Figure 1
Neck ultrasound
Appearances can be deceptive: Burkitt lymphoma mimicking infectious disease in a child
SectionPaediatric radiology
Case TypeClinical Cases
AuthorsCassar Scalia A, Fiocchi F, Gibertini MC, Torricelli P
Connected authors
8 years, male
Clinical History
An 8-year-old boy was admitted to ED with fever, temporal headache and left cervical lymphadenopathy for two weeks. Physical examination and blood work-up were normal except for a left tonsillar phlegmon, which was incised before imaging. Given persistent symptoms, the patient was hospitalised for further investigations.
Imaging Findings
A chest X-ray was negative. Neck ultrasound demonstrated numerous enlarged lymph nodes, especially on the left, the largest measuring 4.3 x 1.3 cm (Fig. 1). Abdominal ultrasound showed numerous hypoechoic lesions in the hepatic and renal parenchyma, with peripheral vascularisation with Doppler interrogation (Fig. 2). No lymphadenopathy or splenomegaly was found.
At abdominal MR the hepatic lesions appeared mildly T2 hyperintense, T1 hypointense (Fig. 3), with high signal on diffusion-weighted imaging (DWI) (Fig. 4), and apparent peripheral enhancement with a central hypovascular component (Fig. 5). Neck MR showed a large solid mass arising from the left pharyngeal tonsil and extending to the rhinopharynx, nasal fossa and pterygo-palatine fossa, and reaching the prevertebral space (Fig. 6), highly suspicious of a malignant process.
Pharyngeal biopsy led to the final diagnosis of Burkitt lymphoma (BL), negative for t (8;14) translocation.
At abdominal MR the hepatic lesions appeared mildly T2 hyperintense, T1 hypointense (Fig. 3), with high signal on diffusion-weighted imaging (DWI) (Fig. 4), and apparent peripheral enhancement with a central hypovascular component (Fig. 5). Neck MR showed a large solid mass arising from the left pharyngeal tonsil and extending to the rhinopharynx, nasal fossa and pterygo-palatine fossa, and reaching the prevertebral space (Fig. 6), highly suspicious of a malignant process.
Pharyngeal biopsy led to the final diagnosis of Burkitt lymphoma (BL), negative for t (8;14) translocation.
Discussion
BL is an aggressive subtype of non-Hodgkin lymphoma with a doubling time of 24-48 hours [1]. In 90-95% of cases it is caused by translocation t(8;14), involving c-myc gene and immunoglobulin heavy-chain locus [2]. Male-to-female ratios range from 1.3:1 to 8.8:1; the median age of affected children is 8 years [3].
The WHO classification of BL describes three clinical variants: endemic, sporadic (the predominant type in non-malarial areas) and immunodeficiency-related [4].
The abdominal and pelvic region is commonly affected by BL, particularly by its sporadic form. The ileocaecal region, specifically Peyer’s patches, is the most common site involved along the gastrointestinal tract, because the terminal ileum has the highest concentration of lymphatic tissue [1].
EBV is found in almost all cases of endemic BL, while it is rarely associated with the sporadic type [4]. In our case EBV IgG search was negative.
Although primary involvement of solid organs is rare, secondary involvement of the liver, spleen, or kidney is not uncommon. Radiologic findings include organomegaly and single or multiple focal hypoechoic masses on ultrasound [1].
Even with its aggressive character, BL is curable and highly sensitive to chemotherapy. Thus, knowledge of proper imaging features is fundamental for early diagnosis and treatment [1].
Sonography plays an important role in differential diagnosis of lymph node swelling in children, caused in most patients by a diagnosable infectious disease [5, 6]. In our case the findings at neck ultrasound led us to consider possible infectious aetiology, considering the global clinical history (fever, phlegmon, cephalea) even if lymphomatous disease could not be excluded. Nonetheless the presence of concurrent multiple hypoechoic lesions in the hepatic and renal parenchyma suggested differential diagnoses such as multiple abscesses, parenchymal localisation of lymphoma and metastases of unknown origin.
Neck and pharyngeal infiltration and enlarged nodes are seen in 25% of children with BL; the primary mass usually appears isointense on T1-weighted MR images, hyperintense on T2-weighted images [7], with high signal on DWI and heterogeneous enhancement after contrast material administration.
Hepatic involvement in BL has been reported in approximately 17% of patients, presenting as hypoechoic lesions on ultrasound. Renal involvement of BL most commonly includes nephromegaly (90%), focal renal masses (30%) and hydronephrosis due to ureteral obstruction (50%). At ultrasound, the kidneys may show increased cortical echogenicity (60%) with or without iso or hypoechoic masses [3].
Our patient, after 6 months of chemotherapy, completely healed from BL and currently undergoes periodic follow-up examinations.
The WHO classification of BL describes three clinical variants: endemic, sporadic (the predominant type in non-malarial areas) and immunodeficiency-related [4].
The abdominal and pelvic region is commonly affected by BL, particularly by its sporadic form. The ileocaecal region, specifically Peyer’s patches, is the most common site involved along the gastrointestinal tract, because the terminal ileum has the highest concentration of lymphatic tissue [1].
EBV is found in almost all cases of endemic BL, while it is rarely associated with the sporadic type [4]. In our case EBV IgG search was negative.
Although primary involvement of solid organs is rare, secondary involvement of the liver, spleen, or kidney is not uncommon. Radiologic findings include organomegaly and single or multiple focal hypoechoic masses on ultrasound [1].
Even with its aggressive character, BL is curable and highly sensitive to chemotherapy. Thus, knowledge of proper imaging features is fundamental for early diagnosis and treatment [1].
Sonography plays an important role in differential diagnosis of lymph node swelling in children, caused in most patients by a diagnosable infectious disease [5, 6]. In our case the findings at neck ultrasound led us to consider possible infectious aetiology, considering the global clinical history (fever, phlegmon, cephalea) even if lymphomatous disease could not be excluded. Nonetheless the presence of concurrent multiple hypoechoic lesions in the hepatic and renal parenchyma suggested differential diagnoses such as multiple abscesses, parenchymal localisation of lymphoma and metastases of unknown origin.
Neck and pharyngeal infiltration and enlarged nodes are seen in 25% of children with BL; the primary mass usually appears isointense on T1-weighted MR images, hyperintense on T2-weighted images [7], with high signal on DWI and heterogeneous enhancement after contrast material administration.
Hepatic involvement in BL has been reported in approximately 17% of patients, presenting as hypoechoic lesions on ultrasound. Renal involvement of BL most commonly includes nephromegaly (90%), focal renal masses (30%) and hydronephrosis due to ureteral obstruction (50%). At ultrasound, the kidneys may show increased cortical echogenicity (60%) with or without iso or hypoechoic masses [3].
Our patient, after 6 months of chemotherapy, completely healed from BL and currently undergoes periodic follow-up examinations.
Differential Diagnosis List
Burkitt lymphoma with hepatic and renal nodular localisations.
Abscess
Lymphoma
Metastases
Final Diagnosis
Burkitt lymphoma with hepatic and renal nodular localisations.
References
[1] Derynkuyu BE, Boyunaga O, Oztunali C, Tekkesin F et al (2016) Imaging features of Burkitt lymphoma in pediatric patients. Diagnostic and Interventional Radiology 22: 95–100 (PMID: 26611257)
[2] Kiaei A, Onsori H, Alijani A, Andalib S, Ghorbian S, Sakhinia E (2016) Detection of t(8;14) c-myc/IgH gene rearrangement by long-distance polymerase chain reaction in patients with diffuse large B-cell lymphoma. Hematology/oncology and stem cell therapy 9, 141– 146 (PMID: 27352258)
[3] Biko DM, Anupindi SA, Hernandez A, Kersun L, Bellah R (2009) Childhood Burkitt Lymphoma: Abdominal and Pelvic Imaging Findings. American Journal of Roentgenology 192:1304-1315 (PMID: 19380555)
[4] Molyneux EM, Rochford R, Griffin B, Newton R, Jackson G, Menon G, Harrison CJ, Israels T, Bailey S (2012) Burkitt's Lymphoma. Lancet 379(9822):1234-44 (PMID: 22333947)
[5] Lang S, Kansy B (2014) Cervical lymph node diseases in children. Head and Neck Surgery 13:1865-1011 (PMID: 25587368)
[6] Ferrer R (1998) Lymphadenopathy: Differential Diagnosis and Evaluation. American Family Physician 58(6):1313-1320 (PMID: 9803196)
[7] Meuwly JY, Lepori D, Theumann N, Schnyder P, Etechami G, Hohlfeld J, Gudinchet F (2005) Multimodality Imaging Evaluation of the Pediatric Neck: Techniques and Spectrum of Findings. RadioGraphics 25:931–948 (PMID: 16009816)
Case information
| URL: | https://eurorad.org/case/15505 |
| DOI: | 10.1594/EURORAD/CASE.15505 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Abdominal ultrasound
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Figure 3
Abdominal MR (T1w and T2w)
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Figure 4
Abdominal MR (DWI)
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Figure 5
Abdominal MR (T1w after contrast administration)
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Figure 6
Neck MR (T1w and T2w)
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Abdominal ultrasound
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Numerous hypo-anechoic lesions in the renal (a) and hepatic (b) parenchyma, with peripheral vascularisation at colour Doppler examination (c).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR (T1w and T2w)
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Department of Radiology, University of Modena, Italy
Department of Radiology, University of Modena, Italy
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Department of Radiology, University of Modena, Italy
Department of Radiology, University of Modena, Italy
Abdominal MRI demonstrates hepatic and renal lesions hyperintense in T2-weighted sequences (a) and T2w fat sat (b), hypointense in T1-weighted ones (c: in phase, d: out of phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR (DWI)
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR demonstrates multiple hepatic and renal lesions with high signal in diffusion-weighted images (a: b value = 0, b: b value = 330, c: b value = 660, d: b value = 1000).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Abdominal MR (T1w after contrast administration)
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Hypovascular hepatic and renal lesions with apparent peripheral rim-enhancement in T1w images after contrast administration (a: no contrast; b: arterial phase; c: portal phase; d: late phase).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Neck MR (T1w and T2w)
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
Large solid mass with origin from the pharyngeal tonsil and extension to rhinopharynx, nasal fossa and pterygopalatine fossa reaching prevertebral space (a: axial T2w; b, c: axial and coronal T1w after contrast administration).
Department of Radiology, University of Modena, Italy.
Department of Radiology, University of Modena, Italy.
