Background: The purpose of the present paper was to carry out an audit of clinicopathological profile and treatment outcome in 13 children with functioning adrenal tumours.



Methods: The medical records of 13 children with functioning adrenal tumours who were managed between June 1990 and January 1999 were reviewed. Demographic data, clinical features, biochemical and localization studies, operative details and follow-up records were studied. Children with neuroblastoma were excluded.



Results: The mean age was 7.4 ± 5.3 years. Seven patients had Cushing’s syndrome (CS), two patients had virilizing tumours, three patients had phaeochromocytoma (PCC) and one patient had Conn’s syndrome. All patients (except one child with CS) were treated surgically. Two children with adrenocortical carcinoma (ACCa) died during the perioperative period. Histopathological diagnosis was adrenal cortical adenoma (ACAd) in four patients, ACCa in five patients and PCC in three patients. Two ACCa patients died of metastases at 12 and 14 months, respectively, while the third is alive and well at 30 months. Children with ACAd are alive and well at 91, 56, 32 and 27 months postoperatively. Children with PCC are free of disease (normal urinary metanephrines) at 63, 18 and 8 months after surgery but require antihypertensive drugs in low doses.



Conclusion: The outcome of surgery is good in cases of ACAd and PCC. Although outcome is poor in ACCa, surgery remains the mainstay of treatment and offers good palliation.

Functioning adrenal tumours in children and adolescents: An institutional experience

Summary Duplications o f gastrointestinal tract are unusual anomalies, especially in the colon. We report a case with two colonic duplications in a 20 year old woman who complained o f recurrent backache associated with left lower quadrant abdominal pain. Neither physical examination nor laboratory test alterations were detected. Roentgenographic examination showed organic scolinosis, no intestinal gas and a mass with central hyperdensity and thickned wall in the descending colon area on the CT scan. Laparotomy was performed in which 22 cm o f ascending colon and terminal ileum sited on left side, was excised. Pathological study revealed two unrelated and cystic duplications without communication to intestinal lumen. Their epithelial lining were o f colonic or gastric types and showed a mucinous adenoma; the remaining layers were normal except for eosino philic inflammatory infiltrate with CharcotLeyden like crystaloids. In conclusion, we present a double spherical colonic duplication type I associated to a mucinous adenoma on a non-rotational ascending colon in a patient with organic scoliosis.

Double duplication in a nonrotational colon. Study of a case associated with mucinous adenoma.

Introduction Colonic duplication is an uncommon congenital anomaly. Bowel obstruction, presence of an abdominal mass, and sometimes bleeding and perforation may be the clinical manifestations. Objective To report a patient with colonic duplication who underwent to surgical treatment due to acute abdominal pain. Report of the case 17 year-old, female patient admitted to the emergency room due to acute abdominal pain and signs of intestinal obstruction, at her initial evaluation she was tachycardic, hyperthermic with 38 degrees C, dehydration, abdominal distention, acute abdominal pain, absent peristalsis and peritoneal signs. Laboratories with Hb 14.2 g/dL, leucocytosis of 16,000 mm3, plain abdominal films showed dilation of intestinal loops and air-fluid levels. A laparotomy for intestinal obstruction was performed; operative findings were a tubular colonic duplication that demands a complete resection of the duplicated segment. Conclusion Colonic duplication is a rare congenital anomaly, the onset of the symptoms can occur during infancy or early childhood. It must be consider as differential diagnoses in patients with intestinal obstruction and palpable abdominal mass in this rank of ages.

Sanchez D

Papers

Colonic duplication in an adult.