Testicular Tumor-Kenan PDF

Testicular tumors Dr.Kenan ÖZTORUN, MD, FEBU Urology  Importance Testicular tumors are rare. 1 – 2 % of all malignant tumors. Most common malignancy in men in the 15 to 35 year age group. Most curable solid neoplasms and serves as a...

Testicular tumors Dr.Kenan ÖZTORUN, MD, FEBU Urology  Importance Testicular tumors are rare. 1 – 2 % of all malignant tumors. Most common malignancy in men in the 15 to 35 year age group. Most curable solid neoplasms and serves as a paradigm for the multimodal treatment of malignancies. Seminoma - most common bilateral primary testicular tumour; Lymphoma - most common bilateral testicular tumour  Anatomy The testis is the male gonad. It is homologous with the ovary in female. It lies obliquely within the scrotum suspended by the spermatic cord The left testis is slightly lower than the right Shape: Oval Size:3.75 cm long, 2.5 cm broad, 1.8 cm thick Weight: about 10-15 gm. Has 2 poles , 2 surface, 2 borders  Etiology Cryptorchidism Intersex disorder Testicular atrophy Chromosomal abnormalities - loss of chromosome 11, 13, 18, abnormal chromosome 12p. Exogenous estrogen administration to mother during pregnancy Carcinoma-in-situ Previous testicular malignancy  Cryptorchidism 7 - 10% patients - history of cryptorchidism Most common - seminoma 5 - 10% tumors - contralateral testis Relative risk - Intraabdominal testis (1 in 20) > Intrainguinal testis (1 in 80) Orchidopexy - does not alter malignant potential - facilitates examination & detection  Malignancy due to Abnormal germ cell morphology. Elevated temperature - abnormal spermatocyte maturation. Endocrinal disturbances. Gonadal dysgenesis.  Carcinoma in-situ Pre malignant precusor of all GCT, except spermatocytic seminoma. Incidence - 0.8%. Testicular CIS develops from fetal gonocytes. Characterized histologically by seminiferous tubules containing only Sertoli cells and malignant germ cells.  Risk Factors for CIS: History of testicular carcinoma (5% to 6%), Extra gonadal GCT (40%), Cryptorchidism (3%), Contralateral testis with unilateral testis cancer (5% to 6%), Somatosexual ambiguity (25% to 100%) Atrophic testis 30 % Infertility (0.4% to 1.1%) TESTICULAR BIOPSY gold standard for diagnoses of CIS  Classification Malignant Testicular Tumors Germ Cell Tumors >90% Non-Germ Cell Tumors Seminomas Non-Seminomatous Tumors Interstitial Cell Tumors Other Tumors Classical 85% Embryonal Carcinoma Leydig Cell Tumors Lymphoma Spermatocytic 5% Yolk Sac Tumor Sertoli Cell Tumors Metastases Anaplastic 10% Choriocarcinoma Teratoma Mixed Tumors  Dramatic Improvement in Survival: Effective diagnostic techniques Improved tumor markers Multi-modal treatment - Surgical + Radiotherapy/Chemotherapy Mortality markedly decreased.  Seminoma Classical: 80 - 85% Anaplastic: 5 - 10% middle age middle age PLAP & B-hCG - raised slow growing aggressive good prognosis higher local & metastatic potential Spermatocytic: 5 - 10% high B-hCG production age > 50 years Inguinal Orchiectomy + Adj therapy low metastatic potential good prognosis  Non-Seminomatous Germ Cell Tumors Embryonal Carcinoma Yolk Sac Tumor/ Endodermal Sinus Tumor 25 - 35 years Most common testicular tumor in infants & children 3 - 6% testicular tumors Raised AFP small, rounded, irregular mass Histologically - cells demonstrate vacuolated invades tunica albuginea cytoplasm secondary to fat and glycogen deposition, resemble 1 - 2 week old embryos  Chorionic Carcinoma Teratoma pure choriocarcinoma - rare raised AFP second - third decades resistant to both chemotherapy & radiotherapy raised PLAP, B-hCG mature teratoma - differentiated elements from 2-3 embryonic germ cell layers high incidence of distant metastases immature teratoma - undifferentiated primitive tissue malignant teratoma - malignant changes  Sertoli Cell Tumors Leydig Cell Tumors rare; < 1% testicular tumors most common non-germ cell cell timor of testis - 1 - 3% testicular tumours bimodal age distribution - < 1 year; 20 - 45 years age bimodal age distribution - 5 - 9 years; 25 - 35 years age presentation - testicular mass; virilization in children; gynecomastia in adults 25% cases - childhood; bilaterally - 5 - 10% cases Gonadoblastomas presentation - prepubertal children - rare virilization; adults - gynecomastia seen in patients with gonadal dysgenesis elevated serum & urinary 17-ketosteroids & estrogens age group - 30 years of age clinical presentation - gonadal dysgenesis  Secondary Tumors of Testis Leukemic Infiltration of Testis Lymphoma relapse of children with acute lymphocytic most common testicular tumour over age of 50 leukaemia years bilateral - 50% cases most common secondary neoplasm of testis treatment - bilateral testicular irradiation with 20Gy & reinstitution od adj chemotherapy presentation - painless enlargement of testis, constitutional symptoms in 25% patients; Metastatic Tumor bilateral - 50% patients rare most common primary - prostate > lung > gastrointestinal tract > melanoma > kidney  Extragonadal Germ Cell Tumor Rare - 3% all germ cell tumors Sites - mediastinum > Retroperitoneum > Sacrocoocygeal > Pineal gland Presentation - site & volume of disease Mediastinal lesions - pulmonary symptoms Sacrococcygeal - neonates - palpable mass, bowel/ urinary obstruction Pineal - headache, visual/ auditory complaints, hypopituitarism Treatment - same as testicular tumors.  Lymphatic drainage Right Testis - Inter-aortocaval nodes > Precaval > Preaortic > Right common iliac > Right external iliac Left Testis - Left Para-aortic > Preaortic > Left common iliac > Left external iliac Cross over from right to left possible. Epididymis - external iliac chain. Inguinal node metastasis - scrotal involvement by the primary tumor, prior inguinal or scrotal surgery, or retrograde lymphatic spread secondary to massive retroperitoneal lymph node deposits. Testicular cancer spreads in a predictable and stepwise fashion, except choriocarcinoma.  Metastatic Spread Distant metastases - Lung > Liver > Brain > Bone > Kidney > Adrenal Glands > Gastrointestinal Tract > Spleen Germ cell tumors - Lymphatic spread Choriocarcinomas - Hematogenous spread - lungs  Clinical Presentation Painless testicular swelling Dull ache/heaviness in Lower Abdomen 10% - Acute Scrotal Pain 10% - Metatstasis Neck Mass /Cough /Anorexia /Vomiting /Back ache/Lower limb swelling 5% - Gynecomastia - Estrogen producing tumors Rarely - Infertility  Physical Examination Firm to hard fixed area within tunica albugenia - suspicious Seminoma expand within the testis as a painless, rubbery enlargement. Embryonal carcinoma or teratocarcinoma may produce an irregular, rather than discrete mass. Choriocarcinoma - no testicular enlargement  Differential Diagnosis Testicular torsion Epididymitis, or epididymo-orchitis Hydrocele Hernia Hematoma Spermatocele Syphilitic gumma  Investigations All patients with a solid, firm intra-testicular mass that cannot be transilluminated should be regarded as malignant unless otherwise proved. Scrotal Ultrasound - rapid, reliable technique hypoechoic area within the tunica albuginea - markedly suspicious for testicular cancer.  Tumor markers Onco-fetal Substances: AFP & HCG Cellular Enzymes: LDH & PLAP  Alfa-fetoprotein Raised AFP : T ½ 5-7 days Pure embryonal carcinoma Teratocarcinoma Yolk sac Tumor Combined tumors AFP not raised in pure choriocarcinoma & pure seminoma  Human Chorionic Gonadotropin T ½ 48-72 hours Raised β hCG 100 % - Choriocarcinoma 60% - Embryonal carcinoma 55% - Teratocarcinoma 25% - Yolk Cell Tumour 7% - Seminomas  Role of Tumor Markers Diagnosis 80 - 85% testicular tumors - positive markers post orchiectomy elevated markers - stage II/III disease post lymphadenectomy residual disease - stage III disease histology of tumor Burden of disease - degree of marker elevation Follow-up markers becoming positive on follow-up - recurrence markers become positive earlier than x-rays  Imaging studies USG Scrotum Chest X ray - Pulmonary Metastasis - 85 - 90% metastases CECT abdomen & pelvis – Retroperitoneal nodes  Staging of Disease Pre-requisites history + clinical examination tumor markers - hCG, AFP Radiology - USG Scrotum, CECT Abd, X-Ray Chest Pathology of tumor specimen  LDH Beta HCG AFP (mIu/ml) (ng/ml) S1 < 1.5 x N 50000 >10000  Prognostic Grouping Stage T N M S Stage 0 Tis N0 M0 S0 Stage 1a T1 N0 M0 S0 Stage 1b T2 - T4 N0 M0 S0 Stage 1c any T N0 M0 S1 - S3 Stage IIa any T N1 M0 S0 - S1 Stage IIb any T N2 M0 S0 - S1 Stage IIc any T N3 M0 S0 - S1 Stage IIIa any T any N M1 S0 - S1 Stage IIIb any T any N M0 - M1 S2 any T any N M0 - M1a S3 Stage IIIc any T any N M1b any S  Treatment Treatment should be aimed at one stage above clinical stage. Seminomas - radio-sensitive Non-seminomatous - radio-resistant Advanced diseases/ metastases - chemotherapy.  Treatment RADICAL INGUINAL ORCHIDECTOMY - first line of therapy Bulky Retroperitoneal Tumours/ Metastatic Tumors - Initially “DOWN- STAGED” with CHEMOTHERAPY The inguinal approach permits early control of the vascular and lymphatic supply as well as en-bloc removal of the testis with all its tunicae.  Stage I Testicular Cancer Risk Factors Seminom Non-seminom Tumor size > 4 cm LVI Rete testis invasion Proliferation rate >70% Embryonal carcinoma rate > 50%  Chemotherapy Chemotherapy Toxicity BEP - Bleomycin Pulmonary fibrosis Etoposide Myelosuppression Alopecia Renal insufficiency (mild) Secondary leukemia Cisplatin Renal insufficiency Nausea, vomiting Neuropathy Lymph Nodes Dissection For Right & Left Sided Testicular Tumours  Prognosis Seminoma Non-Seminoma Stage I 99% 95 - 99% Stage II 70 - 92% 90% Stage III 80 - 85% 70 - 80%