TUMORS OF THE TESTICULAR ADNEXA and SPERMATIC CORD Victor E. Reuter, MD Memorial Sloan-Kettering Cancer Center reuterv@mskcc.org 66 th Annual Pathology Seminar California Society of Pathologists Short Course in Genitourinary Pathology MSKCC Disclosures: none
TESTICULAR APPENDAGES and CORD LESIONS Outline: Rete testis Tubuli efferentes/epididymis Mesothelium Soft tissue Metastatic disease
When confronted with an adnexal testicular mass, what should I be thinking about? Neoplastic or not? Reactive process, infection, infarct If neoplastic, primary or not? If primary, what type? Are there ancillary studies I can depend on? After I type it, what factors must I evaluate to predict progression and how do I stage the tumor? If metastatic, what are the more likely sources? Clinical history
METASTATIC DISEASE TO THE TESTIS Rarely is it the initial presentation of disease Epithelial tumors predominate Prostate Colon Kidney Melanoma Lymphoma Sarcoma
RETE TESTIS Benign Lesions Classification Hyperplasia Adenomatous hyperplasia Adenoma - Sertoliform adenoma Morphologic criteria Circumscribed Cystic or solid Cytologically banal For adenoma; not associated with other neoplasm
Normal anatomy
Pagetoid spread of ITGCN with reactive papillary hyperplasia of the rete
Reactive changes in the rete testis
Hilum Well circumscribed Banal appearing Inhibin /+ Sertoliform adenoma of the rete
PAX8
RETE TESTIS Adenocarcinoma Lesion centered in the hilum* Transition from normal to neoplastic epithelium* Absence of primary elsewhere Morphology incompatible with any other intrascrotal primary Proper immunohistochemical panel excluding other primaries *may be difficult to assess
RETE TESTIS Adenocarcinoma Clinical scenario: Adult males Unilateral painful testicular swelling With or without hydrocele Poor survival Pathology: Solid or cystic Mostly tubulopapillary (solid/spindled/reteform) Cuboidal to columnar cells Eosinophilic to basophilic cytoplasm
CK7 CEA CD15 PAX8
CYSTADENOMA OF THE EPIDIDYMIS vhl syndrome Sporadic Painless, movable mass Wide age distribution Variable size No hydrocele Microscopy: Papillary/tubulopapillary growth Clear cytoplasm Low grade nuclei Nuclei polarized towards the luminal surface
CYSTADENOMA OF THE EPIDIDYMIS CA9 CK7 PAX8
MÜLLERIAN-TYPE EPITHELIAL TUMORS Clinical scenario: Wide age range (adults) No specific medical history Painful testicular swelling Carcinomas may recur and metastasize Pathology: Solid or cystic Wide range of Mülleriantype morphologies Serous features dominate (endometrioid, clear,mucinous, Brenner) Entire histologic spectrum (cystadenomaborderline-carcinoma)
Müllerian rests/metaplasia
ER Müllerian rests/metaplasia within adnexa
Cystadenoma Serous borderline tumor
Papillary serous carcinoma
PARATESTICULAR SEROUS PAPILLARY CARCINOMA Cases 6 Age (yrs) 31 (16-42) Size (cm) 2 (1.5-2.5) Presentation Mass 5 Hydrocele 1 Jones et al. Am J Surg Pathol.1995;19:1359-1365
PARATESTICULAR SEROUS PAPILLARY CARCINOMA: Testicular groove* 4 Paratesticular soft tissue 1 Visceral tunica vaginalis 1 * two cases had extensive visceral tunica vaginalis involvement with invasion of the tunica albuginea Jones et al.am J Surg Pathol.1995;19:1359-1365.
Endometrioid carcinoma
ER WT-1 Positive: PR, CA125, CK7, CD15 Negative: CK20, Calretinin, CEA
LESIONS OF MESOTHELIAL ORIGIN Reactive changes Hyperplasia, cyst Mesothelioma Adenomatoid tumor
When confronted with a hydrocele sac, what should I be thinking about? If the urologist decides to send it to pathology What does the specimen look like? Thin or thick? Smooth or rough surface? What should I submit for morphology? Submit in toto or simply sample? What did the cyst fluid look like? What is the clinical history?
Reactive changes: Thickening Inflammation Mesothelial proliferation parallel to mesothelial surface
Reactive mesothelial hyperplasia Reactive changes: Thickening Inflammation Mesothelial proliferation parallel to mesothelial surface
Reactive mesothelial hyperplasia Respects the underlying muscular coat / no vertical growth
Mesothelioma Haphazard arrangement of cells Nodular of diffuse thickening Vertical growth
Mesothelioma CK 5/6 Calretinin WT-1 Seminiferous tubules
Mesothelioma; morphologic spectrum
What about benign papillary mesothelioma versus WD papillary mesothelioma? Beware of the word benign Minute, focal, cytologically banal, single cell layer, limited to the surface Complexity, solid component, and cytologic atypia warrant a diagnosis of mesothelioma
Papillary mesothelioma
Papillary mesothelioma
MESOTHELIOMA OF TUNICA VAGINALIS Clinicopathologic Features Cases 63 Age (yrs) mean 53.5, range 7-80 Presentation Asbestos exposure 48% Histology Local invasion hydrocele and/or mass 75% epithelial 25% biphasic skin of scrotum/penis, epididymis/testis, spermatic cord Jones et al. Am J Surg Pathol.1995;19:815-825
MESOTHELIOMA OF TUNICA VAGINALIS Sites of Metastasis Site presentation later total (%) Lymph nodes retroperitoneum 9 9 18 (38) inguinal 4 3 7 (15) mediastinal 2 2 (4) Lung 8 8 (17) Peritoneum 3 3 ( 6) Liver 2 2 ( 4) Pelvic soft tissue 3 3 ( 6) Jones et al. Am J Surg Pathol.1995;19:815-825
MESOTHELIOMA OF TUNICA VAGINALIS Outcome of Patients With Follow-up F/U (yrs) Patients DOD AWD NED < 2 19 5 1 13 < 3 15 7 3 5 < 4 4 4 0 0 < 5 7 5 1 1 < 6 3 2 0 1 > 6 4 0 4 0 Total 52 23(44%) 9(17%) 20(39%) Jones et al.am J Surg Pathol.1995;19:815-825
WT1 Calretinin Also: CK5/6 D2-40
ADENOMATOID TUMOR - Well circumscribed, mobile - Not encapsulated Microscopy: - Epithelioid or spindled - Cords, nests, tubules - Microcystic, signet ring - No mitotic activity - May have intervening stroma or adnexal structure - May infarct - atypical myofibroblastic reaction Immunohistochemistry: - Like mesothelioma Differential Diagnosis: Mesothelioma, Adenoca.,YST
ADENOMATOID TUMOR Calretinin WT-1
ADENOMATOID TUMOR
MESENCHYMAL LESIONS OF THE SPERMATIC CORD Fibrous reactive versus neoplastic Lipomatous lipoma liposarcoma dedifferentiated Smooth muscle Squeletal muscle To also consider: Angiolipoma Angiofibroma Angiomyoblastoma Angiomyxoma
NODULAR PERIORCHITIS paratesticular fibrous pseudotumor Non neoplastic Nodular masses single, multiple, confluent Rarely diffuse Plaque-like May be massive and disfiguring Usually unilateral Gross features: Firm to hard, white
NODULAR PERIORCHITIS Microscopic features: Densely hyalinized collagen Clacification Well circumscribed May be somewhat cellular Inflammation Granulation tissue Differential diagnosis: Inflammatory pseudotumor Fibromatosis
Nodular periorchitis Fibrous Pseudotumor
LIPOMA OF THE SPERMATIC CORD
LIPOSARCOMA OF THE CORD - Most common sarcoma at this site - WD liposarcoma / atypical lipomatous tumor Gross appearance: - Variable, based of fatty, fibrous, or dedifferentiated component Microscopy: - Mature appearing adipocytes of varying size - Lipoblasts can be rare - Spindle and florrette-like cells - Fibrous bands are common - Dedifferentiation low grade high grade - Smooth muscle differentiation
Well differentiated Liposarcoma
Dedifferentiated liposarcoma, low grade
Dedifferentiated liposarcoma, high grade
Dedifferentiated Liposarcoma, low grade CDK4 MDM2
Retroperitoneal recurrence Dedifferentiated liposarcoma, now high grade CDK4 MDM2
Adnexal tumors: TESTICULAR APPENDAGES and CORD LESIONS Summary Rare; benign and reactive more common than malignant If epithelioid and malignant, consider a metastasis If primary, location, histology and IHC can help Adenocarcinoma (CEA, CD15, etc) Müllerian (ER, PAX8, WT1, CA125, etc) Mesothelioma (Calretinin, WT1, CK 5/6, Podoplanin) Soft tissue tumors: Beware of the subtle features of WD and low grade dedifferentiated liposarcoma Beware of benign mimics of liposarcoma and embryonal RMS