Tumors of the testis S. Vahidi M.D

Tumors of the testis Introduction: 1-8 new cases/ 100000 male/year 90-95% germ cell tumors Survival of patients has improved dramatically Higher socioeconomic/lower classes 2/1 R>L 1-2%: Bilateral. Seminoma-lymphoma 7-10% of T-tumors develop in cryptorchidism Orchiopexy does not alter the malignant potential Exogenous estrogen Adm. To the mother during pregnancy

Tumors of the testis Classification:  Primary  Benigne  Secondary  Malignant  Germ cell  Non germ cell  Germ cell tumors: Seminoma Nonseminomatous: Embryonal Teratoma Choriocarcimoma Mixed tumors

Totipotential germ cell (totipotential tumor cell) Tumors of the testis Tumorigenic hypothesis: Normal spermatocyte Totipotential germ cell Seminoma ? Embryonal carcinoma (totipotential tumor cell) Extra embryonic differentiation Intra embryonic differentiation Trophoblastic pathways Yolk sac pathways Choriocarcimoma Yolk sac tumor Teratoma

Tumors of the testis Pathology A. Seminoma (35%) Classic (85%) Anaplastic (5-10%) Spermatocytic (5-10%) B. Embryonal cell carcimoma (20%) Adult type Infantile type (yolk sac tumor) C. Teratoma (5%) D. Choriocarcinoma (<1%) E. Mixed cell type (40%) Teratocarcinoma F. Carcimoma in situ (CIS)

Tumors of the testis Patterns of metastatic spread Step wise lymphatic fashion. (T1-T4) RT testis: intra aortocaval at RT K.hilum Precaval-preaortic-Paracaval-RT common Iliac- RT ext Iliac nodes LT testis: paraaortic area at LT K. Hilum Pre aortic- LT common Iliac- LT ext. Iliac nodes RT to LT crossover metastasis: common LT to RT crossover metastasis: No Visceral metastases: lung – liver-brain – bone- kidney-adrenal . GI. Spleen Hematogenous : choriocarcinoma

Tumors of the testis Clinical staging T-primary tumors Cannot be assessed No evidence of primary tumor Intratubular cancer (CIS) Limited to testis and epididymis, no vascular invasion Invades beyond tunica albuginea or has vascular invasion Invades spermatic cord Invades scrotum TX: T0: Tis: T1: T2: T3: T4: N-Regional lymph nodes No regional lymph node metastasis Lymph node metastasis≤ 2cm, or multiple nodes, none more than 2 cm. and<6 nodes positive Nodal mass>2cm and ≤5cm. Or ≥6 nodes positive Nodal mass> 5 cm NX: N1: N2: N3: M-Distant metastasis No distant metastasis Distant metastasis present in nonregional lymph nodes or lungs Nonpulmonary visceral metastases MX: M0: M1: M2: S-Serum tumor markers Markers not available Marker levels within normalimits Lactic acid dehydrogenase (LDH)<1.5xnormal and HCG<500 mlU/ml and AFP<1000ng/ml LDH 1.5-10xnormal or hCG 5000-50000mIU/ml or AFP 1000-10000 ng/ml LDH>10xnormal or hCG>50000 mIU/ml or AFP>10000 ng/ml SX: S0: S1: S2: S3:

Tumors of the testis Clinical findings A-symptoms: Painless enlargment of testis Acute testicalor pain (10%) Symptoms related to metastasis (10%): back pain-cough-dyspnea anorexia-nausea- bone pain- lower ext. edema Asymptomatic (10%)

Tumors of the testis Clinical findings B: signs: Testicular mass or diffuse enlargment Node palpation Gynecomastia Hemoptysis

Tumors of the testis Laboratory findings and tumor markers: Anemia-liver function tests- creatinin Tumor markers: αFP βHCG LDH PLAP-GGT AFP(%) hCG(%) 38 64 70 7 25 57 60 100 Seminoma Teratoma Teratocarcinoma Embryonal choriocarcinoma

Tumors of the testis Imaging Ultrasonography CXR CT-Scan Pedal LAG For staging

Tumors of the testis Differential diagnosis Epdidymitis & Epididymoorchitis Hydrocele Spermatocele Hematocele Granulomatous orchitis Varicocele Epidermoid cyst

Tumors of the testis Treatment Inguinal Exploration & radical orchiectomy Low stage seminoma: retroperitoneal irradiation 95% care high stage seminoma: primary chemotherapy 95% complete response

Tumors of the testis Treament C: low stage NSGCT: R.O +RPLND (N22cycles chemotherapy) Surveillncc: 1)NSGCT confined within tunica albuginea 2)No vascular invasion 3)normalize tumor markers 4)no evidence of disease in radiographic imaging 5)the patient is considered reliable Modified RPLND: Nodal tissue dissection ipsilateral to the tumor below the level of the inferior mesenteric artery Clinical stage I+ vascular invasion: 2 cycles of chemotherapy

Tumors of the testis Treatment High stage NSGCT: primary chemotherapy (+RPLND?) Normal tumor marker+retroperitoneal massmass resection tumor markersalvage chemotherapy (upto 70% care) High risk patients: mediastinal primary tumor non pulmonary visceval metastasis S3 marker levels

Tumors of the testis Follow up: careful exam of remaining testis, abdomen, lymph node area lab (AFP, BHCG-LDH) CXR every 3 month/ first 2 years every 6 month/ until 5 years and then yearly Surveillance follow up: tumor marker at each visit. CXR & CT every 3-4 month Visit: monthly/ first 2 years bimonthly/ third year

Tumors of the testis Prognosis Seminoma: R.O+radiotherapy: stage I: 98% 5 years survival rate stage II: 92-94% 5 years survival rate stage III (chemotherapy): 35-75% NSGCT: stage I: 96-100% low, volume stage II: 90% stage III: 55-80%

Tumors of the testis Non-Germ cell tumors 5-6% of all testis tumor leydig cell tumors most common 5-9 & 25-35 years old no association with UDT clinical finding: virilization (prepubertal) asymptomatic (adults). 10% malignant Treatment : radical orchiectomy- RPLND Sertoli cell tumors exceedingly rare 10% malignant radical orchiecty+RPLND Gonadoblastoma in gonadal dysgenesis(80% female fenotype) radical orchiectomy + contralateral gonadectomy

Tumors of the testis Secondary tumors of the testis 1. lymphoma most common t.t. in>50 years old 2. leukemia t.Biopsy is choice 3. metastatic tumor: prostate- lung –GI-melanoma-kidney

Tumors of the testis Extragonadal germ cell tumors 3% of all germ cell tumors the most common sites: mediastinum-retroperitoneum, sacrococcygeal – pineal gland

Tumors of the testis T. Of epididym: commonly benign: adenomatoid Tumors of the epididymis, paratesticular tissue & spermatic cord T. Of epididym: commonly benign: adenomatoid leiomyoma cystadenoma T. of spermatic cord: lipoma Rabdomyosarcoma leiomyosarcoma. Fibrosarcoma liposarcoma