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1/113. Surgical management of pediatric tumor-associated epilepsy.

    brain tumors are a common cause of seizures in children. Early surgical treatment can improve seizure outcome, but controversy exists regarding the most appropriate type of surgical intervention. Some studies suggest tumor resection alone is sufficient, while others recommend mapping and resection of the surrounding epileptogenic foci to optimize seizure outcome. To address this issue, we reviewed the charts of 34 pediatric patients aged 18 months to 20 years with medically intractable epilepsy and primary brain tumors. The average age at operation was 12.6 years, and patients had seizures for an average of 6.4 years. The majority of tumors were located in the temporal lobe. Seventeen patients, because of tumor location near an eloquent area, underwent extraoperative mapping using subdural electrode grids prior to definitive tumor resection. Fourteen of these patients had a gross total tumor resection, yet only two had a distinct zone of ictal onset identified and resected. The remaining 17 patients had tumors either in the nondominant hemisphere or far removed from speech-sensitive areas, and therefore did not undergo extraoperative subdural electroencephalograph mapping. Fourteen of these patients also had a gross total tumor resection, while none had intraoperative electrocorticography to guide the resection of additional nontumoral tissue. overall, of the 28 patients treated with a gross total tumor resection, 24 (86%) are seizure free, while the other four are significantly improved. Of the six patients who had a subtotal tumor removal, five have persistent seizures. The mean follow-up was 3.6 years. We conclude that in children and adolescents, completeness of tumor resection is the most important factor in determining seizure outcome. The routine mapping and resection of epileptogenic foci might not be necessary in the majority of patients. As a corollary, the use of subdural electrode grids in pediatric patients with tumor-associated epilepsy should be limited to cases requiring extraoperative cortical stimulation for localization of nearby eloquent cortex.
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2/113. First cytogenetic study of a recurrent familial chordoma of the clivus.

    Two recurrences of a familial clivus chordoma, arisen from a patient who developed the primary tumor at age of 8 years, were investigated by cytogenetic and the fluorescence in situ hybridization (FISH) approach. Of the patient's 3 daughters, 2 developed, respectively, a clivus chordoma and an astrocytoma in infancy, a familial aggregation highly suggestive of a genetic background. After a 31-year hiatus, 2 tumor recurrences, developed over 17 months, were removed surgically. Both were hypo- or nearly diploid, and had a pronounced karyotypic heterogeneity with clonal and non-clonal rearrangements affecting several chromosomes. The same rearrangement, a dic(1;9)(p36.1;p21), was shared in both tumor specimens and, in 90% of the cells, chromosome 1p appeared to be involved in unbalanced translocations with different chromosomes, leading to variable losses of 1p. Previous cytogenetic data concerning chordoma are limited to 10 sporadic tumors with an abnormal karyotype; although no tumor-specific rearrangements have been identified, chromosome 1p appears to be involved frequently.
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3/113. Recurrent hepatocellular carcinoma successfully treated with radiofrequency thermal ablation.

    We report a patient with hepatocellular carcinoma (HCC) who was successfully treated with radiofrequency thermal ablation (RFA). A 71-year-old man was admitted to our hospital in August 1996 with recurrence of HCC. Partial hepatic resection had been performed in January 1993 for HCC that had measured 1.3 cm in segment VIII, and subsequently he had received six sessions of percutaneous ethanol injection (PEI) for treatment of recurrence. Dynamic computed tomography (CT) performed in August 1996 showed two recurrent tumors, one measuring 3.8 cm in segment VIII adjacent to the right hepatic vein, and one measuring 2.0 cm in segment V. Three sessions of percutaneous RFA were performed. After this treatment, most of the tumor in segment VIII and all the tumor in segment V showed low density on dynamic CT, and the right hepatic vein was preserved. However, a remnant of the mass appeared near the right hepatic vein 2 months after the treatment. An additional two sessions of RFA were performed. After the end of treatment, serum alpha-fetoprotein level dropped to the normal range, and no sign of recurrence has been observed until September 1998.
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4/113. Recurrent intracranial Masson's vegetant intravascular hemangioendothelioma. Case report and review of the literature.

    In the central nervous system, recurrence of intracranial Masson's vegetant intravascular hemangioendothelioma (MVIH) is rare. To the authors' knowledge, only three recurrent intracranial cases have been reported. The authors report the case of a 75-year-old woman with a recurrent left-sided cerebellopontine angle and middle cranial fossa MVIH. When the patient was 62 years of age, she underwent preoperative embolization and subtotal resection of the intracranial lesion followed by postoperative radiotherapy. She was well and free from disease until 9 years postoperatively when she became symptomatic. At 71 years of age, the patient again underwent preoperative embolization and near-gross-total resection of the lesion. Follow-up imaging performed 15 months later revealed tumor recurrence, and she underwent stereotactic gamma knife radiosurgery. At a 2.75-year follow-up review, the patient's imaging studies revealed stable residual tumor. This case report is unique in that it documents the clinical and pathological features, surgical and postoperative treatment, and long-term follow-up review of a patient with recurrent intracranial MVIH and suggests that this unusual vascular lesion is a slow-growing benign tumor rather than a reactive process. Because the pathological composition of the lesion may resemble an angiosarcoma, understanding this benign vascular neoplasm is crucial so that an erroneous diagnosis of malignancy is not made and unnecessary adjuvant therapy is not given.
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5/113. 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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6/113. Oral cimetidine (Tagamet) for recalcitrant, diffuse conjunctival papillomatosis.

    PURPOSE: To report the efficacy of cimetidine for treatment of conjunctival papillomatosis. methods: An 11-year-old boy had an 8-year history of diffuse conjunctival papillomas, treated previously with standard measures of excisional biopsy and cryotherapy. He developed spontaneous conjunctival bleeding and diffuse tumor recurrence over the entire conjunctival surface. Oral cimetidine liquid (30 mg/kg/day) was prescribed. RESULTS: Within 2 months, dramatic tumor regression was noted, with nearly complete resolution by 4 months. The patient had no local or systemic side effects from the medication. CONCLUSIONS: Oral cimetidine possesses immune modulation properties and may be useful in some cases of virus-induced conjunctival papillomatosis.
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7/113. Cystic hygroma of the skull base.

    Cystic hygroma is a benign developmental tumor formed by lymph vessels that occurs in 1 of 12000 individuals and presents most frequently in the head and neck. It is often difficult to manage because the tumor frequently envelopes nearby vital structures. Lesions that extend significantly above the hyoid are especially difficult to excise because of the complexity of achieving a complete excision. This article describes a series of 3 cases of cystic hygroma that presented in the head and neck and, on further investigation, were found to also involve the skull base. This anatomic location is exceedingly rare, and these 3 cases represent new and difficult challenges not previously discussed in the literature, to our knowledge.
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8/113. Stereotactic radiosurgery. IX. craniopharyngioma: durable complete imaging responses and indications for treatment.

    The previous literature on radiosurgical treatment for craniopharyngioma is sparse, because the involvement of the radiosensitive optic chiasm within the target volume (in most cases) precludes safe high single dosage. The relocatable frame, introduced at St Bartholomew's Hospital in 1989, utilizes the dosimetric advantages of stereotactic isodosimetry to treat with the safer fractionated therapy; this may well be the most advantageous methodology for larger and critically situated tumours around the chiasm. We believe that radical high dose radiosurgery (either single dose or fractioned) has a role in the treatment of selected craniopharyngiomas and our first six patients treated are presented here. There were two patients with discrete, small, solid lesions and both achieved complete responses that maintain at 1-2 years. The other four patients are more difficult to assess: one patient was treated for disease within the clivus and there is no evidence of disease progression at 18 months; one child, who was treated by fractionated radiosurgery to a solid component of a complex partly solid, partly cystic craniopharyngioma enveloping the chiasm, suffered tumour progression and died; one patient died of a hemisphere cerebovascular accident, which we believe was unrelated to the therapy, and one patient suffered malignant change 1 year after radiosurgery (a time point that we consider too early to ascribe to the radiation from this therapy). We discuss the indications for this technology in the multi-disciplinary therapy of this complex disease. It seems clear that, for low lying lesions, well below the optic apparatus, radiosurgery has an important role (possibly a primary radical radiotherapeutic role for small bulk solid masses). Where there is an inoperable solid tumour enveloping the chiasm the dosimetric advantages of the x-knife (10% internal dose gradient versus the 100% internal dose gradient of the gamma knife) plus the fractionation facility using the relocatable frame, argue for the x-knife (linear accelerator) as being the optimal radiosurgery system. These arguments also apply to acoustic neuroma therapy and preservation of hearing, the other clinical situation where a radiosensitive special sensory nerve lies within the target volume.
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9/113. 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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10/113. Video-assisted transaortic resection of left ventricular sarcoma using the ultrasonic surgical aspirator.

    We present a case of recurrent metastatic synovial sarcoma of the left ventricle. The first resection of the tumor was performed through a left ventriculotomy. This time, the tumor was resected through the aortic valve with a video-assisted technique. The ultrasonic surgical aspirator was useful to remove the tumor near the papillary muscle and the chordae.
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