Endodermal sinus tumor of the vulva: successfully treated with high-dose chemotherapy

Abstract

Endodermal sinus tumors (EST) of the lower female genital tract are uncommon malignancies. Most of these tumors are found in the vagina or in the cervix. Only seven cases of EST involving the vulva are reported. We report an eighth case of vulvar EST. The patient recurred and was successfully treated with high-dose chemotherapy and peripheral blood cell transplantation. Because of their location, an early diagnosis of vulvar EST should be possible. Resection of the lesion by using a modified radical vulvectomy followed by cisplatin-based chemotherapy seems the best treatment for these tumors today. Our patient was initially treated with the help of local surgery and adjuvant chemotherapy consisting of three courses of etoposide and cisplatin. Sixteen months after the first diagnosis, pleural and lung metastases were diagnosed. Second-line chemotherapy followed by high-dose chemotherapy with autologous peripheral blood stem cell support was administered after resection of the lung metastases and biopsy of the pleural metastases. There is no evidence of disease 40 months after the diagnosis of the lung and pleural metastases, and 56 months after the primary diagnosis.

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

“…Extrago- nadal YST are uncommon and those involving the female genital tract even more so. The vagina and cervix are more commonly involved [6] , and vulval involvement is extremely rare with only 11 cases reported so far [1][2][3][4] . Histogenesis of extragonadal germ cell tumours has been explained as due to the aberrant migration of germ cells during early embryonic life, which normally occurs from the yolk sac to genital ridges.…”

“…Traen et al [1] reviewed 8 cases in 2004. Since then 3 more cases have been reported [2][3][4] , making a total of 11 cases in the literature.…”

“…Since then 3 more cases have been reported [2][3][4] , making a total of 11 cases in the literature. Vulvar YST has shown some differences in behaviour from similar tumours at other more common sites (like ovary and mediastinum) that include absence of abnormal levels of ␣ -fetoprotein (AFP) in serum and a more aggressive behaviour [1] . This has implications in the management of this tumour.…”

Unusual Presentations of Gynecologic Tumors: 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. 1 However, in the case of yolk sac tumor primary in the vulva, misplaced/aberrant germ cell migration along the gubernaculum is the leading hypothesis.…”

“…None of the reported vulvar yolk sac tumors to date have had an associated somatic component; however, 3 cases did have other germ cell elements. 18,25,29 Recognition of other elements is important in the event they make up the sole component of a recurrence. The clinical and histologic features of the Immunoperoxidase studies used to arrive at a correct diagnosis for the last case described in Table 1.…”