given the propensity these tumors possess for recurrence, a wide bony and dural margin is recommended at surgical resection.
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4/8. Thorotrast-induced hepatic angiosarcoma with 39 years latency. A pathologic and immunohistochemical study.

    An autopsy case of hepatic angiosarcoma which occurred in a 64-year-old man who had been examined by Thorotrast angiography 39 years previously, is reported together with the pathologic, radiologic and immunohistochemical findings. Thorotrast deposits were found in the spleen, bone marrow, right elbow joint and lymph nodes as well as the liver. The angiosarcoma was accompanied by markedly distended sinusoids with hypertrophic and hyperplastic endothelial cells, which were considered to be closely associated with angiosarcoma. The results of immunostaining indicated that only a very limited number of tumor cells and not many sinusoidal endothelial cells showed a positive staining reaction to anti-factor viii-related antigen (anti-F VIII-RAG). On the other hand, endothelial cells lining blood vessels in the portal areas were distinctly positive for anti-F VIII-RAG. This would suggest that production of F VIII-RAG by tumor cells was dependent on the degree of cell dedifferentiation or on the heterogeneity of endothelial cells.
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5/8. Radiation-induced leiomyosarcoma of the great vessels presenting as superior vena cava syndrome.

    A patient with a pleomorphic intravascular leiomyosarcoma of the great vessels of the neck and mediastinum presented clinically with a superior vena cava syndrome. A latent period of 29 years elapsed between receiving orthovoltage radiation to the neck and right side of chest to treat recurrent ganglioneuroblastoma, and the appearance of a leiomyosarcoma and subsequent recurrences. The patient underwent partial resection of the tumor, received adjunct chemotherapy, and was shown to be free of disease by clinical tests and by magnetic resonance imaging (MRI) 17 months after completion of chemotherapy. The criteria for the diagnosis of radiation-induced sarcomas are reviewed in relation to the present case. The critical role of magnetic resonance imaging in both the diagnosis and continued follow-up of the patient is described. This would appear to be the first reported case of radiation-induced intravascular leiomyosarcoma of the great vessels of the neck and mediastinum presenting as a superior vena cava syndrome.
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6/8. Intracranial large vessel vasculopathy and anaplastic meningioma 19 years after cranial irradiation for acute lymphoblastic leukaemia.

    A child was diagnosed in 1969 as having acute lymphoblastic leukaemia (ALL) and received chemotherapy. On bone marrow relapse in 1973, he was treated with cranial irradiation (20 Gy) in addition to chemotherapy. He continues in complete remission 19 years after his relapse. At age 25 years, he presented with headaches and left hemiparesis. Computerised tomograph demonstrated a large, enhancing right-sided intracranial tumour. angiography was performed and showed the right internal carotid artery was occluded. Most of the right hemisphere was supplied from the external carotid via the middle meningeal artery. The left posterior cerebral artery and the left anterior cerebral artery were absent presumably as a result of radiation-induced arteritis. A resection of an anaplastic meningioma arising from the right sphenoidal ridge was achieved. There was a rapid improvement in function and he returned to work. Vasculopathy of the large intracranial arteries has been described after high dose radiation. It may occur as in this case after moderate dose radiation. There is a correlation with meningioma. There is a possibility that large artery vasculopathy will be present in a proportion of patients irradiated for ALL. The long lag time between irradiation and the development of meningioma may mean that, as survivors of childhood ALL enter their third decade since cure, this tumour may be seen increasingly.
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7/8. Post-radiation primary intranodal leiomyosarcoma.

    An unusual case of post-radiation primary intranodal leiomyosarcoma of the cervical lymph node is presented. The patient was a 32-year-old white man who was treated in 1986 with hemiglossectomy and right neck dissection, followed by radiation therapy, for squamous cell carcinoma of the tongue. Six years later, he presented with an enlarged left cervical lymph node which on biopsy showed a high grade spindle cell malignant neoplasm. immunohistochemistry and electron microscopic study verified the smooth muscle origin of the tumour. Extensive work-up for a second primary lesion was negative. Primary intranodal leiomyosarcoma is extremely rare. We briefly discuss the histological differential diagnosis of spindle cell lesions of lymph nodes, leiomyosarcoma of the vessel wall and radiation-associated sarcoma.
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8/8. Metastatic angiosarcoma of the spleen after accidental radiation exposure: a case report.

    Angiosarcoma is a rare malignant tumor arising from endothelial cells of blood vessels or lymphatic channels. Therapeutic irradiation, thoriumdioxide administration, pyothorax, and polyvinyl chloride exposure have been shown to be predisposing factors for developing angiosarcoma. Accidental radiation exposure has not been associated with angiosarcoma. We present an unusual case of angiosarcoma of the spleen, with metastases to bone, liver, breast, and bone marrow, in a woman who lived near the Chernobyl nuclear facility in the former Soviet Union at the time of the reactor accident in 1986. To the best of our knowledge, this is the first report of metastatic angiosarcoma after accidental radiation exposure.
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