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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 250-251
Metastatic primitive neuroectodermal tumor involving testis


1 Department of Surgery, Krishna Institute of Medical Sciences, Karad, Maharashtra, India
2 Department of Pathology, Krishna Institute of Medical Sciences, Karad, Maharashtra, India

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   Abstract 

A 40-year-old male patient presented with enlarging mass over left leg region. Incisional biopsy report revealed an undifferentiated malignant round cell sarcoma, for which above-knee amputation was done. One year later, the same patient presented with left testicular swelling which appeared to be malignant. A left high orchiedectomy was done. Biopsy report showed metastatic round cell tumor, which was confirmed by immunohistochemistry as primitive neuroectodermal tumor. Metastasis to testis is very uncommon.

Keywords: Primitive neuroectodermal cell tumor, round cell sarcoma, testicular metastasis

How to cite this article:
Kshirsagar AY, Langade YB, Ahire MD, Nanivadekar MR, Nikam SR, Neeraj S. Metastatic primitive neuroectodermal tumor involving testis. Indian J Pathol Microbiol 2008;51:250-1

How to cite this URL:
Kshirsagar AY, Langade YB, Ahire MD, Nanivadekar MR, Nikam SR, Neeraj S. Metastatic primitive neuroectodermal tumor involving testis. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Oct 15];51:250-1. Available from: http://www.ijpmonline.org/text.asp?2008/51/2/250/41672



   Introduction Top


Primitive neuroectodermal tumors (PNETs) are a group of neoplasms which arise from pluripotential neural crest cells. [1] These tumors show evidence of neural differentiation on light microscopy, immunohistochemistry and ultrastructural study. They are aggressive tumors and usually metastasize to lungs and skeleton. Metastasis to testis is very uncommon.


   Case History Top


A 40-year-old male patient presented with an enlarging mass over his left leg [Figure 1]. It was small to start with but enlarged progressively to the size of 10 8 cm. It was painless and had no associated symptoms. Results of routine investigations were within normal limits. X-ray of his left leg showed a soft tissue swelling over upper half of the leg without involvement of bone. Incisional biopsy was done, which revealed an undifferentiated malignant round cell sarcoma. An above-knee amputation was performed. The histopathological diagnosis was round cell sarcoma of leg.

One year later, the same patient presented with a painless, nontender, hard testicular swelling which clinically appeared to be malignant. A high inguinal orchiedectomy was performed [Figure 2] and subjected to histopathological examination, which was suggestive of metastatic malignant round cell tumor of testis.

Pathological findings

On histopathological examination, the above-knee - amputated specimen showed tumor over-lined by skin and composed of small round cells arranged in sheets separated by fibrovascular septa. The tumor cell had round vesicular hyperchromatic nuclei and scanty cytoplasm with areas of hemorrhage. The histopathological diagnosis was round cell sarcoma of leg.

A high inguinal orchiedectomy specimen showed testicular tissue infiltrated extensively by a highly vascular tumor composed of small round cells [Figure 3]. The tumor was composed of solid lobules of round cells having rounded vesicular nuclei with distinct nuclear membrane, finely divided chromatin and minute nucleoli with sparing of interstitial tubules. The cytoplasm was ill defined, scanty and pale staining. The histopathological diagnosis was metastatic malignant round cell tumor of testis.

Immunohistochemistry of both biopsies was positive for neuron-specific enolase, synaptosine and epithelial membrane antigen; and negative for leucocytes-common antigen, s-100 protein and cytokeratine. This indicates both these tumors to be of neuroectodermal origin, the primary site being the extremity; and testis, the site of metastasis.


   Discussion Top


It was first in 1918 that Stout reported an undifferentiated small round cell tumor with rosette pattern, followed by Ewing, Jaffe, Enzinger, Seemay and Askin. [2] In 1979, Liberman proposed the present name "primitive neuroectodermal tumor."

PNETs form an entity distinct from other malignant small round cell tumors, such as neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma and lymphoma. They are first distinguished by their light and electron microscopic features. [2] Their neuroectodermal origin is then confirmed by immunohistochemical methods. [3],[4] Cytogenetic studies demonstrated abnormality t (11:22) (q 24 :q 12 ) in relation to PNET and demonstrate Ewing's sarcoma. [5] The demonstration of neurosecretory granules by electron microscopy distinguishes PNET from Ewing's sarcoma. [2],[4]

Primitive neuroectodermal tumor of testis has been reported and is considered monodermal teratoma. [6] In secondary testicular involvement, tumor cells are confined to the interstitium and spare the seminiferous tubules. [7],[8] Extensive lymphatic and vascular invasion is present. [7],[8] Testicular involvement by metastatic PNET is an uncommon event.

Treatment consists of combination of surgery and chemotherapy. Radical surgery if possible should be performed to prevent local recurrence. Anthracyclines and high-dose alkylating agents have shown good response.


   Acknowledgment Top


We would like to thank Dr. H. R. Tata, Head of the Department of Surgery and Medical Director of KIMS, Karad, for allowing us to publish this data. We are also thankful to Mrs. S. D. Kulkarni for her secretariat support in preparing this paper.

 
   References Top

1.Dehner LP. Peripheral and central primitive neuroectodermal tumors: A nasologic concent seeking a consensus. Arch Pathol Lab Med 1986;110:997-1005.  Back to cited text no. 1  [PUBMED]  
2.Askin FB, Rosai J, Sibley RK, Dehner LP, McAllister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: A distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979;43:2438-51.  Back to cited text no. 2    
3.Tsokos M, Linnoila RI, Chandra RS, Triche TJ. Neuron specific enolase in the diagnosis of neuroblastoma and other small round cell tumors in children. Hum Pathol 1984;15:575-84.  Back to cited text no. 3  [PUBMED]  
4.Linnoila RI, Tsokos M, Triche TJ, Marangos PJ, Chandra RS. Evidence of neural origin and PAS-positive variants of the malignant small cell tumors of thoracopulmonary region ("Askin Tumor") Am J Surg Pathol 1986;10:124-33.  Back to cited text no. 4    
5.Whang-Peng J, Thriche TJ, Knustsen T, Miser J, Douglass EC, Israel MA. Chromosome translocation in peripheral neuroepithelioma. N Engl J Med 1984;311:584-5.  Back to cited text no. 5    
6.Aguirre P, Suelly RE. Primitive neuroectodermal tumor of the testis. Arch Pathol Lab Med 1983;107:643-5.  Back to cited text no. 6    
7.Haupt HM, Mann RB, Trump DL, Abewff MD. Metastatic carcinoma involving the testis. Cancer 1984;54:709-14.  Back to cited text no. 7    
8.Price EB, Mostofi FK. Secondary carcinoma of the testis. Cancer 1957;10:592-5.  Back to cited text no. 8    

Top
Correspondence Address:
Ashok Yadavrao Kshirsagar
Department of Surgery, Krishna Institute of Medical Sciences, Malkapur, Karad - 415 110, Dist. Satara, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.41672

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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    Abstract
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