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1/108. Mohs' surgery as an approach to treatment of multiple skin cancer in rhinophyma.

    BACKGROUND: skin cancer arising within a rhinophyma is rare, less than would be expected from the coexisting chronic active inflammatory process. In rhinophyma, multiple coexisting tumours of different histologic types present an unusual challenge and have never been described in the literature. OBJECTIVE: The treatment approach to multiple tumours occurring in rhinophyma, utilizing Mohs' surgery, is reported and discussed. PATIENT: The case of a 64-year-old farmer with basal cell carcinoma, squamous cell carcinoma, and basosquamous carcinoma occurring in the setting of longstanding rhinophyma is described. CONCLUSION: skin cancer, especially basal and squamous cell carcinoma, diagnosed simultaneously in a rhinophyma creates a challenge; the enlarged, inflamed, and hypertrophied tissue masks their margins. In our opinion, Mohs' micrographic surgery is the treatment of choice and should be primarily considered in view of the malignant potential of these tumours, as is shown by the substantial tumour extension in the case described.
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2/108. brain metastases from adenoendocrine carcinoma of the common bile duct: a case report.

    A 68-year-old man with metastatic brain tumors from adenoendocrine carcinoma of the common bile duct is reported. A common bile duct tumor and a metastatic liver tumor had been resected 6 years and 3 years prior to admission, respectively. Microscopically they showed two components; moderately differentiated tubular adenocarcinoma and neuroendocrine carcinoma. He presented with headache and vomiting and MRI revealed two metastatic brain tumors. They were successfully resected and radiotherapy was carried out. Histological diagnosis of the metastatic brain tumors was neuroendocrine carcinoma, but carbohydrate antigen (CA)-19-9 and carcinoembryonic antigen (CEA)-immunoreactive cells were observed without glandular pattern. Immunohistochemically serotonin and pancreatic polypeptide were detected, but somatostatin was not. As the endocrine cells demonstrated in the normal extrahepatic bile ducts are only somatostatin-containing D cells, these cells are considered to originate as part of a metaplastic process. To our knowledge, this represents the second case of adenoendocrine carcinoma of the common bile duct.
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3/108. 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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4/108. Concomitant bihemispheric cerebral ganglioglioma and hemangioma in an 18-month-old child: case report.

    This is a rare case of concomitant bihemispheric cerebral ganglioglioma and hemangioma in an 18-month-old child. There were two identical processes; a combination of ganglioglioma and hemangioma was located in the parieto-occipital region of both cerebral hemispheres. The child underwent diagnostic computed tomographic and magnetic resonance imaging examinations for seizures and right-sided facial palsy, which showed the bilateral presence of parieto-occipital, oval, well-demarcated, subcortically located processes. A biparieto-occipital craniotomy was carried out and two well-demarcated abnormalities were observed and both were removed in toto. Two years after the operation, the boy had normal psychomotor development and remained free from seizures with no therapy required. Repeat magnetic resonance imaging showed no tumor recurrence. Gangliogliomas are relatively uncommon tumors in childhood, located in the supratentorial compartment and usually associated with seizures. Histopathologic and immunohistochemical analysis of the two processes found in our patient showed them to be a ganglioglioma and a hemangioma, confirming the dysontogenetic origin of these alterations and the association between malformations and tumor growth. The combination of ganglioglioma and hemangioma, especially bilateral presentation of two such identical processes in a young child, is very rare and was not found in the recent literature.
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5/108. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.

    The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.
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6/108. Bone metaplasia in a case of bilateral renal cell carcinoma.

    We present a case of synchronous, bilateral renal cell carcinoma with osseous metaplasia. In renal cell carcinoma, bone can originate through two different processes: osseous differentiation or osseous metaplasia. The case we report here represents the second process. We discuss pathological differential diagnosis and prognosis of the infrequent tumor.
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7/108. Malignant fibrous histiocytoma of the ileum at a site of previous surgery: report of a case.

    Sarcomas rarely arise in the intestinum, and leiomyosarcoma represents the majority of cases. With only seven cases reported in the available English literature up to now, malignant fibrous histiocytoma of the small intestine is exceedingly rare. Moreover, follow-up data are almost completely unavailable. We present herein the unique case of a malignant fibrous histiocytoma arising in a postoperatively adherent intestinal loop. To the best of our knowledge, this is the first such case ever to be described. The clinical history and the intraoperative findings suggested that chronic postoperative repair processes might have been a promoting factor in the tumorigenesis of this neoplasm, on the analogy of malignant fibrous histiocytoma arising at different sites. The patient recovered well but 9.5 years after surgical removal, a solitary recurrent tumor developed in the urinary bladder and progressed rapidly, highlighting the need for long-term, possibly life-long, surveillance of patients with this rare type of intestinal cancer.
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8/108. Ovarian endometriosis and clear cell carcinoma, leiomyomatosis peritonealis disseminata, and endometrial adenocarcinoma: an unusual, pathogenetically related association.

    A 42 year-old female with a preoperative clinical diagnosis of ovarian cancer underwent laparotomy which revealed leiomyomatosis peritonealis disseminata (LPD) in the peritoneum and omentum and a left ovarian endometriotic cyst associated with a clear cell carcinoma. A grade 1, superfically invasive villoglandular endometrial endometrioid adenocarcinoma was also found. Microscopically, the endometriotic cyst wall contained an extensive peripheral band-like condensation of stromal cells. These cells were strongly positive for alpha inhibin and may have been the hormonal source responsible for the induction of the simultaneous LPD and endometrial adenocarcinoma. It is proposed that endometriosis is not only a precursor of clear cell carcinoma but, through secondary hormonal induction of the surrounding ovarian stroma, may also provide a hormonal stimulus for diverse proliferative processes.
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9/108. keratitis, ichthyosis and deafness syndrome with development of multiple hair follicle tumours.

    We report multiple occurrences of various kinds of tumours that originate from hair follicles in a patient diagnosed with KID (keratitis, ichthyosis, deafness) syndrome. These tumours are diagnosed as: (i) trichilemmal cysts in early lesions; (ii) proliferating trichilemmal tumours in moderate duration lesions; and (iii) malignant proliferating trichilemmal tumours in advanced lesions that are thought to progress from benign trichilemmal lesions. This three-step process raises the hypothesis of a multihit model that could account for the frequent development of tumours in KID patients.
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10/108. Co-existing cholesteatoma and vestibular schwannoma.

    A 69-year-old man presented with a cholesteatoma in the right mastoid process and a vestibular schwannoma at the left internal acoustic meatus. cholesteatoma co-existing with a vestibular schwannoma has not been documented previously in the contemporary literature. The clinical dilemma in the management of his progressive bilateral hearing loss is discussed. He presented with dizziness and bilateral hearing loss worse on the right side. pressure over the mastoid process elicited vertigo and nystagmus. He had no history of previous operation or infection in the ear canal. Audiograms confirmed high-tone hearing loss. Radiological investigations revealed a symptomatic cholesteatoma on the right side and an incidental vestibular schwannoma on the left. We have elected to manage both lesions conservatively. Bilateral cholesteatoma and bilateral vestibular schwannomas have been previously reported. Co-existing lesions, as in our patient have, however, not been reported previously. The management options of his hearing loss are discussed.
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