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1/396. Follicular dendritic cell tumor of the parapharyngeal region.

    BACKGROUND: Follicular dendritic cell (FDC) tumors are rare. A majority of the reported cases were confined to the lymph nodes. We report a case of FDC tumor occurring in the parapharyngeal region in a 45-year-old woman. methods: Characteristic histopathologic features of the excised primary and recurrent parapharyngeal tumors in conjunction with immunohistochemistry and electron microscopy helped us to arrive at a diagnosis of FDC tumor. RESULTS: Histopathology of primary excision revealed a lobulated tumor with a suggestion of ill-defined whorls. The most striking feature was regular occurrence of aggregates of lymphocytes within the tumor, especially around the blood vessels. The anatomic location together with the histology indicated the possibilities of either a meningioma, a salivary gland tumor, or a nerve sheath tumor. Immunostains for cytokeratin (CK), S-100 protein, and smooth muscle actin (SMA) were negative. However, the tumor cells showed strong immunoreactivity for epithelial membrane antigen (EMA) and vimentin. A diagnosis of parapharyngeal meningioma appeared to be the closest possibility. One year later, the patient developed a recurrence at the same site. A reexcision showed an identical tumor with an additional feature of lymphatic embolization and angioinvasion. A review of the entire case with further immunoreactivity for CD21 and CD35 confirmed the diagnosis of FDC. CONCLUSIONS: Follicular dendritic cell tumor has distinctive morphologic features and immunohistochemical profile. It is also characterized by considerable potential for recurrences.
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2/396. The place of irradiation in the treatment of malignant tumors of the salivary glands.

    1. radiation therapy is not indicated after surgical removal with adequate margins of low-grade tumor. 2. radiation therapy is indicated with a) Inadequate surgical margins in low-grade tumors b) All high-grade tumors c) All recurrent malignant tumors 3. Irradiation of nerve pathways is indicated with demonstrated nerve and perineural invasion and/or with adenoicystic carcinoma. 4. Irradiation of the entire ipsilateral neck is indicated a) Wtih high-grade tumors unless radical neck dissection shows negative nodes b) In the place of radical neck dissection.
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3/396. 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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4/396. 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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5/396. Primary eyelid mucinous adenocarcinoma of eccrine origin.

    To report a case of mucinous adenocarcinoma of the eyelid. A 70-year-old man presented with a lesion of his lower left eyelid, which had been growing over the past few years. An excisional biopsy was performed. The lesion was incompletely excised and recurrence at the excision site was observed at a 3-month follow-up visit. A histopathological examination of the excised specimen showed it to be a mucinous adenocarcinoma of eccrine origin. Primary mucinous adenocarcinoma of the eye lid is a rare malignant sweat gland tumor with a high incidence of local recurrence. It has to be differentiated from other adenocarcinomas which may rarely metastasize to the skin.
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6/396. Histologic transformation of polymorphous low-grade adenocarcinoma of salivary gland.

    Polymorphous low-grade adenocarcinoma of salivary gland origin (PLGA) was initially described in 1984 and has since become an established clinicopathologic entity. Owing to the indolent nature of PLGA and its relatively recent description, the full clinicopathologic spectrum of this entity has not been elucidated fully. Transformation to a histologically different-appearing lesion or progression to a higher histologic grade has not been reported. We describe 2 PLGAs arising in the palate and associated with multiple locoregional recurrences that were treated with excision and radiation therapy. This was followed by histologic transformation to a higher grade neoplasm after 17 and 26 years, respectively. The histologic appearance after transformation was characterized by a predominantly solid and cystic growth pattern, nuclear atypia with prominent nucleoli, and foci of necrosis. High-grade transformation of PLGA may occur after a protracted clinical course with multiple recurrences of typical PLGA. The possible role of radiation therapy as an initiator of this transformation merits further study. Tumors with these histologic features should not be included under the rubric of typical PLGA. Segregation of these neoplasms will allow further study of their biologic potential, particularly with regard to possible increased rates of local recurrence and metastasis.
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7/396. Clinical and morphological features of undifferentiated monomorphous GH/TSH-secreting pituitary adenoma.

    A 41-year-old male presented with progressive visual defects, acromegaly and hyperthyroidism. After clinical evaluation a giant GH/TSH-secreting pituitary adenoma was diagnosed. Administration of the somatostatin analog octreotide at doses of 150 microg s.c. per day inhibited the secretion of both GH and TSH. A three-week treatment with octreotide prior to surgery led to slight visual improvement and CT scan showed some new necrotic areas within the tumor mass. Transcranial surgery was performed. By immunohistochemical analyses of the adenoma tissue GH, prolactin and beta-chorionic gonadotropin were detected; TSH was negative. Electron microscopy revealed an undifferentiated, monomorphous adenoma with morphological features of an acidophil stem cell adenoma such as the presence of misplaced exocytoses, fibrous bodies and mitochondrial gigantism. However, the tumor cells contained small secretory granules (up to 250 nm) accumulated along the cell membrane characteristic of thyrotrope cells. Furthermore, some adenoma cells were fusiform with long cytoplasmic processes resembling thyrotropes. Two months after the operation CT scan revealed a large residual tumor. serum GH and TSH levels had increased again and the TSH level was even higher than before the treatment. The patient died suddenly, most probably of lethal arrhythmia. Specimens of the adenoma tissue obtained at autopsy confirmed the previous findings with the exception of positive immunostaining for TSH which was found in less than 1% of the adenoma cells. This undifferentiated, monomorphous GH/TSH-secreting pituitary adenoma represents an entity that is unusual both in its ultrastructural features and clinical manifestations suggesting a cytogenesis from an early, undifferentiated stem cell.
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ranking = 12.724125990736
keywords = pituitary
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8/396. Extramammary Paget's disease of the vulva: report of five cases and review of the literature.

    Five patients with vulvar extramammary Paget's disease (EMPD) are reported. They accounted for 4.4% of all vulvar malignancies diagnosed in the south of israel between 1961-1997. Mean age was 64.4 years and the predominant symptom was vulvar pruritus. Three patients had intra-epidermal lesions (managed by wide local excision or, at most, simple vulvectomy), one had a minimally invasive lesion (managed by simple vulvectomy) and one had EMPD with an underlying apocrine gland adenocarcinoma (managed by radical vulvectomy and bilateral groin dissection followed by pelvic radiotherapy). EMPD recurred in two patients: 1) local recurrence after simple hemivulvectomy for an intra-epidermal lesion was successfully treated by wide local excision; 2) widespread recurrence at distant skin sites after simple vulvectomy for a minimally invasive lesion was unsuccessfully treated with systemic chemotherapy. It is concluded that vulvar EMPD is an uncommon neoplasm that primarily affects postmenopausal women. Its histogenesis is uncertain and most commonly it is an intra-epidermal lesion. The high rate of recurrent disease remains a challenge for optimal management.
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9/396. Recurrent adrenocortical carcinoma in a child.

    An 8-month-old girl presented with clitoromegaly, cushingoid features and a large abdominal tumour. ultrasonography (US) and computed tomography (CT) of the abdomen revealed a tumour of the left suprarenal gland, 12x11x7 cm in size. serum levels of cortisol, testosterone and DHEA-S, and urinary extretion of 17-ketosteroids and 17-hydroxycorticoids were increased. Complete removal of the tumour was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically. Three months after the first operation, a recurrent tumour of the left renal hilus, 23x15 mm in size, was identified by US and verified by aspiration biopsy. The tumour was removed by the transabdominal route. In this report, we discuss the diagnosis and the treatment of this rare disease.
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10/396. Rapid enlargement and recurrence of a preexisting intrasellar craniopharyngioma during the course of two pregnancies. Case report.

    Enlargement of preexisting pituitary adenomas during pregnancy is well documented, but this phenomenon is unusual for nonendocrine pituitary tumors such as craniopharyngiomas. Only six cases of craniopharyngioma have been reported as presenting during pregnancy. The authors describe a 19-year-old woman who presented with amenorrhea and galactorrhea caused by an intrasellar mass. Seven months later, when she was 20 weeks pregnant, the patient developed sudden visual dysfunction. Emergency transsphenoidal surgery was performed to restore visual function, and the tumor was found to be a craniopharyngioma. The patient had spontaneous labor and delivered a healthy infant at term. The tumor recurred 4 years later, during her second pregnancy, and was again entirely removed via a second transsphenoidal approach. She again had a normal term delivery. During the 5-year follow-up period she has demonstrated no endocrinological or visual dysfunction. Control magnetic resonance images have revealed no recurrence of the tumor. The transsphenoidal approach seems to be the safest procedure to use during pregnancy to achieve an immediate optic nerve decompression and to preserve pituitary function.
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ranking = 6.3620629953681
keywords = pituitary
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