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1/59. Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas.

    Upper gastrointestinal polyps and extraintestinal tumors are well recognized in association with familial adenomatous polyposis (FAP). Although carcinoid tumors have been reported in association with sporadic colonic neoplasms and ulcerative colitis, to date, carcinoids have not been reported in association with FAP. We report a patient with FAP who has recurrent carcinoid tumors located at the bases of duodenal adenomas. The genetic basis of carcinoid neoplasms is still uncertain. This report may represent the clinical effect of the APC gene mutation on the enterochromaffin cell line manifesting as recurrent carcinoid tumors in physical association with intestinal adenomas. Future genetic analysis and epidemiological studies may be of value in determining whether a true association exists.
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2/59. Recurrent primary well-differentiated intrascrotal liposarcoma: case report and review of the literature.

    lipoma-like liposarcomas of the scrotal wall are very rarely reported neoplasms in the surgical and histopathological literature. We treated a well-differentiated liposarcoma of the inside wall of the scrotum in a 62-year-old man. Following local excision, the tumor recurred after three months, and a funiculoorchidectomy was performed. Today, 24 months following secondary surgery, the patient is completely asymptomatic and there is no evidence of tumor recurrence either on physical examination, ultrasonography or abdominal and pelvic computed tomography. In this paper we present the case and a review of the relevant literature.
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3/59. Clinical delivery of intensity modulated conformal radiotherapy for relapsed or second-primary head and neck cancer using a multileaf collimator with dynamic control.

    BACKGROUND AND PURPOSE: Concave dose distributions generated by intensity modulated radiotherapy (IMRT) were applied to re-irradiate three patients with pharyngeal cancer. patients, MATERIALS AND methods: Conventional radiotherapy for oropharyngeal (patients 1 and 3) or nasopharyngeal (patient 2) cancers was followed by relapsing or new tumors in the nasopharynx (patients 1 and 2) and hypopharynx (patient 3). Six non-opposed coplanar intensity modulated beams were generated by combining non-modulated beamparts with intensities (weights) obtained by minimizing a biophysical objective function. Beamparts were delivered by a dynamic MLC (Elekta Oncology Systems, Crawley, UK) forced in step and shoot mode. RESULTS AND CONCLUSIONS: Median PTV-doses (and ranges) for the three patients were 73 (65-78), 67 (59-72) and 63 (48-68) Gy. Maximum point doses to brain stem and spinal cord were, respectively, 67 Gy (60% of volume below 30 Gy) and 32 Gy (97% below 10 Gy) for patient 1; 60 Gy (69% below 30 Gy) and 34 Gy (92% below 10 Gy) for patient 2 and 21 Gy (96% below 10 Gy) at spinal cord for patient 3. Maximum point doses to the mandible were 69 Gy for patient 1 and 64 Gy for patient 2 with, respectively, 66 and 92% of the volume below 20 Gy. A treatment session, using the dynamic MLC, was finished within a 15-min time slot.
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4/59. Multiple organ dysfunction syndrome induced by whole-body hyperthermia and polychemotherapy in a patient with disseminated leiomyosarcoma of the uterus.

    OBJECTIVE: Whole-body hyperthermia (WBH) in combination with chemotherapy is a relatively new promising treatment modality for patients with cancer. The objective of this report is to present the development of an acute systemic inflammatory response syndrome (SIRS) with multiple organ dysfunction syndrome (MODS) following WBH in combination with chemotherapy. Although WBH can also induce cytokine production, MODS has not been described before in association with WBH. DESIGN: Case report. The patient was treated with WBH (core temperature 41.8 degrees C using a radiant heat device (Aquatherm) ) in combination with polychemotherapy (ifosfamide, carboplatin and etoposide (ice) ) in the context of a clinical trial for metastatic sarcomas. SETTING: Department of medical oncology and intensive care unit of a university hospital. PATIENT: A 58-year-old Caucasian woman treated for disseminated leiomyosarcoma of the uterus, who developed SIRS with brain dysfunction, hypotension, respiratory failure and renal dysfunction following WBH/ice. INTERVENTIONS: She was successfully treated in the intensive care unit by mechanical ventilation, inotropics and antibiotics. MEASUREMENTS AND RESULTS: There was a remarkable recovery within 2 days: she regained full conciousness, could be extubated, inotropic support was stopped and creatinine levels returned to pre-treatment levels. All cultures remained sterile. After almost complete recovery, 5 days later a second episode of fever during neutropenia occurred and, despite antibiotic treatment, she died of bacteroides distasonis sepsis. CONCLUSION: WBH should be added as a new cause to the already known list of physical-chemical insults which can result in MODS.
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5/59. Malignant melanoma presenting as an intrathymic tumor: a primary thymic melanoma?

    The identification of malignant melanoma in a visceral organ of nonepidermal origin is not an uncommon occurrence. Frequently, these cases are solitary metastases that present years after a thin epidermal melanoma has been diagnosed (and sometimes forgotten). However, primary visceral melanomas have been reported that have not been preceded by an epidermal lesion. We describe herein a unique case of melanoma presenting as a primary intrathymic tumor. The patient had no previous history of epidermal melanoma, and extensive workup did not reveal evidence for an alternative primary site. The tumor exhibited histologic features characteristic of melanoma, including an abundance of large pleomorphic cells with eosinophilic cytoplasm, prominent nucleoli, and S100 protein and ultrastructural analysis revealed stage II and stage III melanosomes. The patient remained free of disease until intrathoracic recurrence was detected on a computed tomographic scan 14 months later. The lack of clinical history and physical findings of melanoma at presentation, the intrathymic location of the tumor, and the pattern of recurrence suggest that this case likely represents a primary thymic melanoma, a previously unreported entity.
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6/59. Recurrent invasive adenocarcinoma after hysterectomy for cervical adenocarcinoma in situ.

    BACKGROUND: Unlike its squamous counterpart, therapy for cervical adenocarcinoma in situ with positive endocervical cone margin remains controversial. CASE: A 52-year-old gravida 2, para 1,0,1,1, presented with vaginal bleeding. Gynecologic history was significant for cervical cold knife conization with a positive endocervical margin and endocervical curettage with atypical endocervical cells. Repeat cone biopsy was considered unsafe given the large initial cone specimen. An extrafascial hysterectomy was performed 5 weeks later and pathology confirmed a disease-free cervix. Pap smear performed 1 year later was interpreted as recurrent adenocarcinoma but later downgraded to inflammation. Inspection and random biopsies of the vaginal cuff revealed only inflammation. Two subsequent Pap smears also returned inflammation. Seventeen months after the hysterectomy physical examination revealed a 2 x 3-cm smooth mass at the vaginal cuff. biopsy revealed invasive adenocarcinoma. The patient underwent an upper vaginectomy followed by postoperative pelvic radiation. CONCLUSION: This case suggests that despite extrafascial hysterectomy for presumed adenocarcinoma in situ of the cervix, a residual focus could remain and present later as invasive adenocarcinoma.
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keywords = physical examination, physical
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7/59. Rotary atlanto-axial subluxation with torticollis following central-venous catheter insertion.

    Atlanto-axial subluxation with torticollis is an uncommon condition that occurs in children usually as a result of pharyngeal infection, minor trauma, or neck surgery. Passive motion of the head and neck during general anesthesia is probably another etiologic factor. torticollis is the most common presenting physical finding. pain may or may not be present, but is commonly present with passive neck motion. Neurologic sequelae are uncommon. Our case illustrates this condition as a complication of central venous catheter (CVC) insertion in a child under general anesthesia. The surgeon should suspect this pathology when a child presents with torticollis following CVC placement. Precautions should be taken in the operating room to avoid aggressive rotation and extension of the child's neck while under general anesthesia whether or not cervical inflammation is present. Special attention to head and neck positioning should be taken in patients with Down's syndrome since they are at increased risk for atlanto-axial subluxation. The prognosis is excellent when diagnosed early. A delay in diagnosis can result in the need for surgical intervention.
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8/59. Radical surgery in a neonate with craniopharyngioma. report of a case.

    ultrasonography revealed a suprasellar tumor in a fetus at 28 weeks of gestation. The male newborn, delivered 10 weeks later, was operated at the age of 17 days, and a craniopharyngioma was completely removed. Intraoperatively, inappropriate secretion of antidiuretic hormone occurred and was followed by diabetes insipidus causing imbalance of fluid and electrolytes. The tumor recurred and was totally removed 1 year later. Further development was uneventful and, at the age of 8 years, the boy is in generally good mental and physical condition except for a left-sided hemiparesis. In contrast to the poor outcome of neonatal craniopharyngioma reviewed in the literature, this case may encourage radical surgery even in the very young.
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9/59. Extensive ossification in a craniopharyngioma.

    A rare extensive ossification occurred in a suprasellar craniopharyngioma of a man who died at 21 years of age. The tumor produced headache, retarded physical and mental development, visual disturbances, and increased intracranial pressure. The neoplasm recurred after surgical and roentgenological treatments. Differentiation of multipotential mesenchymal cells or mesenchymal type cells within the tumor has been suggested as the mode of occurrence of bone in this craniopharyngioma.
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10/59. Subependymal giant cell astrocytoma: clinical and neuroimaging features of four cases.

    The clinical history, neuroimaging features, treatments, and outcome of 4 patients with histologically verified subependymal giant cell astrocytomas (SEGA) were retrospectively reviewed. The average age at the time of surgery was 13.3 years. headache related to raised intracranial pressure was the first and only sign in 2 patients, with the remaining 2 being admitted because of sequential neuroimaging studies over several years revealing the growth of 'subependymal nodules' into intraventricular tumours. In each case the tumour was in the region of Monro's foramen and was associated with ventricular dilatation. On computed tomography (CT), multiple subependymal nodules were found in 3 patients, and these well circumscribed isodense SEGAs were markedly enhanced by contrast medium. On magnetic resonance imaging (MRI), which was obtained in 3 patients, 2 SEGAs were isointense with the cerebral cortex and one with the white matter on T1-weighted images, and on T2-weighted images, 2 were isointense with the cortex and one with the white matter. At surgery the tumours appeared to originate from the inferolateral wall of the lateral ventricle in the region of the head of the caudate nuclei. Total macroscopic removal was achieved in 3 patients, and subtotal removal in one patient. Follow up ranged from 4.6 to 13.2 years, and all patients have exhibited similar physical and mental conditions to preoperative. So far there has been no evidence of any recurrences. The diagnosis and the surgical indications for SEGA are discussed, with periodic monitoring with neuroimaging studies being recommended even for asymptomatic patients with 'subependymal nodules'.
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