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1/447. 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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2/447. Extraneural metastasizing ependymoma of the spinal cord.

    This paper reports a case of the rare entity of an extraneural metastasizing ependymoma of the spinal cord. The tumor which arose in the conus medullaris and in the cauda equina was first diagnosed in 1956 when a thoracolumbar myeloresection was performed. At autopsy, 40 years after the primary diagnosis, a massive local tumor recurrence with extraneural metastases in the lungs, the pleura, the liver, and the thoracal and abdominal lymph nodes were found. Immunohistochemical stains of the extraneural metastases showed a strong cytoplasmatic expression of glial fibrillary acidic protein (GFAP). Neither the primary tumor nor its metastases showed any of the conventional morphological criteria of malignancy. Reviewing the literature we discuss the possible mechanism of extraneural tumor spread and the incidence of metastases with regard to the tumor type.
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3/447. 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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4/447. Surgical management of chylous fistula after retroperitoneal lymph node dissection.

    Conservative treatment with low-fat diet, medium-chain triglyceride or total parenteral nutrition, depending on the general condition of the patient, is the mainstay in the treatment of chylous ascites. In patients with persistent chylous fistula direct surgical closure is a valid treatment option.
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5/447. 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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6/447. Rapid growth of residual colonic tumor after incomplete mucosal resection.

    We report an 89-year-old man with colon cancer that developed rapidly after an incomplete endoscopic mucosal resection (EMR), and discuss the adverse effect of this maneuver on the tumor biology. A sessile polyp, 15 mm in size, was detected at the hepatic flexure. EMR was performed immediately. Histological examination showed well differentiated adenocarcinoma with an adenomatous component invading the submucosal layer. There was vascular invasion (positive on elastica van Gieson staining) and the surgical margin was positive for cancer. A right hemicolectomy was performed. The surgical specimen showed the residual tumor, 22 mm in diameter. The relevant histopathological findings of the surgical specimen were: well differentiated adenocarcinoma, with partly mucinous carcinoma and a tubular adenomatous component, depth muscularis propria (mp), lymph node (LN) (0/9). Most of the submucosally invasive cancer was resected by the initial EMR, but the small residual tumor showed rapid growth within only 3 months after the EMR. It was assumed that the residual tumor cells had acquired more malignant characteristics after EMR. In regard to EMR we propose that: (1) except for patients who are at high risk for a major operation, EMR should be avoided for carcinoma with massive submucosal invasion, (2) colonic resection should be performed immediately when histology shows a positive surgical margin for carcinoma, and (3) patients operated after an incomplete EMR should be watched very carefully for the detection of recurrence.
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7/447. Recurrent squamous-cell carcinoma arising in sacrococcygeal pilonidal sinus tract: report of a case and review of the literature.

    PURPOSE: Carcinoma arising in a pilonidal sinus is a rare complication. This study reports the case of a patient with recurrent squamous-cell carcinoma arising in a sacrococcygeal pilonidal sinus tract. methods: This patient was treated with a wide local excision and mesh grafts. Three months later the patient was treated with adjuvant radiation therapy. RESULTS: The patient died two years later from recurrence in inguinal lymph nodes, liver, and lungs. CONCLUSION: Some authors propose consideration of treatment with adjuvant chemotherapy and radiation therapy in addition to complete local excision as a possible means to decrease the local recurrence rate.
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8/447. 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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9/447. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

    Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.
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10/447. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

    Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.
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