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1/782. 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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2/782. The effectiveness of chemotherapy with cisplatin and 5-fluorouracil for recurrent small cell neuroendocrine carcinoma of the rectum: report of a case.

    We report herein the case of a 46-year-old-man with small cell neuroendocrine carcinoma (NEC) concomitant with large villous adenoma of the rectum, who underwent abdominoperineal resection with regional lymphnode dissection. The resected specimen was histologically found to contain a small lesion of NEC confined to the submucosa in the large adenoma. A computed tomography scan done 4 months postoperatively revealed recurrences in the liver, lymph nodes, and bone. Therefore, two cycles of sequential intravenous combined chemotherapy with standard doses of cisplatin and 5-fluorouracil (5-FU) were administered, after which the size of each tumor decreased remarkably. Nevertheless, the patient died 8 months after the operation. As there was a fair response of this tumor to the combined chemotherapy of cisplatin and 5-FU, this regimen against NEC of the colon and rectum should be given consideration.
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3/782. Ovarian endometrioid-like yolk sac tumor treated by surgery alone, with recurrence at 12 years.

    We describe the case of a stage Ia endometrioid-like yolk sac tumor (YST) of the ovary, which was originally misdiagnosed as a malignant struma ovarii and not treated with adjuvant chemotherapy. After 12 years, a contralateral dermoid cyst was excised along with a small omental nodule of partially necrotic and calcified endometrioid-like YST. No tumor was detected in several other biopsy specimens, and a peritoneal lavage was negative for tumor cells. Since there was no evidence of remaining tumor and the serum alpha-fetoprotein (AFP) level was normal after the second operation, the patient was followed. Serial serum AFP levels remained normal for 4 months. At a second-look laparotomy after 4 months, a small tumor nodule was removed from the cul-de-sac. Postoperatively, the patient received three cycles of BEP chemotherapy. The long disease-free interval after the first operation in spite of the presence of occult spread to the omentum and to the pouch of Douglas in this case indicates that some endometrioid-like YSTs may have an indolent course. The present case underscores the importance of careful surgical staging and of long-term follow-up in cases of primitive germ cell tumors of the ovary.
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4/782. anesthesia for cesarean section in two patients with brain tumours.

    PURPOSE: To describe two patients with brain tumours where general anesthesia was used for cesarean sections under emergency and urgent conditions. CLINICAL FEATURES (CASE #1): The first patient presented at 38 wk gestation with an acute intracranial tumour herniation, requiring emergency craniotomy and simultaneous cesarean section. General anesthesia was induced with thiopental and vecuronium, maintained with enflurane 1% in O2 100%. Maternal P(ET)CO2 was maintained at 25 mmHg. After delivering a healthy infant, she was given syntocinon, mannitol and dexamethasone i.v. anesthesia was maintained with fentanyl, nitrous oxide 50% in O2 and isoflurane 1% during frontal-lobe tumour resection. CLINICAL FEATURES (CASE #2): The second patient presented at 37 wk gestation for urgent cesarean section because of placental insufficiency. She had had a brain tumour resection four years earlier. An increase in intracranial pressure necessitated craniotomy for decompression at 20 wk gestation. She was further treated with dexamethasone, carbamazepine and radiation for control of cerebral oedema at 34 wk. cesarean section was performed under general anesthesia; rapid-sequence-induction with thiopental and succinylcholine, followed by isoflurane 1% in O2 100%. Syntocinon, fentanyl and atracurium i.v. were administered after delivery of a healthy infant. Although neurosurgeons stood by, their intervention was unnecessary. CONCLUSION: General anesthesia remains safe and dependable for operative delivery in parturients with intracranial tumour. Tracheal intubation allows maternal hyperventilation thereby controlling raised intracranial pressure. Hemodynamic stability is readily achieved to maintain cerebral perfusion. However, a multidisciplinary-team approach is critical for successful patient management.
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5/782. Diagnostic utility of Mullerian inhibiting substance determination in patients with primary and recurrent granulosa cell tumors.

    OBJECTIVES: In this study we evaluated changes in serum Mullerian inhibiting substance (MIS) concentration in a large number of patients with granulosa cell tumors (GCT) to determine whether MIS is elevated at the time of presentation and whether MIS is an index of successful surgical resection and management of recurrences. methods: We retrospectively reviewed MIS levels from 17 subjects prior to tumor resection and studied serial MIS samples from 56 subjects following initial tumor resection. Clinical follow-up information was available for 36 of those with postoperative MIS values. serum MIS was measured by an ELISA. MIS values were compared to a combination of normative values previously established in our laboratory and from more recently obtained samples from older pre- and postmenopausal women, using this assay. RESULTS: serum MIS was elevated pre-operatively in 6 of 8 (75%) subjects with juvenile GCTs and in 7 of 9 (78%) of those with adult GCTs relative to age-matched controls (76% for both types combined). Post-operative clinical correlation was available for 36 patients. There was no clinical recurrence in 21 subjects with normal or undetectable postoperative values, and incompletely resectable tumor or recurrence was identified in 6 of 15 patients with elevated postoperative values. CONCLSUIONS: The results of this study demonstrate that postoperative serum MIS concentrations may be used to evaluate the completeness of tumor removal following initial surgery and that serial MIS determinations may allow the detection of recurrences.
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6/782. Laser ablation of recurrent Paget's disease of vulva and perineum.

    We describe the management by CO 2 laser of a case of recurrent extramammary Paget's disease of vulva and perineum, previously managed on three occasions with conventional surgical excision. Ablation of the whole lesion took less than 30 minutes, and the patient was able to go home on the third postoperative day. There were no significant complications. At follow-up after 12 months, multiple biopsies from the lasered area have shown no recurrence, and the symptomatic improvement has been satisfactory.
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7/782. A glomus tumour with four recurrences.

    A case of a glomus tumour of the distal segment of the ring finger, with four apparent recurrences in an 8-year period, is described. The patient was treated by two different surgeons (two and three times respectively) and obtained pain free intervals of between 4 and 11 months before recurrence. Histological examination confirmed the diagnosis of a glomus tumour in all five procedures. The location of the glomus tumour was defined preoperatively by high resolution MR imaging.
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8/782. Soft tissue masses of the chest wall and axilla: has metastatic melanoma been considered?

    Isolated axillary and chest wall soft tissue masses are an uncommon presentation of metastatic cancer. The authors present three patients in whom malignant melanomas metastatic to these sites had been misdiagnosed, leading to inappropriate oncologic treatment planning in all three cases. The presumed diagnoses, even after fine-needle aspiration or trucut biopsies, were soft-tissue sarcoma (n = 2) and undifferentiated breast cancer (n = 1). The combination of taking a thorough history and performing proper immunohistochemical analysis of the biopsy material would have suggested the presence of malignant melanoma in all cases. As the disease appeared locoregionally limited in all patients, radical surgical resection with extended lymphadenectomy was performed without significant dysfunction of the upper extremity. One patient agreed to postoperative immunotherapy with interferon-alpha. Two patients are currently alive 17 and 14 months after operation. One patient was found to have systemic recurrence at 5 months, one experienced two isolated local recurrences in a prior operative site that were amenable to reresection and presently has no evidence of disease 12 months after resection, and one patient remains free of disease at 14 months. Clinical presentation, suggested diagnostic workup, and therapeutic implications are discussed to avoid misdiagnoses in this setting of possible clinical presentations of metastatic melanoma.
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9/782. Management of adrenal metastasis of hepatocellular carcinoma by asynchronous resection of bilateral adrenal glands.

    We report on a 65-year-old man who received asynchronous bilateral adrenalectomy for adrenal metastasis of hepatocellular carcinoma. Fifteen months after curative resection of right hepatic lobe for hepatocellular carcinoma, a metastatic lesion of the left adrenal gland was detected and left adrenalectomy was performed. Ten months after the second operation, a metastatic lesion in the right adrenal gland, associated with tumor thrombus in the inferior vena cava, was revealed. Transcatheter arterial embolization of the arteries feeding the metastatic tumor was performed, but its effects were incomplete. As there was the tumor thrombus in the inferior vena cava and no other intrahepatic recurrence or extrahepatic metastasis was found, resection of the right adrenal gland with tumor thrombus, without the employment of veno-venous bypass, was performed, followed by postoperative hormonal supplementation. Changes in the patient's alpha-fetoprotein level were clinically useful for the detection of the metastatic lesions and the evaluation of therapeutic effects. Metastasis to adrenal gland from hepatocellular carcinoma should be actively managed, and the appropriate surgical treatment selected, if intrahepatic recurrence and/or other extrahepatic metastasis are controlled. To achieve higher curability and better outcome in patients with bilateral adrenal metastasis of hepatocellular carcinoma, bilateral total adrenalectomy is indicated, accompanied by effective postoperative hormonal supplementation.
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10/782. Thrombelastographic patterns during cryotherapy for recurrent hepatocellular carcinoma.

    A cirrhotic patient with recurrent hepatocellular carcinoma and thrombocytopenia undergoing cryotherapy showed: 1) transient hyperfibrinolysis shortly after platelet transfusion, and 2) evidence of activation of coagulation after freezing and thawing of tumor as measured on the thrombelastography. No anti-fibrinolytic treatment was required for the hyperfibrinolysis which subsided spontaneously in this patient. This case report highlights that: 1) in patients with chronic liver disease, platelet transfusion might potentially worsen rather than improve the hemostatic function by activation of fibrinolysis, and 2) activation of coagulation may underlie the perioperative coagulation changes seen in cryotherapy.
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