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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 6 | Page : 1358-1359
Diffuse large B-cell lymphoma of the testis presenting as a pontine syndrome
Karim M Mahawish
Older Persons Rehabilitation Services, Rotorua Hospital, Rotorua, New Zealand
|Date of Web Publication||11-Nov-2016|
Karim M Mahawish
Older Persons Rehabilitation Services, Rotorua Hospital, Rotorua
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mahawish KM. Diffuse large B-cell lymphoma of the testis presenting as a pontine syndrome. Neurol India 2016;64:1358-9
Testicular lymphoma is one of the most common testicular malignancies among older men. With modern treatment using rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), cure rates for primary testicular diffuse large B-cell lymphoma (DLBCL) are 60%; however, relapse results in dramatically shortened survival times. The prognosis is particularly poor in patients with central nervous system (CNS) involvement.
A 58-year-old gentleman presented with a 2-week history of generalized arthralgia, fever, night-sweats, and weight loss. At admission, he was febrile with a temperature of 38.8°C. An examination of the cardiorespiratory, abdominal, neurological, and rheumatological systems were unremarkable. He underwent a series of investigations including tests for infective causes, solid organ malignancies, and vasculitis. A mildly elevated lactate dehydrogenase (LDH) (390 iu/L) and borderline splenomegaly on abdominal computed tomography (CT) was found. During admission, he developed a progressive pontine syndrome with left-sided V th and VI th cranial nerve palsy, a right VII upper motor neuron palsy, and a right hemiparesis with brisk reflexes. Magnetic resonance imaging (MRI) of the brain demonstrated a lesion in the left pons extending laterally to the left cerebellar penduncle [Figure 1] and [Figure 2]. A repeat physical examination identified a solid enlarged left testicle, that was histologically confirmed, following the orchidectomy, to be harbouring the presence of DLBCL. The pontine lesion was thought to be consistent with secondary CNS spread. The patient was commenced on intravenous methylprednisolone followed by rituximab. After an initial improvement in symptoms, he subsequently deteriorated and finally succumbed to his illness.
|Figure 1: Axial magnetic resonance T2 demonstrating a lesion in the left pons extending laterally to the left cerebellar peduncle|
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|Figure 2: Coronal fluid-attenuated inversion recovery magnetic resonance demonstrating a lesion in the left pons|
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Testicular lymphoma is one of the most common testicular malignancies among older men, with DLBCL accounting for 80–90% of the cases. It has a predilection for extranodal sites, particularly the contralateral testis and the CNS. The most common presentation is a rapidly enlarging painless testis, with constitutional symptoms present in one-third of the patients. CNS dissemination is rare (4–5%); however, once it occurs usually it is regarded as a fatal complication of aggressive lymphomas, with a median survival of 4–5 months. Although, in our patient, the testicular mass was identified late, given the aggressive course of DLBCL, it is unlikely that an earlier detection would offer an improved prognosis.
Testicular lymphoma should be considered in the differential diagnoses of male patients presenting with constitutional symptoms and investigated accordingly. Standard imaging strategies, including a pelvic CT scan, are inadequate to adequately diagnose this entity. Although an ischemic stroke may present with progressive neurological symptoms in a minority of patients, infiltrative processes e.g., granulomatous disease and lymphoma, should also be considered.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
Fletcher CD, Kahl BS. Central nervous system involvement in diffuse large B-cell lymphoma: An analysis of risks and prevention strategies in the post-rituximab era. Leuk Lymphoma 2014;55:2228-40.
Horne MJ, Adeniran AJ. Primary diffuse large B-cell lymphoma of the testis. Arch Pathol Lab Med 2001;135:1363-7.
Ferreri AJ, Assanelli A, Crocchiolo R, Ciceri F. Central nervous system dissemination in immunocompetent patients with aggressive lymphomas: Incidence, risk factors and therapeutic options. Hematol Oncol 2009;27:61-70.
[Figure 1], [Figure 2]