PARATESTICULARPARATESTICULARTUMORSTUMORS

Dr. Vainrib, Urology Department

Meir Hospital

TESTICULAR TUMORSTESTICULAR TUMORS

UNCOMMON (1-2% of all UNCOMMON (1-2% of all pediatric solid tu)pediatric solid tu)

Benign>Malignant in childrenBenign>Malignant in children Incidence peaks at age 2yrs, Incidence peaks at age 2yrs,

tapers after 4yrs and rises tapers after 4yrs and rises again at pubertyagain at puberty

Rare in Asian children and Rare in Asian children and blackblack

Testicular Cancer – one of Testicular Cancer – one of the most curable solid the most curable solid neoplasmsneoplasms

TUMORS OF TESTICULAR ADNEXATUMORS OF TESTICULAR ADNEXA

PARATESTICULAR TUMORS: Rhabdomyosarcoma Leiomyosarcoma Miscellaneous Mesenchymal Tu of the Spermatic

Cord ADENOMATOID TUMORS:

Mesothelioma Cystadenoma

EPITHELIAL TUMORS

PARATESTICULAR TUMORSPARATESTICULAR 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 PARATESTICULAR RHABDOMYOSARCOMA (RMS)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 PARATESTICULAR RHABDOMYOSARCOMA (RMS)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 PARATESTICULAR RHABDOMYOSARCOMARHABDOMYOSARCOMA

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 PARATESTICULAR RHABDOMYOSARCOMA TXRHABDOMYOSARCOMA 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 PARATESTICULAR RHABDOMYOSARCOMARHABDOMYOSARCOMA

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 REPORTCASE REPORT

History: 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 REPORTCASE 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 -

LEIOMYOSARCOMALEIOMYOSARCOMA

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 MISCELLANEOUS MESENCHYMAL TUMORS of the MESENCHYMAL TUMORS of the SPERMATIC CORDSPERMATIC CORD

MISCELLANEOUS MISCELLANEOUS MESENCHYMAL TUMORS of the MESENCHYMAL TUMORS of the SPERMATIC CORDSPERMATIC CORD

Liposarcoma the most frequent (17 case reports)

Pathology: Microscopic: no

differences as in other tissues, uniform pattern of bundles

ADENOMATOID TUMORSADENOMATOID 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 TUMORSADENOMATOID TUMORS

ADENOMATOID TUMORSADENOMATOID TUMORS

Tumor origin – Unknown Tumor behavior: benign, none of the cases with

MTX, sometimes – local invasion TREATMENT: SURGICAL EXCISION

MESOTHELIOMAMESOTHELIOMA

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

CYSTADENOMACYSTADENOMA

CYSTADENOMACYSTADENOMA 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 REPORTCASE REPORT History:

15yrs old, 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 REPORTCASE REPORT

Pathology: Papillary Cystadenoma of Epididymis Follow up: no evidence of varicocele, no

inflammation in scrotum

EPITHELIAL TUMORSEPITHELIAL TUMORS

Rare Epididymis and paratesticular tissues maybe

involved by extensions from 1’ germinal cell testicular tu

OVARIAN-TYPE OVARIAN-TYPE EPITHELIAL TUMORSEPITHELIAL 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 OVARIAN-TYPE EPITHELIAL TUMORSEPITHELIAL TUMORS

OVARIAN-TYPE OVARIAN-TYPE EPITHELIAL TUMORSEPITHELIAL 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 REPORTCASE REPORT

History: 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 REPORTCASE REPORT

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

CASE REPORTCASE 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