TRUE PROSTATIC

TISSUE

WITH ADRENOGENITAL

hl.-\RK

JOHN

II.

KI\‘IAT.

SYNDROME

hl.1).

11. LEONLARD.

From the Departments \Vashington, and the Seattle, Washington

IN 46,xX FEMALE

L1.D. of Urology and Medicine, U.S. Public Health Service

University Hospital,

of

FIGURE 1.

{A) External

genitalia

showing

complete

mcmx&nization

of urethra.

(B) Retrograde

urethrogram.

The buccal smear was positiv,e with a chromatin l)ocly identified in 20 per cent of the cells examined. The blood karyotype was 46,Xx. Skeletal radiographic examination revealed complete fusion of the epiphyses. Retrograde urethrography showed a normal male urethra with an easily recognizalrle \,erumontanuIn in the prostatic urethra (Fig. 1B). Serum electrolytes were normal. Urinary and plasma steroid levels are shown in Table I. At cystourethroscopy the urethra appeared normal with the exception of a somewhat enlarged verui11011talluIll located normally in the prostatic urethra. The utricular lumen did not appear abnormally wide in diameter and accepted a 5 F ureteral catheter snugly. Injection of contrast medium outlined a vagina, uterus, and fallopian tulles on a film of the abdomen. H~sterosalpingo-oophorectonl~, and vaginectoni!. were performed through a transperitoneal approach. The vagina was tense and filled with a mucoicl plug of a rublrery consistency. The uterus, tubes, and ovaries had the TABLE I.

Laboratory

Test CTrin e 17-hydrosycorticosteroids mg./24 hr. 17-ketosteroids mg./24 hr. Pregnanetriol rng.124 hr. Plasma 8 A. 11. cortisol pg./dl. 17-hydroxvprogesterolle /Lg. itll.

76

studicy

Normal

Range

Patient ~_.____

3-10

1.2

9-22 the f&al testis.” For fusion of‘ tllc 1lrethr;;l ant1 labioscrotal folds in thcl f&male cJlrlhr)c) to oc’c’ur. drenul androgens ni~lst .1c1 OII these structures at the ten ;o eleven-week stagy, of clr\-cloplllent. ’ Althoiigh nuiiicrous reports of t! 1x’ \’ gtwitaliu in patients with congenital adrenal hyprrplxia ha\-e apptwed iii the literal i1w 0~ er the past celltllr~~, 1ii.5-2’1in a k-u!; few of t~ic~st~reports has the presence of a prostatr lx*c?n Itoted on ph!.sical esaniinution or autol)s!..“.“.~.“.’ ‘.“’ anal iii onl) 3 other ciis~s has histolq~ic~ \ ~rification lwen ol~taint~cl.x~“~l” Prostatic tissitta lids lwcii clesc*rilxd only, in adrt~nogenit~\l s~7~c.lromt~ in patients \vitli types III to 1’ cxsteri1a1 gc7iitalia. Based on Joh~~m.“~

the

embryologic

ohwr\

ations

of

thcb anlagen of the l)rost;rtic glands in the female are first noted at thy W-min. (ten\vwk) stage. Although present in the timu of rtldimrntar!~ llrethral glands and Slkfb~~c-.s ~lantls in adult life, these struc+urt:s n(h\ t’r procwcl to

d~~vt~lop into the extensi\,e ilcAt\vorl*. of‘ 1)ranchrtl t~~l~i~lo~~l~~~ol~~r glands seen i11 thus riralc~prost:ttc. The a1~c~nce of prostatic tissue ilr tiles tyyras I

and II genital configuration

is t’\ idenw that the androgenic stimulus which pre\wlts the regression of the glantlular anlagen must lw present

l~efore the sixteenth these mild genital

kvwk of de\-elopment b~lien clefor-mitks arc detern~inetl.

One bmild also expect that the patient with a t)ye V genital configuration experiencing the earliest androgenic stimulus woidd demonstrate the most significant degree of prostatic clcvelopmmt. , such as acid phosAlthough acid hydrolases phatase, can be fhnd in lysosomes in most of the cells of the 11otly. only the acinnr cells of the prostate produce acid phosphatase in the form of secretory granules fbr export into the glandiilar this is the first lumina.” To our knowledge, histochemical clernonstration of such activity in the prostatic tissue of a human fende. of L~Idog~, RL-10 University of \Vashington Seattle, \Vashington 98195 (DR. KI\‘IAT)

I)q~“rtlllellt

True prostatic tissue in 46,XX female with adrenogenital syndrome.

TRUE PROSTATIC TISSUE WITH ADRENOGENITAL hl.-\RK JOHN II. KI\‘IAT. SYNDROME hl.1). 11. LEONLARD. From the Departments \Vashington, and the S...
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