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Table of Contents
Epidemiology | Sites | Pathophysiology / etiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Immunohistochemistry | Molecular / cytogenetics description | Differential diagnosisCite this page: Lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testisparatestlymphoma.html. Accessed July 15th, 2017.
Epidemiology
- Paratesticular lymphoma is rare without testicular involvement
- Inadequate staging information in many reported cases
- Involves epididymis in 60% and spermatic cord in 39% of cases (Am J Surg Pathol 1994;18:376)
- Lymphomas comprise 2% of spermatic cord tumors (J Urol 1965;94:445)
- Most patients are elderly, although younger patients have been reported
Sites
- Epididymis and spermatic cord are most common location; involvement of tunica or lymphoma arising in cysts/hydroceles is rare
Pathophysiology / etiology
- Precise pathophysiology of primary paratesticular lymphomas is unknown
- Occurrence in tunica, cysts or hydroceles suggests role of chronic inflammation, demonstrated by EBV DNA in these cases (Am J Surg Pathol 2012;36:1074, Mod Pathol 2010;23:493)
- Most cases are diffuse large B cell lymphoma; other reported types (uncommon) are follicular, Burkitt, plasmacytoma, NK-T cell lymphoma
- Single case of transplacental transmission of NK-T cell lymphoma localizing to paratesticular region (Blood 2011;117:6046)
- Specific translocations are seen with each histological subtype
Clinical features
- Painless scrotal / paratesticular swelling of variable duration
- May have long standing history of hydrocele
- Variable B symptoms
Diagnosis
- Histological examination with immunohistochemistry and FISH/PCR for translocations as necessary
- Systemic workup for disease staging
- Identification at frozen section can prevent unnecessary orchiectomy (Hum Pathol 2012;43:1514)
- Misdiagnoses occur including mistaking lymphoma for seminoma, embryonal carcinoma and embryonal rhabdomyosarcoma (Arch Pathol Lab Med 2007;131:1040)
Radiology description
- Radiology helpful to delineate extent of disease and for staging workup, not for diagnosis
- Lesion is usually diffuse without sharp boundaries
Prognostic factors
- Poor prognosis in general, due to extra-lymphatic disease and high incidence of CNS spread
- Prognostic factors (same as lymphomas in general and specific to each histological type): age, ECOG status, LDH level, stage, association with HIV and other chronic infections
- Mean overall survival is 32-96 months for testicular and paratesticular lymphoma (Arch Pathol Lab Med 2007;131:1040)
Case reports
- 4 year old boy with Burkitt lymphoma of spermatic cord (Zhonghua Nan Ke Xue 2008;14:624)
- 17 year old boy with lymphoma of epididymis (Hinyokika Kiyo 2000;46:291)
- 35 and 61 year old men (Arch Pathol Lab Med 2001;125:428)
- 70 year old man with primary extranodal marginal zone lymphoma (Indian J Urol 2011;27:553)
- 73 and 76 year old men with primary lymphoma of spermatic cord (Indian J Pathol Microbiol 2011;54:588, Leuk Lymphoma 2001;40:663)
- 80 year old man with paratesticular solitary plasmacytoma (J Clin Imaging Sci 2011;1:52)
- 90 year old man with primary follicular lymphoma of epididymis (J Med Case Rep 2012;6:24
Treatment
- Biopsy for diagnosis followed by systemic chemotherapy based on type of lymphoma; radiotherapy to prevent local recurrence
Clinical images
Images hosted on other servers:
MRI of paratesticular lymphoma in infant
Paratesticular plasmacytoma
MALT lymphoma
Gross description
- As a nodule, lesion is fleshy and homegenous
- No confluent necrosis or hemorrhage (Arch Pathol Lab Med 2007;131:1040)
- As a nodule, lesion is fleshy with variable necrosis or hemorrhage
Gross images
Image hosted on other servers:
Well encapsulated
Microscopic (histologic) description
- Usually diffuse sheets of high grade cells with mitotic figures and apoptotic cells
- Morphology depends on type of lymphoma
Microscopic (histologic) images
Images hosted on other servers:
Various images
NK-T cell lymphoma in infant (transplacental spread)
Large, atypical and polymorphic cells
CD79a, CD3
Cytology description
- Cellular smears with scattered single high grade lymphoid cells having prominent nucleoli, frequent mitosis and apoptosis
Immunohistochemistry
- Full immunohistochemistry workup is essential for diagnosis: B, T cell markers, NK cell markers (if needed), BCL2, BCL6, CD10, Ki67, EBV LMP, EBNA, etc.
Molecular / cytogenetics description
- Translocation studies may be essential in some cases
Differential diagnosis
- Inflammatory pseudotumor / inflammatory myofibroblastic tumor: mixed population of cells as compared to the uniform population of high grade cells in non-Hodgkin lymphoma
- Seminoma: similar gross appearance; microscopically has branching fibrous septae, cells with distinct borders, more cytoplasm and an intratubular germ cell neoplasia in situ component
- Embryonal carcinoma: usually has more necrosis and hemorrhage grossly and microscopically; has architectural features such as papillae or trabeculae, higher degree of pleomorphism and an intratubular germ cell neoplasia in situ component
- Embryonal rhabdomyosarcoma
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