1/37. Laparoscopic management of pseudomyxoma peritonei secondary to adenocarcinoma of the appendix.pseudomyxoma peritonei is a rare disease in which the abdominal cavity fills with thick mucoid material secondary to either benign or malignant conditions. We discuss a case where pseudomyxoma peritonei secondary to adenocarcinoma of the appendix was diagnosed and managed laparoscopically. The laparoscopic approach allows thorough exploration of the abdomen, as well as irrigation and aspiration of the thick mucinous material using a 10-mm suction cannula and the instillation of mucolytic agents such as 5% dextrose solution. appendectomy or right hemicolectomy can be performed with minimal disturbance of the anterior abdominal wall, thus minimizing future adhesions as well as possible tumor-cell implantation. Intraperitoneal catheters for chemotherapy can be placed easily through the port sites. These measures offer an alternative to radical peritoneal dissection and can be accomplished during the initial laparoscopic exploration.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/37. 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) |
3/37. Polymorphous low-grade adenocarcinoma of the nasopharynx. Case report and review of the literature.BACKGROUND: The polymorphous low-grade adenocarcinoma of the nasopharynx is a rare disease. Polymorphous low-grade adenocarcinoma is a minor salivary gland neoplasm which occurs frequently in the mucosa of the soft and hard palates, in the buccal mucosa and in the upper lip. To date this entity has been identified within the oral cavity and only one case within the nasopharynx and some cases in the parotid gland. It has a slow infiltrating growing pattern with frequent perineural invasion and low metastatic potential. CASE REPORT: We report on a patient with non-papillary polymorphous low-grade adenocarcinoma in the nasopharynx which extended intracranially. The patient underwent primary radiotherapy. The CT showed partial response to radiotherapy and the patient is alive 51 months after the diagnosis his state being unchanged. CONCLUSION: The treatment for minor salivary gland tumor is primarily surgical. It is reported that the polymorphous low-grade adenocarcinoma has been known to have poor response to radiotherapy. However, we believe that in addition to its favorable biological behavior, the radiotherapy in this localization may result in longer survival.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/37. Colonic carcinoma: a case report in a child and review literature.This article reports one case of child colonic carcinoma. This is a rare disease in children which usually occurs in predisposing conditions, e.g. ulcerative colitis, familial polyposis coli, Gardner's syndrome, Turcot's syndrome and Peutz-Jegher's syndrome. The patient in this report was 12 years old. He presented with chronic intermittent colicky abdominal pain and uncorrectable iron deficiency anemia for 7 months prior to definite diagnosis. This report also reviews the literature about colorectal carcinoma in children. physicians can make an early diagnosis with a high index of suspicion if they cannot explain clearly what causes abdominal pain. Further investigations should be performed, thereby, avoiding delayed diagnosis and improving survival rate.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/37. A case of nephrogenic diabetes insipidus caused by obstructive uropathy due to prostate cancer.Nephrogenic diabetes insipidus (DI) secondary to chronic urinary tract obstruction is a rare disease. The exact cause is unknown but it is likely that increased collecting duct pressures cause damage to the tubular epithelium, resulting in insensitivity to the action of arginine-vasopressin (AVP). A 77-year-old man complaining of polyuria and polydipsia was treated with alpha glucosidase inhibitor under the impression of polyuria due to diabetes mellitus. But his symptoms did not improve. water deprivation and AVP administration study revealed that the patient had nephrogenic DI. urinary tract obstruction due to an enlarged prostate was suggested as a principal cause of nephrogenic DI. The patient underwent transurethral resection of the prostate and bilateral subcapsular orchiectomy. After surgery, the urine osmolarity was normalized and the patient became symptom-free. We report a case of nephrogenic DI due to obstructive uropathy which was cured by surgery eliminating obstruction.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/37. A carcinoembryonic antigen-secreting adenocarcinoma arising within a retrorectal tailgut cyst: clinicopathological considerations.Retrorectal tailgut cysts (TGC) develop from postanal fetal gut remnants. They have specific radiological and histopathological features that distinguish them from dermoid cysts, enteric duplication cysts, and teratomas. We report a patient with a carcinoembryonic antigen-producing adenocarcinoma arising within a TGC who underwent resection through a combined anterior laparotomy/posterior pelvic approach. Despite complete resection and delayed but complete functional recovery, diffusely metastatic disease was encountered 6 months after resection. Diagnostic, therapeutic, histopathological, and oncological implications of this illustrative case are discussed. It seems possible to use carcinoembryonic antigen measurements for treatment planning and for assessing treatment response for this rare disease. The described outcome also suggests that TGC can develop malignant degeneration and should be resected at the time of diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/37. Acrokeratosis paraneoplastica (Bazex syndrome) with adenocarcinoma of the colon: report of a case and review of the literature.Acrokeratosis paraneoplastica is a rare disease and is uncommon even in patients with upper aerodigestive tract cancer. We report a 63-year-old man with a 1-month history of numerous pruritic lesions and vesicles on both feet. Although he had received local therapy, progressive dense scale formation involving both palms and both soles was found. colonoscopy was performed because of hematochezia, and it revealed an early colon cancer. After the resection of the cancer, the skin lesions began to fall off dramatically. To the best of our knowledge, there is no report of acrokeratosis paraneoplastica associated with colon cancer in the literature. This is the first case report of acrokeratosis paraneoplastica associated with early colon cancer.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/37. Multiple inflammatory pseudotumors mimicking liver metastasis from colon cancer: report of a case.A 54-year-old man underwent an operation for colon cancer histologically diagnosed as moderately differentiated adenocarcinoma with clinical staging of Dukes C. He was prescribed carmofur for adjuvant chemotherapy. A follow-up computed tomography scan done 6 months later revealed two new low-density areas in the liver. A diagnosis of metastatic adenocarcinoma from the previous colon cancer was presumed, based on the patient's history and radiological findings, and resection of the affected area of liver was performed. Histological examination of these tumors revealed that they were inflammatory pseudotumors (IPT). The patient had an excellent postoperative course and has shown no further signs of recurrence in the 3 years since his last operation. IPT of the liver is a rare disease, for which no methods of diagnosis and treatment have been established, since it is difficult to distinguish IPT from hepatocellular carcinoma or metastatic carcinoma. We describe this case with a review of the 101 cases of IPT documented in the Japanese literature, in the hope that it will contribute to the diagnosis and treatment of this unusual disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/37. Inflammatory breast cancer presenting with acute central venous thrombosis: a case report.Inflammatory breast cancer is a relatively rare disease associated with varied presentations, which frequently obscure the underlying diagnosis. We discuss a 54-year-old woman who presented to our emergency department with a chief complaint of right arm swelling and was admitted to the surgical service with an initial diagnosis of spontaneous brachial, axillary, and subclavian venous thrombosis. Further workup revealed an extensive inflammatory breast cancer. To our knowledge there have been no literature reports of inflammatory breast carcinoma in which the sole presenting symptom complex was due to central venous thrombosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/37. leiomyomatosis of mesenteric lymph nodes associated with duodenal adenocarcinoma.leiomyomatosis of lymph nodes is an extremely rare disease. Only a few cases have previously been reported in pelvic lymph nodes. They were related to a benign uterine leiomyoma, a metastasizing uterine leiomyoma, an endometrial adenocarcinoma, and an ovarian endometrioid carcinoma. We report on a case of leiomyomatosis of the mesenteric lymph nodes associated with a duodenal adenocarcinoma with no history of uterine leiomyoma or any gynecological malignancy. The patient, a 56-year-old woman, was found to have an adenocarcinoma of the duodenum. All mesenteric lymph nodes removed showed leiomyomatosis, which was verified by immunohistochemical study showing positive immunostaining for smooth muscle actin, desmin, and vimentin, but negative staining for HMB-45. It is necessary to make a differential diagnosis from other examples of spindle cell proliferation involving lymph nodes such as a hemorrhagic spindle cell tumor with amianthoid fibers (palisade myofibroblastoma), angiomyolipoma, lymphangiomyomatosis, inflammatory pseudotumor, and Kaposi's sarcoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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