A 21-year-old female, white race, native resident in western Europe and with no relevant personal history, was admitted with a severe, progressively worsening, abdominal pain lasting for 7 days. She denied vomits, weight loss or alterations in bowel movements. No other extraintestinal symptoms were present. Laboratory tests only revealed mild leukocytosis. Abdominal ultrasonography suggested an abdominal mass in the right flank. Abdominal CT showed a colonic intussusception with cecum, ascending colon, fat and mesenteric vessels (intussusceptum) invaginated into the transverse (intussuscipiens) (Fig. 1); within this area a nodular mass with 4cm was identifiable. After a multidisciplinary discussion a colonoscopy was proposed. It revealed a significant narrowing in the transverse and ascending colon and a congestive and extensively ulcerated pseudopolypoid mass with 4cm in the cecum (Fig. 2). Forceps biopsies were performed. After clinical improvement and before a histological evaluation was available, the patient was referred to our outpatient department. Nevertheless, 8 days after the discharge, she recurred to the emergency department with a similar abdominal pain. An intussusception relapse was again suggested by abdominal ultrasonography. A surgical approach was then decided and a right hemicolectomy performed. Histological evaluation revealed a cecal lymphoproliferative disorder constituted by medium-size cells with high proliferative and apoptotic activities (Fig. 3). Immunostaining revealed CD3 and CD20 positivity (Fig. 4) and CD23, CD30, B cell leukemia/lymphoma 2 (bcl-2), ciclin D1 and Pax 5 negativity. These features were compatible with a Burkitt lymphoma. The patient was then referred to the hemato-oncology department.
Burkitt lymphoma is an aggressive non-Hodgkin B-cell lymphoma related to the translocation and deregulation of the c-myc gene.1
Burkitt lymphoma is rare in Western Europe and North America and it is usually diagnosed in children and young adults. Currently there are three types of Burkitt lymphoma: sporadic, endemic and immunodeficiency-related. Their epidemiology, clinical presentation and genetic features are different. Endemic variant, which is strongly related to Epstein–Barr virus infection, is mainly present in Africa and affects children. Sporadic variant is more common in Europe and United States and it is also more common in children. It rarely affects adult patients. Immunodeficiency-related Burkitt lymphoma occurs mainly in HIV-positive patients. A male predominance occurs in all variants.2
Clinical presentation differs between variants. Endemic variant usually involves facial bones, namely the maxilla, jaw and orbit with a rapid spread to extranodal sites including kidneys, breasts, bone marrow and meninges. Sporadic variant often has an abdominal presentation with bowel obstruction or gastrointestinal bleeding. Distal ileum, cecum, stomach and mesentery are frequently involved. Immunodeficiency-related Burkitt lymphoma often presents with lymph node involvement.3
Although Burkitt lymphoma variants comprise some differences, histological features are similar. Tumor cells are usually monotonous, medium-sized, nuclei with multiple nucleoli and a basophilic cytoplasm. High rates of proliferation and apoptotic activities are typical. A “starry-sky” pattern is characteristic and refers to numerous macrophages ingesting apoptotic tumor cells. Immunostaining also contributes for the diagnosis. Burkitt lymphoma cells lack CD5, bcl-2 and CD23. In contrast they present positivity for CD19, CD20, CD22, CD10 and CD43.4
A definitive diagnosis of intestinal Burkitt lymphoma is usually achieved after histological evaluation of the surgical specimen. Nevertheless, endoscopy may achieve the diagnosis in some cases. Chemotherapy is the mainstay treatment for all variants of Burkitt lymphoma.5 Surgical approach may be recommended in selected cases, namely for symptoms relief or to avoid complications during chemotherapy.6
Intussusception consists in a prolapse of a proximal bowel segment into a distal segment. It is much more common in children than in adults (20:1). Among children intussusception is idiopathic in 90% of the cases. In contrast, a causal lesion is identified in 90% of the cases occurring in adults.7 Tumors, colonic diverticulum or Meckel's diverticulum may cause intussusception. Bowel obstruction with abdominal pain, vomiting and often with a palpable mass is the most common clinical presentation. Currently, diagnosis is mainly based on ultrasonography and/or computed tomography.8 Although a temporary relief of intussusception may be achieved by endoscopy, a surgical approach is recommended for a definitive resolution. Moreover, considering the existence of a leading point, the surgical approach is advisable.9
Authors describe a case of a white adult female with recurrent cecocolic intussusception caused by an intestinal Burkitt lymphoma. Colonoscopy achieved a temporary relief of the intussusception. Nevertheless, an early intussusception relapse occurred and a definitive surgical approach was performed.
