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1/18. 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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2/18. 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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3/18. Fine-needle aspiration biopsy of postradiation epithelioid angiosarcoma of breast.

    Angiosarcoma of breast skin and parenchyma is a rarely reported complication of irradiation for breast carcinoma. We report a case of a subareolar epithelioid angiosarcoma arising 8 years subsequent to lumpectomy and irradiation of the ipsilateral breast for infiltrating carcinoma. The epithelioid appearance of the neoplastic cells on fine-needle aspiration biopsy (FNA) biopsy suggested a recurrence of the primary carcinoma. Careful attention to certain cytomorphologic features and cell block immunohistochemistry were useful in the distinction from recurrent carcinoma. Cytologic features that identified this neoplasm as an angiosarcoma included marked cell discohesiveness, elongate cytoplasmic processes or "pseudopodia," heterogeneous cell size, large nucleoli or macronucleoli, and cytoplasmic lumina. Immunohistochemical markers, including factor viii antigen, CD31, and CD34, were positive, confirming the vascular nature of the neoplasm. Other markers ruled out morphologically similar neoplasms such as recurrent carcinoma and melanoma. Epithelioid angiosarcoma should be included in the differential diagnosis of a suspected recurrence of breast carcinoma several years postirradiation therapy. Diagn. Cytopathol. 2000;22:172-175.
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4/18. Spindle cell angiosarcoma following irradiation therapy for cervical carcinoma.

    BACKGROUND: Angiosarcomas arise in the scalp and face in the elderly, in association with chronic lymphedema (Stewart-Treves syndrome), and in irradiated areas. Rarely in these settings, angiosarcomas exhibit pure spindle cell phenotype. methods: Herein, the clinicopathologic features of a 72-year-old-woman with spindle cell angiosarcoma are described. RESULTS: A 72-year-old woman presented with numerous nodules and diffuse induration from the lower abdomen to the right buttock, corresponding to the area exposed to 60Co-irradiation during treatment for cervical carcinoma 10 years earlier. Histopathological examination revealed inflitrative, atypical, spindle cells that labeled with antibodies to CD31, CD34, and factor viii-related antigen. Ultrastructurally, these malignant spindle cells contained weibel-palade bodies. No features suggesting radiation dermatitis (sclerosis or bizarre, large fibroblasts) were identified, but lymphangiectases and widely spaced collagen bundles(lymphedema) were prominent in the skin surrounding the angiosarcoma. Computed tomographic scan of the abdomen highlighted this histologic finding by demonstrating tumor masses limited to areas of lymphedema. Treatment with intravenous and local injections of recombinant interleukin 2 (rIL 2) followed by electron beam irradiation were initially effective with tumor remission for 2 months. However, the recurrent tumor did not respond to a second course of one-shot injection of rIL 2 through the abdominal aorta and the patient succumbed to her angiosarcoma 19 months after diagnosis. CONCLUSIONS: radiation-induced lymphedema may be a factor in angiosarcoma associated with radiotherapy.
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5/18. radiation-associated synovial sarcoma: clinicopathologic and molecular analysis of two cases.

    Development of a soft-tissue sarcoma is an infrequent but well-known long-term complication of radiotherapy. Malignant fibrous histiocytomas, extraskeletal osteosarcomas, fibrosarcomas, malignant peripheral nerve sheath tumors, and angiosarcomas are most frequently encountered. radiation-associated synovial sarcomas are exceptional. We report the clinicopathologic, immunohistochemical, and molecular features of two radiation-associated synovial sarcomas. One tumor developed in a 42-year-old female 17 years after external irradiation was given for breast carcinoma; the other occurred in a 34-year-old female who was irradiated at the age of 7 years for a nonneoplastic condition of the left hand. Both lesions showed morphologic features of monophasic spindle cell synovial sarcoma, were immunoreactive for cytokeratins, epithelial membrane antigen, CD99, CD117 (c-kit), and bcl-2 and bore the t(X;18) (SYT-SSX1) translocation. We conclude that synovial sarcoma has to be added to the list of radiation-associated soft-tissue sarcomas.
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6/18. radiation-induced laryngeal angiosarcoma after cervical tuberculosis and squamous cell carcinoma: case report and review of the literature.

    Primary laryngeal angiosarcoma (LA) is quite rare with only 13 cases reported in English literature to date. A case of LA after radiation therapy for tuberculosis and squamous cell carcinoma is reported. A 70-year-old woman had a history of radiation therapy for left cervical tuberculosis at the age of 28. At 60 years of age a squamous cell carcinoma of the larynx was found and chemotherapy and radiotherapy, consisting of a total dose of 68.4 Gy, were administered. At the age of 68, recurrent squamous cell carcinoma was suspected from several biopsies, and a total laryngectomy with right thyroidectomy was performed. The tumor cells formed vascular spaces and expressed some endothelial markers, such as CD34, CD31, and ulex europaeus agglutinin I, but no epithelial markers, such as cytokeratins or epithelial membrane antigen. No residual squamous cell carcinoma was found. In the present case, it was suspected that irradiation to the larynx for cervical tuberculosis and squamous cell carcinoma induced angiosarcoma. The patient was still alive despite multiple skin and soft tissue metastasis 3 years and 6 months after the radical operation. Distinction of postirradiation angiosarcoma from pseudoangiosarcomatous carcinoma seems difficult but is important because irradiation is not effective and an initial radical surgery is the only effective treatment. Although irradiation is a common treatment for laryngeal squamous cell carcinoma, this is only the second case of radiation-induced LA in English literature.
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7/18. Two secondary malignancies after radiotherapy for seminoma: case report and review of the literature.

    We report a case of a 50-year-old man with two synchronous second malignancies 25 years after orchiectomy and adjuvant radiotherapy for seminoma. An annual health examination revealed an elevated prostate-specific antigen level. A biopsy was performed revealing Gleason score 9 adenocarcinoma of the prostate. Computed tomography of the abdomen revealed a 2-cm solid mass in the right kidney consistent with renal cell carcinoma. Both of these lesions were within the nonstandard radiation field for seminoma with which this patient was treated. Second malignancies, including prostate cancer, are a very uncommon occurrence but an important consideration in long-term survivors of seminoma treated with radiotherapy.
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8/18. Postirradiation osteosarcoma of the mandible with heterologous differentiation.

    Heterologous differentiation in osteosarcoma is rare, with only 17 cases previously described in the literature. We report a case of a mandibular osteoblastic osteosarcoma with rhabdomyosarcomatous differentiation in a 45-year-old man who had a history of Hodgkin lymphoma that was treated with chemotherapy and radiation. Radiographs showed a destructive osteoblastic tumor of the mandible that was proven by biopsy to be osteosarcoma. After the patient underwent neoadjuvant chemotherapy, the tumor was resected. It contained a high-grade osteosarcoma composed of osteoblastic and chondroblastic elements that had no definitive response to therapy. Within the center of the lesion was a discrete focus of pleomorphic cells with rhabdomyosarcomatous differentiation that was confirmed by immunohistochemical stains for desmin, myogenin, and myogenic differentiation antigen 1. The patient received additional chemotherapy and radiation therapy but developed lung, brain, and spinal metastases and died 7 months after surgery. To our knowledge, this is the first report of osteosarcoma of the mandible with heterologous differentiation.
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9/18. Postradiation angiosarcoma of the small intestine: a case report and review of literature.

    Postradiation angiosarcoma is typically a high-grade sarcoma that presents mainly in the skin and superficial tissues. Postradiation angiosarcoma arising in the small intestine is rare with only 11 cases documented in the English-language literature. Herein, we report a postradiation angiosarcoma of the small intestine 9 years after radiotherapy for uterine cervical adenocarcinoma. The patient presented with symptoms of intestinal obstruction. At exploratory laparotomy, tumor nodules involved the small bowel. Microscopically, the neoplasm was composed of spindled and epithelioid cells arranged in solid aggregates and focally forming vascular channels. The diagnosis of angiosarcoma was confirmed immunohistochemically by tumor cell expression of CD31, CD34, and factor viii-related antigen. The patient died 10 months after laparotomy. The diagnosis of PRA should be entertained for any poorly differentiated neoplasm arising in a previously irradiated site. The correct diagnosis of PRA depends upon histomorphologic identification of vascular differentiation, coupled with immunohistochemical expression of endothelial-related markers.
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10/18. Post-irradiation leiomyosarcoma. Case report with immunohistochemical studies.

    We report here one case of post-irradiation leiomyosarcoma. The diagnosis of this was confirmed using anti-intermediate filament antibodies: tumor cells were positive for anti-vimentin and anti-desmin but negative for anti-prekeratin and anti-epithelial membrane antigen. The positive staining with anti-desmin clearly indicates a muscular origin although the tumor cells were not stained by two anti-actin antibodies, one directed against alpha-smooth muscle actin and the other against alpha-striated muscle actin. Irradiation was motivated by a wrong diagnosis of breast carcinoma when the patient was 13 year-old. The laps of time separating irradiation and the occurrence of the leiomyosarcoma was 13 years. The total dose was 6,000 rads. This is the ninth case of post-irradiation leiomyosarcomas reported in the literature.
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