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Journal of Clinical Oncology, Vol 23, No 22 (August 1), 2005: pp. 5257-5259 © 2005 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.07.031
Problems in Colon Cancer and a Child With Renal LymphomaCASE 3. Mature B-Cell Renal Lymphoma Presenting As a Single Osseous Mass in a ChildDepartments of Pediatric Hematology-Oncology, Radiology, Surgery, and Anatomopathology, G. Gaslini Institute, Genoa, Italy An 8-year-old boy presented with a history of pain in his right arm. The child's mother had noticed humeral swelling a few days earlier. Physical examination revealed a firm, nontender mass involving the humerus. No other organomegaly was clinically detected. Preliminary blood and urine analyses were within normal limits, except for a high lactate dehydrogenase value of 1,882 U/L (normal range, 204 to 474 U/L). At diagnostic work-up, ultrasonography revealed an 11 x 7 x 4-cm mass confined to the right kidney. Sonography and computed tomography scan revealed a large, noncalcified, well-circumscribed mass arising from the right kidney, sharply demarcated from the normal renal parenchyma and the adjacent organs. The mass did not encase the retroperitoneal vessels. It presented as a heterogeneous structure that was mostly hypoechoic at sonography and hypodense at computed tomography (Figs 1A and B). No other lesions were detectable in the abdomen. In particular, the contralateral kidney, the liver, and the spleen were normal. Radiograph of the humerus showed diffuse involvement of the bone (diaphysis and metaphyses), which indicated a malignant growth. The cortex was irregularly sclerotic, with an aggressive periosteal reaction characterized by the presence of "spicules" (Fig 2A, arrow). Magnetic resonance imaging determined the extent of the lesion. On T1, abnormally low signal tissue was evident in the shaft and in both the metaphyses replacing (Fig 2B, arrows) the marrow cavity of the humerus. On T2 and on postcontrast T1, the tissue was homogeneously hyperintense, with an associated soft tissue mass growing through the periosteum (Fig 2C, arrow). Bone scan did not reveal any other bone lesions. A primary renal tumor was suspected; therefore, right nephrectomy was performed. The combined morphologic and immunophenotypic profile of the renal mass was that of a Burkitt-like lymphoma.1 Long-distance polymerase chain reaction (LD-PCR) assay detected the t(8;14)(q24; q32) chromosomal translocation. A lumbar puncture, two bone marrow aspirates, and two biopsies showed no malignant cells, but LD-PCR for t(8;14) revealed minimal bone marrow infiltration.2 The boy underwent polychemotherapy according to the Associazione Italiana Emato Oncologia Pediatrica Lymphoma non-Hodgkin’s B protocol.3 Clinical examination 1 week after starting chemotherapy showed a marked reduction in the size of the humeral mass. The patient is presently alive in complete remission 6 months after diagnosis.
Wilms' tumor (nephroblastoma) accounts for 87% of renal masses in childhood4; therefore, in the presence of a single renal tumor in patients older than 6 months, chemotherapy or primary surgery can be carried out according to the protocol in use.5,6 Less common diagnoses may include renal cell carcinoma, rhabdoid tumor, or clear cell sarcoma.4 Neuroblastoma or mesoblastic nephroma are seldom reported in children older than 1 year.4,5 The presence of a bone lesion suggests either a more aggressive anaplastic Wilms' tumor or a clear cell sarcoma, or a renal cell carcinoma, with the latter being very rare in the first decade of life. Secondary renal involvement in children is frequent in disseminated cases of non-Hodgkin's lymphoma, while primary renal lymphomas are rare, and in most cases, bilateral. Approximately half of the reported cases are mature B-cell lymphomas.7 It is unusual for lymphoma to present as a single renal mass. To our knowledge, only two cases have ever been reported in the literature.8,9 A single renal mass was associated with retroperitoneal lymph node enlargement in both cases, and with multiple bone lesions in one case. In this latter case, as in our patient, a bone mass was the first sign of disease. Imaging cannot definitively identify the various histotypes of renal masses. Fine needle aspirate or biopsy of the renal mass or of the metastatic lesions may be performed in less typical cases so as to avoid nephrectomy.10 Biopsy of the metastasis should be interpreted with caution, as it could be derived from a single line of a multitissue primary lesion. However, if a correct diagnosis of lymphoma is made without performing nephrectomy, a great deal of care is required when starting chemotherapy, since tumor lysis syndrome and renal failure may occur in these cases.11,12 Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES
1. Lones MA, Auperin A, Raphael M, et al: Mature B-cell lymphoma/leukemia in children andadolescents: Intergroup pathologist consensus with the Revised European-American Lymphoma Classification. Ann Oncol 11:47-51, 2000 2. Mussolin L, Basso K, Pillon M, et al: Prospective analysis of minimal bone marrow infiltration in pediatric Burkitt's lymphomas by long-distance polymerase chain reaction for t (8;14) (q24; q32). Leukemia 17:585-589, 2003[CrossRef][Medline] 3. Pillon M, Di Tullio MT, Garaventa A, et al: Long term results of the first AIEOP Protocol for the treatment of Pediatric B-Cell non Hodgkin's Lymphomas-AIEOP LNH92. Cancer 101:635-641, 2004
4. Lowe LH, Isuani BH, Heller RM, et al: Pediatric renal masses: Wilms tumor and beyond. Radiographics 20:1585-1603, 2000
5. Tournade MF, Com-Nougué C, de Kraker J, et al: Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: Results of the Ninth International Society of Pediatric Oncology Wilms' Tumor Trial and Study. J Clin Oncol 19:488-500, 2001 6. D'Angio GJ: Perspective pre-or post-operative treatment for Wilms tumor? Who, what, when, where, how, why-and which? Med Pediatr Oncol 41:545-549, 2003[CrossRef][Medline]
7. Chepuri NB, Strouse PJ, Yanik GA: CT of renal lymphoma in children. AJR Am J Roentgenol 180:429-431, 2003 8. Hugosson C, Mahr MA, Sabbah R: Primary unilateral renal lymphoblastic lymphoma. Pediatr Radiol 27:23-25, 1997[CrossRef][Medline] 9. Capps GW, Das Narla L: Renal lymphoma mimicking clear sarcoma in a pediatric patient. Pediatr Radiol 25:S87-S89, 1995
10. Vujani
11. Sieniawska M, Bialasik D, Jedrzejowski A, et al: Bilateral primary renal Burkitt lymphoma in a child presenting with acute renal failure. Nephrol Dial Transplant 12:1490-1492, 1997 12. Malbrain ML, Lambrecht GL, Daelemans R, et al: Acute renal failure due to bilateral lymphomatous infiltrates. Primary extranodal non-Hodgin's lymphoma (p-EN-NHL) of the kidneys: Does it really exist? Clin Nephrol 42:163-169, 1994[Medline]
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Copyright © 2005 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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Gordan M, Kelsey A, Mitchell C, et al: The Role of Biopsy in the Diagnosis of Renal Tumors of Childhood: Results of the UKCCSG Wilms Tumor Study 3. Med Pediatr Oncol 40:18-22, 2003
