TESTICULAR TUMORS & DISORDERS OF EXTERNAL GENITALIA

1. TESTICULAR TUMORS & DISORDERS OF EXTERNAL GENITALIA DEPARTMENT OF UROLOGY IAŞI – 2010

2. TESTICULAR TUMORS • rare [0.8 (Japan) – 6.7 (Scandinavia) new cases/100,000 males/yr] • 90-95% are germ cell tumors (seminoma & nonseminoma) • effective combination chemotherapy  overall 5-year survival rate: 78% (1975) & 91% (1985) • 1-2% are bilateral – 50% with history of cryptorchidism • risk factors • congenital – cryptorchidism (7-10%) • acquired – exogenous estrogen administration during pregnancy, trauma & infection-related testicular atrophy (?) • tumor development – totipotential germ cell • normal differentiation  spermatocyte • abnormal development  seminoma or embryonal carcinoma

3. TESTICULAR TUMORS • embryonal carcinoma (totipotential tumor cell)  teratoma (intraembryonic diff.), choriocarcinoma or yolk sac tumor (extraembryonic diff.) PATHOLOGY • seminoma (35%) – 4th decade of life; grossly, coalescing gray nodules; syncytiotrophoblastic elements (10-15%)  hCG • embryonal cell carcinoma (20%) – adult & infantile type (yolk sac tumor = endodermal sinus tumor, most common in children); microscopically, embryoid bodies • teratoma (5%) – mature: benign structures from ectoderm, mesoderm and endoderm; imature: undifferentiated primitive tissue • choriocarcinoma (<1%) – aggressive tumors, early hematogenous spread; microscopically, syncytio- & cytotrophoblast

4. TESTICULAR TUMORS • mixed cell type (40%) – teratocarcinomas etc. METASTATIC SPREAD & CLINICAL STAGING • stepwise lymphatic spread – regional lymph nodes at the level of the renal hilum; for the right testis: interaortocaval area  precaval  preaortic  right common iliac  right external iliac; for the left testis: para-aortic area  preaortic  left common iliac  left external iliac • no crossover metastases to the right side, but common right-to-left metastases (!) • choriocarcinoma – early hematogenous spread • visceral metastases: lung, liver, brain, bone, kidney, adrenal, gastrointestinal tract, spleen

5. STADIERE TNM classification for testicular cancer (UICC, 2002, 6th edition) pTis Intratubular germ cell neoplasia (carcinoma in situ) pT1 Tumour limited to testis and epididymis without vascular/lymphatic invasion: tumour may invade tunica albuginea but not tunica vaginalis pT2 Tumour limited to testis and epididymis with vascular/lymphatic invasion, or tumour extending through tunica albuginea with involvement of tunica vaginalis pT3 Tumour invades spermatic cord with or without vascular/lymphatic invasion pT4 Tumour invades scrotum with or without vascular/lymphatic invasion N1 Metastasis with a lymph node mass 2 cm or less in greatest dimension or multiple lymph nodes, none more than 2 cm in greatest dimension N2 Metastasis with a lymph node mass more than 2 cm but not more than 5 cm in greatest dimension, or multiple lymph nodes, any one mass more than 2 cm but not more than 5 cm in greatest dimension N3 Metastasis with a lymph node mass more than 5 cm in greatest dimension M1 Distant metastasis M1a Non-regional lymph node(s) or lung M1b Other sites Sx Serum marker studies not available or not performed S0 Serum marker study levels within normal limits LDH (U/l) hCG (mIU/ml) AFP (ng/ml) S1 < 1.5 x N and < 5,000 and < 1,000 S2 1.5-10 x N or 5,000-50,000 or 1,000-10,000 S3 > 10 x N or > 50,000 or > 10,000

6. TESTICULAR TUMORS CLINICAL FINDINGS • symptoms • painless enlargement of the testis, testicular heaviness • acute testicular pain (10%) – intratesticular hemorrhage or infarction • metastatic disease (10%) – back pain (retroperitoneal); cough or dyspnea; anorexia, nausea or vomiting; bone pain; lower extremity swelling (venacaval obstruction) • signs • testicular mass (firm, nontender) or diffuse enlargement • hydrocele may accompany the tumor • palpation of the abdomen – bulky retroperitoneal disease • supraclavicular or inguinal nodes • gynecomastia (5%)

7. TESTICULAR TUMORS INVESTIGATIONS • elevated serum creatinine – ureteral obstruction • biochemical markers • AFP – NSGCTs • β-hCG – NSGCTs (choriocarcinoma – 100%) & seminomas (7%) • LDH – NSGCTs & seminomas • imaging • scrotal US • chest x-ray • CT scan (abdomen & pelvis) • inguinal orchiectomy

8. TESTICULAR TUMORS AJCC (American Joint Committee on Cancer) STAGE GROUPING 0 Tis N0 M0 S0 I T1-4 N0 M0 SX II T1-4 N1-3 M0 SX III T1-4 N0-3 M1 SX IA T1 N0 M0 S0 IIA T1-4 N1 M0 S0-1 IIIA T1-4 N0-3 M1a S0-1 IB T2-4 N0 M0 S0 IIB T1-4 N2 M0 S0-1 IIIB T1-4 N1-3 M0/M1a S2 IS T1-4 N0 M0 S1-3 IIC T1-4 N3 M0 S0-1 IIIC T1-4 N1-3 M0/M1a S3 sau M1b S0-3 DIFFERENTIAL DIAGNOSIS • epididymitis or epididymoorchitis, granulomatous orchitis • hydrocele, spermatocele, hematocele, varicocele, epididymal cysts

9. TESTICULAR TUMORS TREATMENT • radical orchiectomy • low-stage seminoma (I, II-A/B)  retroperitoneal irradiation (25-30 Gy) • high-stage seminoma (II-C/III) primary chemotherapy (PEB, VAB-6, cisplatin + etoposide) • low-stage NSGCT (I, II-A/B)  RPLND or surveillance • high-stage NSGCT (II-C/III) primary chemotherapy ± RPLND

10. PRIAPISM • prolonged painful erection; no sexual excitement or desire • idiopathic (60%) • secondary (40%) – leukemia, sickle cell disease, pelvic tumors, pelvic infections, penile trauma, spinal cord trauma or use of medications (intracavernous injection) • obstruction of the venous drainage  highly viscous, poorly oxigenated blood within the corpora cavernosa  interstitial edema and fibrosis of the corpora cavernosa  impotence • epidural or spinal anesthesia, evacuation of sludged blood from the corpora cavernosa through a large needle, intracavernous adrenergic agents (norepinephrine, Levophed), shunt between the glans penis and corpora cavernosa (biopsy needle), anastomosing the superficial dorsal vein to the corpora cavernosa, corpora cavernosa to corpus spongiosum and saphenous vein to corpora cavernosa

11. PEYRONIE DISEASE • plastic induration of the penis – painful erection, curvature of the penis and poor erection distal to the involved area • examination – palpable dense, fibrous plaque, usually near the dorsal midline, involving the tunica albuginea of the penile shaft • spontaneous remission ≈50% of cases • p-aminobenzoic acid (powder or tablets) or vitamin E (tablets) for several months • refractory cases – excision of the plaque with replacement with a dermal graft, the use of tunica vaginalis grafts after plaque incision and incision of the plaque with insertion of penile prostheses in the corpora cavernosa • additional methods – radiation therapy and injection of steroids, dimethyl sulfoxide or parathyroid hormone into the plaque

12. PHIMOSIS • the contracted foreskin cannot be retracted over the glans • cause - chronic infection (balanoposthitis) from poor local hygiene • calculi and squamous cell carcinoma may develop under the foreskin • signs – edema, erythema and tenderness of the prepuce, purulent discharge • incision of the dorsal foreskin • circumcision (posthectomy), after the infection is controlled

13. PARAPHIMOSIS • the foreskin, once retracted over the glans, cannot be replaced in its normal position • cause – chronic inflammation under the redundant foreskin  contracture of the preputial opening (phimosis) • tight ring behind the glans  venous congestion  edema and enlargement of the glans  arterial occlusion and necrosis of the glans • squeeze of the glans for 5 min, then reduction (phimosis) • incision of the constricting ring, under local anesthesia • circumcision – after inflammation has subsided

14. VARICOCELE • 10% of young men • dilatation of the pampiniform plexus above the testis (left side most commonly affected !) • ! sudden development of a varicocele in an older man ≈ late sign of renal tumor, that has invaded the renal vein, occluding the spermatic vein • examination – mass of dilated, tortuous veins lying posterior to and above the testis; degree of dilatation can be increased by the Valsalva maneuver; testicular atrophy (impaired circulation); sperm concentration and motility are significantly decreased (65-75%) infertility • ligation of the internal spermatic veins; percutaneous methods (balloon catheter, sclerosing fluids) to occlude the veins, following percutaneous spermatic venography

15. HYDROCELE • collection of fluid within the tunica or processus vaginalis • may develop rapidly secondary to local injury, radiotherapy, acute nonspecific or tuberculous epididymitis or orchitis and testicular neoplasm ! • diagnosis – rounded cystic intrascrotal mass, that is not tender; the mass transilluminates • differential diagnosis – testicular tumor – US • indications for treatment – tense hydrocele that embarrass circulation to the testicle or large, bulky mass, uncomfortable for the patient • hydrocele sac is opened and stitched together to collapse the wall (Lord’s procedure)

16. SPERMATIC CORD - TORSION • most often seen in adolescent males • congenital abnormality (voluminous tunica vaginalis, that inserts well up on the cord and allows the testis to rotate) + contraction of the cremaster muscle  left testis rotate counterclockwise and right testis clockwise  vascular occlusion  ischemic death of the testis and epididymis • diagnosis – young boy suddenly develops severe pain in one testicle, followed by swelling of the organ, reddening of the scrotal skin, lower abdominal pain, nausea and vomiting • examination – swollen, tender organ, that is retracted upward (shortening of the cord by volvulus); pain may be increased by lifting the testicle up (pain from epididymitis is usually alleviated) – Prehn’s maneuver

17. SPERMATIC CORD - TORSION • differential diagnosis – acute epididymitis, acute orchitis and trauma – color Doppler US (absence of arterial flow in torsion, hypervascularity in inflammatory lesions); scintillation scan (99mTc-pertechnetate) – avascular (torsion), increased vascularity (testicular tumor) or decreased vascularity (trauma) • manual detorsion may be attempted (the right testis should be “unscrewed” and the left one “screwed up”) after local anesthesia; ! surgical fixation of both testes should be done within the next few days • if manual detorsion fails  immediate surgical detorsion & orchydopexy • detorsion within 6 h of onset – good result; delayed beyond 24 h –orchiectomy