Friday, August 08, 2008

19 year-old male with hypertension and renal insufficiency

Clinical History

A 19 year-old Hispanic man presented with hypertension and renal insufficiency (serum creatinine of 7.1 mg/dl). The peripheral blood smear showed pancytopenia but no circulating blasts.

A CT scan demonstrated bilateral renal enlargement with lymphadenopathy in the retroperitoneum and neck.

A core biopsy was obtained.

Immunostains were performed.






Pre-B acute lymphoblastic lymphoma involving the kidneys


A bone marrow biopsy was also obtained which showed a diffuse infiltration by tumor cells with a similar morphology as the renal biopsy.

The renal biopsy showed a diffuse infiltrate of monotonous blastoid cells obliterating the normal renal architecture. The bone marrow biopsy showed a similar population of cells replacing most of the marrow. Immunophenotyping by flow cytometry and immunohistochemistry showed that the tumor cells co-express CD19, CD20, CD10, CD34, CD38, CD79a, HLA-DR and TdT.

Precursor B-cell acute lymphoblastic leukemia/lymphoma (ALL) is a common pediatric hematologic malignancy. Although renal failure due to tumor lysis is a recognized complication of treatment, initial presentation with renal failure is distinctly uncommon. ALL must be considered among the causes of acute renal failure when the kidneys are enlarged. Careful morphologic study and immunophenotyping by flow cytometry or immunohistochemistry is helpful to arrive at the correct diagnosis, and to avoid confusion with other small blue cell tumors which may involve the kidney, such as Wilm’s tumor (J Pediatr Hematol Oncol 2008;30:471), small cell carcinoma or Ewing’s sarcoma/primitive neuroectodermal tumor.

Bilateral nephromegaly simulating wilms tumor: a rare initial manifestation of acute lymphoblastic leukemia.
Pradeep R, Madhumathi DS, Lakshmidevi V, Premalata CS, Appaji L, Patil SA, Swapnil B.
A 7-year-old boy was referred with a provisional diagnosis of bilateral Wilms tumor. Peripheral smear revealed elevated leukocyte count with 90% blasts. Bone marrow aspiration and biopsy were hypercellular with sheets of blasts. Immunohistochemistry on paraffin sections showed a pre-B phenotype of acute lymphoblastic leukemia. Computerized tomographic scan of the abdomen showed moderate bilateral renal enlargement. Ultrasound-guided fine needle aspiration cytology of both kidneys showed blasts similar to those seen in the bone marrow. Finally, a diagnosis of pre-B acute lymphoblastic leukemia infiltrating both the kidneys was made. This case is being presented because of its rarity.

Additional references:

1. Weinstein Howard J, Tarbell Nancy J. Leukemias and lymphomas of childhood. Cancer Principles and Practice of Oncology. 5th edition; Ch.44: Section 2, 2145-65

2. Boueva A, Bouvier R. Precursor B-cell lymphoblastic leukemia as a cause of a bilateral nephromegaly. Pediatr Nephrol 2005;20:679

Precursor B-cell lymphoblastic leukemia as a cause of a bilateral nephromegaly.
Boueva A, Bouvier R.
Nephromegaly and non-oliguric acute renal failure is an unusual manifestation of lymphoblastic infiltration of the kidneys. We report the clinical history of a female child where a precursor B-cell lymphoblastic proliferation was diagnosed at the age of 21 months by a surgical renal biopsy for an unexplained bilateral nephromegaly. Lymphoblastic infiltration should be suspected in any patient presenting with unexplained renal failure and enlarged kidneys. The importance of renal biopsy to identify the etiology of renal failure and nephromegaly is emphasized.

3. Mehta A, Gulati K, Jain M, Gulati S. Non-Hodgkin lymphoma in a child presenting as nephromegaly and acute renal failure. Indian Pediatr 2001;38:407

4. Gilboa N, Lum GM, Urizar RE. Early renal involvement in acute lymphoblastic leukemia and non-Hodgkin lymphoma in children. J Urol 1983;129:364

Early renal involvement in acute lymphoblastic leukemia and nonHodgkin's lymphoma in children.
Gilboa N, Lum GM, Urizar RE.
Clinical manifestations of kidney disease, particularly renal failure, caused by malignant infiltration in patients with acute lymphoblastic leukemia or nonHodgkin's lymphoma have been described rarely. We report 1 case of acute lymphoblastic leukemia and 3 cases of nonHodgkin's lymphoma in which renal disease was the only or one of the presenting manifestations of malignancy. Of these patients 2 had rapidly progressive renal failure with nephromegaly, 1 presented with bilateral abdominal masses caused by severe nephromegaly and with microscopic hematuria, and 1 had microscopic hematuria without nephromegaly. In all 4 patients kidney biopsy revealed malignant infiltration. In the 2 patients who presented with renal failure kidney function promptly returned to normal after chemotherapy and irradiation of the kidneys. Prompt and correct diagnosis of nephropathy, when it is the only or one of the presenting signs of acute lymphoblastic leukemia or nonHodgkin's lymphoma, is necessary to expedite initiation of specific antitumor therapy.

5. Nizze H et al. Primary renal manifestations in malignant lymphomas and leukemia. Pathologe 2003;24:460

[Primary renal manifestation in malignant lymphomas and leukemia]
Nizze H, Prall F, Wigger M, Eggers G, Knieling K, Parwaresch R.
Primary manifestation of malignant lymphoma and/or leukaemia rarely occurs in the kidney. It can be the cause of a hitherto unexplained acute renal failure or it is incidentally detected as shown in the three cases under report.1.A 68-year-old man was operated on because of a symptomatic tumour in his right kidney. At nephrectomy, a conventional (clear cell) renal cell carcinoma was found simultaneously with an occult mantle cell lymphoma infiltrating the adjacent renal and extrarenal tissue. Clinical follow-up uncovered nodal and bone marrow involvement, so that a primary renal manifestation of mantle cell lymphoma was apparent.2.A 69-year-old man with suspected vertebral metastasis underwent partial renal resection because of a mass in his left kidney. Histologically and immunohistochemically, the renal infiltration was diagnosed as a precursor B-lymphoblastic lymphoma. After chemotherapy and irradiation, leukaemic blood cell counts with 50% lymphoblasts proved a primary renal manifestation of precursor B-lymphoblastic leukaemia/lymphoma.3.A 13-year-old boy presented clinically with renal failure, enlarged kidneys, and normal urinalysis. Renal biopsy showed a diffuse interstitial infiltration with atypical T-lymphoblasts compressing tubules and surrounding preserved glomeruli. Subsequent clinical bone marrow smears presented 60% T-lymphoblasts, so that the final diagnosis of a primary renal manifestation of acute T-lymphoblastic leukaemia of mature thymic cortex type was made. Immediate chemotherapy resulted in total recovery of renal function and bone marrow findings.

6. John William J, Foon Kenneth A, Patchell Roy A. Paraneoplastic syndromes Cancer Principles and Practice of Oncology. Vol.2; Ch.46: 2397-422.

7. - Leukemia, Acute chapter

(Source: Pathology Outlines by Dr. Nat Pernick)


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