Gastrointestinal Burkitt lymphoma (BL) is a highly aggressive malignancy in childhood, and early treatment is critical for its favorable prognosis. Ultrasonography is a widely accepted initial imaging workup; therefore, recognition of the sonographic features of BL should contribute to its early diagnosis and initiation of treatment. We present a 4-year-old boy with primary jejunal BL with intussusception mimicking presentation, in which initial abdominal US allowed sustainable detection and characterization of the intestinal lesion. Jejunotomy was performed and histopathological analysis revealed a ‘starry sky’ pattern and c-myc split signals characteristic of BL. The patient remains disease-free following chemotherapy. INTRODUCTION CASE REPORT Burkitt lymphoma (BL) is a highly aggressive cancer and the A previously healthy 4-year-old boy presented with a 10-day his- most frequent subtype of non-Hodgkin’s lymphoma in child- tory of intermittent abdominal pain, and 1-day history of nausea hood. The gastrointestinal tract, especially the terminal ileum, and vomiting. On examination, the patient had no fever and the cecum and appendix, are frequent locations of extranodal abdomen was soft with slight distension. Supine abdominal radio- lesions of the sporadic BL found mostly in non-African patients graph demonstrated gaseous distension of small bowel loops in . The lesions also are reported occasionally as causes of left-upper abdomen (Fig. 1a). Focused sonography was performed pathological lead points (PLP) of intussusception . Early using a Toshiba Aplio 400 PVT-375BT transducer with the pediat- imaging diagnosis of PLPs is, of course, crucial for the choice of ric abdominal setting at a 5-MHz frequency. This revealed a cir- reduction procedures, but also contributes to the prompt surgi- cumscribed area of homogenously low echogenicity without wall cal diagnosis and early treatment of aggressive malignant dis- stratiﬁcation in the submucosal area of the intestine (Fig. 1b). eases such as BL. Here, we report a case of primary jejunal BL Power Doppler sonography detected abundant blood ﬂow signals with intussusception mimicking presentation, in which initial in the same area (Fig. 1c). These ﬁndings prompted a strong suspi- abdominal ultrasonography (US) allowed sustainable detection cion of intestinal malignant lymphoma. The size of the lesion was and characterization of the intestinal lesion. ~20 × 5mm .A ‘target pattern’ was observed in the adjacent Received: February 27, 2018. Accepted: April 24, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact firstname.lastname@example.org Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy090/4995819 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 T. Okamoto et al. DISCUSSION (d) (a) (b) Abdominal manifestation with intussusception is one of the major presentations of BL in childhood . However, in a recent study, only three cases of BL were identiﬁed in 543 pediatric intussusception cases , which suggests that BL is actually rare as a cause of pediatric acute abdomen. US is a widely (c) (e) accepted initial imaging workup; therefore, recognition of the sonographic features of BL should contribute to its early diag- nosis and treatment induction. In this case, that it is a (i) cir- cumscribed [6, 7], (ii) submucosal uniformly hypoechoic mass [8, 9], (iii) without wall stratiﬁcation  and (iv) abundant blood (f) (g) ﬂow  signs matches the crucial sonographic characteristics for intestinal BL, which corresponds histologically to diffuse inﬁltration of lymphoma cells in intestinal submucosal layers. Those sonographic signs are rather clearly discernable in small-sized tumors, which is not the case with CT scanning detection as shown in this case. In the case of a relatively large-sized tumor, previously reported US signs such as a ‘doughnut sign’,ora ‘pseudo-kidney sign’  might also pro- Figure 1: (a) Supine abdominal radiograph at initial presentation demonstrates vide a clue to the diagnosis. In addition, as shown in this case, a bowel gas pattern with gaseous distension of the left-upper small bowel the imaging feature of sporadic gastrointestinal mass with the loops. (b) Focused sonography of the abdomen demonstrates a well- lack of nodal disease will different BL from other forms of circumscribed area of low echogenicity in the lumen of the intestine, (c) and lymphoma. The physician should consider the beneﬁts of ini- power Doppler sonography detected abundant blood ﬂow signals in that area. The lesion was ~20 × 5mm in size. (d)A ‘target pattern’ was observed in the tial sonographic imaging not only for solid organs, but for the adjacent intestinal loops, which suggested intussusception. (e) CT scanning detection of intramural lesions. conﬁrmed an intestinal intraluminal mass in the left-upper quadrant (white arrowhead). (f) Freshly exophytic tumor bulging into the jejunal lumen, involv- ing half the circumference of the wall. (g) The tumor is composed of scattered pale tiny body macrophages on a dense background of monomorphic, CONFLICT OF INTEREST STATEMENT intermediate-sized lymphocytes, creating a ‘starry sky’ pattern (lower left). The authors declare no conﬂict of interest. intestinal loops, and therefore intestinal intussusception with PLP REFERENCES was suspected (Fig. 1d). Subsequently, computed tomographic (CT) scanning of chest, abdomen and pelvis conﬁrmed an intes- 1. Biko DM, Anupindi SA, Hernandez A, Kersun L, Bellah R. tinal intussusception in the left-upper quadrant (Fig. 1e), but no Childhood Burkitt Lymphoma: abdominal and pelvic intraluminal mass was evident on US. There was no evidence of imaging ﬁndings. Am J Roentgenol 2009;192:1304–15. lymphadenopathy. Laboratory values showed an elevated level of 2. Navarro O, Daneman A. Intussusception Part 3: diagnosis soluble IL-2 receptor (sIL2R) of 854 U/ml. 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Journal of Surgical Case Reports – Oxford University Press
Published: May 14, 2018
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