Papillary cystadenomas of the epididymis (PCE), are seen in approximately 10-26% of men with VHL (12,17,50). PCEs are rarely found as unilateral lesions in the general population but when they are bilateral they are virtually pathognomonic of VHL. Epididymal cysts with no solid component are also commonly reported in VHL but are seen in 23% of the general population (105) making epididymal cysts an unreliable diagnostic characteristic for VHL.
The PCE can be unilateral or bilateral and is most often found in the globus
major, the head of the epididymis. PCE may involve the spermatic cord as
well (57). The lesions range in size from 1 5cm in diameter but are typically
2-3cm (56,57). PCEs are firm and easily palpable but can contain cystic
spaces containing clear yellow or hemorrhagic fluid (106). Histologically,
PCE resembles endolymphatic sac cysts and renal cysts lined by clear cells
containing fat and glycogen with tubular and papillary structures, a fibrous
stroma and surrounding pseudo-capsule of dense collagenous tissue (57).
The patient may report a hard smooth "pebble" in the scrotum (17).
Infertility, presumably due to obstructive azospermia has been reported
and atrophy of the seminiferous tubules of the testicle may be seen (18,50,56).
Imaging is not generally required for these lesions since they are often palpable. Ultrasound will demonstrate a solid mass occasionally showing some distinct cystic spaces (Figure 20).
Figure 20. Papillary cystadenoma of the epididymis. This lesion was
palpable as a firm mass in the globus of the epididymis. On sonography it
is principally solid and has a non-shadowing echogenic focus within it (arrow).
E is the epididymal mass, T is the testicle.Echogenic shadowing or calcifications may be seen. The cysts may be so closely spaced as to make the cystic areas appear echogenic. Dilated rete testes may be seen with ectatic ductules within the testicle. Testicular atrophy may be seen. There is no malignant potential and surgery is not performed unless the patient exhibits severe pain.
A histologically identical lesion to the PCE in males is rarely found in the broad ligament, the embryologic counterpart in women (75). A report describes a partially calcified, 3cm multiloculated cyst in the midportion of mesosalpinx which contained cystic chambers (107). In our experience such lesions are exceedingly rare and more commonly represent incidental ovarian cysts or endometriomas.
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