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1/606. 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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2/606. multiple myeloma on polycythemia vera following radioactive phosphorus therapy.

    A 74-year-old white man with established polycythemia vera was treated with radioactive phosphorus after phlebotomies alone failed to control his disease. About 2 3/4 years later he died of multiple myeloma. The mutagenic effect of radioactive phosphorus may have caused or possibly accelerated preexisting myeloma. Basic nonmalignant disease deserves careful consideration before radiation or radiomimetic agents are used. One might consider a probably less mutagenic drug such as hydroxyurea in patients with polycythemia vera when phlebotomy alone does not give good control of red cell mass and thrombocytosis.
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3/606. 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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4/606. Increased risk of lymphoma in sicca syndrome.

    The risk of cancer was ascertained in 136 women with sicca syndrome followed at the National Institutes of health (NIH). Seven patients developed non-Hodgkin's lymphoma from 6 months to 13 years after their first admission to NIH. This was 43.8 times (P less than 0.01) the incidence expected from the rates of cancer prevailing among women of the same age range in the general population during this time. In addition, three cases of Waldenstrom's macroglobulinemia occurred in this study group. Eight patients developed cancers other than lymphoma, similar to the number expected based on the rates prevailing in the general population. patients with a history of parotid enlargement, splenomegaly, and lymphadenopahy had an increased risk of lymphoma. These clinical conditions did not appear to be early manifestations of undiagnosed lymphoma but rather seemed to identify a subgroup of patients with sicca syndrome with marked lymphoid reactivity, who had a particularly high risk of subsequently developing lymphoma.
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5/606. radiation-induced esophageal carcinoma 30 years after mediastinal irradiation: case report and review of the literature.

    A 54-year-old man who had been irradiated in 1964 for cervical involvement by Hodgkin's disease was admitted in December 1994 to our clinic with strong complaints of dysphagia. The reason was a moderately differentiated squamous cell carcinoma of the proximal esophagus in the previously irradiated region. The patient had no risk factors (abuse of nicotine or alcohol) for the developement of esophageal carcinoma. A reirradiation was performed, but the disease progressed locally and two weeks after the beginning of the therapy the patient developed two tracheoesophagocutaneous fistulae. The radiation therapy was discontinued and the tumor stenosis was bridged by a tube closing the fistulae. A retrospective dose analysis to evaluate the applied doses will be performed. Furthermore, an overview of 66 cases of the literature with radiation-induced esophageal carcinoma analysed concerning applied dose and latent interval will be given. In conclusion the reported case fits the criteria for radiation-induced malignancies (Chudecki Br J Radiol 1972;45:303-4) known from literature: (1) a history of previous irradiation, (2) a cancer occurring within the irradiated area, (3) gross tissue damage due to an excessive dose of radiation, and (4) a long latent interval between irradiation and development of cancer. Esophageal carcinomas belong to the rare secondary malignancies after the therapeutic use of ionizing radiation. Nevertheless in patients with dysphagia they should be suspected as a differential diagnosis even many years after mediastinal irradiation. The treatment of these tumors is very difficult and is associated with a poor prognosis.
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6/606. Microcystic adnexal carcinoma: collaborative series review and update.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a malignant appendageal tumor first described in 1982. It can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, leading to inadequate initial treatment. This neoplasm is locally aggressive and deeply infiltrating, characterized by high morbidity and frequent recurrence. Mohs micrographic surgery has been used to conserve tissue and improve the likelihood for cure. OBJECTIVE: We report our experience using Mohs micrographic surgery for the treatment of MAC and compare with earlier reports in the literature. In addition, we review the epidemiology, clinical and histologic characteristics, and optimal treatment of this rare neoplasm. We also describe a 15-year-old white male patient with MAC on the scalp occurring only 7 years after radiation exposure. methods: The medical records of 11 patients with MAC who were treated by Mohs micrographic surgery were reviewed at both departments, and follow-up data were obtained. RESULTS: In all patients treated with Mohs micrographic surgery, there were no recurrences after a mean follow-up of 5 years. CONCLUSION: Mohs technique enables the detection of clinically unrecognizable tumor spread and perineural invasion often encountered with MAC. Aggressive initial treatment by microscopically controlled excision appears to offer the greatest likelihood of cure for this neoplasm, while providing conservation of normal tissue. In addition, we describe the second youngest patient with MAC and readdress the issue of previous radiotherapy as an important predisposing factor.
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7/606. 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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8/606. radiation-associated rectal cancer: report of four cases.

    BACKGROUND/AIMS: radiation-associated rectal cancer is a remarkable clinical entity. We demonstrate 4 patients (mean age 68 years, range 63-74) who had undergone pelvic radiotherapy for cervical cancer. We indicate some characteristics of radiation-associated rectal cancer. RESULTS: Two patients had received intracavitary and external pelvic radiotherapy, while the remaining 2 had external pelvic radiotherapy following hysterectomy. The mean total radiation dose was 63 Gy, though radiation dose information was not available for 1 patient. Colorectal cancer developed at a mean time of 20.7 years (range 11-30) after radiation therapy. All patients presented with chronic radiation colitis, and 3 demonstrated abnormal tumor markers. colonoscopy revealed an ulcerative, localized well-differentiated adenocarcinoma of the rectosigmoid colon in 1 patient, and diffusely infiltrating cancers of the lower rectum, one signet-ring cell carcinoma and two mucinous carcinomas in the remaining 3. One case was stage I, 2 were stage IIIa, and the remaining case was stage IV. Three patients underwent abdominoperineal resection. The remaining patient was felt to be inoperable. The colorectal wall demonstrated the changes of chronic radiation injury. Two patients died within a short time because of their advanced cancers. CONCLUSION: radiation-associated rectal cancer has a tendency to be diagnosed in the advanced stage and to have a poor prognosis. A literature review and our case report suggest that since there are no reliable clinical or laboratory indicators of the presence of a curable colorectal cancer in the setting of chronic radiation proctocolitis, surveillance with a colonoscope should be done 10 years after irradiation in patients with previous pelvic radiotherapy.
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9/606. Invasive cutaneous squamous cell carcinoma associated with actinic keratosis: a case with orbital invasion and meningeal infiltration.

    BACKGROUND: It is suggested that most squamous cell carcinomas in sun-exposed areas arise from preexisting solar keratosis. Actinic keratosis is thought of as being a precursor to squamous cell carcinoma. This form of squamous cell carcinoma has been considered to be a relatively benign lesion. We report a case of invasive squamous cell carcinoma associated with actinic keratosis leading to orbit destruction and meningeal infiltration. OBJECTIVE: To demonstrate that well-differentiated tumors can act extremely aggressively with the potential toward infiltrative growth patterns. methods: Histologically controlled surgery along with multiple radiation therapy was performed. RESULTS: The tumor progressed inducing perineural invasion, orbit infiltration, osseous destruction, and meningeal invasion. CONCLUSION: The association of squamous cell carcinoma and actinic keratosis supports the concept of a causal relation. Excision with histologic examination of actinic keratosis seems to be useful for accurate diagnosis. Squamous cell carcinoma can represent an aggressive tumor with infiltrative growth pattern and should not be considered a benign lesion.
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10/606. 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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