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Journal of Neuroscience Nursing:

Stroke Versus Primary CNS Lymphoma in the Immunocompromised Patient

Manfredi, Tanya; Grietens, Ann‐Marie; Lambert, Teresa; Machado, Miguel

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Questions or comments about this article may be directed to Tanya Manfredi, MSN RN CNRN. She is director of surgical nursing at Flagler Hospital, St. Augustine, FL.

Ann‐Marie Grietens, RN CMSRN, is a charge nurse at Flagler Hospital, St. Augustine, FL.

Teresa Lambert, RN CNRN, is a nurse manager at Flagler Hospital, St. Augustine, FL.

Miguel Machado, MD, is a neurosurgeon at Flagler Hospital, St. Augustine, FL, and president‐elect of the Florida Neurosurgical Society.

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Primary central nervous system (CNS) lymphomas are an uncommon form of brain neoplasm that should be considered in the immunocompromised patient with neurological changes. It can be difficult to distinguish primary CNS lymphoma from stroke on initial radiographic images. Neuroscience nurses who are involved in the care of these patients should be prepared to assist the patient and family in coping with an evolving diagnosis and in explaining the related diagnostic procedures, treatment options, and eventual discharge plan.

When caring for immunocompromised patients who present with stroke symptoms, healthcare professionals need to consider the possibility of other disease processes, such as neoplasms, as the basis for neurological changes. Primary central nervous system (CNS) lymphoma accounts for only 3% of all intracranial neoplasms and 7% of all malignant lymphomas (Rowland, 2005). The incidence is more prevalent in adults ages 50 to 70 years, with a male‐to‐female ratio of 3:2 (Rowland). Single or multiple intraparenchymal lesions may occur. Clinical presentation may include headache, signs and symptoms of increased intracranial pressure, and lateralizing signs appropriate to the area(s) of involvement (Samuels & Feske, 1996). These symptoms can include behavioral, cognitive, and personality changes. Focal symptoms include hemiparesis, aphasia, and visual field defects. Ataxia, seizures, and cranial nerve palsies can also occur but are not as common (Hickey, 2002; Rowland).

Primary CNS lymphoma is a diffuse non‐Hodgkin lymphoma typically of the intermediate‐ or highgrade variety. Diffuse large B‐cell lymphoma is the most common type of lymphoma worldwide. The tumor cells tend to grow in the perivascular spaces, although they do not destroy or obliterate the vascular lumen (Samuels & Feske, 1996). Systemic dissemination of primary CNS lymphoma is rare. Regardless of where the malignant cells originate, once within the CNS, these cells proliferate in the subarachnoid space, the choroid plexus, or both and subsequently disseminate throughout the CNS (Shibata, 1989). Primary CNS lymphomas have a different appearance on computed tomography (CT) scan or magnetic resonance imaging (MRI), depending on whether the patient is immunocompetent or immunocompromised. In the immunocompetent patient, the lesion may appear as a solitary mass, but it may also take the form of multiple masses or a diffuse, infiltrating lesion. On CT or T2‐weighted MRI, edema may be visible and surround the lymphoma lesion, producing mass effect (Batchelor, Buchbinder, & Harris, 2005; Rowland, 2005). Primary CNS lymphomas enhance with the administration of contrast material. When central necrosis is present, an irregular ring enhancement is visible. In the immunocompromised patient, multiple lesions, necrosis, ring enhancement, and surrounding edema are more common findings than in the immunocompetent patient (Batchelor et al.; Rowland). This ring‐enhancing pattern correlates with the high incidence of tumor necrosis seen pathologically in the immunocompromised patient (Samuels & Feske).

Diagnosis requires histologic confirmation from brain tissue, cerebrospinal fluid (CSF), or vitreous humor because primary CNS lymphoma can involve all of these areas (Rowland, 2005). Elevated protein and mild mononuclear pleocytosis in the CSF are indicative of CNS lymphoma (Samuels, 1999). Given the deep periventricular location of most primary CNS lymphomas, image‐guided stereotactic biopsy may be the safest means to obtain a tumor sample. After primary CNS lymphoma has been diagnosed, contrast‐enhanced CT scans of the chest, abdomen, and pelvis are recommended to rule out extraneural sites (Rowland). However, in most individuals, lymphoma is confined to the brain parenchyma. Furthermore, each patient should also be tested for the presence of human immunodeficiency virus anti‐bodies bodies as a cause for immunodeficiency resulting in this disease process.

In the immunocompetent patient, survival rate is 12‐60 months, depending on treatment regimens and state of health at the time of diagnosis. For the immunocompromised patient, survival rate depends on state of health at the time of diagnosis and treatment regimen. Following the diagnosis, the standard approach is whole‐brain radiation therapy (WBRT), but patients may also be started on a regimen of high‐dose methotrexate (Rowland, 2005). For the immunocompromised patient, the median survival rate is approximately 4 months with radiotherapy, whereas patients who receive high‐dose methotrexate may survive for years. The patient's survival correlates closely with his or her performance status at diagnosis and the presence of coexistent opportunistic infections (Rowland).

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Case Study

JS was a well‐nourished, 83‐year‐old white female who had experienced multiple falls during the last 2 months. She was living at home with her daughter, who brought her into the emergency department (ED). The daughter reported that during the previous few days, JS had begun to decline physically, showed occasional facial weakness, and leaned to the left as she sat in a chair or attempted to ambulate.

Vital signs on admission to the ED were as follows: blood pressure 140/76 mm Hg, pulse oximetry 96% on room air, heart rate 90 beats per minute, and respiratory rate 16 breaths per minute. JS's physical examination revealed mild left facial droop; full extraocular movements; pupils that were equal, round, and reactive to light and accommodation; normal facial sensation; and a midline tongue and palate. She was alert and oriented times three and answered questions appropriately, but she appeared withdrawn and had a flat affect. She exhibited minimal left upper extremity and left lower extremity weakness. Her reflexes were symmetric, with sensation to light and touch intact. JS's gait was not tested. Her complete blood count and electrolyte panel were within normal limits.

JS's medical history consisted of hypertension; seizure disorder (diagnosed in childhood); and no history of tobacco, alcohol, or recreational drug use. Her surgical history was positive for breast cancer (which was treated with tamoxifen after a mastectomy), unspecified facial skin cancer, left nasal cancer surgery for a separate basal cell carcinoma, and a total abdominal hysterectomy because of an ovarian cyst. She had a history of frequent falls during the past few months resulting in compression fractures of the 4th and 10th thoracic vertebrae, for which she underwent subsequent kyphoplasty. A CT scan of the head in the ED demonstrated an acute to subacute ischemic infarct in the right frontal and frontoparietal region extending into the right subinsular region (Fig 1). There was also moderate associated mass effect.

Fig 1
Fig 1
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With this finding, JS was admitted to the medical intensive care unit (MICU) with a diagnosis of stroke. The stroke protocol orders initiated included neurological assessments every hour, complete bed rest, and nothing by mouth (NPO) status until speech pathology evaluated her ability to swallow safely. She was scheduled for an MRI of the brain with contrast.

When caring for immunocompromised patients who present with stroke symptoms, healthcare professionals need to consider the possibility of other disease processes, such as neoplasms, as the basis for neurological changes.

Nurses caring for the patient monitored vital signs and noted the slight facial droop and left‐sided weakness with left pronator drift on examination. JS was now noted to be somnolent and at times disoriented to time and place. Nursing interventions included performing frequent neurological assessments, monitoring for aspiration and ability to protect her airway, monitoring for increasing intracranial pressure, maintaining the patient's blood pressure within the range for sufficient cerebral perfusion pressure, providing frequent reorientation, and monitoring laboratory values and diagnostic results. The nurses educated JS and her family concerning the pathophysiology of stroke and short‐term and long‐term implications of her course of treatment and care.

Nurses reviewed JS's plan of care with her and her family and involved both in her care decisions. The nurses frequently found they had to reorient JS to the MICU environment and made every effort to respond quickly to her needs. Other interventions included speaking slowly and repeating important concepts. The nurses made sure that JS and her family understood the neurological tests, laboratory tests, and procedures to be performed, and informed them of results as they became available.

After assessment by the neurologist, the patient was started on intravenous dexamethasone to treat her cerebral edema and fosphenytoin sodium to prevent seizures. The neurosurgeon also assessed the patient and the MRI findings (Fig 2), which showed a moderate to large amount of perilesional edema with a moderate amount of regional mass effect but no shift of the midline structures. The pattern of contrast enhancement was in keeping with a significant ischemic insult but could also represent a neoplastic process, either metastatic or primary. Continued clinical correlation and imaging follow‐up was advised. The radiologist's impression was that there was a large lesion involving the right basal ganglia, thalamic, capsular, and sylvian regions with extension into the adjacent portions of the medial anterior right temporal lobe, as well as the posterior and inferior right frontal lobe, with a considerable associated perilesional edema with confluent areas of marked contrast enhancement. The radiologist's impression was that these findings were more in keeping with a large area of significant ischemic injury than with a neoplastic process.

Fig 2
Fig 2
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On review of the MRI films, the neurosurgeon believed that the findings on the MRI might be more compatible with a neoplasm and that with JS's history of breast cancer the possibility of brain metastasis should be considered. The surgeon discussed the possibility of a right deep hemispheric lesion with JS and her family and explained the necessity, risks, and relative safety of a stereotactic biopsy. The patient was scheduled for the biopsy the following day. The nurses reinforced the information the neurosurgeon had discussed and answered further questions about the impending procedure, which included supporting JS and her family in their decisions to pursue biopsy of the lesion.

Prior to surgery, several reference markers were fixed to JS's head, and she underwent CT scanning once again. These images were imported into the CT‐guided Stealth workstation in which the surgeon developed a surgical approach plan with the software. After anesthesia was administered, the computerized images were merged with the patient's cranial anatomy by touching each reference marker on the skull with a probe containing light‐emitting diodes (LEDs). A digitizing camera linked to the computer senses the LEDs, allowing the computer to “see” the probe's position in space. Special surgical instruments containing LEDs can also be followed in space by the computer. The position of the instruments is indicated on the workstation monitor. This allows precision in the surgical exposure and optimizes the biopsy.

Because of JS's history of breast cancer, the initial concern was that the mass would turn out to be a metastasis. Frozen and permanent sections of the biopsied tissue were examined and demonstrated uniform lymphoid cells. Surprisingly, results showed a diffuse large B‐cell lymphoma. A diagnosis of primary CNS lymphoma was then determined.

Postoperative nursing interventions included monitoring for decline in neurological condition, monitoring urine output and electrolytes, controlling pain, assessing for dysphagia, encouraging good nutrition, supporting the family, and monitoring the biopsy site for bleeding and infection. Postoperatively, JS developed dysphagia and was placed on aspiration precautions with the appropriate diet. Her leftsided weakness became more pronounced, and she required greater assistance with ambulation and activities of daily living. Difficulties for the nurses and other care providers at this point included explaining that brain metastasis had been ruled out by the stereotactic biopsy and that JS had been diagnosed with a different diagnosis: primary CNS lymphoma. The nurses were quick to acquaint themselves with this disease process and to provide JS and her family with information about her diagnosis and possible treatment regimen.

With the support of her family, JS progressed well through her hospitalization. Nursing and social services worked with the family, who wished to take JS home. The nurses instructed JS on her corticosteroid and antiepileptic medications to be taken at home and how to safely use her walker and shower chair. On the fifth day, JS was discharged home. Follow‐up appointments were made with her primary physician and neurosurgeon within a week of discharge. JS was also referred to a radiation oncologist and medical oncologist for discussion of further treatment options.

This case was presented to our Tumor Board 10 days postdischarge. The Tumor Board recommended a positron emission tomography (PET) scan to ensure that no systemic lymphoma was present, which could change the course of her therapeutic regime. However, the family reported that JS had quickly declined while at home and requested the assistance of hospice and palliative measures only.

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This patient, who was initially diagnosed with stroke, provided a learning experience for all involved with her care. Her presenting symptoms along with her preliminary test results all mimicked the obvious diagnosis of stroke. However, her significant history of cancer produced a degree of suspicion for the neurosurgeon that could not be disregarded. The nurses assisted the physician in providing the patient the necessary information to make an informed decision about treatment options. Today's technology provides a viable and reasonably safe means of a definitive diagnosis. The nurses who cared for JS and her family were forced to adapt to the difficulty of facing a patient who had been given numerous diagnoses, ranging from stroke to possible breast cancer metastasis to a rare brain tumor. The nurses were required to research and educate themselves about the disease process, treatment plan, and outcomes of each new diagnosis in order to care for the patient and family and assist them in coping with their situation. It is vital for nurses to keep themselves, the patient, and the family well informed. The nurse must be aware of test results to provide necessary explanations and emotional support during changes in treatment and plan of care.

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This case study demonstrates that nurses must be open‐minded and vigilant with assessment because each patient is an individual with his or her own complex history, and what may appear to be routine can actually turn out to be quite complex. For JS's nurses, regardless of their years of experience, this patient was challenging in every aspect of nursing care. The clinical challenge was to assess and monitor the patient's physical status to include her neurological status, vitals, laboratory results, and other test results. Another challenge was being able to adapt the plan of care and prepare the family for what seemed at first to be a routine stroke but evolved into a situation of an unusual brain tumor that had a great impact on the lives of the patient and her family.

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Batchelor, T. T., Buchbinder, B. R., & Harris, N. L. (2005). Case records of the Massachusetts General Hospital: Case 1-2005. New England Journal of Medicine, 352, 185-195.

Hickey, J. V. (2002). The clinical practice of neurological and neurosurgical nursing (5th ed.). Philadelphia: Lippincott, Williams & Wilkins.

Rowland, L. P. (2005). Merritt's neurology (11th ed.). Philadelphia: Lippincott, Williams & Wilkins.

Samuels, M. A. (1999). Hospitalist neurology. Woburn, MA: Butterworth-Heinemann.

Samuels, M. A., & Feske, S. (1996). Office practice of neurology. New York: Churchill Livingstone.

Shibata, S. (1989). Sites of origin of primary intracerebral malignant lymphoma. Neurosurgery, 25, 14-19.

© 2007 American Association of Neuroscience Nurses


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