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1/34. Surgically created fourth-third cranial nerve communication: temporary success in a child with bilateral third nerve hamartomas. Case report.

    Shortly after birth, an otherwise healthy infant developed eye deviation and ptosis due to a hamartomatous lesion of the interpeduncular segment of the right oculomotor nerve. The left nerve became similarly involved when the child was 1.5 years of age. Direct nerve repair was not possible. Instead, the trochlear nerve was divided and its proximal end was attached to the distal end of the third nerve. Elevation of the upper eyelid and partial adduction of the eye developed gradually over the ensuing 3 to 5 months. Both functions were lost after an additional 2 months, presumably as a result of tumor recurrence or neuroma formation. This case report shows that surgically created fourth-third cranial nerve communication is feasible and may merit consideration under similar circumstances.
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2/34. p53 and p16INK4A mutations during the progression of glomus tumor.

    Glomus tumors are significantly rare tumors of carotid body. The great majority of these tumors are benign in character. Here we present two brothers with hereditary glomus jugulare tumor who had consanguineous parents. radiotherapy was applied approximately 8 and 10 years ago for treatment in both cases. Eight years later, one of these cases came to our notice due to relapse. The mutation pattern of p53, p57KIP2, p16INK4A and p15NK4B genes which have roles in the cell cycle, was analyzed in tumor samples obtained from the two affected cases in the initial phase and from one of these cases at relapse. The dna sample obtained from the case in initial diagnosis phase revealed no p53, p57KIP2, p16INK4A or p15INK4B mutation. He is still in remission phase. Despite the lack of p53, p57KIP2, p16INK4A and p15INK4B mutation at initial diagnosis the tumor dna of the other case in relapse revealed p53 codon 243 (ATG-->ATC; met-->ile) and p16 codon 97 (GAC-->AAC; asp-->asn) missense point mutations. No loss of heterozygosity in p53 and p16INK4A was observed by microsatellite analysis of tumoral tissues in these cases. P53 and p16INK4A mutations observed in relapse phase were in conserved regions of both genes. No previous reports have been published with these mutations in glomus tumor during progression. The mutation observed in this case may due to radiotherapy. In spite of this possibility, the missense point mutations in conserved region of p53 and p16INK4A genes may indicate the role of p53 and p16INK4A in tumor progression of glomus tumors.
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3/34. Surgical treatment for recurrent tumors of primary malignant melanoma of the esophagus: a case report and review of the literature.

    The purpose of this communication is to present a case of resection performed for local recurrent tumors of primary malignant melanoma of the esophagus (PMME) and to review the relevant literature. The patient was a 54 year-old man who had received an intraabdominal esophagectomy with a total gastrectomy for primary malignant melanoma of the abdominal esophagus in another hospital, in November 1995. After the initial operation, he was treated as an outpatient. In August 1997, computed tomography and ultrasonography revealed recurrent tumors in the dorsal pancreatic lymph node and in the right adrenal gland. The recurrent tumor of the dorsal pancreas directly invaded the dorsal pancreas parenchyma and occluded the superior mesenteric vein and splenic vein, and the other metastatic tumor in the right adrenal gland existed in the absence of circumference invasion. Metastases of the PMME were confirmed in the dorsal pancreas, the superior mesenteric vein, splenic vein, and right adrenal gland, and were removed by a total pancreatectomy on October 7, 1997. By immunohistochemical staining, we found that the focal areas expressed S-100 protein and HMB-45 antibody. Currently (February 1998), the patient is alive and disease-free. PMME is an extremely rare tumor with a poor prognosis for survival. Only 2 cases of removal of recurrent tumors, including the present case, have been reported. The treatment of choice is surgical resection, even in cases of recurrence, because radiotherapy and/or chemotherapy have not been proven to be beneficial; however, they may play a palliative role if surgery is not possible.
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4/34. Local tumor recurrence or emergence of a new primary lesion? A molecular analysis.

    The distinction between a new primary oral tumor and recurrence may bear significant prognostic implications. Currently, this differentiation relies mainly on tumor location: when both lesions are at or near the same site, the new one is regarded as a recurrence; when the two are at different sites, the second lesion is regarded as a new primary. Recent investigations using molecular analysis have demonstrated that some oral squamous cell carcinomas (SCC) arising from different sites show the same clonogenical changes. In this case report, we studied the clonality of three SCC (one primary, two apparent recurrences) from the right lateral tongue of a young, non-smoking woman by using microsatellite analysis for loss of heterozygosity. The results showed that while the first two tumors were clonogenically similar, the third tumor was clonogenically different and was consistent with the development of a new primary. This result indicates that location of tumors alone is not always reliable in determining whether a new tumor is a recurrence or a new primary lesion.
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5/34. A long-term survivor undergoing extensive microwave coagulation for unresectable hepatocellular carcinoma.

    Surgery for advanced hepatocellular carcinomas (HCCs) has not been standardized. We report on a long-term tumor-free survivor who underwent extensive microwave coagulation therapy (MCT) for multiple bilobar HCCs. A 61 year-old woman was diagnosed to have bilobar HCCs, including a large tumor, 9 cm in diameter, and 4 small satellite nodules, associated with chronic hepatitis b. The patient had received repeated chemoembolizations using iodized oil, but the increased alpha-fetoprotein level did not fall to normal. The main tumor was unresectable because the tumor involved the caval vein and hepatic veins. The patient underwent extensive MCT with a total of 134 electrode insertions. The paracaval portion of the main tumor was meticulously coagulated under sonographic guidance to avoid vascular injury. The post-operative course was uneventful. Post-operative computed tomography (CT) showed complete necrosis of all tumors. The patient is alive without tumor recurrence for 4 years after MCT. This case proves that extensive MCT can provide a chance of cure in selected patients with multiple bilobar HCCs and centrally located HCCs near the caval vein.
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6/34. Sedation for intractable distress of a dying patient: acute palliative care and the principle of double effect.

    Shortly before his death in 1995, Kenneth B. Schwartz, a cancer patient at massachusetts General Hospital (MGH), founded the Kenneth B. Schwartz Center at MGH. The Schwartz Center is a nonprofit organization dedicated to supporting and advancing compassionate health care delivery, which provides hope to the patient, support to caregivers, and encourages the healing process. The Center sponsors the Schwartz Center Rounds, a monthly multidisciplinary forum where caregivers reflect on important psychosocial issues faced by patients, their families, and their caregivers, and gain insight and support from fellow staff members. The case presented is of a young man dying of recurrent epithelioid hemangioendothelioma, distressed with stridor and severe pain, whose poorly controlled symptoms were successfully treated with an infusion of propofol, titrated to provide effective comfort in the last few hours of the patient's life. The tenet of double effect, which allows aggressive treatment of suffering in spite of foreseeable but unintended consequences, is reviewed. The patient's parents were invited and contributed to the Rounds, providing compelling testimony to the power of the presence of clinicians at the time of death and the importance of open communication about difficult ethical issues.
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7/34. Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature.

    Malignant chondroid syringoma, or mixed tumor of the skin, salivary gland type, is an uncommon neoplasm believed to originate in sweat glands. This neoplasm occurs mostly in women and is typically seen in the extremities and torso. A case of recurrent malignant chondroid syringoma of the right foot in a man aged 34 years is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology, immunohistochemistry, and transmission electron microscopy. The malignant chondroid syringoma showed microscopic dermal satellite tumor nodules. Immunohistochemical staining was positive for keratin and S100 and negative for actin and p53. Ki-67 showed <10% positive staining. Ultrastructurally, the neoplasm was composed of epithelial cells with tonofilaments, cell junctions, and electron-dense amorphous keratin-like substance in the intercellular spaces. No evidence of myoepithelial differentiation was noted. Given the tumoral size, acral location, and histologic findings, the neoplasm was classified as a malignant chondroid syringoma. After reviewing the literature, it became apparent that wide surgical excision, adjuvant radiation therapy as well as patient education are critical in facilitating long-term survival.
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8/34. frontal sinus adenocarcinoma.

    CONTEXT: Paranasal sinus cancer is considered rare, with an incidence of less than 1 per 100,000 per year, with the frontal sinus being the primary site in only 0.3%. We report a case of adenocarcinoma arising in the frontal sinus. DESIGN: Case report. CASE REPORT: A 59-year-old woman, secretary, came in February 1998 with a 4-month history of low intensity frontal headache. She denied contact with wood dust. On examination a non-tender swelling was noted over her right forehead next to the medial aspect of the right orbit. CT scan showed a soft-tissue mass involving frontal sinus with intracranial invasion through the posterior wall. The anterior ethmoid sinus and the medial aspect of the right orbit were also involved. MRI demonstrated dural thickening in communication with the frontal mass. She underwent an en-bloc tumor resection by craniotomy including orbital clearance. histology revealed an adenocarcinoma. After surgery she had tumor recurrence, and chemotherapy and radiotherapy were started resulting in partial improvement.
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9/34. Bladder cancer genotype stability during clinical progression.

    genomic instability is manifested by the accumulation of large numbers of mutations. The rate at which mutations accumulate has been difficult to estimate because serial comparisons are required. For further insight into how quickly mutations accumulate during clinical progression, cell lines sequentially isolated 6 or 11 months apart from two patients with metastatic bladder cancer were compared for loss of heterozygosity (LOH). The genomes were scanned at approximately 200 polymorphic microsatellite loci to increase the resolution and sensitivity for losses. The cell lines from both patients had evidence of genomic instability, with aneuploidy, chromosomal instability, and fractional allelic losses of 0.15 and 0. 48, respectively. However, additional changes were relatively infrequent, with more than 90% identity between the initial and recurrent cell lines. Allelic losses were not randomly scattered, but clustered on specific chromosomes. Therefore, the numbers of loci with further LOH during the clinical progression of some bladder cancers are small relative to the total number of loci with LOH, suggesting that most allelic losses accumulate before clinical presentation.
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10/34. A novel type of metastatically spreading subcutaneous aspergillosis without epidermal lesions following allogeneic stem cell transplantation.

    Systemic mycosis is among the most feared opportunistic infections in the immunocompromised host. Difficulty and delay in diagnosis and treatment often result in poor outcomes. In this communication a metastatically spreading form of subcutaneous aspergillosis developed in a patient with a history of allogeneic stem cell transplantation for relapsed Hodgkin's lymphoma. Strikingly, necrotizing cutaneous papules or ulcerating lesions were absent. diagnosis was accomplished after excision of a clinically non-suggestive subcutaneous nodule. Despite prompt initiation of antimycotic therapy the outcome was fatal; dosage of conventional and liposomal amphotericin b was limited due to treatment-related toxicities. This case report describes a novel form of aspergillosis and underlines the need for an aggressive diagnostic approach in severely immunocompromised patients.
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