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LETTER TO EDITOR
Year : 2011  |  Volume : 48  |  Issue : 2  |  Page : 274-276
 

Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium


1 Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India
2 Department of Surgery, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India

Date of Web Publication11-Jul-2011

Correspondence Address:
G S Pathak
Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.82906

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How to cite this article:
Ashturkar A V, Pathak G S, Deshmukh S D, Neve R S. Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium. Indian J Cancer 2011;48:274-6

How to cite this URL:
Ashturkar A V, Pathak G S, Deshmukh S D, Neve R S. Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium. Indian J Cancer [serial online] 2011 [cited 2019 Oct 20];48:274-6. Available from: http://www.indianjcancer.com/text.asp?2011/48/2/274/82906


Sir,

We report a rare case of renal primitive neuroectodermal tumor (PNET) with tumor thrombus extending from right renal vein to inferior vena cava and right atrium in a 44-year-old female. She presented with complaint of palpable, soft, and non-tender mass in the right lumbar region for the past 3 months. Computed tomography (CT) of the abdomen showed a large, lobulated heterogeneously enhancing soft tissue mass, measuring 17.4 × 11.5 × 9.3 cm arising from mid and lower pole of right kidney. The tumor thrombus was extending into the right renal vein, inferior vena cava (IVC) and occupying a significant proportion of the right atrial volume [Figure 1]. The patient underwent right radical nephrectomy with optimal resection of tumor thrombus within the IVC. Postoperative USG was carried out to exclude presence of tumor embolus. The patient was referred to a specialty centre for further management, which she refused and subsequently died 2 months later.
Figure 1: Computed tomography (axial view) of the abdomen showing a large, heterogeneously, enhancing soft tissue mass arising from mid and lower pole of right kidney. Inset shows extension of the tumor thrombus into the right renal vein and IVC

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Gross examination revealed an encapsulated, grayish white, lobulated mass, replacing almost all the kidney. On cut surface, the tumor showed extensive areas of necrosis and hemorrhage, with extension in the right renal vein. Microscopic examination showed a tumor composed of cohesive sheets of monomorphic, small, round cells separated by fibrous bands. The malignant cells had scanty cytoplasm, inconspicuous nucleoli, and brisk mitotic activity. True as well as pseudo-rosetting of tumor cells were noted [Figure 2]. Immunohistochemically, the tumor cells were positive for neuron-specific enolase, chromogranin-A and CD 99 [Figure 3], and negative for cytokeratin (CK), and leukocyte common antigen (LCA), establishing the diagnosis of primitive neuroectodermal tumor (PNET).
Figure 2: Photomicrograph showing renal tissue with the tumor comprised of cohesive sheets of tumor cells separated by fibrous bands (H and E, ×40). Inset shows formation of rosette by the tumor cells (H and E, ×400)

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Figure 3: Tumor cells showing strong membrane positivity for CD99 immunostain (×100)

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Differential diagnoses of primary small round cell tumors of kidney include include blastemal predominant Wilms' tumor, PNET, rhabdomyosarcoma, neuroblastoma, lymphoma, and desmoplastic round cell tumor. Out of all these, renal PNET is a rare entity and most of the reported cases have occurred in young adults with mean age of 28−34. [1] In our case, the diagnosis was confirmed by positive immunohistochemical marker specially for CD99, which is cell-surface glycoprotein highly expressed by Ewing's sarcoma and PNET. [2]

The cases of PNET of kidney with venous thrombus are extremely rare. [3] A PNET with a level IV thrombus in a 55-year-old woman has been reported which was managed successfully with deep hypothermic circulatory arrest. [4] In another report involving a 17-year-old girl, radical nephrectomy was performed, but the caval thrombus was left behind. [5] Our patient underwent right radical nephrectomy with optimal resection of tumor thrombus within the IVC and was further referred to a specialty hospital, but the patient refused further treatment. Similar observations have been documented in literature. [6] Our experience highlights the unusual clinical presentation in this case and importance of immunohistochemical study in the differential diagnosis of renal round cell tumors.

 
  References Top

1.Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, et al. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: A clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol 2002;26:320-7.  Back to cited text no. 1
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2.Ong PH, Manikandan R, Philip J, Hope K, Williamson M. Primitive neuroectodermal tumor of the kidney with vena caval and atrial tumor thrombus: a case report. J Med Case Reports 2008;2:265.  Back to cited text no. 2
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3.Sivaramakrishna B, Mundada OP, Aron M, Aron M, Vijayaraghavan M. Primary primitive neuroectodermal tumor (PNET) of the kidney with venous thrombus. Int Urol Nephrol 2003;35:311-2.  Back to cited text no. 3
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4.Thomas JC, Sebek BA, Krishnamurthi V. Primitive neuroectodermal tumor of the kidney with inferior vena cava and atrial tumor thrombus. J Urol 2002;168:1486-7.   Back to cited text no. 4
[PUBMED]    
5.Juan CJ, Wu CJ, Chen CY, Lee SS, Chen A, Fan HC. Primitive neuroectodermal tumor of the kidney associated with Budd-Chiari syndrome in a 17-year-old girl. J Formos Med Assoc 2001;100:628-30.  Back to cited text no. 5
[PUBMED]    
6.Gonlusen G, Ergin M, Paydas S, Bolat FA. Primitive neuroectodermal tumor of the kidney: A rare entity. Int Urol Nephrol 2001;33:449-51.  Back to cited text no. 6
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