Traumatic Rupture of Jejunal Burkitt's Lymphoma with Intestinal Transection.

Burkitt's lymphoma (BL) is an aggressive non-Hodgkin lymphoma.[1] BL of the small intestine, presenting as a surgical emergency needing emergency laparotomy, is an uncommon presentation of this tumor. We present a case of BL presenting as perforation peritonitis after blunt trauma abdomen.

INTRODUCTION

Burkitt's lymphoma (BL) was first described in African children by Dennis Burkitt.[2] Etiology of BL is thought to be infestation with Epstein–Barr virus and chromosomal translocations of MYC gene on chromosome 8.[3] Three different clinical variants of BL have been described: endemic, sporadic, and immunodeficiency. The usual presentation of endemic variant is jaw swelling and cervical lymphadenopathy. The sporadic type presents as abdominal lump or intestinal obstruction. BL presenting as perforation peritonitis due to blunt trauma abdomen is a rare presentation.

CASE REPORT

Herein, we report a 10-year-old child who presented to casualty as perforation peritonitis after a blunt trauma abdomen resulting from road traffic accident. He had no significant past medical history. On examination, abdomen was grossly distended, with signs of peritonitis and features of shock. Baseline investigations were carried out including X-ray and ultrasonography (USG) of the abdomen. X-ray suggested gas under the diaphragm. USG abdomen revealed moderate free fluid in the peritoneal cavity. After initial resuscitation, the patient was subjected to emergency exploratory laparotomy which revealed a ruptured mass [Figure 1], moderate amount of blood and bile with complete transection of the distal jejunum. All the ruptured pieces of mass scattered throughout the abdomen were removed. The residual tumor attached with gut was resected and primary jejunojejunal anastomosis was done. The abdomen was closed without any drain after thorough peritoneal toilet. Postoperative period was uneventful and the patient was discharged on oral feed after 10 days.

Figure 1

Intraoperative picture showing ruptured tumor mass

Histopathological study of the resected tumor mass revealed tumor cells arranged in diffuse sheets admixed with numerous macrophages giving a “starry-sky” pattern [Figure 2] diagnostic of BL. The patient was later referred to medical oncologist where he received chemotherapy. On routine follow-up after 3 months, the patient was well.

Figure 2

Histopathology showing starry-sky appearance (H and E, ×40)

DISCUSSION

BL is an aggressive non-Hodgkin lymphoma (NHL).[1] Three different clinical variants of BL have been described: endemic, sporadic, and immunodeficiency. The endemic form is most commonly observed in Africa, with frequent involvement of the jaw and kidneys. In contrast, in other geographic areas, most patients present with abdominal tumors. Lymphoma constitutes 15%–20% of all small bowel neoplasms. The ileum is the most common site (60%–65%), followed by the jejunum (20%–25%), duodenum (6%–8%) and other sites (8%–9%). The clinical presentation of small intestinal lymphoma is nonspecific, and the patient may have symptoms, such as colicky abdominal pain, abdomen distention, nausea, vomiting, acute intestinal obstruction, or gastrointestinal bleeding. Preoperative diagnosis of intestinal lymphoma is very difficult. Diagnosis is made usually after surgery. In the present case, the patient was apparently asymptomatic. The blunt trauma abdomen due to road traffic accident resulted in complete transection of distal jejunum and rupture of the tumor mass. Signs of peritonitis and shock necessitated emergency exploratory laparotomy which revealed the pathology.

On reviewing English language literature, we could find a few cases of spontaneous tumor rupture resulting in shock[4] but none after blunt trauma abdomen.

The tumor-free survival has improved with intensive chemotherapy. Four-year event-free survival is up to 98% in localized or low-grade BL and in advance stage varies from 66% to 91%. In the previous studies, prognosis of perforated NHL was extremely poor.[5] In the presented case, chemotherapy was started after surgery and the patient was well on follow-up at 3 months.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.