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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 606-608
Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: A diagnostic dilemma


1 Laboratory Oncology Unit, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
2 Department of Medical Oncology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

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Date of Web Publication20-Sep-2011
 

   Abstract 

Granulocytic sarcoma (GS) usually presents concomitantly with or after the onset of acute myeloid leukemia, blastic phase of chronic myeloid leukemia (CML), or myelodysplastic syndromes. Rarely, it may present even before the onset of overt leukemia and when so, it is often misdiagnosed. We are reporting a case of GS of kidney presenting as an isolated renal mass with normal laboratory investigations including a normal peripheral blood smear. It was initially misdiagnosed as lymphoma as the blasts, in addition to the morphological similarity with lymphoma cells, also showed positive immunohistochemistry for B cell markers. Based on further investigations including immunophenotyping and cytogenetic studies, a final diagnosis of CML-blast crisis (mixed phenotype) presenting initially as GS was made. To the best of our knowledge, this is the first antemortem report of nonleukemic GS presenting as kidney mass that later on progressed to CML-blast crisis with mixed phenotype blasts.

Keywords: CML-BC, extramedullary myeloid tumor, immunohistochemistry, lymphoma

How to cite this article:
Agrawal V, Gupta A, Gupta R, Sharma MC, Das P. Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: A diagnostic dilemma. Indian J Pathol Microbiol 2011;54:606-8

How to cite this URL:
Agrawal V, Gupta A, Gupta R, Sharma MC, Das P. Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Oct 24];54:606-8. Available from: http://www.ijpmonline.org/text.asp?2011/54/3/606/85111



   Introduction Top


Granulocytic sarcoma (GS) is a localized tumor mass composed of immature cells of granulocytic series at extramedullary sites. It usually presents concomitantly with or after the onset of acute myeloid leukemia, blastic phase of chronic myeloid leukemia (CML), or myelodysplastic syndromes with a reported incidence of 2 to 8%. [1],[2] On rare occasions, it may present even before the onset of overt leukemia (nonleukemic GS) and when so, it is often misdiagnosed as lymphoma due to morphological similarity of both. [1],[2],[3],[4],[5] We report an unusual case of nonleukemic GS presenting initially as renal mass which later on progressed to overt leukemia.


   Case Report Top


A 21-year-old boy presented with complaints of low-grade fever and right-sided flank pain of two-week duration to the surgery out patient department of this hospital. Physical examination revealed a vague right-sided lumbar mass. On investigating, his hemoglobin was 12.2 gm%; total leukocyte count (TLC), 7.6 Χ 10 9 /l; and platelet count, 198 Χ 10 9 /l. The peripheral blood smear showed 36% neutrophils, 60% lymphocytes, 2% eosinophils, and 2% monocytes. The biochemical parameters were within normal limits.

Ultrasound abdomen revealed a hypoechoic mass measuring 5 Χ 3.4 cm in the middle and lower pole of right kidney partially attenuating the right renal pelvis. A small hypodense lesion measuring 1 cm was present in the lower pole of left kidney. Computer tomographic scan of abdomen showed a well-defined lobulated mass measuring 5 Χ 4.3 Χ 4 cm in size with mild homogenous enhancement arising from medial cortex of mid and lower pole region of the right kidney partially attenuating right renal pelvis and para-aortic lymph node enlargement. Renal vessels were free of tumor. A small hypodense lesion of approximately 10 mm was also noted in anterior cortex of the left kidney. A radiological diagnosis of renal cell carcinoma was entertained and a radical right nephrectomy was performed. On gross examination, kidney measured 11 Χ 6 Χ 4 cm and multiple grayish white ill-circumscribed nodules varying in size from 1 to 5 cm were seen throughout the renal parenchyma. Histopathological examination of nodules showed sheets of large round cells with vesicular nuclei and prominent nucleoli which were immunopositive for LCA, CD20 and were negative for CD3, pancytokeratin, and epithelial membrane antigen [Figure 1]a-d and f. A diagnosis of diffuse large B cell non Hodgkin lymphoma (NHL) was made and the patient was referred to cancer center for further management.
Figure 1: Photomicrographs showing (a) and (b): Tumor cells infiltrating the renal parenchyma but sparing few normal glomeruli and tubules (Hematoxylin and Eosin, x40 [a]; x20 [b]). Tumor cells are immunopositive for (c) LCA; (d) CD 20; (e) MPO and are negative for (f) CD 3 (x40 each)

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At this time, that was after a span of almost 3 months from his initial presentation at the hospital, patient had fever, marked pallor, generalized lymphadenopathy with largest node measuring 1 cm, and mild hepatosplenomegaly. A repeat hemogram done showed hemoglobin of 4 g/dl; TLC, 54.7 Χ 10 9 /l; and platelet count, 81 Χ 10 9 /l. Coagulation profile was normal. Peripheral blood smear showed 65% blasts, 5 to 6 nucleated red blood cells/100 leukocytes, and thrombocytopenia. Bone marrow aspirate was replaced by blasts and reactivity for myeloperoxidase (MPO) was seen in 20% blasts. At this stage, a diagnosis of acute myeloid leukemia was considered. On immunophenotyping, the blasts were positive for CD10, CD19, CD34, CD13 and were negative for CD33 and CD7. The blasts stained positive for CD20 on bone marrow biopsy. Immunohistochemistry for MPO was then done on renal mass and was found to be positive [Figure 1]e. Cytogenetic studies done on bone marrow revealed translocation t (9; 22). On reverse transcriptase-polymerase chain reaction, bcr-abl transcript of p210 size was identified. A final diagnosis of CML-blast crisis (mixed phenotype) presenting initially as GS was made. The patient was advised to take 600 mg daily dose of imatinib and provided supportive care. Patient, however, left the hospital against medical advice and was lost to follow-up.


   Discussion Top


Common sites of involvement by GS as described in literature are skin, bone, soft tissue, and other organs like lymph apparatus, reproductive organs, digestive organs, and central nervous system. [1],[4] Although kidney has been described as the most common organ involved by GS in a few postmortem studies, antemortem studies have rarely described this which is probably due to paucity of symptoms related to renal involvement in antemortem cases. [6] On thorough search of English literature (antemortem), we could find only one case of nonleukemic GS presenting as isolated renal mass that developed acute myeloid leukemia after few months. [7] To the best of our knowledge, ours is the first case report of nonleukemic GS presenting as kidney mass that later on progressed to overt leukemia with mixed phenotype blasts. The duration for GS progressing to overt leukemia in our case was about 3 months, similar to various other studies that have shown that nonleukemic GS almost invariably progresses to acute leukemia with a mean duration of 10 months or less. [1],[3],[5]

The morphology of GS is variable and they have been sub-classified into well-differentiated, poorly differentiated, and blastic subtypes according to the degree myeloid differentiation. When GS occurs as a presenting feature before the onset of overt leukemia, it is often misdiagnosed as lymphoma which also happened in our case. A review of 72 patients of nonleukemic GS showed that 35 patients (47%) were initially misdiagnosed, most often (31/35) as malignant lymphoma. [8] The morphological similarity of blasts with lymphoma cells, especially in the blastic and undifferentiated variant of GS and its rarity resulting in low index of suspicion, have been reported as the main reasons for the misdiagnosis in such cases. Majority of extramedullary blastic tumors can be classified as pre B, pre T, or GS using a panel of monoclonal antibodies including myeloid (MPO, lysozyme, CD68 and CD 43), together with B lineage markers (CD20 and CD79a) and T lineage markers (45RO and CD3). [4],[5],[9],[10] In our case, a normal TLC with a normal differential leukocyte count on peripheral blood smear at presentation, morphological similarity of the tumor cells to high-grade NHL, and in addition, use of an abbreviated panel of antibodies based on morphological assessment without adding a myeloid marker resulted in initial misdiagnosis of this case. Use of complete panel of antibodies could have avoided this error in our case also. Treatment of nonleukemic GS if given on time with intensive chemotherapy can help to achieve clinical remission and reduces the subsequent risk of development of overt leukemia. [4],[5],[8] Therefore, correct pathological diagnosis at the right time is a must and requires a pathologist to maintain a high index of suspicion of GS when viewing a lesion that resembles large cell lymphoma and carry out special studies with complete panel of antibodies.


   Acknowledgements Top


We gratefully acknowledge support of Dr. V. Kochupillai in carrying out this work.

 
   References Top

1.Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell RE, et al. Granulocytic sarcoma: A Clinicopathologic study of 61 biopsied cases. Cancer 1981;48:1426-37.  Back to cited text no. 1
    
2.Paydas S, Zorludemir S, Ergin M. Granulocytic sarcoma: 32 cases and review of the literature. Leuk Lymphoma. 2006;47:2527-41  Back to cited text no. 2
    
3.Meis JM, Butler JJ, Osborne BM, Manning JT. Granulocytic sarcoma in nonleukemic patients. Cancer 1986;58:2697-709.  Back to cited text no. 3
    
4.Yamauchi K, Yasuda M. Comparison in treatments of nonleukemic granulocytic sarcoma: Report of two cases and a review of 72 cases in the literature. Cancer 2002;94:1739-46.  Back to cited text no. 4
    
5.Pileri SA, Ascani S, Cox M-C, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: Clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients Leukemia 2007;21:340-50.  Back to cited text no. 5
    
6.Menasce LP, Banerjee SS, Beckett E, Harris M. Extra-medullary myeloid tumour (granulocytic sarcoma) is often misdiagnosed: A study of 26 cases. Histopathology 1999;34:391-8.  Back to cited text no. 6
    
7.Liu PI, Ishimaru T, McGregor DH, Okada H, Steer A. Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia, Hiroshima-Nagasaki 1949-1969. Cancer 1973;31:948-55.  Back to cited text no. 7
    
8.Bagg MD, Wettlaufer JN, Willadsen DS, Ho V, Lane D, Thrasher JB. Granulocytic sarcoma presenting as a diffuse renal mass before hematological manifestations of acute myelogenous leukemia. J Urol 1994;152:2092-3.  Back to cited text no. 8
    
9.Quintanilla-Martínez L, Zukerberg LR, Ferry JA, Harris NL. Extramedullary tumors of lymphoid or myeloid blasts. The role of immunohistology in diagnosis and classification. Am J Clin Pathol 1995;104:431-43.  Back to cited text no. 9
    
10.Traweek ST, Arber DA, Rappaport H. Extramedullary myeloid cell tumors: An immunohistochemical and morphologic study of 28 cases. Am J Surg Pathol 1993;17:1011-9.  Back to cited text no. 10
    

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Correspondence Address:
Ritu Gupta
Laboratory Oncology Unit, Dr. B.R.A. IRCH, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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DOI: 10.4103/0377-4929.85111

PMID: 21934235

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