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LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  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

Correspondence Address:
Karim M Mahawish
Older Persons Rehabilitation Services, Rotorua Hospital, Rotorua
New Zealand




How to cite this article:
Mahawish KM. Diffuse large B-cell lymphoma of the testis presenting as a pontine syndrome.Neurol India 2016;64:1358-1359


How to cite this URL:
Mahawish KM. Diffuse large B-cell lymphoma of the testis presenting as a pontine syndrome. Neurol India [serial online] 2016 [cited 2019 Aug 20 ];64:1358-1359
Available from: http://www.neurologyindia.com/text.asp?2016/64/6/1358/193828


Full Text

Sir,

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.[1] 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}{Figure 2}

Testicular lymphoma is one of the most common testicular malignancies among older men, with DLBCL accounting for 80–90% of the cases.[2] 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,[3] 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Fletcher 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.
2Horne MJ, Adeniran AJ. Primary diffuse large B-cell lymphoma of the testis. Arch Pathol Lab Med 2001;135:1363-7.
3Ferreri 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.