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Year : 2013  |  Volume : 56  |  Issue : 3  |  Page : 329-331
Extragonadal GCT: A rare case report of sacrococcygeal pure yolk sac tumor


Department of Pathology, MVPS Dr. Vasantrao Pawar Medical College and Research Centre, Nashik, Maharashtra, India

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Date of Web Publication24-Oct-2013
 

How to cite this article:
Pawar NP, Mahajan SV, Chaudhari RA, Chavan SD. Extragonadal GCT: A rare case report of sacrococcygeal pure yolk sac tumor . Indian J Pathol Microbiol 2013;56:329-31

How to cite this URL:
Pawar NP, Mahajan SV, Chaudhari RA, Chavan SD. Extragonadal GCT: A rare case report of sacrococcygeal pure yolk sac tumor . Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Feb 20];56:329-31. Available from: http://www.ijpmonline.org/text.asp?2013/56/3/329/120421


Sir,

Germ cell tumors (GCTs) are heterogeneous groups of neoplasm with diverse variation in age, site, clinical presentation, histopathological features, and treatment modalities. GCTs in children are relatively uncommon, presenting approximately in 3% of all pediatric malignancies. [1] Extragonadal GCTs constitute only 1-5% of all GCTs. [2] Amongst these yolk sac tumors is a rare GCT presenting as the third most common variant. We present the case of a 2 year-old female child diagnosed as "extragonadal pure yolk sac tumor" after extensive workup.

A 2year-old female child presented with complaints of low-grade fever and excess cry since 2 months. The child gradually developed dribbling micturition and difficulty during defecation. Ultrasonography revealed heterogeneous mass in the pelvis with bilateral hydronephrosis and hydroureter suggestive of bladder outlet obstruction. Magnetic resonance imaging (MRI) of lumbosacral spine [Figure 1], [Figure 2] showed large, 11 × 9.5 × 7 cm, irregular lobulated soft tissue mass in lower sacrum and coccyx, with pre, para, and post sacrococcygeal components causing spinal canal compression. Radiologically, differential diagnoses of presacral neuroblastoma, sacrococcygeal GCT, rhabdomyosarcoma, primitive neuroectodermal tumor, and chordoma were considered.
Figure 1: Magnetic resonance image of lumbosacral spine showing irregular lobulated soft ti ssue mass in lower sacral and coccyx regions (transverse plane)

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Figure 2: Magnetic resonance image of lumbosacral spine showing bilateral hydronephrosis with heterogeneous irregular mass extending into lateral pelvic wall muscles (coronal plane)

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Histopathological examination [Figure 3]a and b revealed tumor composed of round to oval cells having mildly pleomorphic, hyperchromatic nuclei and moderate amount of eosinophilic cytoplasm arranged predominantly in microcystic pattern. Well vascularized intervening stroma showed myxoid degeneration. No other germ cell elements were identified. Immunohistochemistry examination was strongly positive for Alpha-fetoprotein (AFP) [Figure 4]a and cytokeratin [Figure 4]b and negative for epithelial membrane antigen (EMA) [Figure 4]c. High level of serum AFP (>350 ng/ml) with normal serum beta human chorionic gonadotropin (HCG) and urinary vanillyl mandelic acid (VMA) were detected.
Figure 3: Histopathologic photomicrograph of yolk sac tumor predominantly arranged in microcystic pattern with many areas of myxoid degenerati on: (a) H and E stain, ×100; (b) H and E stain, ×400

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Figure 4: Immunohistochemical images: Tumor cells showing strong positi vity for (a) AFP, ×400 and (b) CK, ×400 and (c) Negative staining for EMA, ×400

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All other organs, including ovaries, were free from any pathology, hence the diagnosis of "extragonadal - sacrococcygeal pure yolk sac tumor" was made. By the time the diagnosis was made, the tumor had become inoperable and patient was started on cisplatin-based chemotherapy.

Extragonadal pure GCTs are vary rare. In Norway, Dueland et al. estimated the incidence of extragonadal GCTs as 0.5 per 1,00,000 population per year. [3] Extragonadal GCTs make up 2-5% of all GCTs and are mostly found in three body sites: Mediastinum, CNS, and retroperitoneum, [4] with sacrococcyx being the less common location.

The pathogenesis of extragonadal yolk sac tumor (YST) is not completely understood. The classical theory suggests that it arises from local transformation of misplaced primordial germ cells. From the fourth to the sixth week of embryogenesis, germ cells migrate through midline dorsal mesentery; a remnant of tissue anywhere along the migration course can be a site of GCT in future. [5] To diagnose a case of malignant extragonadal GCT, criteria laid down by Einhorn [6] must be fulfilled which include absence of a detectable including stigmata of burnt-out lesion or subsequent appearance of a gonadal tumor and absence of nodal metastasis in para-aortic and iliac regions.

Most gonadal and extragonadal YSTs are part of mixed GCTs; in pure form, they are uncommon. In pathological examination, Schiller - Duval bodies which are pathognomonic of YST have low sensitivity. Schiller - Duval bodies are seen in less than 20% of cases. Immunohistochemically, YST stains with AFP, cytokeratin, AE1/3, PLAP, and is negative for EMA. Presence of tumor markers such as serum AFP and serum beta HCG aids in the diagnosis. Malignant sacrococcygeal YSTs are usually advanced at the time of diagnosis. Pre- and postoperative cisplatin-based regimens have dramatically improved patients' outcome. Completely surgically resectable tumors have good prognosis. During the postoperative period, frequently serum AFP level should be checked to evaluate the response of chemotherapy.

In conclusion, yolk sac tumors, whether gonadal or extragonadal, are highly aggressive. Combined approach of radioimaging, tumor markers, and histopathologic examination is essential for early diagnosis of YST. In our case, the patient presented late in course of the disease, resulting in inoperable tumor with poor prognosis. Recognizing such tumors early is important because these tumors are sensitive to chemotherapy with increased patient survival. This case highlights the importance of clinical suspicion and early diagnosis of YST at unusual location, requiring aggressive treatment subsequently.

 
   References Top

1.Deb M, Mohanty S, Ananthamurthy A, Garg I, Das K. Atypical extragonadal germ cell tumors. J Indian Assoc Pediatr Surg 2012;17:9-15.   Back to cited text no. 1
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2.McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: A review with emphasis on pathologic features, clinical prognostic variables, and differential diagnostic considerations. Adv Anat Pathol 2007;14:69-92.   Back to cited text no. 2
    
3.Dueland S, Stenwig AE, Heilo A, Høie J, Ous S, Fosså SD. Treatment and outcome of patients with extragonadal germ cell tumours - the Norwegian Radium Hospital's experience 1979-94. Br J Cancer 1998;77:329-35.   Back to cited text no. 3
    
4.Iczkowski KA, Butler SL, Shanks JH, Hossain D, Schall A, Meiers I, et al. Trials of new germ cell immunohistochemical stains in 93 extragonadal and metastatic germ cell tumors. Hum Pathol 2008;39:275-81.   Back to cited text no. 4
    
5.Dede M, Pabuccu R, Yagci G, Yenen MC, Goktolga U, Gunhan O. Extragonadal yolk sac tumor in pelvic localization. A case report and literature review. Gynecol Oncol 2004;92:989-91.   Back to cited text no. 5
    
6.Vaideeswar P, Deshpande JR, Jambhekar NA. Primary pleura-pulmonary malignant germ cell tumours. J Postgrad Med 2002;48:29-31.  Back to cited text no. 6
[PUBMED]  Medknow Journal  

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Correspondence Address:
Nilesh P Pawar
"Atharva" Shree Nagar, Near Dream Niwas, Old Sai Kheda Road, Jail Road, Nashik Road, Nashik - 422 101, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.120421

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