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CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 2  |  Page : 252-254
Primary primitive neuroectodermal tumor of the kidney


1 Department of Pathology, Krishna Institute of Medical Sciences Deemed University, Karad, Maharashtra, India
2 Department of Urosurgery, Medical Research Centre, Krishna Hospital, Karad, Maharashtra, India
3 Department of Physiology, Krishna Institute of Medical Sciences Deemed University, Karad, Maharashtra, India

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Date of Web Publication20-Apr-2018
 

   Abstract 


Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor. On immunohistochemistry, CD99 positivity confirmed the diagnosis of primary PNET of the kidney. We are presenting this case for its rarity, clinical presentation, and pathological findings.

Keywords: Peripheral primitive neuroectodermal tumor, renal tumor, round cell tumor

How to cite this article:
Jagtap SV, Kale PP, Huddedar A, Hulwan AB, Jagtap SS. Primary primitive neuroectodermal tumor of the kidney. Indian J Pathol Microbiol 2018;61:252-4

How to cite this URL:
Jagtap SV, Kale PP, Huddedar A, Hulwan AB, Jagtap SS. Primary primitive neuroectodermal tumor of the kidney. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Sep 26];61:252-4. Available from: http://www.ijpmonline.org/text.asp?2018/61/2/252/230545





   Introduction Top


Primitive neuroectodermal tumor (PNET) of the kidney is extremely rare entity, and less than 100 cases have been reported in literature.[1],[2] PNET is a malignant small round cell tumor that has the neural crest regarded as the most likely progenitor.[3] PNET most commonly occurs in bones and is equivalent to Ewing's sarcoma microscopically, immunohistochemically, and genetically.[4]


   Case Report Top


A 45-year-old male admitted to our hospital in urosurgery unit with complaints of fever, pain in the abdomen, and hematuria for 15 days. He had a history of intermittent pain in the abdomen for 6 months. There was no contributory significant family or past history. Ultrasonography of the abdomen and pelvis revealed large solid and cystic right renal mass measuring 16 cm × 10 cm × 3 cm. There was no evidence of any metastasis. Urine analysis showed mild proteinuria, hematuria, and pus cells. Other investigations were normal. Right nephrectomy was done. On gross examination, we received right nephrectomy specimen totally measuring 20 cm × 10 × cm 5 cm with large mass at the upper half of the right kidney 15 cm × 13 cm × 3.5 cm. Cut section showed solid grayish-brown tumor with fleshy areas and large cystic cavity filled with necrotic material and blood [Figure 1]. Pelvic, ureter, and renal vessels were unremarkable. Perinephric fat was adherent to underlying kidney. Three lymph nodes were noted, largest measuring 0.5 cm in diameter. On microscopy showed highly cellular tumor consists of malignant small round cells arranged in cohesive sheets, lobules, nest, rosettes separated by thin fibrous bands [Figure 2]. Homer Wright pseudorosettes were present. Predominantly, perivascular arrangement was noted [Figure 3]. Tumor showed extensive areas of hemorrhage, cyst formation, and necrosis. Tumor does not show any tubule or glomeruloid formation. High mitotic activity was noted. Tumor with oval or spindle cells was noted. Tumor infiltrating the capsule, perinephric fat, pelvis, and lymph nodes was noted [Figure 4]. On histopathology reported as malignant small blue round cell tumor suggestive of primary PNET right renal mass. The immunohistochemical study was done. On immunohistochemistry, tumor showed diffuse membrane positivity for CD99 (MIC-2 gene product) and immunonegativity for creatine kinase, neuron-specific enolase (NSE), leukocyte common antigen (LCA), and desmin.
Figure 1: Gross photograph of the right nephrectomy specimen totally measuring 15 cm × 13 cm × 3.5 cm showing gray-brown tumor with necrotic material

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Figure 2: Photomicrograph showing renal tissue with malignant small round blue cell tumor with areas of congestion (H and E, ×100)

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Figure 3: Photomicrograph of showing angiocentric tumor (H and E, ×400)

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Figure 4: Photomicrograph of the right kidney showing small round blue cell tumor infiltrating surrounding perinephric fat (H and E, ×100)

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   Discussion Top


Renal PNET is extremely rare tumor and considered as Ewing's sarcoma family-related group having monotonous small round cells. It is characterized by a translocation resulting in a fusion of the EWS gene and ETS-related family of oncogenes.

PNET is a malignant small round cell tumor and typically arises from the bone or soft tissue in the trunk or axial skeleton in adolescent and young adults. Various other primary sites are pelvis, retroperitoneum, limbs, neck, and paraspinal region. PNETs as primary genitourinary system have been reported rarely in the kidney, bladder, and prostate.[5],[6]

Renal PNET clinically shows nonspecific clinical features such as vague abdominal pain, lump in groin, fever, weight loss, and fever with hematuria. Many cases with aggressive clinical course with metastatic disease stage and poor prognosis have been reported. Many cases may show rapid progression with death within 1 year.

Our patient underwent nephrectomy which showed large tumor mass measuring 15 cm × 13 cm × 3.5 cm. Reported cases showed more than 10 cm mass in 65% of cases.[6] Histopathology showed features of malignant round cell tumor with extensive cystic change, necrosis, and hemorrhage. The differential diagnosis for renal PNET includes other small round cell tumors such as adult nephroblastoma, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma, small cell variant of osteosarcoma, desmoplastic round cell tumor, and malignant rhabdomyosarcoma.[7],[8]

For better confirmatory diagnosis, immunohistochemistry analysis is necessary. In our case, tumor cells express MIC-2 gene product CD99 and were immunonegative for cytokeratin, LCA, desmin, and synaptophysin.

PNET is immunoreactive to CD99 and is clue for the diagnosis. For other malignant round cell tumors, cytokeratin (for nephroblastoma, small cell carcinoma, and synovial sarcoma), LCA (for lymphoma), and NSE/chromogranin (for neuroblastoma) will be helpful for differentiation from PNET.

Moreover, cytogenetic studies demonstrated that PNET and Ewing's sarcoma are analogous to a translocation of the long arm of the chromosome t (11;22)(q24;q12) [8].

As the PNET is highly aggressive, it is often diagnosis in advance stage. The 5-year disease-free survival rate is 45%–55% in early disease cause while only 2 years for advance stage.[9] The treatment modalities include surgery with early and aggressive chemotherapy.


   Conclusion Top


Renal PNET is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gupta S, Billadello L, Casalino DD. Renal primitive neuroectodermal tumor. J Urol 2012;187:2211-2.  Back to cited text no. 1
    
2.
Chakrabarti N, Dewasi N, Das S, Bandyopadhyay A, Bhaduri N. Primary Ewing's sarcoma/Primitive neuroectodermal tumor of of kidney – A diagnostic dilemma. Iran J Cancer Prev 2015;8:129-33.  Back to cited text no. 2
    
3.
Dehner LP. Primitive neuroectodermal tumor and Ewing's sarcoma. Am J Surg Pathol 1993;17:1-13.  Back to cited text no. 3
    
4.
Brinkhuis M, Wijnaendts LC, van der Linden JC, van Unnik AJ, Voûte PA, Baak JP, et al. Peripheral primitive neuroectodermal tumour and extra-osseous Ewing's sarcoma; a histological, immunohistochemical and DNA flow cytometric study. Virchows Arch 1995;425:611-6.  Back to cited text no. 4
    
5.
Kumar V, Khurana N, Rathi AK, Malhotra A, Sharma K, Abhishek A, et al. Primitive neuroectodermal tumor of prostate. Indian J Pathol Microbiol 2008;51:386-8.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Mohsin R, Hashmi A, Mubarak M, Sultan G, Shehzad A, Qayum A, et al. Primitive neuroectodermal tumor/Ewing's sarcoma in adult uro-oncology: A case series from a developing country. Urol Ann 2011;3:103-7.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Friedrichs N, Vorreuther R, Poremba C, Schafer KL, Böcking A, Buettner R, et al. Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors. Pathol Res Pract 2002;198:563-9.  Back to cited text no. 7
    
8.
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. 8
    
9.
Casella R, Moch H, Rochlitz C, Meier V, Seifert B, Mihatsch MJ, et al. Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol 2001;39:613-7.  Back to cited text no. 9
    

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Correspondence Address:
Sunil Vitthalrao Jagtap
Department of Pathology, Krishna Institute of Medical Sciences Deemed University, Karad, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_277_16

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