|Year : 2015 | Volume
| Issue : 3 | Page : 133-135
Primary Burkitt lymphoma of kidney: A rare presentation in a child
Shilpi Agarwal, Gunjan Mahajan, Pallavi Prakhar Rana, Riti Yadav
Department of Pathology, Lady Hardinge Medical College, New Delhi, India
|Date of Web Publication||18-Sep-2015|
Department of Pathology, Lady Hardinge Medical College, Shaheed Bhagat Singh Marg, New Delhi - 110 001
Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.
Primary renal Burkitt lymphoma (BL) is an unusual occurrence in children. Although the prognosis of this tumor is poor, early diagnosis on biopsy confirmed by immunohistochemistry (IHC) and followed by chemotherapy improves survival of such patients. We present a case of 4-year-old male child with bilateral renal lump suggestive of lymphoma on computerized tomography. Renal biopsy and subsequent IHC was suggestive of bilateral BL in the kidney; following which the child was started on FAB LMB 96 based treatment protocol.
Keywords: Burkitt lymphoma, kidney, pediatric neoplasms, primary
|How to cite this article:|
Agarwal S, Mahajan G, Rana PP, Yadav R. Primary Burkitt lymphoma of kidney: A rare presentation in a child. J Appl Hematol 2015;6:133-5
| Introduction|| |
Primary renal Burkitt lymphoma (PRBL) is extremely rare. Only four cases have been reported in the world literature. The diagnostic criteria for primary renal Non-Hodgkin lymphoma suggested by Malbrain et al. include: (1) Renal failure as the initial presentation, (2) bilateral enlargement of the kidneys without obstruction and other organ or nodal involvement, (3) diagnosis made by renal biopsy, (4) absence of other causes of acute renal failure, and (5) rapid improvement of renal function after radiotherapy or systemic chemotherapy. Primary renal lymphoma (PRL) usually affects adults. In this report, we describe a child affected by primary Burkitt lymphoma (BL), bilateral kidney.
| Case Report|| |
A 4-year-old male child presented to our hospital with 2 months history of abdominal distension, abdominal pain, and fever. On examination, the patient had bilateral lower abdominal lump measuring 7–8 cm and extending from loin to the groin. These lumps were ballotable, and bilateral renal angle tenderness was present. The liver, spleen, and peripheral lymph nodes were not enlarged. Laboratory data disclosed a hemoglobin 10.1 g/dL, leukocyte count of 7180/µL (36% neutrophil, 60% lymphocyte, 02% monocyte, and 02% eosinophil), platelet count 2.46 lac/µL, and a hematocrit of 31.8%. Other laboratory data were as follows: Serum creatinine concentration 4 mg/dL, blood urea 181 mg/dL; and serum uric acid 15.6 mg/dL, serum sodium 140 mmol/L, serum potassium 3.8 mmol/L, serum calcium 9.0 mmol/L, serum phosphates 4.80 mmol/l, SGOT: 103 IU/L, SGPT: 18 IU/L, and ALP: 104 IU/L, serum protein level 6.3 g/L. Serology for HIV was nonreactive. Urinanalysis showed albumin 1+, no sugar, and 1–2 leukocytes/high power field. Urine cultures were negative. The chest X-ray showed bilateral pleural effusion, however, no mediastinal lymphadenopathy was present. Abdominal ultrasonography showed bilateral enlargement of the kidneys (17 cm) with the loss of the corticomedullary differentiation and hypoechoic mass within the bilateral renal parenchyma. Contrast-enhanced computed tomography (CECT) abdomen showed bilateral renal mass confined to the kidney with hyper and hypoechoic areas. No evidence of the involvement of other organs or lymph nodes was seen. CECT abdomen was suggestive of lymphoma, bilateral kidney. Flow cytometry of bilateral renal aspirate showed a discrete population of cells on CD45/side scatter gate with a bright expression of CD45. These cells showed moderate expression of CD10, CD20, and dim to moderate co-expression of CD19 and CD20. However, these cells were negative for CD34, TdT, CD3, and CD5. These cytometric findings were suggestive of non-Hodgkin lymphoma, B-cell type. Trucut biopsy of the bilateral renal mass was performed which showed similar morphology with diffuse infiltration of lymphoid cells between normal renal parenchyma [Figure 1]a and [Figure 1]b. These lymphoid cells were of medium size with scant to moderate amount of cytoplasm, squared off cell borders, round nucleus, coarse chromatin, and multiple small nucleoli. A "starry-sky" pattern was present [Figure 1]c. Many mitotic figures and apoptotic bodies were seen interspersed between the lymphoid cells. On immunohistochemistry (IHC), lymphoid cells were positive for CD20, CD10, Bcl6, and negative for CK, CD3, CD34, and Bcl2. These lymphoid cells had strong and homogenous Ki67 expression with an index of >95% [Figure 1]d. Furthermore these cells were strongly positive for c-myc on IHC [Figure 2]. Bone marrow was not infiltrated by atypical lymphoid cells. Hence a final diagnosis of primary BL, bilateral kidney was given. The patient was started on French-American-British/lymphoma malignancy B (FAB-LMB) 96 treatment protocol  with two induction cycles of COPADM (cyclophosphamide, vincristine, prednisolone, adriamycin, and methotrexate) and two consolidation courses of CYVE (cytarabine, etoposide). The renal function tests of the patient became normal and size of kidney reduced less than twice of initial before the start of the second cycle of chemotherapy. Presently, the patient is in the maintenance phase of treatment with improved general condition.
|Figure 1: (a) Diffuse infiltration of lymphoid cells between renal parenchyma (glomeruli); H and E ×400. (b) Diffuse infiltration of lymphoid cells between renal tubules; H and E ×400. (c) Monotonous population of medium-sized cells with squared off cell borders along with starry sky pattern; H and E ×1000. (d) These cells had a Ki67 index >95%|
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|Figure 2: The medium-sized cells were strongly positive for c-myc on immunohistochemistry; ×400|
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| Discussion|| |
Primary renal non-Hodgkin lymphoma (PRL) is exceedingly rare with only approximately 65–70 cases being reported and even rarer cause of acute renal failure in children. Most common reported cases of PRL are of diffuse large B-cell lymphoma (DLBCL). Nonetheless, only four cases of primary BL in the kidney are reported in the literature.,,,
The kidney is not a lymphoid organ. Hence, the very existence of lymphomas of the kidney was questioned by some investigators. The proposed pathogenetic mechanisms include: Origin in the subcapsular lymphatics, seeding via the hematogenous route, an extension of retroperitoneal disease or inflammatory disease with a lymphoplasmacytic infiltrate.
Renal imaging is not sufficient to diagnose lymphomatous infiltration of the kidney.,, A kidney biopsy was needed to establish the phenotype of lymphoma using IHC techniques. BL has overlapping cytological, histological, and IHC findings with DLBCL and lymphoblastic lymphoma (LBL). The differentiating features are discussed in [Table 1].
Sieniawska et al. did IHC for B and T cell markers to diagnose BL but c-myc was not done. Our case was positive for c-myc further supporting our diagnosis. Baran et al. diagnosed BL in kidney based on computed tomography findings along with a cytological examination of intra-abdominal fluid obtained via paracentesis, but IHC findings were not discussed.
The prognosis of PRBL is poor with a median survival of less than a year. The mainstay of treatment is chemotherapy. Early diagnosis by renal biopsy and subsequent treatment may improve prognosis. Our patient was given two cycles of COPADM regimen followed by two cycles of CYVE based therapy in accordance of FAB-LMB 96 treatment protocol and has improved clinically, radiologically and biochemically.
This case is important to the practicing oncopathologists to get enlightened about primary BL, kidney which can cytologically and histopathologically mimic DLBCL and LBL. Making a correct diagnosis is imperative for the treatment of such patients.
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Conflicts of interest
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[Figure 1], [Figure 2]