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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2012  |  Volume : 17  |  Issue : 1  |  Page : 37-39
 

Endodermal sinus tumor of testis with acute intestinal obstruction: A rare presentation


1 Department of Pediatric Surgery, NRS Medical College, Kolkata, India
2 Department of Pathology, Institute of Postgraduate Medical Education and Research, Kolkata, India

Date of Web Publication22-Dec-2011

Correspondence Address:
Biswanath Mukhopadhyay
Department of Pediatric Surgery, NRS Medical College, Kolkata - 700 014
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.91087

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   Abstract 

A 7-year-old boy reported with endodermal sinus tumor of left testis, jejunal metastasis and intestinal obstruction. He was treated by high inguinal orchidectomy and resection of jejunal mass followed by chemotherapy.


Keywords: Alpha fetoprotein, endodermal sinus tumor, jejunal metastases, radical orchidectomy, yolk sac tumor


How to cite this article:
Mukhopadhyay B, Shukla RM, Mukhopadhyay M, Mandal KC, Roy D. Endodermal sinus tumor of testis with acute intestinal obstruction: A rare presentation. J Indian Assoc Pediatr Surg 2012;17:37-9

How to cite this URL:
Mukhopadhyay B, Shukla RM, Mukhopadhyay M, Mandal KC, Roy D. Endodermal sinus tumor of testis with acute intestinal obstruction: A rare presentation. J Indian Assoc Pediatr Surg [serial online] 2012 [cited 2019 Dec 13];17:37-9. Available from: http://www.jiaps.com/text.asp?2012/17/1/37/91087



   Introduction Top


Pediatric germ cell tumors are rare and they account for 3-4% of childhood solid tumors. [1] Endodermal sinus tumor (EST) is the most common testicular neoplasm in the pediatric population and accounts for approximately half of the prepubertal testis tumors. [2],[3],[4] ESTs in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. In adults, the clinical course is poor. [5],[6] Herein, we report an unusual case of metastasis of testicular tumor to the small bowel and its management.


   Case Report Top


A 7-year-old boy was admitted with a history of colicky pain in the abdomen and bilious vomiting for last 2 days. He was dehydrated with abdominal distension and visible peristalsis. The patient was stabilized first with adequate resuscitative measures. The left testis was enlarged (6 cm × 4 cm) and nontender. Right testis was normal. The blood counts were normal. The serum alpha fetoprotein (AFP) level was 756 IU/ml. Plain radiograph of the abdomen revealed multiple air-fluid levels. An ultrasonography of the testis showed a heterogeneous mass arising from the left testis.

On exploratory laparotomy, a mass was noted arising from the jejunum. The mass was completely occluding the jejunal lumen [Figure 1]. The mass measured 5 cm × 5 cm in the jejunum and was about 2.5 feet away from the duodenojejunal flexure. Resection of jejunum with adequate margins, end-to-end anastomosis, biopsy of mesenteric and retroperitoneal lymph nodes and high inguinal left orchidectomy were performed. The postoperative period was uneventful. The patient was discharged on 6 th postoperative day. The histology of the resected jejunum showed reticular and endodermal sinus patterns with Schiller Duval bodies [Figure 2]. The resection margins of the intestine were tumor free. The mesenteric and retroperitoneal lymph nodes showed features of reactive hyperplasia. Both the intestine and testis were periodic acid-Schiff (PAS) positive as well immunoreactive for AFP [Figure 3]. The patient received six cycles of chemotherapy with Cisplatinum (1.3 mg/kg/dose), Etoposide (3 mg/kg/dose) and Bleomycin (15 units/m 2) . After six cycles of chemotherapy, the patient had ultrasonography of the whole abdomen and serum AFP, and both were normal. The patient is doing well, though the follow-up is only for 10 months.
Figure 1: The left picture shows the resected jejunum with metastases at two sites and the right picture shows cut open specimen

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Figure 2: The left picture shows the cut open specimen of the testicular tumor and the right picture shows photomicrograph of endodermal sinus tumor with Schiller Duval bodies (x400)

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Figure 3: Photomicrograph of immunohistochemistry showing positive for AFP (x400)

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   Discussion Top


About 80% of pediatric ESTs present with stage I disease, [2],[3],[6],[7] but our patient presented atypically with intestinal metastases (Stage IV disease). Childhood EST does not appear to be associated with cryptorchidism. [8] Besides baseline investigations, all patients of testicular tumors should have ultrasonography of testis and abdomen, computed axial tomography of abdomen, chest X-ray and estimation of serum AFP. Testicular tumors are treated with radical inguinal orchidectomy and multi-agent chemotherapy. [9],[10]

EST is a highly malignant neoplasm of germ cells, which grows rapidly and metastasizes early via the lymphatic and hematogenous routes. The pattern of metastatic disease of yolk sac tumors in childhood differs from the pattern in adult germ cell tumors, owing to a higher incidence of hematogenous spread. [11] Approximately 95% of yolk sac tumors are confined to the testis. The lungs are the most common site of distant metastasis, which are affected in 20% of patients. Metastasis of yolk sac tumors to the retroperitoneum is uncommon in children (4-6%), whereas in adults, spread is usually to the retroperitoneal nodes. The tumor tends to metastasize to retroperitoneal lymph nodes below renal vessels first because of the anatomy of lymphatic flow from testis. It also frequently causes hematogenous spread. Abnormal sites of testicular metastasis are liver, brain, bone, bowel, spinal cord, cauda equina and eye. [8],[11],[12],[13],[14] The most commonly observed metastatic sites of the gastrointestinal (GI) tract include the small intestine and duodenum. [14],[15]

In a review article by Grady et al., 33 patients presented with metastatic disease. Nine patients had retroperitoneal metastases, 13 had hematogenous spread alone, lymphatic and haematogenous spread was seen in 6 patients and the sites could not be clearly documented in 5 patients. [16] This study shows a hematogenous predilection in the spread of metastases and suggests that retroperitoneal lymph node dissection has no routine value in the treatment of children with yolk sac tumor of the testis.


   Conclusion Top


Our case presented with features of acute intestinal obstruction due to metastasis to small bowel, which is a very rare presentation and so should be kept in mind in the differential diagnosis when a patient of testicular tumor presents with acute abdomen. The survival has increased significantly in the last two decades. Improvements in outcome are related to the use of multi-agent chemotherapy and to a better understanding of tumor biology. [8]

 
   References Top

1.Miller RW, Young JL Jr, Novakovic B. Childhood Cancer. Cancer 1995;75:395-405.  Back to cited text no. 1
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2.Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: A summary of the Prepubertal Testis Tumor Registry. J Urol 2002;168:1675-8.  Back to cited text no. 2
[PUBMED]    
3.Bahrami A, Ro JY, Ayala AG. An overview of testicular germ cell tumors. Arch Pathol Lab Med 2007;131:1267-80.  Back to cited text no. 3
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4.Martinazzi M, Crivelli F, Zampatti C. Immunohistochemical study of hepatic and enteric structures in testicular endodermal sinus tumors. Basic Appl Histochem 1988;32:239-45.  Back to cited text no. 4
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5.Olsen MM, Raffensperger JG, Gonzalez-Crussi F, Luck SR, Kaplan WE, Morgan ER. Endodermal sinus tumor: A clinical and pathological correlation. J Pediatr Surg 1982;17:832-40.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Woodtli W, Hedinger C. Endodermal sinus tumor or orchioblastoma in children and adults. Virchows Arch A Pathol Anat Histol 1974;364:93-110.   Back to cited text no. 6
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7.Cushing B, Perlman EJ, Marina NM, Castleberry RP. Germ Cell Tumors. In: Pizzo PA, editor. Principles and Practice of Pediatric Oncology. 5 th ed. Philadelphia: Lippincott Williams and Wilkins; 2006. p. 1130.  Back to cited text no. 7
    
8.Ro JY, Amin MB, Sahin AA, Ayala AG. Tumors and tumorous conditions of the male genital tract. In: Fletcher CD, editor. Diagnostic Histopathology of Tumors. 2 nd ed. London: Churchill Livingstone; 2000. p. 733-838.  Back to cited text no. 8
    
9.Zynger DL, Dimov ND, Luan C, Teh BT, Yang XJ. Glypican 3: A novel marker in testicular germ cell tumors. Am J Surg Pathol 2006;30:1570-5.  Back to cited text no. 9
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10.Rogers PC, Olson TA, Cullen JW, Billmire DF, Marina N, Rescorla F, et al. Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: A Pediatric Intergroup Study - Pediatric Oncology Group 9048 and Children's Cancer Group 8891. J Clin Oncol 2004;22:3563-9.  Back to cited text no. 10
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11.Gooneratne S, Keh P, Sreekanth S, Recant W, Talerman A. Anterior mediastinal endodermal sinus (yolk sac) tumor in a female infant. Cancer 1985;56:1430-3.  Back to cited text no. 11
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12.Lee JK, Kim SH, Kim JH, Kim IY, Kim TS, Jung S, et al. Metastatic spinal cord compression of testicular yolk sac tumor. Childs Nerv Syst 2002;18:171-4.  Back to cited text no. 12
[PUBMED]  [FULLTEXT]  
13.Unal O, Beyazal M, Avcu S, Akbayram S, Akgun C. Metastasis of testicular yolk sac tumor to cauda equina. Fetal Pediatr Pathol 2011;30:150-5.   Back to cited text no. 13
[PUBMED]  [FULLTEXT]  
14.Sweetenham JW, Whitehouse JM, Williams CJ, Mead GM. Involvement of the gastrointestinal tract by metastases from germ cell tumors of the testis. Cancer 1988;61:2566-70.  Back to cited text no. 14
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15.Johnson DE, Appelt G, Samuels ML, Luna M. Metastases from testicular carcinoma. Study of 78 autopsied cases. Urology 1976;8:234-9.  Back to cited text no. 15
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16.Grady RW, Ross JH, Kay R. Patterns of metastatic spread in prepubertal yolk sac tumor of the testis. J Urol 1995;153:1259-61.  Back to cited text no. 16
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
   Introduction
   Case Report
   Discussion
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    References
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