1/51. Granulocytic sarcoma (chloroma) of the small intestine.Granulocytic sarcoma or Chloroma may develop before, at the time or after presentation of acute myeloid leukemia. We report the case of a 66-year old man presenting with intermittent abdominal pain during one month before developing a peritonitis due to perforation of small bowel followed by irreversible shock and death. Nearly the entire length of small bowel and bone marrow were infiltrated by giant promyelocytic cells. Abnormal circulating cells were never discovered. The literature is briefly reviewed.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/51. Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2b: implications for treatment.Three infants, who presented with intestinal obstruction due to diffuse transmural intestinal ganglioneuromatosis, are described. mutation analysis of exon 16 of the RET proto-oncogene revealed germline M918T and thus, a molecular diagnosis of multiple endocrine neoplasia type 2b (men 2B). Two infants developed medullary carcinoma of the thyroid. The third had a prophylactic thyroidectomy despite no obvious thyroid masses and normal calcitonin concentrations, but microscopic multifocal medullary carcinoma was found on histological examination. Early recognition of intestinal ganglioneuromatosis with germline RET M918T mutation in pseudo-Hirschsprung's disease is an indication for prophylactic thyroidectomy.- - - - - - - - - - ranking = 704.66964060264keywords = endocrine (Clic here for more details about this article) |
3/51. Enterotherapy associated T-cell lymphoma: a case report and literature review.T-cell lymphoma of the intestine is a form of extranodal, non-Hodgkins lymphoma. Enteropathy associated T-cell lymphoma (EATCL) is a unique form of T-cell lymphoma involving the gastrointestinal tract. This disorder is rare and there is no consensus on guidelines for diagnosis or treatment. CASE: This 47 year old patient presented to the hospital with increasing abdominal girth and swelling of the lower extremities over the four days prior to admission. He complained of dyspnea on exertion, anorexia, nausea and vomiting, 40 pound weight loss over half a year, accompanied by intermittent diarrhea. On physical examination, the patient was found to have anasarca, an abdominal fluid wave with shifting dullness, mild abdominal tenderness, marked pitting edema of the extremities, and necrotic toes. A small bowel follow through revealed an abnormality in the mid-jejunum and a colonoscopy revealed two erythematous lesions near the cecum. Subsequent esophagoduodenoscopy and enteroscopy identified multiple white patchy lesions in the proximal jejunum. Biopsies were consistent with a T-cell lymphoma. The patient underwent a partial small bowel resection. Biopsies of the jejunum had ulceration and necrosis with a lymphomatous infiltrate. The lymphocytes were found to be a monoclonal CD2 positive T-cell population. A bone marrow biopsy was unremarkable. Enteropathy associated T-cell lymphoma is a rare, and this may be the first reported case of EATCL presenting as anasarca. DISCUSSION: The etiology and pathophysiology of intestinal lymphomas, particularly enteropathy associated T-cell lymphoma, is reviewed. CONCLUSION: EATCL is a rare and unique form of lymphoma without an exact treatment regimen which carries a high mortality rate.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
4/51. Diffuse large B-cell non-Hodgkin's lymphoma presenting as a vaginal mass in a patient with a history of intestinal mucosa-associated lymphoid tissue (MALT) lymphoma.Vaginal involvement in non-Hodgkin's lymphoma is uncommon and is often secondary to disseminated disease. Primary disease at this site is quite rare. We present here an unusual case of a patient who developed a diffuse large B-cell lymphoma presenting as a vaginal mass after having been treated for primary intestinal mucosa-associated lymphoid tissue (MALT) lymphoma 4 years earlier. Combined chemotherapy and radiation therapy for the intestinal MALT lymphoma produced complete remission that lasted for 2 years. Chemotherapy given for the diffuse large B-cell lymphoma with secondary vaginal involvement produced a second complete remission. The second remission was consolidated with high-dose chemotherapy and autologous bone marrow transplantation. Although the patient died from complications related to the transplant procedure, the disease was in complete remission at the time of her death. Given the rarity of primary intestinal MALT lymphoma and primary vaginal lymphoma, no standard treatment has been established. Treatment options have included chemotherapy, radiation therapy, or surgery, given alone or in combination.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
5/51. Intestinal carcinoid tumours in a father and daughter.Familial cases of carcinoid tumours that are not associated with any known syndrome or disease are extremely rare. All cases reported in the world literature have involved carcinoid tumours of the gastrointestinal tract. Two cases of carcinoid tumours of the small intestine in a father and daughter are presented. Laboratory analyses did not support the hypothesis that the occurrence of carcinoid tumours in this family is a variant of the multiple endocrine neoplasia type 1 syndrome. A review of the literature on familial occurrence of intestinal carcinoid tumours in the absence of any other known carcinoid tumour-predisposing genetic syndrome is provided.- - - - - - - - - - ranking = 140.93392812053keywords = endocrine (Clic here for more details about this article) |
6/51. Malignant carcinoid in two brothers.Familial occurrence of malignant carcinoid is rare (about 3%). Authors describe occurrence of the malignant carcinoid in two brothers. In the older one the diagnosis was estimated in 1991. He had multiple intestinal carcinoid tumor with multiple liver metastases histological type III by Soga classification. Patient is intermittently treated with somatostatin analogue--lanreotid and with interferon alfa. By this therapy the disease is stabile. In the younger of brothers the diagnosis was estimated in 1999. The disease had rapid progression and in ten months patient died despite of the therapy. Definitive diagnosis was a malignant neuroendocrine tumor of pancreas-mixed low differentiated carcinoid with calcitonin overproduction. (Fig. 4, Ref. 15.)- - - - - - - - - - ranking = 140.93392812053keywords = endocrine (Clic here for more details about this article) |
7/51. Extraskeletal Ewings sarcoma of the small bowel: case report and literature review.Extraskeletal Ewings sarcoma is a tumour of neuroectodermal origin sharing close similarities with Ewings sarcoma of bone.We report the case of a 21 year old 16 week pregnant woman presenting with vomiting and weight loss and found to have an extraskeletal Ewings sarcoma of the small bowel. In a review of the literature there are no previous reports of extraskeletal Ewings sarcoma occurring in the small bowel. The diagnosis of extraskeletal Ewings sarcoma and the complicated management of a young pregnant woman with a malignant tumour are described.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
8/51. Small intestinal bleeding, secondary to metastatic malignant fibrous histiocytoma of the rib: report of a case.Malignant fibrous histiocytoma (MFH) is an aggressive bone tumor with a high propensity for metastasis. We describe herein the case of a 73-year-old man found to have MFH of the rib with a metastasis in the small intestine. The patient complained of gastrointestinal bleeding as the first symptom. Computed tomography imaging demonstrated a tumor in the left chest wall, and abdominal magnetic resonance imaging revealed thickening of the wall in the internal cavity of the small intestine. Histopathological and immunohistochemical examination confirmed that the tumor in the small intestine was an MFH that had metastasized from the rib.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
9/51. A hemophagocytic syndrome-like condition after emergency colectomy for perforated colon cancer: report of a case.Hemophagocytic syndrome is a rare but often fatal condition, and little is known about why this disorder can occur following surgery. We report herein the case of a patient successfully treated for a hemophagocytic syndrome-like condition that developed after emergency right hemicolectomy for a retroperitoneal abscess secondary to perforated colon cancer. The 62-year-old man initially presented after the sudden development of severe right back pain, and computerized tomography scans revealed a retroperitoneal abscess continuous with a tumor in the ascending colon. An emergency right hemicolectomy was subsequently performed. On postoperative day (POD) 2, his blood platelet count suddenly dropped to 1 x 10(4)/microl and histological examination of a bone marrow specimen taken on POD 5 showed abnormal histiocytes that had phagocytosed not only megakaryocytes, but also erythrocytes and leukocytes, and a normocellular marrow with a normal number of megakaryocytes. Hemophagocytic syndrome was suspected, and predonine was administered. The patient's condition improved remarkably and he was discharged on POD 51.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
10/51. Diffuse neuroendocrine tumor of the small bowel: an exceptional case with long survival and literature review.Malignant tumors of the small intestine are uncommon. Carcinoid tumors represent 20% of all malignancies occurring in this segment. We report the case of a 53-year-old female who was treated surgically for intestinal obstruction secondary to carcinoid tumors diffuse to the small intestine. This is the first case described in the literature. Carcinoids are considered less aggressive than the more common intestinal adenocarcinomas, but because of the extensive localization of the neoplasm this case can be considered a high-grade malignancy with an aggressive pattern of growth. Surgical resection, although noncurative in this case, can provide the patient with a long survival rate and a good quality of life.- - - - - - - - - - ranking = 563.73571248211keywords = endocrine (Clic here for more details about this article) |
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