1/15. Clinicopathological and immunohistochemical study of cancer arising from Barrett's esophagus.In japan, Barrett's esophageal cancer is a very rare disease. We examined clinicopathologically and immunohistologically 4 patients with Barrett's esophageal cancer who underwent surgical resection in our department. Barrett's esophageal mucosa was classified into 3 types for detailed observation. Specialized columnar epithelium (SCE) remained on the orifice side of carcinoma, and progression to adenocarcinoma was observed in some dysplastic glands. positive findings were detected on p53 immunohistochemical staining, and the ki-67 labeling index was higher than other types. SCE-type Barrett's esophagus may be a precancerous lesion arising prior to the development of adenocarcinoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/15. Giant esophageal polyp: a rare and benign neoplasm.Giant esophageal polyp is a very rare neoplasm, usually benign but often demanding both for diagnostic and/or therapeutical procedures and for dramatic symptomatologic onset in the patient (regurgitation and asphyxia). The authors present a brief clinical report of a patient with a 20 cm.-long esophageal polyp removed by left lateral cervicotomy and esophagotomy for 8 cm. below the upper esophageal sphincter (histologic examinations showed a pedicled polypoid fibrolipoma with a remarkable presence of myxoid and vascular components). The authors also describe diagnostic and therapeutic methods to manage this rare disease and they gave a brief review of recent literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/15. A case of primary intestinal T-cell lymphoma involving entire gastrointestinal tract: esophagus to rectum.Primary intestinal T-cell lymphoma is a rare disease entity, which is approximately 10% to 25% of intestinal lymphomas, and most of the lymphomas occur in the small intestine. We report here a case of a 56-year-old woman who has been suffering from chronic diarrhea and weight loss for 6 months. Abdominal CT scan and small bowel series showed diffuse wall thickening of the small bowel. Gastroscopic examination showed diffuse erythematous lesions on the esophagus and small gastric ulcerations on the antrum of the stomach, and colonoscopic examination also showed multiple punched-out ulcerations and erosions on the entire colon, including the sigmoid colon to the terminal ileum. Diffuse infiltration of CD 3 positive lymphoma cells was found on biopsy. The patient was diagnosed as primary intestinal T-cell lymphoma with diffuse involvement of the entire gastrointestinal tracts from the esophagus to the rectum. Although the patient received systemic combination chemotherapy and achieved partial response initially, the lymphoma relapsed repeatedly.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/15. Primary malignant melanoma of the esophagus.is a rare disease, with only 200 cases being reported since this condition became an established clinical entity in 1963. This tumor, which accounts for only 0.1-0.2% of all esophageal neoplasms, is typically aggressive and disseminates early via the bloodstream and lymphatics, with only some 30% of patients surviving > 1 year after diagnosis. Management of patients with esophageal melanomata is unsatisfactory, as most tumors are advanced at diagnosis, and therapeutic options are limited by inaccessibility and early dissemination of the neoplasms. Poor survival rates reflect the inoperability of many tumors and the ineffectiveness of radiation and chemotherapy in eradicating advanced tumors and metastases. We present two patients with primary melanoma of the esophagus and discuss the treatment options currently available.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/15. Extraosseous accumulation of 99mTc-MDP in lymph node metastases of small cell carcinoma of the esophagus.We report a case of esophageal carcinoma that showed extraosseous accumulation of 99mTc-MDP in lymph node metastases to the cervical and paracardial lymph nodes. There are few cases showing abnormal extraosseous accumulation of 99mTc-MDP in esophageal cancer lesion. The patient was a 53-year-old man with advanced esophageal cancer. Bone scintigraphy demonstrated extraosseous accumulations in left supraclavicular and paracardial lymph node metastases. The histopathological diagnosis was small cell carcinoma of the esophagus, which is a rare disease with aggressive behavior and poor prognosis. Our patient underwent 2 courses of systemic chemotherapy (CDDP VP16), but died of rapidly growing systemic metastases 5 months after the initial treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/15. Idiopathic eosinophilic oesophagitis: atypical presentation of a rare disease.A 72 year-old man presented severe dysphagia and weight loss of recent onset. Repeated oesophageal endoscopy and biopsies with macroforceps were normal. Oesophageal manometry disclosed features compatible with achalasia. Oesophageal EUS endoscopy localized an infiltrating process between muscular layers of the oesophageal wall and CT scan delimited a circular thickening in the inferior part of the oesophagus. Because of severe clinical presentation mimicking a possible oesophageal neoplasm like a lymphoma, partial oesophagectomy was performed and revealed eosinophilic oesophagitis. This unusual presentation emphasizes that idiopathic eosinophilic oesophagitis must be proposed in the differential diagnosis of dysphagia, even in old patient without apparent oesophageal lesion at endoscopy.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
7/15. Amelanotic malignant melanoma of the esophagus: report of a patient with recurrence successfully treated with chemoendocrine therapy.We report a case of primary amelanotic malignant melanoma of the esophagus, an extremely rare disease. A 58-year-old man was diagnosed as having middle esophageal cancer with lymph node metastasis, which was classified as esophageal cancer, Stage III:T3N1M0, by International Union Against Cancer (UICC) criteria. Preoperative chemotherapy was performed, but the response assessment was no change (NC). The patient underwent a subtotal esophagectomy via right thoracotomy and laparotomy. Reconstruction was performed by pulling up the stomach via the retrosternal route; the site of anastomosis was the neck. Adjuvant chemotherapy consisted of five courses of dacarbazine (DITC), nimustine (ACNU), vincristine (VCR), and interferon-beta. Eleven months after the surgery, computed tomography (CT) demonstrated recurrence in the upper mediastinum. The patient received chemoendocrine therapy, consisting of the first planned course of DITC, ACNU, and cisplatin (CDDP), given intravenously; and tamoxifen (TAM), given orally. Subsequently with a modified regimen of this therapy he attained a complete response (CR). In general, the prognosis of esophageal malignant melanoma is very poor. Although our patient had a recurrence, he is alive 4 years and 5 months after the surgery and 3 years and 6 months after the recurrence. The chemoendocrine therapy probably contributed to this outcome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/15. Primary esophageal T-cell non-Hodgkin's lymphoma.Primary non-Hodgkin's lymphoma of the esophagus is a rare disease. We report a 52-year-old man who had a polypoid mass in the esophagus at endoscopy. histology was suggestive of non-Hodgkin's lymphoma; immunohistochemistry was positive for CD3, CD45 RO, LCA. He was treated with 6 cycles of CHOP and is disease-free 14 months later.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/15. (18)FDG-PET-scan in staging of primary malignant melanoma of the oesophagus: a case report.Primary malignant melanoma of the oesophagus is a rare disease, only 262 cases being reported up to June 2005. In general, the prognosis is dismal because of its tendency to present as an advanced neoplasm with aggressive biological behaviour. (18)FDG-PET-scan is a useful tool for evaluation of metastatic disease and locoregional lymph node metastasis. We present herein a case of a young adult with primary malignant melanoma of the oesophagus, followed by a literature review on the subject.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/15. radiation treatment of superficial spreading carcinoma of the esophagus.Superficial spreading carcinoma of the esophagus is a relatively rare disease. A 78-year-old man with this lesion was treated with high-dose-rate intracavitary irradiation (6 Gy) following external irradiation (48 Gy). The irradiated length was very long: 21 cm for intracavitary irradiation, which covered almost the entire thoracic esophagus, and 24 cm for external irradiation, which covered the entire thoracic esophagus. After treatment, he was well and could eat solid foods until his death, without tumor, two years and five months after radiotherapy. Intracavitary irradiation with a long irradiation length following external irradiation with a long irradiation length is recommended for the treatment of superficial spreading carcinoma of the esophagus.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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