| A patient with the classic lesions of pyoderma gangrenosum was found on autopsy to have a carcinoid tumor of the ileum. The simultaneous occurrence of a carcinoid tumor with pyoderma gangrenosum is interesting because both conditions are associated with immunologic defects. ( info) |
2/662. Natural killer cell lymphoma of the small intestine: diagnosis by flow cytometric immunophenotyping of paracentesis fluid. Natural killer (NK) cell lymphoma is a mass-forming neoplasm of putative NK cell lineage that typically appears in extranodal locations and has the following immunophenotype: CD2 positive, surface CD3 negative, cytoplasmic CD3 positive, and CD56 positive. We report a case of small-intestinal NK cell lymphoma that was originally diagnosed as an enteropathy-associated t-cell lymphoma based on paraffin immunohistochemistry. However, subsequent flow cytometric immunophenotyping of paracentesis fluid resulted in the correct diagnosis. We describe the case to illustrate the usefulness of this technique, which has not previously been described in such a case. ( info) |
3/662. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology. ( info) |
4/662. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis. A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed. ( info) |
5/662. rhodotorula rubra fungaemia in an immunosuppressed patient. We report the case of a patient who, following surgical removal of an extensive tumour of the bowel, developed fungaemia. The yeast was isolated from repeated blood and urine cultures and identified as rhodotorula rubra on the basis of macroscopic and microscopic features. Following treatment with amphotericin b, the patient's condition improved and the cultures became sterile. ( info) |
6/662. 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. ( info) |
7/662. Poorly differentiated adenocarcinoma with extensive rhabdoid differentiation: clinicopathological features of two cases arising in the gastrointestinal tract. This paper describes the pathological features of two cases of composite extra-renal rhabdoid tumor; that is, poorly differentiated carcinoma with extensive rhabdoid differentiation, arising in the small intestine of a 64-year-old man and in the pancreas of a 77-year-old woman. In both cases the tumor behaved aggressively and the patients died from disseminated malignancy within 3 months of original presentation and diagnosis. To the best of our knowledge these are the first examples of such tumors in the small intestine and pancreas. ( info) |
8/662. intussusception in the older child- suspect lymphosarcoma. Examination of the records of 378 children with intussusception at our institution revealed that 29 cases were caused by an identifiable intestinal lesion. A Meckel's diverticulum was the causative agent in 21 children, all of whom were under 2 yr of age. A previously undiagnosed ileal lymphosarcoma produced the intussusception in six other children, all between 6 1/2 and 9 yr of age. Our experience indicates that any child over 6 yr of age with the clinical findings of colicky abdominal pain, bloody stools, and a palpable mass plus the radiographic evidence of intussusception must be considered to have ileal lymphosarcoma until proven otherwise. Hydrostatic reduction of the intussusception must be accompanied by extensive small bowel reflux of barium in order to effectively rule out a small intestinal lesion. If this is not accomplished, surgery should be planned with the suspicion that a malignancy may be present. If this suspicion is confirmed by frozen section, the operation procedure should include wide surgical excision of the lesion along with the regional lymph nodes. ( info) |
| 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. ( info) |
10/662. Adenosquamous carcinoma of the small intestine. Report of a case and review of the literature. Primary adenosquamous carcinomas of the intestine are rare tumors, particularly those occurring in the small bowel. We report the third case of an adenosquamous carcinoma of the ileum in a 55-year-old-man. Histologically, the tumor consisted of malignant glandular and squamous elements. A review of the literature is presented. ( info) |