DOI: 10.1148/rg.251045041
RadioGraphics 2005;25:243-248
© RSNA, 2005
Best Cases from the AFIP
Primary Diffuse Large B-Cell Lymphoma of the Epididymis and Testis1
Jason M. Zicherman, MD,
David Weissman, MD,
Christopher Gribbin, MD and
Robert Epstein, MD
1 From the Departments of Radiology (J.M.Z., C.G., R.E.) and Pathology (D.W.), University of Medicine and Dentistry of New Jersey Robert Wood Johnson Medical School, 1 Robert Wood Johnson Place, Medical Education Building, Room 404, New Brunswick, NJ 08903. Received March 15, 2004; revision requested April 6 and received May 18; accepted May 18. All authors have no financial relationships to disclose. Address correspondence to J.M.Z. (e-mail: zicherja@yahoo.com).
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History
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A 25-year-old African-American man presented with testicular swelling following a motor vehicle accident. The patient had noticed swelling for several weeks prior to the accident but had dismissed the finding. There was no reported history of testicular pain, night sweats, fever, chills, or other constitutional symptoms. Scrotal examination demonstrated a firm, palpable testicular mass within the right hemiscrotum. The remainder of the clinical examination was noncontributory. Computed tomography (CT) performed to assess the traumatic injuries was unremarkable except for a soft-tissue mass occupying the right hemiscrotum. Subsequent ultrasonography (US) demonstrated a right epididymal mass invading the testis. It was thought that these findings may have represented a complex hematoma, a neoplasm, or an infectious bacterial, viral, or granulomatous process. The patient was treated with antibiotics and was scheduled for a 2-week follow-up evaluation. The patient did not return until 6 months later, when he complained of increased swelling within the right hemiscrotum. Magnetic resonance (MR) imaging performed at that time demonstrated an interval increase in the size of the right epididymal mass with significant extension into the surrounding testis.
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Imaging Findings
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Contrast material–enhanced CT of the abdomen and pelvis performed to assess for trauma demonstrated a soft-tissue mass within the right hemiscrotum (Fig 1a). US showed a 2.4 x 2.8-cm complex, heterogeneous vascular mass involving the right epididymis and infiltrating the adjacent testis (Fig 1b). The remainder of the testis and the contralateral testis demonstrated normal homogeneous echotexture at gray-scale US and normal blood flow at Doppler US. Scrotal MR imaging performed 6 months after initial US and CT demonstrated an enlarged, infiltrative mass measuring 3.3 x 3.3 x 3.2 cm within the right epididymal head (Fig 2a, 2b). The mass extended into the superior aspect of the right testis. The signal intensity of the right epididymis and testis did not suggest the presence of blood products. Multiple lymph nodes were seen along the course of the superficial femoral artery, the largest of which measured 2.8 x 8 mm.
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Figure 1a. (a) Axial contrast-enhanced CT scan shows a soft-tissue mass within the right hemiscrotum (arrow). (b) Doppler US image shows a 2.4 x 2.8-cm hypoechoic hypervascular mass occupying the right epididymis (white *) and infiltrating the superior pole of the adjacent testis (black *).
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Figure 1b. (a) Axial contrast-enhanced CT scan shows a soft-tissue mass within the right hemiscrotum (arrow). (b) Doppler US image shows a 2.4 x 2.8-cm hypoechoic hypervascular mass occupying the right epididymis (white *) and infiltrating the superior pole of the adjacent testis (black *).
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Figure 2a. (a) Sagittal fast spin-echo T2-weighted MR image (repetition time msec/echo time msec = 3,116/112) demonstrates a hypointense mass within the right epididymis (*). Note the margin between the infiltrating epididymal mass and the superior pole of the right testis (arrows). (b) Coronal MR image (3,116/112) demonstrates the mass occupying the epididymis (*) and invading the superior pole of the right testis (arrow). (c) Photograph of the resected specimen obtained at right orchiectomy demonstrates a tan-white indurated mass replacing the right epididymis (*). The mass is irregular in shape and focally invades the superior pole of the testis (arrow).
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Figure 2b. (a) Sagittal fast spin-echo T2-weighted MR image (repetition time msec/echo time msec = 3,116/112) demonstrates a hypointense mass within the right epididymis (*). Note the margin between the infiltrating epididymal mass and the superior pole of the right testis (arrows). (b) Coronal MR image (3,116/112) demonstrates the mass occupying the epididymis (*) and invading the superior pole of the right testis (arrow). (c) Photograph of the resected specimen obtained at right orchiectomy demonstrates a tan-white indurated mass replacing the right epididymis (*). The mass is irregular in shape and focally invades the superior pole of the testis (arrow).
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Figure 2c. (a) Sagittal fast spin-echo T2-weighted MR image (repetition time msec/echo time msec = 3,116/112) demonstrates a hypointense mass within the right epididymis (*). Note the margin between the infiltrating epididymal mass and the superior pole of the right testis (arrows). (b) Coronal MR image (3,116/112) demonstrates the mass occupying the epididymis (*) and invading the superior pole of the right testis (arrow). (c) Photograph of the resected specimen obtained at right orchiectomy demonstrates a tan-white indurated mass replacing the right epididymis (*). The mass is irregular in shape and focally invades the superior pole of the testis (arrow).
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Pathologic Evaluation
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The right testis was excised and measured 6 x 3 x 2.5 cm, weighed 49 g, and had an attached spermatic cord 6.5 cm long. The epididymis had been replaced by a tan-white indurated mass 3 cm in diameter that was irregular in shape and focally invaded the testis (Fig 2c).
Light microscopy demonstrated a lymphoma with variable morphologic features. The lymphoma extensively involved the adnexa, including the epididymis, ductuli efferentes, and rete testis. It tended to infiltrate these tubular structures and sometimes directly invaded and effaced them (Fig 3). The lymphoma also extended into the testicular parenchyma with an infiltrative border (Figs 4, 5). The spermatic cord was not involved. In some foci, the malignant cells were large with scant cytoplasm and large vesicular nuclei, the classic appearance of a diffuse large B-cell lymphoma (Fig 6). In other foci, the cells were larger, with bizarre, often folded or lobate nuclei and resembled the cells of an anaplastic large cell lymphoma. The most unusual cytologic findings in this tumor were areas of elongated or spindly lymphoid cells with a twisted or irregular cellular outline (Fig 3). This uncommon morphologic variant of large cell lymphoma has been called "sarcomatoid" or "spindle cell" lymphoma (1), although it is not included in the World Health Organization classification of lymphomas (2). Most sarcomatoid lymphomas have been T-cell or null-cell anaplastic large cell lymphomas (3), although this morphologic variant has also been reported in B-cell lymphomas (4). In all foci, the malignant cells were accompanied by scattered small, mature lymphocytes with clumped chromatin.
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Figure 3. Photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) demonstrates sarcomatoid lymphoma cells surrounding a sclerosed and predominantly effaced seminiferous tubule (*). The lymphoma cells are spindled or twisted, with an irregular nuclear contour (arrow).
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Figure 5. Photomicrograph (original magnification, x10; H-E stain) demonstrates the interface between the uninvolved testis (top) and the portion of the testis involved by lymphoma (bottom).
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Figure 6. Photomicrograph (original magnification, x40; H-E stain) shows diffuse large cells, most of which have large vesicular nuclei with nucleoli that tend to cling to the nuclear envelope (arrow). A few larger, more anaplastic forms with lobate nuclei are also present.
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Given the unusual cytologic features of much of the mass, paraffin immunohistochemical staining was useful in demonstrating the B-cell lineage of the cells, which showed positivity for leukocyte common antigen (CD45) and the B-cell marker CD20, in addition to focal positivity for CD30. The smaller scattered cells were positive for the T-cell marker CD3.
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Discussion
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Testicular lymphoma was first reported by Malassez (5) and was subsequently defined by Curling (6) as a clinical entity. Testicular lymphomas constitute 1%–9% of all testicular neoplasms and 1% of non-Hodgkin lymphomas (7). Although uncommon in general, they are the most common testicular malignancy in men 60 years of age and older (7). Testicular lymphoma may be the primary and only manifestation of malignant lymphoma, the initial sign of generalized disease, or may occur during the clinical course of a patient with established lymphoma (8). Secondary involvement of the testis in patients with lymphoma is far more common than primary testicular lymphoma. Although predisposing causes for the development of testicular lymphoma remain unclear, various reports have implicated prior trauma, chronic orchitis, cryptorchidism, and filariasis of the spermatic cord as potential risk factors (7). Immunosuppressed patients are at increased risk for developing extranodal lymphoma, including testicular lymphoma (7). This patient subset typically presents at an earlier age (median age, 37 years) with an aggressive histologic tumor grade (immunoblastic or small noncleaved cell subtype) and is associated with a poorer prognosis (7).
Primary testicular lymphoma most commonly manifests as painless testicular swelling that develops over a span of weeks to months (7). Patients can present with painful testicular swelling or with systemic symptoms such as weight loss, night sweats, anorexia, fever, and weakness (7). At physical examination, there is usually a firm, nontender nodular mass that is inseparable from the testis. When the epididymis is involved, it too can appear enlarged, firm, and nodular at physical examination (7).
Most testicular lymphomas are diffuse large B-cell lymphomas (9), which are high-grade neoplasms with homogeneous sheets of atypical cells. Less common hematolymphoid malignancies that can be found at this site include follicular lymphomas (10–12), mucosa-associated lymphoid tissue (MALT) lymphoma (13), plasmacytoma (14), and granulocytic sarcoma (15). Lymphoma of the testis is the most common bilateral testicular neoplasm, with an overall prevalence of synchronous involvement approaching 19.5% (7). Asynchronous involvement is far more common and may manifest months to years following orchiectomy (8).
Testicular lymphoma is locally aggressive and can typically infiltrate the epididymis, spermatic cord, or scrotal skin (7). Testicular lymphoma has a predilection for widespread dissemination to unusual sites, including the central nervous system (CNS), Waldeyer ring, skin, and lung (7). Less common sites of metastasis include the prostate, kidney, liver, bone marrow, pleura, and bone (7). The prevalence of CNS involvement varies from 6% to 16.5% and is determined by the histologic subtype of the lymphoma, with lymphoblastic and diffuse undifferentiated lymphoma being the most prevalent (7). The meninges, epidural space, and brain parenchyma can all be affected (7). Involvement of the Waldeyer ring and surrounding structures in the oropharynx and nasopharynx is seen in about 6% of patients at the time of diagnosis. These patients typically present with nasal obstruction and with frequent ear infections (7). Metastasis to the skin implies systemic disease and carries a poor prognosis (7). Skin lesions can manifest as papules, nodules, tumors, or plaques. Pulmonary metastases manifest as discrete nodules and are thought to arise by means of hematogenous dissemination. Pulmonary symptoms during the course of the disease are rare (7).
The imaging features of testicular lymphoma reflect its infiltrative but nondestructive characteristics as seen at pathologic analysis (16). At US, the normal homogeneous echogenic testis is replaced focally or diffusely with hypoechoic vascular lymphomatous tissue (16). Lymphomas typically demonstrate increased intralesional blood flow regardless of tumor size (16). Lymphoma typically infiltrates the epididymis, which then becomes enlarged and hypoechoic. Unlike in this case, however, the testicular component is usually more extensive than the epididymal component (10). Nevertheless, a small number of cases have been reported in which the epididymis is the primary site, including cases of MALT lymphoma (17), follicular large cell lymphoma, "histiocytic" lymphoma, and two other types of neoplasms (10).
The differential diagnosis for infiltrative processes involving both the epididymis and the testis include epididymo-orchitis, sarcoid, lymphoma, tuberculosis, and leukemia. Testicular germ cell tumors are not included in this differential diagnosis because they appear more lobulated with well-defined borders at US (16) and rarely involve both the testis and epididymis. Inflammatory processes involving the testis are usually tender at physical examination and improve following treatment, whereas lymphomas and other malignancies are usually painless and either do not resolve or demonstrate interval growth (7). Genitourinary involvement by sarcoid usually manifests as a unilateral, painless nodular mass within the epididymis (17). Extension to the ipsilateral testis is rare, occurring in less than 1% of reported cases (17). Testicular sarcoid is far more common in African-American males than in white males (20:1) and does not respond to a typical course of antibiotics (17). Testicular leukemia can manifest as an infiltrative epididymal-testicular mass but often afflicts patients with a prior history of treated leukemia.
In this case, the patient presented with an infiltrating scrotal mass that predominantly involved the epididymis. The mass extended into the ipsilateral testis and enlarged over time despite antibiotic treatment. There was no documented history of, or laboratory evidence to suggest, secondary testicular involvement by leukemia or prior infection with tuberculosis. Because of the patient’s young age and race, the most likely diagnosis was epididymal-testicular sarcoid, followed by lymphoma.
Patients who are treated for primary testicular lymphoma usually demonstrate widespread dissemination within the first 2 years of treatment. Testicular lymphoma carries a worse prognosis than its nodal counterpart, with a 5-year survival rate of about 12% and a median survival time of less than 12 months (8). Factors that have been linked to more favorable outcomes include younger patient age, localized disease, the presence of sclerosis at pathologic analysis, smaller tumor size, lower histologic tumor grade, and lack of epididymal or spermatic cord involvement (7). It is a curious fact that two independent groups have described a superior disease-free survival if the malignancy involves only the right testis (11). Although treatment is evolving, mainstay treatment includes orchiectomy and systemic chemotherapy. Prophylactic irradiation of the scrotum and administration of intrathecal methotrexate are usually offered to patients who experience complete remission to prevent recurrence in the contralateral testis and CNS (7).
After undergoing orchiectomy, the patient underwent full staging for lymphoma, including CT of the chest, abdomen, and pelvis, positron emission tomography, and bone marrow biopsy, none of which revealed any evidence of extratesticular involvement by lymphoma. The patient was treated into remission with three cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), rituximab, and prophylactic intrathecal methotrexate and 3 weeks of radiation therapy to the testis (3,000 cGy in 15 fractions).
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References
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- Nozawa Y, Wang J, Weiss LM, et al. Diffuse large B-cell lymphoma with spindle cell features. Histopathology 2001; 38:177-178.[CrossRef][Medline]
- Jaffe ES, Harris NL, Stein H, et al. Tumors of haematopoietic and lymphoid tissues Lyon, France: IARC, 2001.
- Bueso-Ramos CE, Pugh WC, Butler JJ. Anaplastic large cell lymphoma presenting as a soft-tissue mass mimicking sarcoma. Mod Pathol 1994; 7:497-500.[Medline]
- Wang J, Sun NC, Nozawa Y, et al. Histologic and immunohistochemical characterization of extranodal diffuse large-cell lymphomas with prominent spindle cell features. Histopathology 2001; 39:476-481.[CrossRef][Medline]
- Malassez M. Lymphadenome du testicle. Bull Soc Anta (Paris) 1877; 52:176-178.
- In: Curling TB, eds. A practical treatise on diseases of the testis. 4th ed. London, England: Churchill, 1878.
- Shahab N, Doll D. Testicular lymphoma. Semin Oncol 1999; 26:259-269.[Medline]
- Doll D, Weiss R. Malignant lymphoma of the testis. Am J Med 1986; 81:515-523.[CrossRef][Medline]
- Nonomura N, Aozasa K, Ueda T, et al. Malignant lymphoma of the testis: histological and immunological study of 28 cases. J Urol 1989; 141:1368-1371.[Medline]
- McDermott MB, O’Briain DS, Shiels OM, Daly PA. Malignant lymphoma of the epididymis: a case report of bilateral involvement by a follicular large cell lymphoma. Cancer 1995; 75:2174- 2179.[CrossRef][Medline]
- Ferry J, Harris N, Young R, et al. Malignant lymphoma of the testis, epididymis, and spermatic cord. Am J Surg Pathol 1994; 18:376-390.[Medline]
- Pakzad K, MacLennan G, Elder J, et al. Follicular large cell lymphoma localized to the testis in children. J Urol 2002; 168:225-228.[CrossRef][Medline]
- Kausch I, Doehn C, Buttner H. Primary lymphoma of the epididymis. J Urol 1998; 160:1801-1802.[CrossRef][Medline]
- Ferry JA, Young RH, Scully RE. Testicular and epididymal plasmacytoma: a report of 7 cases, including three that were the initial manifestation of plasma cell myeloma. Am J Surg Pathol 1997; 21:590-598.[CrossRef][Medline]
- Ferry JA, Srigley JR, Young RH. Granulocytic sarcoma of the testis: a report of two cases of a neoplasm prone to misinterpretation. Mod Pathol 1997; 10:320-325.[Medline]
- Mazzu D, Jeffrey R, Jr, Ralls P. Lymphoma and leukemia involving the testicles: findings on gray-scale and color Doppler sonography. AJR Am J Roentgenol 1995; 164:645-647.[Abstract/Free Full Text]
- Ryan DM, Lesser BA, Crumley LA, et al. Epididymal sarcoidosis. J Urol 1993; 149:134-136.[Medline]
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History
Imaging Findings
