|
Advertisement |
|
|
||
 |
|
|||||
|
|||||
|
 |
||||||
|
||||||
|
||||||
|
Journal of Clinical Oncology, Vol 25, No 24 (August 20), 2007: pp. 3783-3785 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.12.5112
Primary Renal Lymphoma Presenting With Paraneoplastic Limbic EncephalitisDepartment of Medical Oncology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India
Yashoda Hospital, Hyderabad, India A 58-year-old nonhypertensive, nondiabetic presented with headache and short-term memory loss of 4 months. He had generalized clonic tonic seizures 1 day before his hospitalization. There was no history of fever, vomiting, trauma, drug intake, or anything suggestive of any other neurological deficit. He complained of decreased appetite and weight loss of 5 kg over the previous 4 months. His past medical history was unremarkable. His general and systemic examination was normal. The neurological examination was unremarkable except for recent memory loss. The complete blood picture and biochemistry were within normal limits. His HIV serology was negative. A contrast enhanced magnetic resonance imaging (MRI) and magnetic resonance spectroscopy of the brain revealed abnormalities involving the corpus callosum extending into bifrontotemporal white matter and both thalamocapsulo-ganglionic regions (Fig 1). The CSF analysis showed lymphocytic pleocytosis with mild elevation of proteins and normal sugar, and lactate dehydrogenase and was negative for malignant cells. The anti-Ro, Hu, Yo, and Ma2 antibodies were negative. Because the clinical and radiological features were suggestive of paraneoplastic limbic encephalitis (PLE), computed tomography scans of the chest and abdomen were done to rule out occult malignancy. The computed tomography scan of the abdomen showed a large lobulated hypodense mass arising from the mid and lower pole of the right kidney without enlarged lymph nodes or invasion of adjacent organs (Fig 2). A provisional diagnosis of PLE, possibly secondary to a renal cell carcinoma, was made.
The patient underwent a laproscopic right radical nephrectomy. Histopathology showed a cellular tumor with increased mitotic activity (Fig 3) and large areas of necrosis suggestive of a poorly differentiated malignancy, possibly high-grade non-Hodgkin's lymphoma (NHL). The diagnosis of diffuse large B-cell lymphoma was confirmed by immunohistochemistry, which was positive for CD20 (Fig 4), CD30 with high MIB-1 labeling index, and negative for CD3. A [18F]fluorodeoxyglucose (FDG) positron emission tomography scan done after surgery showed FDG uptake in the peri callosal white matter and both corona radiata, with hypometabolic area in the anterior corpus callosum. The rest of the body was negative for any abnormal foci of FDG uptake. The bone marrow biopsy was normal. A final diagnosis of primary renal lymphoma with PLE was made. His memory loss recovered during the following 3 weeks. He is now on chemotherapy with rituximab and cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisolone. At the time of publication, the patient continues to be well on chemotherapy.
Primary renal lymphoma is extremely rare and constitutes 0.7% of extra nodal lymphomas1 and 0.1% of all malignant lymphomas.2 Because the kidney is devoid of lymphatics, the existence of this entity is often debated.3 However, the negative positron emission tomography scan after surgery in our patient confirms the diagnosis and adds evidence to its existence. Common symptoms at presentation include flank pain, renal insufficiency, hematuria, and systemic symptoms like fever and weight loss.4 To our knowledge, this is the first report of a primary renal lymphoma presenting with symptoms unrelated to local effects of the primary tumor and with only PLE. PLE is a rare disorder characterized by subacute onset of short-term memory loss, seizures, confusion, and symptoms of hypothalamic dysfunction.5 Loss of short-term memory, as in our patient, is a hallmark of PLE. It commonly precedes the diagnosis of malignancy and is a diagnosis of exclusion. It may be associated with abnormalities in the temporal lobes on MRI.5,6 CSF shows features of inflammation in 80% of patients with lymphocytic pleocytosis and elevated protein, oligoclonal bands, or elevated immunoglobulin G. Antineuronal antibodies (anti-Hu and anti-Ma2) are present in 60% of patients with PLE.7 PLE has been commonly associated with small-cell lung cancer and testicular tumors.8 Other cancers associated with PLE include ovarian teratoma, breast cancer, thymoma, and Hodgkin's disease. Our patient presented with short-term memory loss, seizures, evidence of inflammation in the CSF, MRI abnormalities, and a diagnosis of primary renal NHL with complete neurologic recovery after removal of the renal lymphoma. With the exception of Hodgkin's disease and multiple myeloma, the incidence of paraneoplastic neurological syndromes in hematological malignancies is rare—less than 1%.5 The association of NHL with PLE is extremely rare, with only two instances reported.9,10 To our knowledge, it has never been reported with primary extranodal NHL. This case illustrates the occurrence of two uncommon features in a rare tumor—the presentation of primary renal lymphoma with symptoms unrelated to local effects of the primary tumor and the association of PLE with an extranodal NHL. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES 1. Freeman C, Berg JW, Cutler SJ: Occurrence and prognosis of extrarenal lymphomas. Cancer 29:252-260, 1972[CrossRef][Medline] 2. Aozasa K, Tsujimoto M, Sakurai M, et al: Non-Hodgkin's lymphoma in Osaka, Japan. Eur J Cancer Clin Oncol 21:487-492, 1985[CrossRef][Medline] 3. Kandel LB, Mc Cullogh DL, Harrison LH, et al: Primary renal lymphoma. Does it exist? Cancer 60:386-391, 1987[CrossRef][Medline] 4. Stallone G, Infante B, Manno C, et al: Primary renal lymphoma does exist: Case report and review of the literature. J Nephrol 13:367-372, 2000[Medline] 5. de Beukelaar JW, Sillevis Smitt PA: Managing paraneoplastic neurological disorders. Oncologist 11:292-305, 2006 6. Scheid R, Lincke T, Voltz R, et al: Serial 18F-flouro-2-deoxy-d-glucose positron emission tomography and magnetic resonance imaging of paraneoplastic limbic encephalitis. Arch Neurol 61:1785-1789, 2004 7. Rees JH: Paraneoplastic syndromes: When to suspect, how to confirm and how to manage. J Neurol Neurosurg Psych 75:43,2004 8. Gultekin SH, Rosenfeld MR, Voltz R, et al: Paraneoplastic limbic encephalitis: Neurological symptoms, immunological findings and tumor association in 50 patients. Brain 123:1481-1494, 2000 9. Semnic M, Jovanovic D, Petrovic D, et al: Paraneoplastic limbic encephalitis in a patient with non Hodgkin's lymphoma. Arch Oncol 12:71-73, 2004[CrossRef] 10. Thuerl C, Muller K, Laubenberger J, et al: MR imaging of autopsy proved paraneoplastic limbic encephalitis in non-Hodgkin lymphoma. Am J Neuroradiol 24:507-511, 2003
This article has been cited by other articles:
|
|||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|
|
|||||||||||
|
|||||||||||
|
|
Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
|
