Intestinal ganglioneuromatosis with peri-intestinal neurofibroma limited to the gastrointestinal tract mimicking Crohn’s disease

Abstract

S S141 characteristic. As patients with TDT-negative T-ALL/LBL tend to show worse outcome, it is important to remember that lack of TDT expression is expected in subsets of T-ALL/LBL. This pitfall should not preclude a correct diagnosis. 71. INTESTINAL GANGLIONEUROMATOSIS WITH PERI-INTESTINAL NEUROFIBROMA LIMITED TO THE GASTROINTESTINAL TRACT MIMICKING CROHN’S DISEASE Sushmitha Malpe Gopal, Anuradha C.K. Rao, Ranjini Kudva, Rajgopal Shenoy 1Department of Pathology, Melaka Manipal Medical College, and 2Department of Pathology and Surgery, Kasturba Medical College, Manipal, Manipal University, Karnataka, India Introduction: Ganglioneuroma is a benign neurogenic tumor composed of ganglion cells, nerve fibers and Schwann cells. Intestinal ganglioneuromas are rare tumors more often identified in the adults as solitary polypoidganglioneuromas, ganglioneuromatous polyposis and diffuse ganglioneuromatosis, presenting with abdominal pain, diarrhoea, thickening of the bowel wall and stricture formation. Diffuse ganglioneuromatosis is typically associated with multiple endocrine neoplasia, neurofibromatosis type 1 and Cowden syndrome. In this case report, we describe a case of diffuse and poylpoidganglioneuromatosis along with peri-intestinal neurofibroma in a 30 year old female with no syndromic afflictions. Case report: 30 year old female was investigated for recurrent alternating episodes of constipation and diarrhoea since 15 days associated with abdominal distension and palpable mass per abdomen. On CECT abdomen and pelvis, multifocal strictures with subacute intestinal obstruction was noted with possibilities of Crohns disease or tuberculosis. Surgically resected terminal ileum and ascending colon showed multiple linear ulcers, pseudopolyps and cobblestone appearance. On histopathological examination of the resected specimen, diagnosis of intestinal ganglioneuromatosis (polypoid and diffuse) with suture granuloma and peri-intestinal neurofibroma was given. History elicited for similar prior complaints showed nonspecific ileocolitis on biopsy 2 years earlier. She also gave history of surgery for resection and anastomosis 6 years back. Conclusion: We report a patient with intestinal ganglioneuromatosis in whom clinical and radiological findings mimicked those of Crohn’s disease/tuberculosis. Despite its rarity, intestinal ganglioneuromatosis should be considered in patients with findings of Crohn’s disease/tuberculosis on radiological investigations who have intractable symptoms despite medical treatment. 72. HISTOPATHOLOGICAL STUDY OF UPPER GASTROINTESTINAL ENDOSCOPIC BIOPSY IN PATIENTS WITH DYSPHAGIA AND DYSPEPSIA IN SOUTH WESTERN COAST OF INDIA Sushmitha Malpe Gopal, G.N. Srinivas, Rashmi Vaidya 1Melaka Manipal Medical College, Manipal University, Manipal, 2SRL Diagnostics, Mysore, and 3AJ Institute of Medical Sciences, Mangalore, India Background: Patients with non-neoplastic and neoplastic lesions of upper gastrointestinal tract (GIT) exhibit important alarming symptoms like dysphagia and dyspepsia. Upper gastrointestinal endoscopic biopsy is an effective and appropriate investigation to assess these patients. Aims: The aim of this study is to identify various lesions of the upper GIT in patients presenting with dysphagia and dyspepsia along with endoscopic and clinical correlation. For early identification of malignant lesions, screening of premalignant lesions and to differentiate non neoplastic lesions from neoplastic lesions and thus help in its appropriate management. Method: 200 consecutive endoscopic biopsy samples received in histopathology laboratory in and around Mangalore, India for a period of two years was subjected for histopathological examination. All the samples received in the histopathology laboratory sent in 10% formalin was processed, haematoxylin and eosin staining was done. Special stains were done wherever necessary. Results: 80% of the patients presented with dyspepsia. The most common lesion encountered in the oesophagus was malignant lesions followed by premalignant lesions, benign neoplasms and non-neoplastic lesions. The most commonly encountered gastric lesion were non neoplastic lesions followed by malignancy, premalignant lesions and benign neoplasm. Among the duodenal biopsies, non-neoplastic lesions were most common followed by malignancy. Conclusion: The fibreoptic diagnostic upper GI endoscopic biopsy is relatively less invasive, simple, safe, well tolerated procedure, cost effective and provides good diagnostic yield in confirming various upper GIT lesions. There is concordance of endoscopic biopsy findings with post biopsy resected specimens. In routine clinical practice, histopathology is the ‘gold standard’ for the definitive diagnosis of various lesions. 73. INTRAVASCULAR HISTIOCYTOSIS (IVH): A CASE REPORT K. Manoharan, B. Dutta Department of Anatomical Pathology, Laverty Pathology, NSW,