Pediatric caecal lymphoma involving ovary in a 12 year old girl - A bad prognostic sign: A rarity

Primary caecal lymphoma or the colonic lymphoma is a rare tumor of the gastrointestinal (GIT) tract and comprises only 0.2-1.2% of all colonic malignancies, both in adults and pediatric age group. The most common variety of colonic or caecal lymphoma is Non-Hodgkin’s Lymphoma (NHL) which arises from the lymphoid elements of the intestine. GIT is the most frequently involved site, accounting for 50-60% of all extra nodal lymphomas, and most of them are NHL. In adults, the stomach is the most common location of GIT lymphomas, followed by the small intestine, but the most common GI site of NHL in children is the terminal ileum and the ileo-caecal region. Diagnosis is difficult since lymphoma presents with vague abdominal pain with loss of weight and appetite. It may present as lump abdomen with complications such as intestinal obstruction, bleeding, perforation and peritonitis and intussusseption. Histologically it is B or T cell type with small or large cell variation, but frequently encountered is diffuse large B- cell lymphoma (DLBCL). DLBCL has low incidence but favorable outcome in young adults, lesions localized to one area or organ and children below 5 years of age, but has an aggressive course in children between 10-15 years of age and also in adults above 55 years of age. DLBCL or GIT Lymphomas in general have male preponderance. We present a rare case of caecal lymphoma (DLBCL) involving appendix and right ovary in a 12 year old girl who presented with vague symptoms of abdominal pain and abdominal mass. Clinically and radiologically, provisional diagnosis of Ileo-caecal tuberculosis with possibility of adhesions leading to a mass lesion was considered. Histopathological examination (HPE) revealed the diagnosis and prognosis of the case.