Year : 2009 | Volume
: 52 | Issue : 3 | Page : 403--404
Endodermal sinus tumor of the vagina in children: A report of two cases
Mimi Gangopadhyay1, Kalpana Raha1, Swapan K Sinha1, Anuradha De1, Pranati Bera1, Shyamapada Pati2,
1 Department of Pathology, North Bengal Medical College and Hospital, Sushrutanagar, Darjeeling, India
2 Department of Gynecology and Obstetrics, North Bengal Medical College and Hospital, Sushrutanagar, Darjeeling, India
Department of Pathology, North Bengal Medical College and Hospital, Sushrutanagar, Darjeeling
Malignant tumors of the vagina in infants and children are rare, with primary germ cell tumor (endodermal sinus tumor [EST]) being rarer, which carries a dismal prognosis. This tumor is often clinically mistaken as botryoid rhabdomyosarcoma and, on histopathological examination, is often misdiagnosed as clear cell adenocarcinoma. Two cases of EST of the vagina in infants aged 9 and 17 months are reported. Both the patients presented with bleeding per vagina and clinically and by ultrasonography were diagnosed as sarcoma botryoides. Both were diagnosed as primary yolk sac tumors. The serum alfa fetoprotein (AFP) level was 4325 and 9328 ng/ml, respectively. One patient aged 9 months expired 2 months after receiving chemotherapy. The other, aged 17 months, was given chemotherapy followed by surgery with reduction of the AFP levels.
|How to cite this article:|
Gangopadhyay M, Raha K, Sinha SK, De A, Bera P, Pati S. Endodermal sinus tumor of the vagina in children: A report of two cases.Indian J Pathol Microbiol 2009;52:403-404
|How to cite this URL:|
Gangopadhyay M, Raha K, Sinha SK, De A, Bera P, Pati S. Endodermal sinus tumor of the vagina in children: A report of two cases. Indian J Pathol Microbiol [serial online] 2009 [cited 2021 Dec 1 ];52:403-404
Available from: https://www.ijpmonline.org/text.asp?2009/52/3/403/55008
Malignant germ cell tumors are rare tumors of childhood accounting for less than 3% of pediatric malignancies, with endodermal sinus tumor (EST) being the most common histological subtype.  The vagina is an extremely rare site for primary germ cell tumors.
Clinically, it is commonly diagnosed as sarcoma botryoides and microscopically, often misdiagnosed as clear cell adenocarcinoma. We report two cases of EST of the vagina, a rare pediatric malignancy primarily affecting children less than 3 years of age.
A 9-month-old female (Case 1) weighing 6 kg was admitted with a history of a friable polyploid growth in the vagina. A 17-month-old female (Case 2) weighing 9 kg presented with bleeding per vagina for 3 days followed by purulent discharge that was tinged with blood. An abdominal ultrasonography revealed a solid echogenic mass of size 3.5 cm × 4.0 cm × 3.5 cm and 3.3 cm × 3.0 cm × 3.2 cm in the regions of the cervix and vagina in Cases 1 and 2, respectively. The kidney, bladder, liver, uterus and ovaries were free in both the cases. A magnetic resonance imaging scan was performed in Case 2. Per vaginal examination under anesthesia was carried out in both cases, which revealed a mass in the upper lateral vaginal wall in Case 1 and in the posterior fornix in Case 2. Punch biopsies were taken followed by serum alfa fetoprotein (AFP) assay. Serum AFP level in Case 1 was 4325 ng/ml and in Case 2 it was 9328 ng/ ml. Routine hematological investigations were normal except for a mild degree of hypochromia. X-ray of the chest showed no abnormality. Both the cases were diagnosed as EST on histopathological examination. Both received chemotherapy followed by surgery. Case 1 died after 2 months. Case 2 showed a decrease of the AFP level with regression of tumor size and is doing well after conservative surgery.
Grossly, the tumors were friable with sizes of 4 cm 3 cm 2.5 cm and 3 cm 2.5 cm 2.5 cm, respectively. The cut surface was grayish brown, with cystic areas. The sections showed a loose meshwork of microcystic spaces, anastomosing glands and papillae lined by columnar cells with vacuolated cytoplasm and hyperchromatic nuclei and pseudopapillary structures with a fibrovascular core lined by columnar cells projecting into the cystic spaces (Schiller Duval body) [Figure 1]. Numerous hyaline globules were seen within the cytoplasm of the tumor cells. They were periodic acid Schiff (PAS)- positive and diastase resistant [Figure 2]. The tumor cells were arranged in an irregular tubuloalveolar formation. The structures were closely apposed with little intervening stroma in many foci, often being arranged in a reticular pattern. Solid areas were also present. The cells were cuboidal with vacuolated cytoplasm and hyperchromatic nuclei. Intracellular and extracellular hyaline bodies were present, which were diastase resistant and PAS positive.
The type of tumor exemplified by the present cases has usually been reported as mesonephroma or mesonephriccarcinoma.  Norris et al .  and Allyn et al .  concluded that it was possible to distinguish both the groups of tumors as clear cell adenocarcinoma and EST based on histopathological features. EST develops exclusively in children less than 3 years of age. 
Age of the patients in the present cases was 9 months and 17 months, respectively, and presented with blood-tinged vaginal discharge. The origin of the tumor remains speculative and controversial. The midline position, histological pattern and presence of AFP are consistent with germ cell origin.  Among the 50 reported cases of EST in the world literature, 10-15% originate in the cervix and 50% in the vagina, whereas the vulva and corpus are extremely rare sites. ,
The histopathological diagnosis of EST is based on the presence of Schiller Duval bodies and PAS-positive eosinophilic hyaline globules.  Assay of AFP helps in preoperative diagnosis, monitoring the effectiveness of the therapy and recurrence before appearance of clinical symptoms. In the present cases, the AFP level was markedly raised and b-hCG was negative. EST is locally aggressive and capable of metastasis via hematogenous as well as lymphatic routes. A number of treatment modalities have been described, including surgery, radiation and chemotherapy or in combination, with varying degrees of success. 
EST is chemosensitive and various regimens are currently available for treatment. Although more conservative surgery is advised to maintain sexual and reproductive function, radical surgery is sometimes mandatory depending on the extension of the tumor and involvement of the surrounding structures. Long-term survival is achieved in at least 60% of the cases. 
Both the patients in the present report were first subjected to chemotherapy. Case 2 underwent radical hysterectomy and vaginectomy. Case 1 expired 2 months after chemotherapy and Case 2 is healthy and alive after 6 months of initial diagnosis and surgery.
EST, although rare, requires a proper diagnosis. This tumor must be differentiated from clear cell adenocarcinoma, which is more aggressive and less chemosensitive. Diastase-resistant and PAS-positive hyaline intracytoplasmic globules are diagnostic along with high levels of AFP.
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