PARATESTICULAR TUMORS Dr. Vainrib, Urology Department Meir Hospital

TESTICULAR TUMORS UNCOMMON (1-2% of all pediatric solid tu) Benign>Malignant in children Incidence peaks at age 2yrs, tapers after 4yrs and rises again at puberty Rare in Asian children and black Testicular Cancer – one of the most curable solid neoplasms

TUMORS OF TESTICULAR ADNEXA PARATESTICULAR TUMORS: Rhabdomyosarcoma Leiomyosarcoma Miscellaneous Mesenchymal Tu of the Spermatic Cord ADENOMATOID TUMORS: Mesothelioma Cystadenoma EPITHELIAL TUMORS

PARATESTICULAR TUMORS 7-10% among Primary Genitourinary Tu Occasional reports of purely testicular mesenchymal tumor ~40% of all paratesticular tu – juvenile form of rhabdomyosarcoma followed by leiomyosarcoma

PARATESTICULAR RHABDOMYOSARCOMA (RMS) Peak age of presentation: 1-5yrs Location: distal portion of cord, may invade testis Clinical presentation: unilateral, painless scrotal swelling/mass above scrotum Physical examination: firm mass, usually distinct from the testis At Dx: 60% of paratesticular RMS – Stage I >90% of paratesticular RMS – embryonal hystology (good prognosis)

PARATESTICULAR RHABDOMYOSARCOMA (RMS) Evaluation: CT to evaluate retroperitoneum for node MTX (FN rate of CT = 14%) *** - up to 30% of patients have extension to lymph nodes

PARATESTICULAR RHABDOMYOSARCOMA Macroscopic Pathology: circumscribed lesion – solid, white/gray, firm, hemorrhagic(rare) Microscopic Pathology: from totally undiff. mesenchymal elements to features of skeletal muscle fibers (ussually combination)

PARATESTICULAR RHABDOMYOSARCOMA TX Should be removed by ingiunal orchiectomy +/– RPLND Adjuvant TX: RADIATION TX – 4000-6000cGy 5-8wks CHEMOTHERAPY: Vincristine, Cyclophosphamide, Dactinomycin <10yrs + clinically LN-: CHEMOTHERAPY ALONE >10yrs: RPLND before CHEMOTHERAPY (50% chance of LN involvement) and if LN+ >>> RADIATION TX Survival: Stage I: 91% Stage II: 86% Stage III: 35% Stage IV: 5.2%

PARATESTICULAR RHABDOMYOSARCOMA RPLND: controversial RPLND+: Staging of the disease before radiation tx for involved area AGAINST: Grossly involved LN are seen in CT Significant morbidity associated with surgery Intestinal obstruction Ejaculatory dysfunction Edema of lower extremity Microscopic LN disease effectively treated by chemotherapy Diagnostic Laparoscopy - ???

CASE REPORT History: Physical examination: 5yrs old, healthy Occasional finding of painless lt scrotal mass Tumor markers: AFP, BHCG – negative, LDH=mild elevation Physical examination: Paratesticular mass in lt hemiscrotum US: paratesticular mass, w/o involvement of testis Operation: Lt inguinal orchiectomy

CASE REPORT Pathology: Rhabdomyosarcoma Follow up: by CT of chest+abdomen Enlarged lymph node in ext iliac LN, susp enlarged LN in subcarinal region Referral to pediatric oncologist Mediastinal Bx: negative Chemotherapy F/U by CT: LN -

LEIOMYOSARCOMA Rare Patient’s Range: 40-70 90% of the tu: in the cord (25 cases of leiomyosarcoma reported), 70% of them – benign Distant spread: hematogenous TX: Standard: RADICAL ORCHIECTOMY

MISCELLANEOUS MESENCHYMAL TUMORS of the SPERMATIC CORD

MISCELLANEOUS MESENCHYMAL TUMORS of the SPERMATIC CORD Liposarcoma the most frequent (17 case reports) Pathology: Microscopic: no differences as in other tissues, uniform pattern of bundles

ADENOMATOID TUMORS Most common tu of paratesticular tissues (PTT) 30% of all PTT Distribution: epididymis>>tunicae>>cord Max occurance: 20-30 years old (range 20-80) Clinical appearance: small, solid, asymptomatic Occasional appearance: mild pain, discomfort The tumors ussualy attached to the tunicae Macroscopic Pathology: white, yellow or tan with fibrous apparence Microscopic Pathology: epithelium-like cells, fibrous stroma

ADENOMATOID TUMORS

ADENOMATOID TUMORS Tumor origin – Unknown Tumor behavior: benign, none of the cases with MTX, sometimes – local invasion TREATMENT: SURGICAL EXCISION

MESOTHELIOMA More common in older, but maybe seen in children Clinical Presentation: firm, painless scrotal mass +/- hydrocele (sometimes gradual enlargement of hydrocele) 15% MTX to inguinal LN or abdominal structures Macroscopic Patholoogy: poorly demarcated, wite/yellow, intermittent firm, friable mass Microscopic Pathology: papillary&solid structures + dense fibroconnective tissue, no mitotic activity, sometimes psammoma bodies are seen TREATMENT: Surgical Exsicion+/- local bx if MTX are suspected

CYSTADENOMA

CYSTADENOMA Cysadenoma of the epididymis – benign epithelial hyperplasia 20 case reports up to date (1/3 as part of von Hippel-Lindau dis) Most often in young people Clinical Presentation: partially cystic lesion with mild discomfort/asymptomatic Macroscopic Pathology: multicystic, encapsulated or circumscribed Microscopic Pathology: glands&papillary structures, Glycogen-staining cytoplasm as in RCC (DD - RCC MTX) TX: Surgical excision

CASE REPORT History: 15yrs old, b-Talassemia major, s/p BMT failure Urological f/u of bilateral simple epididymal cysts Tumor markers: negative Physical Examination: 2 small testis in scrotum, epididymal cysts bilateral (lt>rt), lt varicocele US: bilateral small testes, 2 lt epididymal cysts (previousle known), rt large (9mm) epididymal cyst Operation: Lt Epididymal Cyst Excision+Lt Low ligation of spermatic Vein

CASE REPORT Pathology: Papillary Cystadenoma of Epididymis Follow up: no evidence of varicocele, no inflammation in scrotum

EPITHELIAL TUMORS Rare Epididymis and paratesticular tissues maybe involved by extensions from 1’ germinal cell testicular tu

OVARIAN-TYPE EPITHELIAL TUMORS Rare Location: testicular tunics or parenchyma Tumor origin: Mullerian metaplasia of mesothelium Mullerian structures Classification: Papillary serous tumor of low malignant potential (most) Serous carcinoma Endometrioid adenocarcinoma Benign/malignant mucinous tumors Benign/malignant Brenner tumors Clear cell carcinoma

OVARIAN-TYPE EPITHELIAL TUMORS

OVARIAN-TYPE EPITHELIAL TUMORS Papillary serous tumor of low malignant potential: Paratesticular tu when involve tunica vaginalis are often accompanied by hydrocele Pathology (DD of Mesothelioma): Single/multifocal, exophytic, papillary on tunica vaginalis Like in ovary: arborizing pattern of epithelium, detached buds of epithelium Mucinous tu: same pathologic pattern as in ovary USUAL TX: RADICAL ORCHIECTOMY

CASE REPORT History: Physical Examination: 12yrs old, usually healthy Main complaint: pain and swelling in LT testis during 1mo prior admission to the ER Physical Examination: Lt testis isn’t palpable because of Lt Hydrocele US in ER: Complicated Lt Hydrocele Negative markers

CASE REPORT US before Operation: Lt hydrocele with paratesticular cyst with solid papillary lesion of 10mm diameter in it

CASE REPORT Operation: Inguinal excision of paratesticular cystic lesion with hydrocele Pathology: Papillary serous cystadenoma, borderline malignancy in tunica vaginalis Elective admission for Radical orchiectomy Patient released after operation filling well Currently: follow up by abdominal CT