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CASE REPORT Table of Contents   
Year : 2011  |  Volume : 8  |  Issue : 2  |  Page : 241-243
Yolk sac tumour of the penile shaft: A rare primary extra-gonadal presentation


1 Department of Morbid Anatomy and Pathology, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria
2 Department of Surgery, Urology Unit, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria
3 Department of Pathology, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria

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Date of Web Publication14-Oct-2011
 

   Abstract 

Germ cell tumours (GCTs) commonly involve the ovaries, testes, and other midline structures in children and adolescents and comprised a variety of tumours that have a common histiogenetic origin. The yolk sac tumour (YST) variant is the most common one seen in over 80% of testicular GCTs in children. Other sites of occurrence of these tumours include the mediastinum, prostate, retroperitoneum, and sacrococcygeal region. Penile malignancies account for less than 10% of male malignancies in the elderly, while its occurrence in children is rare. We present the case of a 5-year-old child with YST of the penile shaft and uninvolved testes.

Keywords: Extra-gonadal, penis, unusual, yolk sac tumour

How to cite this article:
Samaila MO, Maitama HY, Abdullahi K, Mbibu H, Waziri GD. Yolk sac tumour of the penile shaft: A rare primary extra-gonadal presentation. Afr J Paediatr Surg 2011;8:241-3

How to cite this URL:
Samaila MO, Maitama HY, Abdullahi K, Mbibu H, Waziri GD. Yolk sac tumour of the penile shaft: A rare primary extra-gonadal presentation. Afr J Paediatr Surg [serial online] 2011 [cited 2017 Mar 28];8:241-3. Available from: http://www.afrjpaedsurg.org/text.asp?2011/8/2/241/86074

   Introduction Top


Germ cells have the ability to differentiate along multiple lines at different stages of development; thus, tumours that arise from these cells are varied despite having a common histiogenetic origin. [1],[2],[3],[4] These tumours often localise in the gonads and along midline structures due to the path of descent of germ cells during development. [1],[2],[3],[4],[5],[6],[7] The yolk sac tumour (YST) variant is the most common tumour seen in over 80% of testicular germ cell tumours (GCTs) in children. Its extra-gonadal presentation is uncommon, though some cases have been reported in the mediastinum, lung, prostate, liver, retroperitoneum, and sacrococcygeal region. [7],[8],[9],[10],[11] Penile cancer is a disease of the elderly and accounts for less than 10% of all male malignancies with varied geographic incidence. [1],[3] YST of the penis is a rare occurrence and less than five cases have been reported in the literature. [12],[13]


   Case Report Top


A 5-year-old boy presented to the surgical outpatient clinic of the Ahmadu Bello University Teaching Hospital, Shika-Zaria, with a 9-month history of a large distal penile shaft fungating mass associated with multiple groin swellings [Figure 1]. He had presented at a general hospital when the lesion was first noticed and was offered circumcision with excision of the penile mass. However, the excised tissue was not sent for histopathological diagnosis. The penile lesion recurred within a few weeks following excision and rapidly increased in size. Examination revealed a polypoid exophytic friable fungating mass of size 10 × 10 × 8 cm, which bled upon touching and encircled the glans penis. The urethra was uninvolved while both testes were palpable and situated within the scrotum. Also, multiple firm masses were seen in the inguinal region bilaterally. No other palpable masses and abdominal ultrasound revealed any anomaly. An incisional biopsy sent for histology based on the clinical diagnosis of carcinoma of the penis was reported as a poorly differentiated squamous cell carcinoma (SCC). Definitive surgery with excision of the inguinal masses was performed a week later, and the excised tissue was sent for histopathological assessment.
Figure 1: Penile polypoid mass

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Figure 2: Mixed pattern with Schiller-Duval body

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Grossly, there were two ulcerated nodular narrow based polypoid masses measuring 10×8×6 cm and 6×6×4cm and weighing 110 and 40 g, respectively. The larger mass had an attached 3×1.5 cm skin strip at its base. The accompanying two right and three left inguinal masses/lymph nodes measured 8×4×1.5 cm and 5×2.5×1.5 cm and weighed 10 and 8 g, respectively. Cut sections of all tissue specimens were similar and revealed solid grey tan surfaces.

Microscopy revealed a polymorphous tumour composed of poorly differentiated moderate-sized cells with predominantly hyperchromatic to vesicular, round to elongate nuclei and variable amount of cytoplasm arranged in the form of small nests, papillae, tubular, alveolar, reticular, micro- and macrocystic patterns. Glomeruloid structures consisting of tumour cells arranged in round vascular channels in macrocystic spaces ( Schiller-Duval Bodies More Details)[Figure 2], periodic acid-Schiff (PAS)-positive eosinophilic colloid bodies, vascular emboli and necrosis were seen within the fibro-vascular myxoid stroma. Two lymph nodes from the groins showed effacement by similar tumour, while the remaining nodes showed reactive hyperplasia only. It was diagnosed as primary penile YST. Serum alpha fetoproteins (AFP) and tissue immunostaining for AFP were not done due to resource constraints. The patient had an uneventful postoperative period and three courses of chemotherapy regimen consisting of cisplatin, bleomycin and etoposide. However, he died shortly after the third cycle as a result of complications.


   Discussion Top


GCT is an uncommon condition, with only 2.4 million children developing one of the tumours annually, and accounts for 4% of all cancers in children and adolescents under the age of 20 years. [1],[3],[14] In a series of 189 malignant childhood tumours in Zaria, the same centre where the index case was from, 15 had GCT. This accounted for 7.9% of childhood malignancies and four of these were YST located in the ovary, testis, and sacrococcygeal region. [15]

The YST variant of GCT, also known as endodermal sinus tumour, is a primitive teratoid tumour composed of many epithelial and mesenchymal patterns as well as endodermal structures. [1],[4] Its pure form described by Teilum [1],[16],[17] is a classic organoid lesion seen in infants and children of age less than 2 years, while the other form is an aggressive, less-differentiated malignant tumour, which may occur with components of other GCTs in older children and adults. However, the microscopic appearance is same, irrespective of the age of patient. [18]

The most common site of occurrence of the tumour is the testis in males and accounts for more than 80% of testicular GCTs. The pathogenesis of its extra-gonadal presentation, as with other GCTs, has been linked with the neoplastic degeneration of embryonic germ cells along the path of descent into the gonads and inherited defects, while cryptorchidism is an added risk factor. [12],[19],[20]

The incidence of penile malignancy is variable for geographic regions and populations and accounts for less than 10% of all male malignancies with a peak age distribution of 60 years. The most common tumour type is SCC, which may affect the glans, coronal sulcus or foreskin epithelium and present as an exophytic or ulcerated mass. [3] The risk factors for development of SCC include phimosis, lack of circumcision and human papillomavirus infection. [21],[22],[23] The clinical presentation of this patient and circumcision history may lead to an erroneous clinical diagnosis.

The microscopic appearance of YST is heterogeneous and may consist of foci of other GCTs, especially teratoma. Such myriad features may cause a misdiagnosis, as with the incisional biopsy, which was reported as poorly differentiated SCC. Thus, adequate tissue sampling and extensive macroscopic and microscopic descriptions are mandatory for accurate diagnosis and patient management because of the aggressive nature of the tumour. The definitive diagnosis is based on the presence of malignant tumour cells arranged around thin-walled vascular channels simulating glomerulus (Schiller-Duval bodies) and PAS-positive diastase-resistant hyaline or eosinophilic intra/extra-cytoplasmic inclusions, which contains and tests positive for AFP. [17],[24] Cytokeratin positivity and GATA-4, a transcription factor, will further confirm the diagnosis. [25] Other diseases that may also cause elevated AFP level should be screened for in affected patients.

Primary GCTs often metastasise to the lungs, liver, lymph nodes, and central nervous system, while penile tumours will preferentially spread to the groin nodes. The only palpable nodes in our patient were the bilateral inguinal nodes, which showed effacement by tumour cells.

The management of YST is a combination of surgery to debulk tumour, chemotherapy inclusive of cisplatin and radiotherapy depending on the stage of disease and presence of metastasis. [8],[11],[26] However, surgery and chemotherapy are necessary for aggressive recurrent tumours, as seen in this case irrespective of the presence of metastasis.

Extra-gonadal YST is a rare occurrence in the penile shaft and this index case makes it an important differential of penile tumours, particularly in children. Adequate tissue sampling, characteristic morphologic features, and AFP positivity will aid diagnosis.


   Acknowledgement Top


We wish to acknowledge Dr Mustapha Kura, Senior Registrar, Department of Surgery, A.B.U.T.H., Shika-Zaria, for his assistance with the recovery of the clinical photograph.

 
   References Top

1.Rosai J. Germ Cell Tumors. In: Rosai J, editor. Ackerman's surgical Pathology. 8 th ed. Philadelphia: Mosby; 1999. p. 463, 1280, 2090.  Back to cited text no. 1
    
2.Schofield D, Cotran RS. Diseases of infancy and childhood. In: Cotran RS, Kumar V, Tucker C, editors. Robbins Pathologic Basis of Disease. 6 th ed. Philadelphia: W.B Saunders Company; 1999. p. 459-90.  Back to cited text no. 2
    
3.Woodward PJ, Heidenreich A, Looijenga LJ, Oosterhuis JW, McLeod DG, Moller H, et al. Germ cell tumours. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. Lyon IARC Press; 2004. p. 221-40.  Back to cited text no. 3
    
4.Nogales F, Talerman A, Kubik-Huch RA, Tavassoli FA, Devouassoux-Shisheboran M. Germ cell tumours. In: Tavassoli FA, Devilee P, editors. Pathology and Genetics, Tumours of the Breast and Female Genital Organs. Lyon IARC Press; 2003. p. 163-79.   Back to cited text no. 4
    
5.Basoglu A, Sengul AT, Buyukkarabacak YB, Yetim TD, Yildiz L. Pure Yolk-Sac Tumor of the Lung. Asian Cardiovasc Thorac Ann 2008;16:410-1.  Back to cited text no. 5
    
6.Camacho F. Benign cutaneous teratoma. J Cutan Pathol 1982;9:345-51.  Back to cited text no. 6
    
7.Pont J, Pridun N, Vesely N, Kienzer HR, Pont E, Spital FJ. Extragonadal malignant germ cell tumor of the lung. J Thorac Cardiovasc Surg 1994;107:311-2.  Back to cited text no. 7
    
8.Wacker A, Neunhoeffer F, Fuchs J, Warmann S, Schwarze C, Sotlar K, et al. A yolk sac tumor in a female newborn with a subcutaneous metastasis of the abdominal wall. Internet J Pediatr Neonatol 2009. Vol. 10.  Back to cited text no. 8
    
9.Bokemeyer C, Nichols CR, Droz JP, Schmoll HJ, Horwich A, Gerl A, et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: Results from an international analysis. J Clin Oncol 2002;20:1864-73.  Back to cited text no. 9
    
10.Wong NA, D'Costa H, Barry RE, Alderson D, Moorghen M. Primary yolk sac tumour of the liver in adulthood. J Clin Pathol 1998;51:939-40.  Back to cited text no. 10
    
11.Guiping H, Katsutoshi M, Tatsuya T, Yoshihiro T. Primary prostatic endodermal sinus tumor (Yolk Sac Tumor) combined with a small focal seminoma. Am J Surg Pathol 2003;27:554-9.  Back to cited text no. 11
    
12.Nakazawa R, Soneda S, Kinoshita A, Kitagawa H, Koike J, Chikaraishi T. A case report: Primary extragonadal yolk sac tumor of penile shaft in a 2-year-old child. Int J Urol 2009;16:413-5.  Back to cited text no. 12
    
13.Alurkar SS, Dhabhar BN, Jambhekar NA, Kulkarni JN, Advani SH. Primary endodermal sinus tumor of the penis: A case report. J Urol 1992;148:131-3.  Back to cited text no. 13
    
14.Olson T. Germ cell Tumours. Available from: http//www.CureSearch.org Medical Editorial Board [last accessed on 2006 May].  Back to cited text no. 14
    
15.Samaila MO. Malignant tumours of childhood in Zaria. Afr J Paediatr Surg 2009;6:19-23.  Back to cited text no. 15
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16.Green DM, Brecher ML, Grossi M, Simpson L, Fisher JE, Allen JE, et al. The use of different induction and maintenance chemotherapy regimens for the treatment of advanced yolk sac tumors. J Clin Oncol 1983;1:111-6.  Back to cited text no. 16
    
17.Teilum G. Endodermal sinus tumors of the ovary and testis: Comparative morphogenesis of the so-called mesoephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer 1959;12:1092-105.  Back to cited text no. 17
    
18.Harms D, Janig U. Germ cell tumours of childhood: Report of 170 cases including 59 pure and partial yolk-sac tumours. Virchows Arch A Pathol Anat Histol 1986;409:223-39.  Back to cited text no. 18
    
19.Hart WR. Primary endodermal sinus tumor of the liver: First reported case. Cancer 1975;35:1453-8.  Back to cited text no. 19
    
20.Gilbert JB, Hamilton JB. Studies in malignant testis tumours. Surg Gynecol Obstet 1940;71:731-43.  Back to cited text no. 20
    
21.Dillner J, von Krogh G, Horenblas S, Meijer CJ. Etiology of squamous cell carcinoma of the penis. Scand J Urol Nephrol Suppl 2000;205:189-93.  Back to cited text no. 21
    
22.Hemminki K, Dong C. Cancer in husbands of cervical cancer patients. Epidemiology 2000;11:347-9.  Back to cited text no. 22
    
23.Maden C, Sherman KJ, Beckmann AM, Hislop TG, Teh CZ, Ashley RL, et al. History of circumcision, medical conditions, and sexual activity and risk of penile cancer. J Natl Cancer Inst 1993;85:19-24.  Back to cited text no. 23
    
24.Luther N, Edgar MA, Dunkel IJ, Souweidane MM. Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors. J Neurooncol 2006;79:45-50.  Back to cited text no. 24
    
25.Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O, et al. Transcription factor GATA-4 is expressed in pediatric yolk sac tumors. Am J Pathol 1999;155:1823-9.  Back to cited text no. 25
    
26.Wiltshaw E, Stuart-Harris R, Barker GH, Gowing NF, Raju S. Chemotherapy of endodermal sinus tumour (yolk sac tumour) of the ovary: Preliminary communication. J R Soc Med 1982;75:888-92.  Back to cited text no. 26
    

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Correspondence Address:
Modupeola Omotara Samaila
Department of Morbid Anatomy/Pathology, Ahmadu Bello University Teaching Hospital, Shika-Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.86074

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    Abstract
   Introduction
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