Primary Yolk Sac (Endodermal Sinus) Tumour of the Vulva: A Case Report

Abstract

Objective: To report a case of primary yolk sac (endodermal sinus) tumour (YST) of the vulva which is very rare and to highlight some unusual features of this tumour in the vulva. Clinical Presentation and Intervention: A 23-year-old woman presented with a 1-month swelling in the right labium majus that was non-tender and rapidly increasing in size. The mass was excised; it grossly measured 6 × 5 × 3 cm and was firm with a grey white cut surface. Microscopically it showed features of YST. The patient returned to the clinic only after 2 months with a recurrent mass of almost the same size with ipsilateral lymphadenopathy. At that time serum α-fetoprotein showed only a mild increase (13.4 µg/l, normal: 0–10 µg/l). Further management was not possible as she decided to return to her native country for treatment. A literature review shows that local excision with lymphadenectomy followed by cisplatin-based chemotherapy is the best mode of treatment for primary YST of the vulva. Conclusion: This case is presented for its rarity and to highlight the fact that serum α-fetoprotein estimation may not aid in the primary diagnosis or detection of recurrence in patients on follow-up for YST arising in the vulva unlike those seen in the gonads.

Vulvar yolk sac tumor diagnosed during pregnancy, with recurrence during subsequent second pregnancy

“…In 9 of 15 cases in the reported literature, recurrent or metastatic disease occurred within 12 months of primary surgery, often despite adjuvant chemotherapy. The most common site of recurrence is the inguinal lymph nodes ( Subrahmanya et al, 2011 , Krishnamurthy and Sampat, 1981 , Basgul et al, 2006 , Craighead and du Toit, 1993 , Khunamornpong et al, 2005 , Traen et al, 2004 , Ungerleider et al, 1978 , Yin-Yi Chang et al, 2011 ); with lungs being the most common site of distant metastases ( Krishnamurthy and Sampat, 1981 , Traen et al, 2004 , Yin-Yi Chang et al, 2011 , Mochizuki et al, 2012 ).…”

“…The origin of primary vulva YST is believed to be misplaced germ-cells during embryonic life entering the vulva through the gubernaculum ( Flanagan et al, 1997 ). Reported cases of primary vulvar YST ranged in age from 22 months to 52 years, with most cases reported in the 2 nd and 3 rd decade of life ( Subrahmanya et al, 2011 ), at a female’s reproductive peak. Only one vulvar YST has previously been reported in pregnancy ( Krishnamurthy and Sampat, 1981 ).…”

Unusual Presentations of Gynecologic Tumors: Extragonadal Yolk Sac Tumor of the Vulva

“…This review details the body of literature with respect to extragonadal yolk sac tumor of the vulva. 6,[18][19][20][21][22][23][24][25][26][27][28][29][30] There are several theories regarding the histogenesis of extragonadal yolk sac tumor, including (1) arrested migration of or misplaced germ cells during embryogenesis, (2) reverse migration of germ cells, (3) abnormal differentiation of somatic cells, (4) derivation from pluripotential stem cells within a somatic tumor, (5) origination from residual fetal tissue following incomplete abortion (for primary endometrial yolk sac tumor), and (6) metastasis from an occult gonadal primary. 1,4,5,21,25 The association with somatic tumors such as endometrioid adenocarcinoma or carcinosarcoma, seen in some cases arising in the gynecologic tract of older patients, supports a non-germ cell origin (points 3, 4) for a subset of extragonadal yolk sac tumors.…”