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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 11  |  Issue : 1  |  Page : 105-108  

Primitive neuroectodermal tumor of kidney with Level III inferior vena cava thrombus


Department of Urology and Renal Transplantation, SMS Medical College and Hospital, Jaipur, Rajasthan, India

Date of Submission01-Jun-2015
Date of Acceptance05-Aug-2015
Date of Web Publication21-Jan-2019

Correspondence Address:
Dr. Haris Ansari
Department of Urology and Renal Transplantation, SMS Medical College and Hospital, Jaipur - 302 004, Rajasthan
India
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DOI: 10.4103/0974-7796.250558

PMID: 30787583

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   Abstract 

Primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare renal neoplasm with only about 50 reported cases in the literature. These tumors behave aggressively and carry a poor prognosis. A 22 years female patient presented with right lumber and right hypochondrium lump of 4 months duration. Commutated tomography revealed large right renal mass with renal vein and inferior vena cava (IVC) thrombus. Magnetic resonance imaging abdomen demonstrated the extension of tumor thrombus up to the junction of hepatic vein and IVC. Preoperative percutaneous needle biopsy was performed. Histopathology demonstrated small round to oval cells with scanty cytoplasm and cells are arranged in clusters. Immunohistochemical staining demonstrated a highly specific cluster of differentiation 99, confirming the diagnosis of a PNET.

Keywords: Cluster of differentiation 99, inferior vena cava thrombus, primitive neuroectodermal tumor


How to cite this article:
Ansari H, Banerjee I, Tomar V, Yadav SS. Primitive neuroectodermal tumor of kidney with Level III inferior vena cava thrombus. Urol Ann 2019;11:105-8

How to cite this URL:
Ansari H, Banerjee I, Tomar V, Yadav SS. Primitive neuroectodermal tumor of kidney with Level III inferior vena cava thrombus. Urol Ann [serial online] 2019 [cited 2019 Apr 24];11:105-8. Available from: http://www.urologyannals.com/text.asp?2019/11/1/105/250558


   Introduction Top


The term primitive neuroectodermal tumor (PNET) is used for a group of small round cell neoplasm which are a type of sarcoma. They have been considered to be neural crest derivatives.[1] They were first described by Stout in 1918 in the case report that involved an ulnar nerve tumor.[2] In 1994, Mor described the first case of a renal Ewing's sarcoma (ES)/PNET.[3] These tumors are closely related to ES due to the same chromosomal abnormality: t(11;22) (q24:q12).[4],[5] PNET/extraskeletal ES (PNET/ES) rarely present as organ based neoplasm rather, it is seen typically in the soft tissue of extremities, chest wall (Askin's tumor) and paravertebral region. ES/PNET is an extraordinarily rare primary tumor in the kidney with only about 50 cases described in the literature.[6] The majority of the patients present in the second and third decades of life with nonspecific signs and symptoms similar to those of other renal mass lesion. Both males and females have been shown to be affected by some work documenting a predominance in males,[7] although, this has not been universally confirmed.[8] Patients with renal PNET may present with malaise, an increase in abdominal circumference, weight loss, renal colic, fever, flank pain, hematuria, and night sweats.[8],[9],[10],[11] Dysuria, testicular pain, and varicocele have also been reported.[9],[10] Dyspnea and dizziness may also be seen if the tumor features atrial involvement.[12]


   Case Report Top


A 22-year-female patient presented with right lumber and right hypochondrium lump 4 months duration. On physical examination, a large solid mass was palpable with restricted mobility. Initial ultrasonography of kidney revealed 8.5 cm × 8.5 cm renal mass predominantly cystic with mixed echogenicity. Computed tomography revealed heterogeneously enhancing soft tissue right large renal mass crossing midline with renal vein and inferior vena cava (IVC) thrombus. Magnetic resonance imaging (MRI) abdomen demonstrated the extension of tumor thrombus up to the junction of hepatic vein and IVC [Figure 1]a and [Figure 1]b. Based on physical examination and imaging studies tumor was found to be unresectable. Preoperative percutaneous needle biopsy was performed. Histopathology demonstrated small round to oval cells with scanty cytoplasm and cells were arranged in clusters [Figure 2]. Immunohistochemical staining showed a highly specific cluster of differentiation (CD99) [Figure 3], thus confirmed the diagnosis of a PNET. Neoadjuvant chemotherapy (adriamycin, vincristine, ifosfamide with mesna; four cycle) was planned in this patient and completed safely with a resolution of radiographic disease. MRI abdomen demonstrated hemorrhagic cystic mass (bright signal intensities on T1 and T2 image) and no tumor thrombus extension visualized renal vein or IVC [Figure 4]a, [Figure 4]b, [Figure 4]c. Radical nephrectomy was performed with the anterior subcostal approach. The patient is disease free at 6 months of follow-up.
Figure 1: On MRI (a) Tumor shows heterogenous signal; intensity on Axial T2 -Weighted image (b) coronal Recostruction on gadolinium enhanced T2- weighted image the tumor thrombus invading right renal vein and inferior vena cava up to junction of inferior vena cava with hepatic vein

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Figure 2: High power vision of smear shows lobules made of all round cells with indistinct cytoplasm, round nuclei with vesicular chromatin arranged around eosinophilic fibrillary materials and cells were arranged in clusters. (H and E, ×400)

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Figure 3: An immunohistochemical stain for CD99 was positive in the tumor cells. (Immunohistochemistry, ×400)

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Figure 4: After completion of four cycle of neoadjuvant chemotherapy (IVAD regimen) MRI shows (a) bright signal intensities on T1-weighted and T2-weighted imaging (b); (c) No extension of tumor thrombus seen in right renal vein and inferior vena cava, and on gadolinium contrast no abnormal enhancement seen

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   Discussion Top


PNET of the kidneys are exceedingly rare. The exact number of cases is difficult to determine because these tumors are sometimes not clearly differentiated from extraskeletal ES.[13] PNETs of the kidney are found equally in both sexes and tend to occur in relatively young patients and often with metastatic disease.[14] Patient present with flank and abdominal pain hematuria or palpable mass.

Histologically, the tumor is composed of small dark cells arranged in cords, nets or clusters with or without rosettes and pseudorosettes. The most important histologic criterion is the formation of rosettes like structures formed by tumor cells [Figure 2].[15],[16] Well-formed rosettes can also be identified in neuroblastoma, therefore carefully selected immunohistochemical panel is important for differentiating this tumor from other small round cell tumors of the kidney such as rhabdomyosarcoma, neuroblastoma, desmoplastic small round cell tumor, nephroblastoma, and ES.[16],[17] Immunohistochemically PNET cells express vimentin, NSE, and CD99. S-100 protein, synaptophysin neurofilament protein, CD57, and chromogranin show variable reactivity. CD99 (MIC-2 gene product) positivity is a universal features of PNET.

On computed tomography PNETs typically presents as a large heterogenous mass with central low density areas due to necrosis. Areas of high attenuation with in tumor, presumably due to hemorrhage have been observed. Intratumoral calcification, multiple, peripheral, or linear have been reported. Tumor extension into the renal vein, IVC, right atrium have been described in few reports. There are at least two reports of psoas muscle invasion. Hepatic metastases, when present, manifest as low attenuation lesions.[18],[19],[20]

PNET belongs to Ewing's family of tumors. A series of well planned, prospective randomized multicenter trials, clearly document the evolution of modern multimodality treatment regimens and have shown the advantages of multidisciplinary approach to treatment, including chemotherapy “up front” and using surgery where possible for local control.[21] Verrill et al. recorded 34 patients with ES/PNET, received the IVAD chemotherapy regimens. Drug dose intensity, toxicity, and survival data were collected. Author concluded that although the IVAD chemotherapy regimens are myelotoxic in adults, they can be given safely.[22]

The current treatment for renal PNET include a combination of surgery, chemotherapy, and radiotherapy [Table 1].[23],[24],[25],[26] Cavotomy and thrombectomy have also been performed in cases with a IVC tumor thrombus.[19] In addition to nephrectomy surgical resection of other involved organs or vasculature are occasionally indicated.[18],[19] Chemotherapy recommendations to treat PNET include alternating cycles of ifosfamide and etoposide with vincristine, doxorubicin, or dactinomycin.[27] Radiotherapy have been reported for locally advanced disease or when there is involvement of Gerota's fascia.[20]
Table 1: Combination of therapy of surgery and chemotherapy of renal PNET

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The 5 years disease free survival rate for patients presenting with well confined extraskeletal PNET is around 45–55%, and cases with advanced disease at presentation have a median relapse free survival of only 2 years.


   Conclusion Top


Although most authors recommend surgery followed by multidrug chemotherapy, we suggest that chemotherapy prior to surgery (IVAD regimen: Adriamycin, vincristine, ifosfamide with mesna; four cycle) is safe in locally advanced, inoperable PNET of kidney with good response.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Dehner LP. Peripheral and central primitive neuroectodermal tumors. A nosologic concept seeking a consensus. Arch Pathol Lab Med 1986;110:997-1005.  Back to cited text no. 1
    
2.
Stout AP. A tumor of the ulner nerve. Proc NY Pathol Soc 1918;18:2-12.  Back to cited text no. 2
    
3.
Mor Y, Nass D, Raviv G, Neumann Y, Nativ O, Goldwasser B. Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney. Med Pediatr Oncol 1994;23:437-40.  Back to cited text no. 3
    
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Enzinger FM, Weiss SW. Primitive neuroectodermal tumors and related lesions. Soft Tissue Tumors. 3rd ed. St. Louis: CV Mosby; 1995. p. 929-64.  Back to cited text no. 4
    
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Fietchner CD. Peripheral neuroectodermal tumors. In: Fletcher CD, editor. Diagnostic Histopathology of Tumors. Vol. 2. Edinburgh: Churchill Livingstone; 1995. p. 1221-50.  Back to cited text no. 5
    
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Pomara G, Cappello F, Cuttano MG, Rappa F, Morelli G, Mancini P, et al. Primitive neuroectodermal tumor (PNET) of the kidney: A case report. BMC Cancer 2004;4:3.  Back to cited text no. 6
    
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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. 7
    
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Chu WC, Reznikov B, Lee EY, Grant RM, Cheng FW, Babyn P. Primitive neuroectodermal tumour (PNET) of the kidney: A rare renal tumour in adolescents with seemingly characteristic radiological features. Pediatr Radiol 2008;38:1089-94.  Back to cited text no. 8
    
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Angel JR, Alfred A, Sakhuja A, Sells RE, Zechlinski JJ. Ewing's sarcoma of the kidney. Int J Clin Oncol 2010;15:314-8.  Back to cited text no. 9
    
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Cuesta Alcalá JA, Solchaga Martínez A, Caballero Martínez MC, Gómez Dorronsoro M, Pascual Piédrola I, Ripa Saldías L, et al. Primary neuroectodermal tumor (PNET) of the kidney: 26 cases. Current status of its diagnosis and treatment. Arch Esp Urol 2001;54:1081-93.  Back to cited text no. 10
    
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Ong PH, Manikandan R, Philip J, Hope K, Williamson M. Primitive neuroectodermal tumour of the kidney with vena caval and atrial tumour thrombus: A case report. J Med Case Rep 2008;2:265.  Back to cited text no. 11
    
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Pomara G, Cappello F, Cuttano MG, Rappa F, Morelli G, Mancini P, et al. Primitive neuroectodermal tumor (PNET) of the kidney - A case report. BMC Cancer 2004;4:3.  Back to cited text no. 12
    
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Marley EF, Liapis H, Humphrey PA, Nadler RB, Siegel CL, Zhu X, et al. Primitive neuroectodermal tumor of the kidney – Another enigma: A pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 1997;21:354-9.  Back to cited text no. 13
    
14.
Takeuchi T, Iwasaki H, Ohjimi Y, Kaneko Y, Ishiguro M, Fujita C, et al. Renal primitive neuroectodermal tumor: An immunohistochemical and cytogenetic analysis. Pathol Int 1996;46:292-7.  Back to cited text no. 14
    
15.
Maccioni F, Della Rocca C, Salvi PF, Manicone AM, Ascarelli A, Longo F, et al. Malignant peripheral neuroectodermal tumor (MPNET) of the kidney. Abdom Imaging 2000;25:103-6.  Back to cited text no. 15
    
16.
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. 16
    
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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. 17
    
18.
Ellinger J, Bastian PJ, Hauser S, Biermann K, Müller SC. Primitive neuroectodermal tumor: Rare, highly aggressive differential diagnosis in urologic malignancies. Urology 2006;68:257-62.  Back to cited text no. 18
    
19.
Ng AW, Lee PS, Howard RG. Primitive neuroectodermal kidney tumour. Australas Radiol 2004;48:211-3.  Back to cited text no. 19
    
20.
Doerfler O, Reittner P, Groell R, Ratscheck M, Trummer H, Szolar D. Peripheral primitive neuroectodermal tumour of the kidney: CT findings. Pediatr Radiol 2001;31:117-9.  Back to cited text no. 20
    
21.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003;348:694-701.  Back to cited text no. 21
    
22.
Verrill MW, Judson IR, Wiltshaw E, Thomas JM, Harmer CL, Fisher C. The use of paediatric chemotherapy protocols at full dose is both a rational and feasible treatment strategy in adults with Ewing's family tumours. Ann Oncol 1997;8:1099-105.  Back to cited text no. 22
    
23.
Habermann H, Benesch M, Schips L, Pummer K, Ratschek M, Uggowitzer MM, et al. Findings and clinical course of a localized primitive peripheral neuroectodermal tumor of the kidney. Urol Int 2003;71:319-21.  Back to cited text no. 23
    
24.
Wu YJ, Zhu YC, Chen H, Huang Y, Wei Q, Chen HJ, et al. Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus during pregnancy response to sorafenib. Chin Med J (Engl) 2010;123:2155-8.  Back to cited text no. 24
    
25.
Ohgaki K, Horiuchi K, Mizutani S, Sato M, Kondo Y. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin. Int J Clin Oncol 2010;15:210-4.  Back to cited text no. 25
    
26.
Kuczynski AP, Gugelmin ES, Netto RA. Primitive neuroectodermal tumor of the kidney in children J Pediatr (Rio J) 2001;77:49-51.  Back to cited text no. 26
    
27.
Ayhan O, Ali FC, Seref B, Bulmet K, Ugur B, Omer G. The immunohistochemical features of the renal primary primitive neuroectodermal tumor mimicking adult blastemal Wilm's tumor: A case report. Gulhane Tip Derg 2009;51:112-5.  Back to cited text no. 27
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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