Wolffian

In the early mammalian female embryo the absence of the Mullerian inhibitory substance MIS permits continuing development of the Mullerian duct, while the absence of testosterone permits the Wolffian duct to degenerate. However, positive developmental signals are also required. Among these is the protein Wnt-4, a member of a large family of locally acting signal molecules (Section 4). Wnt-4 may be needed both for oocyte development and for further suppression of male development.261... 

Turner KJ, McIntyre BS, Phillips SL, et al. (2003) Altered gene expression during rat Wolffian duct development in response to in utero exposure to the antiandrogen linuron. Toxicological Science 74(1) 114-128. 

In the genotypic female, lack of testosterone and MIS causes regression of the wolffian ducts and maintenance of the mul-lerian ducts, thus forming the female reproductive tract. " Gonadotropin activity in utero is suppressed because of high concentrations of circulating estrogens derived from the mother. ""... 

After the fetal gonads are converted into testes by SRY, testosterone and Miillerian-inhibiting substance (MIS) control subsequent sexual differentiation. MIS causes the regression of the Mullerian duct, which would otherwise develop into female genital tracts. Testosterone promotes the development of the Wolffian duct into male internal genitalia, including epididymis, vas deferens, and seminal vesicles. 

The female adnexal tumor of Wolffian origin (FATWO) is a distinctive tumor that arises in the broad ligament, attached to the mesosalpinx, or, rarely, in the ovary. 

Kariminejad MH, Scully RE. Female adnexal tumor of probable Wolffian origin A distinctive pathologic entity. Cancer. 1973 31 671-677. 

Young RH, Scully RE. Ovarian tumors of probable Wolffian origin A report of 11 cases. Am J Surg Pathol. 1983 7 125-136. 

Tavassoli FA, Andrade R, Merino M. Retiform wolffian adenoma. In Fenoglio-Preiser CM, Wolffe M, Rilke F, eds. Progress in surgical pathology, vol. XL New York Field and Wood Medical Publishers 1990 121-136. 

Karpuz V, Berger SD, Burkhardt K, et al. A case of endometrioid carcinoma of the fallopian tube mimicking an adnexal tumor of probable Wolffian origin. APMIS. 1999 107 550-554. 

Fukunaga M, Bisceglia M, Dimitri L. Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin. Adv Anat Pathol. 2004 11 269-272. 

Devouassoux-Shisheboran M, Silver SA, Tavassoli FA. Wolffian adnexal tumor, so-called female adnexal tumor of probable Wolffian origin (FATWO) Immunohistochemical evidence in support of a Wolffian origin. Hum Pathol. 1999 30 856-863. 

Rahilly MA, Williams ARW, Krausz T, et al. Female adnexal tumour of probable Wolffian origin A clinicopathological and immunohistochemical study of three cases. Histopathology. 1995 26 69-74. 

Tiltman AJ, Allard U. Female adnexal tumours of probable Wolffian origin An immunohistochemical study comparing tumours, mesonephric remnants and paramesonephric derivatives. Histopathology. 2001 38 237-242. 

Defects in the ENS are significant in view of the association of RET with Hirshsprung s disease. The ENS derives from neural crest cells which express Ret when in the developing gut and both Gdnf and Gfr-al are expressed in the gut mesenchyme. Lack of kidneys in mutant mouse embryos is due to the failure of the ureteric bud (which expresses Ret) to develop from the Wolffian duct and expand into the metanephric blastema (which expresses Gdnf). This would normally initiate the formation of the metanephric (adult) kidney, but in the absence of ligand or receptor the blastemal cells eventually die. 

---