Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome: A case report

Table 1 Summary of primary cervical or vaginal carcinoma in Herlyn-Werner-Wunderlich syndrome

	Ref.	Age (years)	Gravida/para	DES exposure	Symptoms	Site	Stage	Side (obstructed or non-obstructed)	Pathology	Adenosis	Treatment	Outcome
												Follow-up time (years)	Outcome
1	GRANT et al[5]	35	G0P0	/	Irregular vaginal bleeding	Cervix	II	Non-obstructed side	AC	/	Hysterectomy	/	NED
2	Cordoba et al[6]	37	G7P2	/	Menorrhagia and irregular vaginal bleeding	Cervix	IIIa	Obstructed side	AC	/	Aortic lymphadenectomy + concomitant chemoradiotherapy	3	NED
3	Kaba et al[7]	49	P2	/	Menorrhagia and intermenstrual bleeding	Cervix	Ib1	Obstructed side	EAC	/	Radical hysterectomy + bilateral salpingo-oophorectomy + pelvic-paraaortic lymphadenectomy + omentectomy; radical parametrectomy and proximal vaginectomy	1.3	NED
4	Watanabe et al[8]	33	G2P2	No	Irregular vaginal bleeding	Cervix	IVa	Obstructed side	EAC	/	Chemotherapy+ external radiation; anterior pelvic exenteration + total vaginectomy	/	/
5	Kusunoki et al[9]	65	G3P2	No	Postmenopausal bleeding	Cervix	I	Obstructed side	CCA	/	Hysterectomy + tumor resection + concurrent Chemoradiation	1	NED
6	Zong et al[10]	20	/	/	/	Cervix	I	/	CCA	/	Radical hysterectomy + bilateral salpingo-oophorectomy + pelvic lymphadenectomy + total vaginectomy	3.0	NED
7	Zong et al[10]	27	/	/	/	Cervix	IIb	/	/	/	Concurrent chemoradiation, hysterectomy + bilateral salpingo-oophorectomy + total vaginectomy, chemotherapy	2	Local recurrence and kidney failure 1 year later; DOD
8	Zong et al[10]	31	/	/	/	Cervix	IIa	/	CCA	/	Chemotherapy,hysterectomy + bilateral salpingo-oophorectomy + pelvic-paraaortic lymphadenectomy, chemotherapy, concurrent Chemoradiation	1.5	Local recurrence and distant metastases 4 months later; DOD
9	Zong et al[10]	38	/	/	/	Cervix	IIa	/	/	/	Radical hysterectomy + bilateral salpingo-oophorectomy + pelvic-paraaortic lymphadenectomy, radiotherapy	2	Distant metastases after 1 year; DOD
10	Zeeshan-ud-din et al[11]	27	/	No	Irregular vaginal bleeding	Vagina	III	Obstructed side	CCA	/	Wertheim's hysterectomy + partial vaginectomy + pelvic lymphadenectomy	/	/
11	Watanabe et al[8]	53	G2P2	No	Vaginal pain and irregular vaginal bleeding	Vagina	I	Obstructed side	CCA	/	Radical hysterectomy	/	/
12	Uehara et al[12]	54	G4P3	No	Vaginal bleeding	Vagina	I	Obstructed side	CCA	Yes	Anterior pelvic exenteration	3.6	NED
13	Present case	40	G1P1	No	Irregular vaginal bleeding	Vagina	I	Obstructed side	CCA	Yes	Hysterectomy + tumor resection + chemotherapy (1 cycle)	5	Lung metastasis 1 year post-operative, had it resected; NED till now

AC: Adenocarcinoma; EAC: Endometrioid adenocarcinoma; CCA: Clear cell adenocarcinoma; NED: No evidence of disease; DOD: Died of disease; DES: Diethylstilbestrol.