Cases reported "Rectal Neoplasms"

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1/54. Secretory villous adenomas that cause depletion syndrome.

    Secretory villous adenomas of the colon have been known to cause a depleting syndrome characterized by dehydration, prerenal azotemia, hyponatremia, hypokalemia, metabolic acidosis, obtundation, and, in severe cases, death. We describe 1 case of classic depleting syndrome and review the literature on possible mechanisms. Both cyclic adenosine monophosphate and prostaglandin E2 have been implicated as possible secretagogue compounds in the pathogenesis of this syndrome unique to the secretory variant form of villous adenomas. indomethacin as a prostaglandin inhibitor has been used with apparent benefit in controlling the volume of rectal effluent in patients with secretory villous adenomas.
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2/54. Paget's disease of the vulva associated with local adenocarcinoma and previous breast adenocarcinoma: report of two cases.

    We report two women in whom vulval Paget's disease occurred in association with local adenocarcinoma and previous breast adenocarcinoma. The first patient presented at the age of 83 years with moist erythematous changes over the perineum and an indurated area near the anus. biopsy of the indurated area showed Paget's cells throughout the epidermis and, below, adenocarcinoma infiltrating the dermis. Ten years previously, she had undergone a left mastectomy for infiltrating ductal carcinoma of the breast. The second patient was diagnosed as having Paget's disease at the age of 74 years. A vulval biopsy showed Paget's cells in the epidermis but, in addition, there were changes suggestive of adenocarcinoma of the sweat glands. Her symptoms of vulval itching had started at the age of 45 years and had led to a simple vulvectomy at the age of 57 years. Retrospective review of this vulvectomy specimen showed Paget's disease. She had also previously been treated for infiltrating ductal adenocarcinoma of the breast and adenocarcinoma of the rectum. The management of Paget's disease is difficult because of its high recurrence rate and, as illustrated by our two cases, treatment is difficult if the patients are elderly and in poor general health.
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3/54. Rectal adenocarcinoid with lymph node metastasis.

    We describe a case of a rare variant of a rectal carcinoid tumor that showed mucous gland differentiation accompanied by a lymph node metastasis with a histological appearance similar to that of the primary site. The tumor consisted of a typical argyrophilic carcinoid component and of goblet cell glands. The carcinoid component was positive for neuron-specific enolase, chromogranin a and synaptophysin. The goblet cells stained positively with periodic acid-Schiff (PAS) and alcian blue, and expressed carcinoembryonic antigen, but were negative for neuroendocrine markers. This case suggests that carcinoid tumor can differentiate towards mucus glands, which can also be found in the metastatic site.
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4/54. adenocarcinoma arising within a tailgut cyst: clinicopathological description and follow up of an unusual case.

    Full clinicopathological details and clinical follow up of a case of malignant transformation within a tailgut cyst are presented. A 43 year old woman presented with signs and symptoms of an imminent threatened abortion. Routine examination identified a coincidental, asymptomatic retrorectal/presacral mass. Following imaging studies, surgical resection was carried out and an adenocarcinoma arising within a pre-existent tailgut cyst was identified by microscopy. Four years later the patient presented with neurological symptoms consistent with local recurrence of the tumour. Surgical biopsies confirmed this diagnosis and she was subsequently started on chemotherapy. She died soon after from a cause unrelated to the disease, after declining further active intervention. Differential diagnosis of such cases includes (cystic) teratoma, epidermal cyst, rectal duplication cyst, anal gland cyst and carcinoma, extension of local carcinoma, and metastatic disease. It is recommended that these lesions be completely excised when detected incidentally.
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5/54. Submucosal dermoid cyst of the rectum: report of a case.

    Despite the relatively common incidence of sacrococcygeal dermoids, rectal cysts are uncommon. We report the case of a submucosal dermoid cyst occurring in the rectum. A 30-year-old woman visited the gynecology Department because of pregnancy. A pelvic tumor was accidentally found during the checkup after miscarriage. A barium enema showed an anterior shift of the rectum by the presence of the tumor. Computed tomography and magnetic resonance imaging revealed a tumor located posterior to the rectum occupying almost the entire pelvic cavity, and the tumor was resected. The tumor was located in the submucosal layer of the posterior rectal wall and was well circumscribed. The resected tumor was a cyst entirely covered with a fibrous and firm capsule, which was filled with an amorphous white creamy substance. The histological findings showed the cyst consisting of a keratinizing stratified squamous epithelium with sebaceous gland and hair follicles, which was compatible with benign cystic teratoma. Primary rectal teratoma is very rare and only 36 cases have been reported in the literature worldwide. Furthermore, while the majority of cases were polypoid-shaped dermoid cysts protruding into the rectal lumen, only 3 cases were submucosal dermoid cysts. Therefore, such cases are considered to be extremely rare.
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6/54. adenocarcinoma of the rectum with various grades of atypia in association with Crohn's disease: a case report and immunohistochemistry of p53 and Ki-67.

    A case of adenocarcinoma of the rectum in a 41-year-old woman, in association with Crohn's disease is presented. The patient had suffered diarrhea and constipation, and Crohn's disease was suspected. Although the endoscopy did not reveal the presence of any tumors, biopsy specimens demonstrated adenocarcinoma. A Miles' operation was performed. The adenocarcinoma was composed of various grades of atypia and had invaded the non-peritonealized perirectal tissues. The infiltration of lymphocytes and plasma cells was moderate at the perimeter of the carcinoma and mild in the distant regions. Epithelioid cell granulomas were found. The p53 labeling index (LI) increased with the grade of atypia over the entire length of the carcinomatous gland. In carcinomas with high grade atypia, the p53 LI was high in both the upper and the lower halves of the gland. In carcinomas with low or moderate grade atypia however, the p53 LI was high in the lower half and low in the upper half of the gland. The Ki-67 LI over the entire gland was higher in carcinomas with high grade atypia than in carcinomas with low or moderate grade atypia.
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7/54. Unusually aggressive rectal carcinoid metastasizing to larynx, pancreas, adrenal glands, and brain.

    Rectal carcinoids are slow-growing tumors. They metastasize when their size is more than 2 cm. Common sites of metastasis are the liver, lungs, and bones. Metastases to thyroid, pancreas, kidneys, adrenal glands, pituitary glands, posterior fossa, and spleen are very rare. We present the case of a 79-year-old white man with dysphagia and left vocal cord paralysis from a rapidly growing mass in his neck. Needle biopsy suggested thyroid anaplastic carcinoma, and the patient underwent total laryngectomy, total thyroidectomy, and left radical neck dissection. pathology showed undifferentiated carcinoid of the larynx. biopsy of a rectal mass suggested poorly differentiated carcinoma. Postoperatively the patient developed cardiac arrhythmias and died after 5 weeks. autopsy showed a 5-cm carcinoid of the rectum with extensive vascular invasion extending into the perirectal fat. There was metastatic disease to both lungs, liver, pancreas, both adrenal glands, peritoneum, subcutaneous tissues of thorax and abdomen, ribs, vertebrae, skull, and the leptomeninges of the cerebrum. Rectal carcinoids may present a variable histologic picture. Poorly differentiated tumors can present with widespread metastases and have poor prognosis. Extensive surgery may not improve the survival of patients with this pattern of unusually aggressive carcinoid.
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8/54. Cronkhite-canada syndrome associated with advanced rectal cancer treated by a subtotal colectomy: report of a case.

    A 41-year-old man with Cronkhite-canada syndrome presented with multiple juvenile polyps with hyperplastic and adenomatous changes throughout his stomach and entire colorectum. dysgeusia was recognized and the degree of hypoproteinemia was remarkable. A barium enema study and colonofiberscopy also revealed an advanced cancer in the rectum. Chronic hepatitis B and membranous glomerulonephritis were also present. It was difficult to design a conservative protocol using steroids for the treatment of protein-loosing enteropathy because the patient was a hepatitis b virus carrier. As a result, a subtotal colectomy while preserving the cecum with cecorectal anastomosis was performed. Pathologically, the ulcerated rectal tumor was a moderately differentiated adenocarcinoma with invasion into the muscularis propria. Most polyps showed cystically dilated glands without dysplasia or edematous stroma with inflammatory cell infiltration. A few polyps were juvenile-type polyps with adenoma components. Although no remarkable improvement was observed in the hypoproteinemia postoperatively, an alpha1-antitrypsin clearance test showed a significant decrease in protein loss from the gastrointestinal tract, which was only about one third of the loss seen preoperatively. These findings lead us to conclude that when improvement using conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with Cronkhite-canada syndrome.
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9/54. Attenuated familial adenomatous polyposis associated with advanced rectal cancer in a 16-year-old boy: report of a case.

    We herein present a case of attenuated familial adenomatous polyposis (AFAP) with advanced rectal cancer in a 16-year-old boy. His mother and younger brother both had subcutaneous soft tissue tumors in the back and sparse-type colorectal polyposis. His mother also had dental anomalies and gastric fundic gland polyposis. The patient was admitted to our hospital for investigation of bloody stools. barium enema and colonofiberscopy revealed advanced rectal cancer and sparse (<50) colorectal polyps. He also had dental anomalies, a subcutaneous soft tissue tumor in the back, and gastric fundic gland polyposis as extracolonic manifestations. A total proctocolectomy and ileoanal anastomosis were performed, and histological examination of the resected specimens confirmed moderately differentiated adenocarcinomas of the rectum with metastases to the regional lymph nodes. The other colorectal polyps were tubular adenomas with no evidence of malignancy. Germline mutations in the APC gene were observed in codons 486, 545, 1493, and 1556. This case serves to demonstrate that a total proctocolectomy with ileoanal anastomosis should be the procedure of choice for young patients found to have advanced rectal cancer associated with FAP.
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10/54. Implantation of rectal cancer cells in a fistula in ano: report of a case.

    We report a case of implantation of tumor cells within a fistula in ano. A 36-year-old man with a 16-year history of an anal fistula underwent an operation for rectal carcinoma. Three weeks later, the anal fistula was resected. A histological examination of the specimen showed atypical cells; moreover, rectal carcinoma had proliferated in the granulation tissue lying underneath the intact squamous epithelium. Because there was no continuity to the rectal carcinoma or the anal glands, we diagnosed implantation of rectal cancer cells in a fistula in ano.
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