1/31. Diffuse schwannoma involving the entire large bowel with huge extramural development: report of a case.Schwannoma of the large bowel is a rare clinical entity, which has reportedly been recognized to arise from one place with a submucosal tumor morphology. We present herein the unique case of a 25-year-old woman who suffered from a schwannoma diffusely involving the entire large intestine. The patient complained of abdominal distension and imaging studies revealed a giant tumor occupying the whole abdomen, but no confirmed preoperative diagnosis could be made. A laparotomy proved the huge tumor detected preoperatively to be the markedly wall-thickened entire large bowel itself due to the diffuse extramural development of a neoplasm, but no other organs were involved. biopsy specimens from the tumor were histologically diagnosed as benign schwannoma. However, because of the possibility of malignancy, we later performed a total proctocolectomy followed by an ileal J-pouch-anal canal anastomosis. The final pathological diagnosis was also that of a benign schwannoma originating from the large intestine. The patient remains well without recurrence 15 months after surgery. To the best of our knowledge, no such case of a schwannoma in the entire large bowel has yet been reported in the literature, and the pathogenesis of its occurrence remains unknown.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/31. T cell lymphoma involving the graft of a multivisceral organ recipient.Posttransplant lymphoproliferative disorders are typically of B cell origin, whereas T cell lymphomas have been rarely documented. We present a case of a non-Hodgkin's T cell lymphoma involving the intestinal graft of a multivisceral transplant patient. The patient was a 7-year-old girl who underwent at age 5 a multivisceral transplant secondary to short gut syndrome. Baseline immunosuppressive therapy consisted of FK506, methylprednisone, and mycophenolate mofetil. At 2 years posttransplant she presented with fever, diarrhea, nausea, and vomiting. Multiple endoscopic biopsies revealed a severe intensity, diffuse and focally nodular lymphocytic infiltrate composed predominantly of small, monomorphic lymphoid cells with scattered plasma cells and abundant eosinophils. Immunohistochemically, the majority of the lymphoid cells expressed the pan T cell marker CD3. Southern blot analysis revealed rearrangement of the T cell receptor beta chain gene, with germline configuration of the heavy immunoglobulin chain gene, confirming a clonal T cell genotype. in situ hybridization for Epstein Barr virus revealed rare positive lymphoid cells, that were negative with CD3 by immunohistochemical staining. A detailed clinico-radiological work-up revealed no other sites of involvement by the lymphomatous process. After the diagnosis of posttransplant lymphoproliferative disorder, immunosuppression was reduced with a subsequent partial improvement in the endoscopic appearance of the graft and a focal decrease in the lymphocytic infiltrate seen in the follow-up biopsies. Repeat gene rearrangement studies demonstrated germline configuration of both the T cell receptor beta chain gene and the heavy chain immunoglobulin. gene. To our knowledge, this represents the first description of a T cell lymphoma affecting the intestinal allograft of a multivisceral transplant patient.- - - - - - - - - - ranking = 4keywords = organ (Clic here for more details about this article) |
3/31. Small bowel metastases of malignant melanoma: palliative effect of surgical resection.Malignant melanoma shows an unusual predilection to metastasize to the small intestine. Three patients with malignant melanoma involving the small bowel are reported. One patient was operated on for small bowel obstruction and the other two for gastrointestinal bleeding. Two patients remained well 6 and 2 years, respectively, after surgery. One patient died of metastatic melanoma 4 years post-operation. Metastatic melanoma in the small bowel should be suspected in any patient with a previous history of malignant melanoma who develops GI symptoms or chronic blood loss. Surgical treatment was the first choice; the prognosis after surgical resection was much better than for other organ metastases or simultaneous metastases of the small bowel and other organs.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
4/31. Malignant schwannoma of the stomach in a patient with von Recklinghausen's disease.A patient with von Recklinghausen's disease died with a malignant schwannoma of the stomach and was found at autopsy to have neurofibromatosis of the gastrointestinal tract, a plexiform neurofibroma of the myocardium and a phaechromocytoma. Malignancy of the gastrointestinal tract in von Recklinghausen's disease is rare, and this case highlights the difficulties in histological diagnosis of malignant nerve sheath tumours.- - - - - - - - - - ranking = 0.27616577528535keywords = nerve (Clic here for more details about this article) |
5/31. Epstein-Barr virus-associated T-lymphoproliferative disease with hemophagocytic syndrome, followed by fatal intestinal B lymphoma in a young adult female with WHIM syndrome. warts, hypogammaglobulinemia, infections, and myelokathexis.A rare association of Epstein-Barr virus-associated T- and B-lymphoproliferative disease (EBV( ) T- and EBV( ) B-LPD) in a patient with WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is reported. A 26-year-old Japanese female, who had been treated for WHIM syndrome since early childhood, developed hemophagocytic syndrome associated with EBV( ) T-LPD at the lymph nodes and spleen. The disease rapidly resolved in response to prednisolone therapy. However, 6 weeks later, fatal EBV( ) B lymphoma unresponsive to chemotherapy occurred in the intestine and other organs. Caution must be exercised that the patient with WHIM syndrome may be at risk for EBV-LPD.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/31. Extranodal multiple involvement of enteropathy-type T-cell lymphoma without expression of CC chemokine receptor 7.Enteropathy-type T-cell lymphoma (ETCL) is a rare extranodal lymphoma that tends to disseminate into the intestines and other extranodal organs. We present a case of ETCL with involvement of the lungs and kidneys and report CC chemokine receptor 7 (CCR7) expression of lymphoma cells. A 73-year-old man was admitted to the hospital with a complaint of abdominal pain. Multiple ulcers and perforations were observed in the small intestine, and partial resection of the ileum was performed. Histological examination of the resected specimen revealed diffuse proliferation of atypical large lymphoid cells. The diagnosis was ETCL with dissemination into the lungs and kidney. lymphoma cells of the small intestine and in pleural effusion were CD3 , CD4 , CD7 , CD8-, CD25-, CD56-, CD103 /-, and TIA-1 . Rearrangement of the T-cell receptor beta gene was detected, and human T-lymphotropic virus was not integrated. Combination chemotherapy did not result in a sustained response. The results for CCR7 expression of lymphoma cells in the lung and pleural effusion were negative. Therefore we concluded that lymphoma cells did not migrate into the lymph nodes but instead spread into the extranodal organs.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
7/31. Case of optic nerve involvement in metastasis of a gastrointestinal stromal tumor.BACKGROUND: The gastrointestinal stromal tumor (GIST) belongs to the leiomyosarcoma or leiomyoma group, and metastasis of leiomyosarcoma to the orbit is extremely rare. The most common sign of the metastasis of leiomyosarcoma to the orbit is proptosis; the primary lesion is usually found in skin or soft tissue. CASE: A 60-year-old man, who had a history of multiple metastases of GIST, suffered sudden unilateral blindness. OBSERVATIONS: His history and computed tomographic scans suggested that the lesion was a metastasis of GIST to the cavernous sinus around the root of the optic nerve. CONCLUSION: To the best of our knowledge, this is the first case of metastasis of GIST to the orbit.- - - - - - - - - - ranking = 1.3808288764267keywords = nerve (Clic here for more details about this article) |
8/31. Abdominal mass secondary to actinomyces infection: an unusual presentation and its treatment.Abdominal actinomycosis may appear as an abdominal mass and/or abscess. This mass can mimic a malignant tumour. The diagnosis and management of abdominal actinomycosis will be discussed through a review of the literature and a case report from our own institution. The patient was a 17-year-old boy who presented with abdominal discomfort and a palpable right lower quadrant mass defined on CT scan. He underwent en bloc resection of the mass for a presumed diagnosis of tumour of uncertain type with intestinal involvement. The diagnosis was reversed, when histology revealed filamentous organisms consistent with actinomyces. He was treated with high dose penicillin for several weeks and was discharged from the hospital taking penicillin orally. Preoperative diagnosis of abdominal actinomycosis is difficult. An accurate diagnosis is always obtained in a histological or microbiological examination, often requiring surgical resection. Recognition is important because successful treatment requires combined surgery and prolonged penicillin treatment.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/31. peutz-jeghers syndrome: case reports and update on diagnosis and treatment.OBJECTIVE: To analyze the etiology and diagnostic methods of peutz-jeghers syndrome (PJS) and thus establish a treatment strategy. methods: Clinical data from six patients with PJS were evaluated from the aspect of familial history, carcinogenesis and recurrence of polyps. RESULTS: The fathers of four and the mother of one of the six patients had PJS. The grandfather of three of the six patients had PJS. There was a history of cancer in three of the five families. Case 4 underwent two laparotomies for intussusceptions caused by recurrent polyps of the small intestine. Case 5 also had recurrent small intestinal polyps and required a laparotomy after 1 year of initial treatment. Polyps in cases 1 and 4 showed adenomatous changes and those in case 2 were associated with gastric cancer. CONCLUSIONS: patients with PJS have a strong family history of cancer and a high incidence of recurrence of small intestinal polyps. Malignant changes of polyps may follow the hamartoma-adenoma-carcinoma sequence. Careful follow-up is mandatory for gastrointestinal tract symptoms, and other solid organs that are susceptible to malignant change.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/31. Natural killer-like T cell lymphoma of the small intestine: report of a case.We report the case of a 77-year-old Japanese man with natural killer (NK)-like T cell lymphoma of the small intestine diagnosed after an emergency laparotomy for perforated peritonitis. Immunohistochemical staining of the tumor showed that the patient had CD3 CD8 CD30- CD56 CD68- CD79a- UCHL-1 EMA- LMP-1 NK-like T cell lymphoma. The patient had a history of hepatocellular carcinoma (HCC) and was also diagnosed with T cell non-Hodgkin's lymphoma associated with T cell receptor (TCR) reconstruction in the Jgamma chain. Intestinal T cell lymphoma is uncommon and very few cases of CD56 T cell lymphoma, otherwise known as NK-like T cell lymphoma, have been reported. The patient did not have a history of gluten-sensitive enteropathy (celiac disease). Multiple lesions appeared within months after the initial operation and his condition deteriorated rapidly. We think that this patient probably had NK-type granular lymphocyte-proliferative disorder (NK-GLPD) because the percentage of CD16 CD56 cells among peripheral blood mononuclear cells was elevated, at 21%. We report this case to help elucidate the relationship between underlying digestive organ disease and the development of intestinal NK-like T cell lymphoma. An accumulation of other such cases is needed to determine the etiology of this disease.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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