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GENITOURINARY IMAGING Table of Contents   
Year : 2004  |  Volume : 14  |  Issue : 2  |  Page : 149-151
Endodermal sinus tumor of vagina in an infant : A case report


Department of Radio Diagnosis, and Imaging, Kasturba Medical College and Hospital Manipal-576104, India

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Keywords: Malignant germ cell tumour, Endodermal sinus tumour, vagina

How to cite this article:
Lakhkar B N, Rajgopal K V, Ramachandran R N. Endodermal sinus tumor of vagina in an infant : A case report. Indian J Radiol Imaging 2004;14:149-51

How to cite this URL:
Lakhkar B N, Rajgopal K V, Ramachandran R N. Endodermal sinus tumor of vagina in an infant : A case report. Indian J Radiol Imaging [serial online] 2004 [cited 2019 Jul 18];14:149-51. Available from: http://www.ijri.org/text.asp?2004/14/2/149/28572

   Introduction Top


Malignant germ cell tumor accounts for only three percent of neoplasms in children, the most common histologic subtype is the Endodermal Sinus Tumour (EST) [1]. EST of the vagina is rare and primarily occurs in infants [2]. In a recent series of EST in children [3],[4], only eight percent occurred in vagina with sacrococcygeal, testicular and ovarian sites being more common [1].Vaginal EST has a very poor prognosis, despite radical surgical therapy [2].A combination of conservative surgery and chemotherapy is recommended for better prognosis [2].

Herein, we report a proven case of vaginal EST and its imaging features in an infant.


   Case report Top


A previously healthy one year old child was brought to the hospital with history of bleeding from the genital region for the past one month .There was no other significant history .Child was afebrile with normal activity. General condition was good.

On physical examination, mild pallor was noticed. Abdominal examination was unremarkable . All other systems were also normal. Per rectal examination revealed a firm mass ,4x 3 cms anterior to the rectum . Rectal mucosa was normal. Per vaginal examination revealed the same mass to be fleshy and reddish which bled on touch.

Routine lab investigations showed hemoglobin = 9.8 gm % and markedly elevated AFP levels = 9331 ng/ml( normal 0-15 ng/ml ). Beta HCG was normal.

Chest radiograph was unremarkable. Pelvic sonography showed a echogenic soft tissue mass posterior to the urinary bladder and inferior to the uterus which extended inferiorly, measuring 4x 3.5 x 2.7 cms, suggestive of an intravaginal mass. Rest of the abdomen was normal.

MRI pelvis, plain & contrast, axial, sagittal and coronal images were done; it showed a heterogeneously enhancing mass with necrosis in the region of vagina, which was hypointense on T1,hyperintense on T2. The mass was seen posteroinferior to urinary bladder and anterior to rectum. Mass was abutting the urinary bladder anteriorly and rectum posteriorly with loss of fat planes.The mass was abutting the obturator internus on both sides with lateral displacement of levator ani on the right. Uterus was not well delineated from the mass.

Histopathological examination of the biopsy material revealed nodules with compact center with polygonal cells having pleomorphic, hyperchromatic nuclei with prominent nucleoli and vacuolated cytoplasm surrounded by pink basement like material forming parietal pattern. At periphery of nodule, microcystic spaces were seen giving the tumour a reticulated appearance. Nodules were separated by dilated capillaries ,extracellular deposits of eosinophilic material. These features were suggestive of Endodermal Sinus Tumour Patient underwent four courses of chemotherapy. Symptomatically, there was complete cessation of bleeding per vagina after the first course of chemotherapy.

The AFP level repeated during the third course of chemotherapy had drastically reduced to 32.6 ng/ml from 9331ng/ml. A repeat MRI at the end of four courses revealed near complete resolution of the tumour.


   Discussion Top


Endodermal sinus tumor of the vagina is a rare and a highly malignant germ cell tumor that primarily occurs in infants. In females, EST usually is encountered in the ovary, either in the pure form or as a component of a mixed germ cell tumor[2].

Infantile EST of vagina is an extremely rare tumor that exhibits distinctive clinicopathologic features .The tumor develops exclusively in children less than three years of age[4],[5].The origin is thought to be embryonic remnants of midline mesonephric structures[1].Beller etal reviewed the literature in 1979 and collected twenty five cases of vaginal EST [2].Young and Scully[4] in 1984 expanded the number of cases to forty one cases.Liann etal between 1990 to 2001 has reported three cases [ total of 44 ].

The clinical presentation includes vaginal bleeding with a polypoidal friable mass. With this clinical presentation, many cases of EST are misdiagnosed as sarcoma botryoides.

Imaging cannot differentiate the two.

The diagnosis of EST is based on histologic findings of Schüller Duval bodies which are glomeruloid bodies characterised by papillae with a single central vessel .PAS positive hyaline globules are also seen. The immunohistochemical study is positive for AFP[5].

The assay of serum AFP can potentially aid in the preoperative diagnosis ,monitor effectiveness of treatment ,detect recurrences before clinical manifestation and staging. The treatment of EST requires conservative surgery and combination chemotherapy because it has improved prognosis and preserves child bearing potential[2].

The surgery eradicates local tumor cells and makes subsequent chemotherapy more effective. Simple tumor excision is not sufficient, as residual tumor cells induce early recurrence and make chemotherapy ineffective.

Staging -because of the different primary sites it is difficult for a staging system to be applied uniformly to all malignant germ cell tumours. The staging system by Brodeur is used to classify tumours based on resectability of the primary lesion ,extent of regional lymphnode involvement and presence or absence of distal spread see [Table - 1][4].

On imaging ,EST of vagina are seen as soft tissue mass with irregular margins and heterogenous enhancement .Extension through the uterus and  Fallopian tube More Detailss may be seen and in large lesions the site of origin may not be identified. On sonography, the mass appears as a. hyperechoic mass[6].MR imaging has been suggested as superior to CT for defining the anatomic setting[3].

In conclusion, vaginal endodermal sinus tumour is a rare, highly malignant germ cell tumour that exclusively involves children less than three years of age. The imaging features are nonspecific with MR being superior to CT for defining the tumour extent. The final diagnosis is based on histology and raised AFP.

 
   References Top

1.Laura A.Grygotis and Felix S.Chew , Endodermal sinus tumour of vagina. AJR1997;169:1632.  Back to cited text no. 1    
2.Eui Ho Hwang,Seok Joo Han,Myo Kyung Lee,Chuhi-Joo L yu, and Byung Soo Kim, Clinical experience with conservative surgery for vaginal endodermal sinus tumour . Journal of Pediatric Surgery 1996;31: 219-222.  Back to cited text no. 2    
3.Imai A,Furui T,Yokoyama ,et al ,Endodermal sinus tumour of the vagina in an infant -Magnetic resonance imaging evaluation . Gynaec Oncol 1993;48:402-405.  Back to cited text no. 3    
4.Handel NL, Scott SM ,Giller RH et al, New perspectives on therapy for vaginal EST. Journal Of Urology 2002 August; 168:687-690.  Back to cited text no. 4    
5.Copeland LJ ,Sneige N ,Ordonez NG ,et al,Endodermal sinus tumour of the vagina and cervix Cancer 1985;55:2558-2565.  Back to cited text no. 5    
6.Arora M, Shrivastav RK, Jaiprakash MP,A rare germ-cell tumor site: vaginal endodermal sinus tumor. Pediatr Surg Int. 2002 Sep;18(5-6):521-3.   Back to cited text no. 6    

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Correspondence Address:
B N Lakhkar
Department of Radio Diagnosis, and Imaging, Kasturba Medical College and Hospital Manipal-576104
India
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Source of Support: None, Conflict of Interest: None


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    Figures

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

    Tables

[Table - 1]

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