Primary gastrointestinal tract lymphoma in childhood.

Abstract

We have recently reviewed the experience of the Hospital for Sick Children, Toronto, The Princess Margaret Hospital, and the Department of Radiotherapy, Toronto General Hospital, with malignant lymphoma in childhood (patients less than 16 years of age at time of diagnosis) (1, 2). Of 196 children who presented in the years 1930 to 1965, 75 had Hodgkin's disease, and 121 had lymphosarcoma, reticulum-cell sarcoma, or other malignant lymphoma. In Hodgkin's disease no child presented with the primary site in the gastrointestinal tract, but of the 121 children with other malignant lymphomas (Table I) 56 presented with abdominal symptoms and signs. Of these, 55 required a laparotomy to establish the diagnosis. The laparotomy findings in 40 cases indicated the primary site of origin to be gut. In one instance the lesion has been reclassified as pseudolymphoma of the small intestine (3). Ten of the remaining 39 cases have previously been reviewed (4). We present this analysis because a marked improvement in survival is apparent in our recent experience with this uncommon and usually fatal tumor. Clinical Data (a) Age and Sex: The age distribution is seen in Figure 1. There is a peak incidence in the age range of 5 to 8 years. Boys outnumbered girls in a ratio of 9:1. The age distribution of the survivors follows the incidence pattern. (b) Mode of Presentation: These children presented with abdominal pain and vomiting, most often as episodes of small bowel obstruction of greater or less degree. In over half of the cases a definite mass was palpable in mid or right abdomen. Fifteen children presented with intussusception and one with a perforated viscus. The diagnosis of primary malignant lymphoma of gut is not easily made preoperatively. Eight of our children had had prior surgery, 2 for reduction of an intussusception and 6 on a diagnosis of appendicitis or appendiceal abscess, without the lymphomatous nature of their disease being appreciated. (c) Stage at Diagnosis—Early Stage: In 20 children the laparotomy findings indicated that the lymphoma was limited to a segment of gut plus or minus the associated mesenteric nodes only. This group is defined as having “early stage” disease. Late Stage: In 19 children the disease was more extensive. Most often, spread was to the para-aortic and retroperitoneal nodes by implants and plaques in mesentery or peritoneum, or by direct infiltration of structures adjacent to the primary tumor. In all these children the primary involvement of gut dominated the clinical picture at laparotomy. This group of patients is defined as having “late stage” disease. (d) Primary Site: In 35 of the 39 children the primary site was in the lower ileum, cecum, appendix, ascending colon, or some combination of these sites (Table II). Treatment (a) Surgery: The diagnosis is made at laparotomy, often for an acute abdomen at a time when malignant tumor is not anticipated.