Testicular tumours Udeh Emeka I 2013

Introduction Rare but most curable solid tumour Marked variation in incidence: Scandinavian countries 6.7/ Japan 0.8/ USA: incidence in black 1/4 th of whites Commoner in high socio economic class; right testis; Primary tumour : 1-2% are bilateral; 50% in cryptorchidism Udeh Emeka I 2013

Pathology ( classification) Germ cell tumour Sex cord/ Gonadal stromal tumour Unclassified Forms  Tumours containing germ cells/ gonadal stromal elements  Miscellaneous tumours  Lymphoid and hematopoietic tumours  Tumours of collecting ducts and rete  Tumours of tunica epididymis, spermatic cord, supporting structures and appendices Udeh Emeka I 2013

Soft tissue tumours Secondary tumours Udeh Emeka I 2013

Germ cell tumour Seminoma: Three sub types viz classic, anaplastic and spermatocytic  Classic comprises 82-85% 0f seminomas  Large cells with clear cytoplasm and densely staining nuclei  Syncytiotrophoblastic elements occur in 10-15%  Both spermatocytic and classic have low metastatic potential and favourable prognosis Udeh Emeka I 2013

Non seminomatous gem cell tumours Embryonal carcinoma: Highly malignant Neoplastic epithelioid cells arrange in glandular tubules Choriocarcinoma Presence of syncytiotrophoblast and cytotrophoblast Syncytiotrophoblast are multinucleated, irregular nuclei, eosinophilic cytoplasm Cytotrophoblast are closely packed, intermediate size Udeh Emeka I 2013

Teratoma more than one germ cell( ectoderm, mesoderm & endoderm) Appear in various stages of maturation and differentiation Yolk sac tumour: Most common in infants and children Occur rarely in adult usually in mixed forms Udeh Emeka I 2013

Non germ cell tumours Leydig cell tumour Commonest of NGCT Bimodal age distribution(5-9; 25-35) 25% occur in adult hood Bilaterality seen in 5-10% Cells are hexagonal with granular eosinophilic cytoplasm Sertoli cell tumour Rare(1%) Bimodal age distribution(0-1; 20-45years) Udeh Emeka I 2013

10% are malignant Heterogenous ( epithelial and stromal components) Gonadoblastomas Rare(0.5%) Occur commonly in gonadal dysgenesis Common in ages below 30years Three cell types sertoli cell, interstitial cell and germ cells Intratubular germ cell neoplasia ( carcinoma in situ): pre-invasive form; 50% progress;low prevalence Udeh Emeka I 2013

Aetio-pathogenesis Congenital causes  Cryptochidism : orchidopexy does not prevent Acquired causes  Trauma: still controversial  Hormones: exposure of mothers to DES or oral contraceptives  Atrophy Udeh Emeka I 2013

Malignant transformation to CIS invasion of basement membrane Replacement of testicular parenchyma Lymphatic or haematogenous spread Local involvement of epididymis and spermatic cord limited by tunica albuginea Involvement result in spread to pelvic or inguinal lymph nodes Udeh Emeka I 2013

Spread Haematogenous spread: lung, bone, liver Lymphatic spread: (predictable step wise fashion)  group of lymph node in the inter aortocaval region And paraaortic lympnodes  Cephalad drainage: cisterna chyli, thoracic duct, supraclavicular lymph nodes.  Retrogade spread to common iliac, external iliac and inguinal lympnodes  Lymphatics of epididymis: external iliac lymphnodes Udeh Emeka I 2013

Scrotal involvement: inguinal lymph nodes Udeh Emeka I 2013

Clinical features Testicular Nodules and painless swelling; scrotal heaviness, sensation of scrotal fullness, testicular pain. Infertility : antisperm antibodies, defects in spermatogenesis Metastases Neck mass Respiratory symptoms: chronic cough, hemoptysis Git disturbances Lumbar back pain Bone pain Udeh Emeka I 2013

Central and peripheral lymph nodes Gynaecomastia( βHcG or estrogen) Virilization in prepubertal children (sertoli cell and leydig cells) Udeh Emeka I 2013

Examination Palpate the testis Palpate the abdomen Udeh Emeka I 2013

Differential diagnosis Testicular torsion Epididymitis Haematoma Spermatocele hernia Udeh Emeka I 2013

Investigations Scrotal ultrasound: well defined hypoechoic lesion within the tunica albuginea; inhomogeneous with calcifications; diff testicular from extra testicular; detects intratesticular(2mm) Chest radiography: PA & Lateral views: assess the lung parenchyma and mediastinum Abdominal CT Detects retroperitoneal lymph nodes about 2cm Involvement of soft tissue and viscera Udeh Emeka I 2013

Serum tumour markers α feto protein(embryonal, teratocarcinoma, yolk sac, mixed tumours) β hcG(choriocarcinoma manily) Lactic acid dehydrogenase(all germ cell tumours; low specificity) Placental alkaline phosphatase( 40% advanced tumour have elevated value) Serum and urinary 17-keto steroids(raised in leydig cell tumours) Udeh Emeka I 2013

Staging : AJCC Primary Tumor (T) pTXPrimary tumor cannot be assessed (if no radical orchiectomy has been performed, TX is used) pT0No evidence of primary tumor (e.g., histologic scar in testis) pTisIntratubular germ cell neoplasia (carcinoma in situ) pT1Tumor limited to the testis and epididymis and no vascular/lymphatic invasion pT2Tumor limited to the testis and epididymis with vascular/lymphatic invasion or tumor extending through the tunica albuginea with involvement of tunica vaginalis pT3Tumor invades the spermatic cord with or without vascular/lymphatic invasion pT4Tumor invades the scrotum with or without vascular/lymphatic invasion Udeh Emeka I 2013

Regional Lymph Nodes (N) Clinical NXRegional lymph nodes cannot be assessed N0No regional lymph node metastasis N1Lymph node mass 2 cm or less in greatest dimension or multiple lymph node masses, none more than 2 cm in greatest dimension N2Lymph node mass, more than 2 cm but not more than 5 cm in greatest dimension, or multiple lymph node masses, any one mass greater than 2 cm but not more than 5 cm in greatest dimension N3Lymph node mass more than 5 cm in gr Udeh Emeka I 2013

Pathologic pN0No evidence of tumor in lymph nodes pN1Lymph node mass, 2 cm or less in greatest dimension and ≤6 nodes positive, none >2 cm in greatest dimension pN2Lymph node mass, more than 2 cm but not more than 5 cm in greatest dimension; more than 5 nodes positive, none >5 cm; evidence of extranodal extension of tumor pN3Lymph node mass more than 5 cm in greatest dimension Distant Metastases (M) M0No evidence of distant metastases M1Nonregional nodal or pulmonary metastases M2Nonpulmonary visceral masses Serum Tumor Markers (S) Udeh Emeka I 2013

Serum Tumor Markers (S) LDHhCG (mIU/mL)AFP (ng/mL) S0≤N S1<1.5 × N<5,000<1,000 S21.5–10 × N5,000–50,0001,000–10,000 S3>10 × N>50,000>10,000

Stage GroupingTNMS Stage 0pTisN0M0S0 Stage IT1-T4N0M0SX IaT1N0M0S0 IbT2N0M0S0 T3N0M0S0 T4N0M0S0 IsAny TN0M0S1-S3 Stage IIAny TAny NM0SX Udeh Emeka I 2013

IIaAny TN1M0S0 Any TN1M0S1 IIbAny TN2M0S0 Any TN2M0S1 IIcAny TN3M0S0 Any TN3M0S1 Udeh Emeka I 2013

Stage IIIAny TAny NM1SX IIIaAny TAny NM1S0 Any TAny NM1S1 IIIbAny TAny NM0S2 Any TAny NM1S2 IIIcAny TAny NM0S3 Any TAny NM1aS3 Any TAny NM1bAny S

Boden and Gibbs staging Stage A: lesion confined to testis Stage B: demonstrated regional lymph node involvement Stage C: spread beyond retroperitoneal lymph nodes Udeh Emeka I 2013

Treatment Depends on stage of tumour Low stage seminoma(I,II-A): radical orchiectomy + retroperitoneal irradiation± retroperitoneal lymph node dissection High Stage seminoma(II-B,III): radical orchiectomy+primary chemotherapy( platinum based regimen: PEB; cisplatinum, etoposide, bleomycin. VAB-6 Udeh Emeka I 2013

Low stage NSGCT:  Stage A disease: Radical orchiectomy+surveillance High stage NSGCT:  Stage B and C: Radical orchiectomy + chemotherapy Non germ cell tumours: Leydig cell tumour: radical orchiectomy ± RPLND Sertoli cell tumour: radical orchiectomy ± RPLND Gonadoblastomas : radical orchiectomy ± RPLND Udeh Emeka I 2013

Complications of treatment Chemotherapy : Nephrotoxicity(cisplatinum) Raynaud's phenomenon, vascular occlusive disease(bleomycin& vinblastine) Neurotoxicity( cisplatinum & vinblastine) Pnemonitis; pulmonary fibrosis (bleomycin) Infertility: cryopreservation of sperm Retroperitoneal lymph node dissection(fertility; ejaculation dysfunction) Udeh Emeka I 2013

Radiotherapy  Myelosuppression Udeh Emeka I 2013