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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 18  |  Issue : 1  |  Page : 54-56

Primary testicular lymphoma


Department of General Surgery, Apollo BGS Hospital, Mysore, Karnataka, India

Date of Submission21-May-2020
Date of Acceptance08-Feb-2021
Date of Web Publication02-Mar-2021

Correspondence Address:
Milan Joshi
Department of General Surgery, Apollo BGS Hospital, Mysore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_42_20

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  Abstract 


Primary testicular lymphoma is a rare lymphoid neoplasm. It accounts for 1%–2% of non-Hodgkin's lymphoma, occurring in the elderly with high incidence of bilateral involvement. Ipsilateral orchiectomy plays an important role in diagnosis and can achieve therapeutic effect. This is a case report of testicular lymphoma which was incidentally detected after orchidectomy.

Keywords: Lymphoma, orchidectomy, testis


How to cite this article:
Joshi M, Subbarayappa S, Nagaraja JB. Primary testicular lymphoma. Apollo Med 2021;18:54-6

How to cite this URL:
Joshi M, Subbarayappa S, Nagaraja JB. Primary testicular lymphoma. Apollo Med [serial online] 2021 [cited 2021 Apr 11];18:54-6. Available from: https://www.apollomedicine.org/text.asp?2021/18/1/54/310642


  Introduction Top


Primary testicular lymphoma (PTL) is a uncommon tumor that accounts for less than 5% of all testicular malignancies.[1],[2],[3] They are a form of extranodal lymphoid malignancy. It is most commonly seen in men aged more than 60 years (mean age of 67 years).[4] Histologically, the most common form is diffuse large B-cell lymphoma (DLBCL).[2] They present as a painless testicular mass or swelling.[4],[5],[6] Bilaterality is common. Secondary involvement of the testis by non-Hodgkin's lymphoma (NHL) is more common than primary extranodal disease.[4] These tumors are characterized by a high risk to disseminate to the central nervous system (CNS), thereby warranting routine CNS prophylaxis with chemo- or radio-therapy.[7] After adequate locoregional and systemic treatment, the CNS remains the most frequent site of recurrence (up to 30%).


  Case Report Top


History and physical examination

A 70-year-old gentleman with no comorbidities came with complaints of a sudden-onset spontaneous swelling in the left groin which was rapidly increasing in size. He was accompanied by dull-aching pain and progressed to involve the left hemiscrotum. There was no history of trauma or previous malignancies. Examination revealed a 15 cm × 6 cm hard mass in the left inguinoscrotal region extending to the base of the scrotum with no expansile cough impulse. Ultrasound Doppler scrotum showed a grossly enlarged and heterogeneously hypoechoic left testis with increased vascularity. The left spermatic cord was grossly thickened with a bulky epididymis. Overall impression was given as chronic epididymo-orchitis with funiculitis. The patient was treated conservatively for 15 days, but the swelling rapidly increased in size and the patient had heaviness, pain, and discomfort in the left hemiscrotum. At this point, the decision to do a left inguinal radical orchidectomy was taken due to high suspicion of neoplasia.

Operative course

After informed consent for orchidectomy, under spinal anesthesia, a left-sided high inguinal orchidectomy was done. Intraoperatively, there was a hard mass involving the whole of the spermatic cord and extending distally to involve the whole of left testis. The left testis was delivered into the inguinal incision after clamping the spermatic cord at the deep inguinal ring. The spermatic cord was divided close to the clamp and was ligated. Left testis was removed with the spermatic cord and hemostasis achieved. Wound was closed in layers. Specimen was sent for histopathological analysis.

Histopathology

Histopathology revealed a specimen consisting of left testis with cord structures weighing 300 g and measuring 15 cm × 7 cm × 6 cm. The cut section showed nodular gray white growth occupying the testis, epididymis, and cord structures. Sections from testis and cord structures and resected margin show sheets of medium-to-large lymphoid cells with coarse chromatin [Figure 1], few prominent nucleoli, mitotic figures, intervening fibrocollagenous and fibromuscular tissue areas of necrosis, destructing and replacing the normal architectural pattern [Figure 2]. Impression being of NHL high grade. Immunohistochemistry for subtyping showed DLBCL.
Figure 1: Gross bi-halved specimen of left inguinal orchidectomy

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Figure 2: Microscopy showing diffuse lymphoid stroma in testicular specimen

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  Discussion Top


PTL is a rare and lethal entity and is primarily a disease of the elderly with incidence at 0.09–0.26/100,000 per year.[8] Testicular lymphoma carries a poor prognosis (5-year survival rate of approximately 12%) compared to other NHL and extranodal lymphomas[9] and may require a more prolonged course of chemotherapy compared to other extranodal lymphomas. They are associated with high morbidity and mortality. A typical clinical presentation is a firm, painless testicular mass associated with hydrocele in 40% of cases.[8] Other sites of metastasis were skin, Waldeyer ring, contralateral testis, and lung. Ultrasonography (US) is the most widely used imaging method for testicular mass. The scrotal sonography of testicular lymphoma might appear as a diffusely hypoechoic but hyperemic testicle or a focal testicular mass with increased blood flow.[10] The role of transscrotal biopsy of testicular lesion is controversial due to concerns for sampling error, the threat of seeding, and missing carcinoma in situ (CIS).

Ipsilateral orchiectomy plays an important role in diagnosis and can achieve therapeutic effect through better local control for relapse.[3] DLBCL is the most common histological subtype, but T-cell and Burkitt lymphoma have also been described.[2] Orchiectomy alone should not be considered as the sole treatment of DLBCL as it would cause a relapse in the contralateral testis within 2 years in 5%–35% cases.[3] Ann-Arbor staging is the staging system for lymphomas, and stage and pathologic grading are the most important predictive factors for outcome.[11] Ahmad et al.[12] reported that up to 90% of patients have Stage I or II disease at diagnosis (60% and 30%, respectively), and 35% had bilateral testicular involvement.

Optimal treatment remains unclear due to rarity of the disease and due to few studies and randomized trials being done on it. Fluorodeoxyglucose (FDG) positron emission tomography (PET)-computed tomography is recommended to stage FDG-avid lymphomas that is DLBCL subtype, which is FDG-avid. It can be used to rule out involvement of chest, abdomen, skin, bone marrow, or other sites of lymph nodes.[13] For CNS involvement, brain magnetic resonance imaging and lumbar puncture for cerebrospinal fluid analysis by cytology and flow cytometry should be performed.[13]

In a large retrospective study conducted by The International Extranodal Lymphoma Study Group and a prospective study for PTL, treatment promoted combined treatment for PTL regardless of stages, including radical orchiectomy, followed by anthracycline-based chemotherapy plus rituximab, intrathecal methotrexate CNS prophylaxis, and contralateral prophylactic testicular irradiation.[3],[14] The risk of testicular relapse without prophylactic contralateral radiation is at least 25%; this risk is essentially eliminated with radiation treatment. The chimeric monoclonal anti-CD20 antibody, rituximab, has been shown to give improvement in progression-free and overall survival in cases of DLBCL.[15]

Our patient had a testicular lymphoma, subtype – DLBCL. After discussion in a multidisciplinary team meeting, he is planned to undergo a FDG-PET scan and receive doxorubicin-based chemotherapy.


  Conclusion Top


Elderly patients (>60 years) presenting with a testicular mass of short duration should be considered as neoplasm unless proven otherwise more so as a lymphoid neoplasm. Due to the high incidence of CNS metastasis, patient requires a close follow-up posttreatment for early diagnosis of CNS metastasis. The effectiveness of rituximab in increasing overall survival in patients with PTLs should be studied. PTL being an uncommon entity requires prospective, randomized trials and comparative effectiveness studies to further explore the best local treatment modality.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer 1972;29:252-60.  Back to cited text no. 1
    
2.
Linassier C, Desablens B, Lefrancq T, Le Prise PY, Harousseau JL, Jacob C, et al. Stage I-IIE primary non-Hodgkin's lymphoma of the testis: Results of a prospective trial by the GOELAMS Study Group. Clin Lymphoma 2002;3:167-72.  Back to cited text no. 2
    
3.
Zucca E, Conconi A, Mughal TI, Sarris AH, Seymour JF, Vitolo U, et al. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol 2003;21:20-7.  Back to cited text no. 3
    
4.
Shahab N, Doll DC. Testicular lymphoma. Semin Oncol 1999;26:259-69.  Back to cited text no. 4
    
5.
Ferry JA, Harris NL, Young RH, Coen J, Zietman A, Scully RE. Malignant lymphoma of the testis, epididymis, and spermatic cord. A clinicopathologic study of 69 cases with immunophenotypic analysis. Am J Surg Pathol 1994;18:376-90.  Back to cited text no. 5
    
6.
Darby S, Hancock BW. Localised non-Hodgkin lymphoma of the testis: The Sheffield Lymphoma Group experience. Int J Oncol 2005;26:1093-9.  Back to cited text no. 6
    
7.
Chen WJ, Kuo JY, Lin CC, Chung HJ, Huang WJ, Wu HH, et al. Primary testicular lymphoma–A single center experience and review of literature. Urol Sci 2016;27:96-100.  Back to cited text no. 7
    
8.
Cheah CY, Wirth A, Seymour JF. Primary testicular lymphoma. Blood 2014;123:486-93.  Back to cited text no. 8
    
9.
Avilés A, Neri N, Huerta-Guzmán J, Pérez F, Fernández R. Testicular lymphoma: Organ-specific treatment did not improve outcome. Oncology 2004;67:211-4.  Back to cited text no. 9
    
10.
Coursey Moreno C, Small WC, Camacho JC, Master V, Kokabi N, Lewis M, et al. Testicular tumors: What radiologists need to know—Differential diagnosis, staging, and management. Radiographics 2015;35:400-15.  Back to cited text no. 10
    
11.
Doll DC, Weiss RB. Malignant lymphoma of the testis. Am J Med 1986;81:515-24.  Back to cited text no. 11
    
12.
Ahmad SS, Idris SF, Follows GA, Williams MV. Primary testicular lymphoma. Clin Oncol (R Coll Radiol) 2012;24:358-65.  Back to cited text no. 12
    
13.
Cheson BD, Fisher RI, Barrington SF, Cavalli F, Schwartz LH, Zucca E, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: The Lugano classification. J Clin Oncol 2014;32:3059-68.  Back to cited text no. 13
    
14.
Vitolo U, Chiappella A, Ferreri AJ, Martelli M, Baldi I, Balzarotti M, et al. First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOP, CNS prophylaxis, and contralateral testis irradiation: Final results of an international phase II trial. J Clin Oncol 2011;29:2766-72.  Back to cited text no. 14
    
15.
Sehn LH, Donaldson J, Chhanabhai M, Fitzgerald C, Gill K, Klasa R, et al. Introduction of combined CHOP plus rituximab therapy dramatically improved outcome of diffuse large B-cell lymphoma in British Columbia. J Clin Oncol 2005;23:5027-33.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2]



 

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