Primary Adrenal Lymphoma Presenting   with Cauda Equina Syndrome

In Keun Choi 1, Eui Bae Kim1, Sang Dae Kim 2, Yoon Seok Choi 1, Kyong Hwa Park 1, Ju-Han Lee 3, Sang Cheul Oh 1, Jae Hong Seo 1,  Sang Won Shin 1, Yeul Hong Kim 1, and Jun Suk Kim 1 

1Department of Internal Medicine, Division of Hematology/Oncology,

2Department of Neurosurgery

3Department of Pathology,

College of Medicine, Korea University, Seoul, Korea

*Correspondence to: Dr. Jun Suk Kim,

Professor of Internal Medicine, Division of Hematology/Oncology, College of Medicine, Korea University Guro Hospital, 97 Gurodong-gil, Guro-ku, Seoul, Korea, Zip Code: 152-703

E-mail: kjs6651@empal.com

SMJ 2008 53(4): 10

 

Abstract

Primary adrenal lymphoma (PAL) is an extremely rare extranodal lymphoma. Moreover, cauda equina syndrome (CES) as a first sign of a primary adrenal lymphoma is very exceptional. Here, we report a 35-year-old man was admitted because of primary adrenal lymphoma presenting with CES. He was treated with chemotherapy including rituximab, cyclophosphamide, vincristine, adriamycin, and prednisolone (R-CHOP). At present 30 months after chemotherapy of R-CHOP, the patient is considered free of tumor. The good results obtained for our case could be due to the relative early detection by CES and the use of combination of monoclonal antibody (R-CHOP).

Key words: Primary adrenal lymphoma, Cauda equina syndrome, Rituximab chemotherapy

 

Introduction

Primary adrenal lymphoma (PAL) is an extremely rare extranodal lymphoma 1 . It usually presents with large, bilateral adrenal masses, and predominantly diffuse large B-cell histology and may be accompanied by adrenal insufficiency in some cases 2 . Cauda equina syndrome (CES) as a first sign of a primary adrenal lymphoma is very exceptional. Here we report a case of primary bilateral adrenal NHL presenting with CES that showed complete remission and prolonged survival as a result of treatment with chemotherapy including rituximab, cyclophosphamide, vincristine, adriamycin, and prednisolone (R-CHOP).

 

Case History 

A 35-year-old man was admitted to our hospital because of nausea and poor oral intake, fever, night sweats, weight loss and general weakness that had lasted for 10 days. Right sciatica and limping gate developed 6 month ago. He initially had suffered paresthesia and tingling sensation on the posterior region of the right lower extremity, which slowly progressed up the back of the leg to the buttock. Erectile dysfunction and urinary retention were also developed recently. Physical examination revealed temperature of 38.8 °C , heart rate of 72/min, respiratory rate of 20/min, and blood pressure of 110/60 mmHg. There was no lymphadenopathy, organomegaly, or other significant findings on physical examination. Neurologic examinations were normal except in the right lower extremity where there was 4/5 [Medical Research Council (MRC) Grading Scale] weakness. There was diminished sensation to all modalities over the posterior aspect of the right leg, the lateral aspect of right foot, and the right perianal region. The right knee jerk was normoactive response, while the ankle jerk was diminished. The performance status was ECOG 2. 

 

The laboratory studies showed a complete blood count with hemoglobin of 9.89 g/dL, a white blood cell count of 8,000/μL with 18.0% segmented neutrophils, 69.0% Lymphocytes, and 9.97% monocytes, and a platelet count of 160,000/ μL. The serum sodium was 129 mEq/L, potassium 4.3 mEq/L, chloride 96 mEq/L, bicarbonate 29 mEq/L and glucose 96 mg/dL. There was a high serum LDH of 1,926 IU/L (nl 263-450) and beta 2 microglobulin was 2.91 mg/dL (nl 0-2.4). The protein was 6.39 mg/dL, albumin was 3.0 mg/dL. There was no serological evidence to support a concurrent CMV infection.

 

The serum cortisol level was 17 μg/dL (5-25 μg/dL), and the ACTH level was 18.3 pg/mL (6.0-76.0pg/mL) at 8 am. The cortisol level was 196 μg/dL 1 h after the cosyntropin stimulation test (250 μg intramuscular injection). There was no remarkable finding in CSF study. Bilateral enlargement of the adrenal glands was found on an abdominal computed tomography (CT) scan (fig 1).

 

Fig. 1. Bilateral adrenal enlargement on initial abdominal CT scan. 

 

 

The Right adrenal mass was 6.2 × 3.3 cm in size, and the left one was 5.8 × 2.8 cm in size. Chest CT was normal. Spine MRI showed diffuse multiple pathologic nerve root enhancement from conus medullaris to sacral nerve level (fig 2). 

 

Fig. 2. Spine MRI showed diffuse multiple pathologic nerve root enhancement from conus medullaris to sacral nerve level. (T1 enhanced saggital view)  

 

 

Nerve conduction studies and needle electromyography showed right S1 radiculopathy nearly complete axonotmesis state. An ultrasound-guided needle biopsy of the right adrenal gland showed malignant diffuse large B cell lymphoma. On immunohistochemical staining, these tumor cells expressed CD20 and CD79a (B cell marker), but negative for CD3, alpha-fetoprotein, PLAP, hCG, C-Kit, Cytokeratin and EMA. There was no bone marrow involvement of lymphoma.

The patient received chemotherapy with cyclophosphamide, vincristine, adriamycin, oral prednisolone and rituximab (R-CHOP). After two cycles, complete remission was achieved. And we added 4 cycles of combination chemotherapy. His neurologic deficits  were also much improved. At present 30 months after chemotherapy of R-CHOP the patient is considered free of tumor.

 

Discussion

Direct involvement of the cauda equina by lymphoma is extremely rare. There were a few cases of CES due to infiltration of angiotrophic lymphoma of B cell origin 3,4 . In MRI of our case, diffuse multiple pathologic nerve root enhancement from conus medullaris to sacral nerve level suggested direct involvement of cauda equina. Moreover, neurological deficits of the patient were improved after R-CHOP chemotherapy.

So far about 100 cases of primary adrenal lymphoma (PAL) have been reported in both Western and Eastern countries over the past 40 years 5 . The most common type of lymphomas encountered in PAL is diffuse large B cell lymphoma 2 . Treatment of this lymphoma has been similar to treatment for other types of lymphoma 1 . Many of them, however, relapsed usually during the first few months of observation 5 . Recently there are reports that the addition of rituximab to the CHOP regimen (R-CHOP) increases the complete-response rate and prolongs overall survival in patients with diffuse large-B-cell lymphoma 6,7 . We treated the patient with R-CHOP chemotherapy because R-CHOP is expected to improve the result of diffuse large B cell lymphoma in PAL, too.

 

Our case had not adrenal insufficiency. Adrenal insufficiency has been identified in half of the reported PAL cases 8 . More than 90% of the adrenal glands are destroyed before clinical or biochemical evidence of adrenal insufficiency 9 . So the patient with adrenal insufficiency can be associated with advanced stage of PAL. After treatment the outcome of these patients has generally been poor.

 

Several prognostic factors of primary adrenal Non-Hodgkin’s lymphoma (NHL) have been addressed. Old age, initial presentation with adrenal insufficiency, huge tumor size, elevated serum LDH level, and involvement of other organs generally carry a poor prognosis 10,11 . In terms of age, adrenal insufficiency, tumor size the patient might be diagnosed in his early stage.

 

Poor prognosis of PAL may reflect a publication bias or the presence of adverse prognostic factors at diagnosis in most patients 12 . This diagnosis is frequently not suspected antemortem, and long delays between the onset of symptoms and the diagnosis are frequent 2,13 . During the early stage, particularly when the lesion is small, primary adrenal lymphoma is likely to be missed 14 . Therefore, if early diagnosis and intervention is possible, this may dramatically affect the clinical outcome 13 .

 

Conclusion

Cauda equina syndrome (CES) as a first sign of a primary adrenal lymphoma is very rare. The good results obtained for our case were likely due to the relatively early detection by CES and the use of combination with monoclonal antibody (R-CHOP).

 

Reference  

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