Primary central nervous system lymphoma in acquired immune deficiency syndrome. A clinical and pathologic study with results of treatment with radiation

Abstract

Primary central nervous system (CNS) lymphoma occurs frequently in patients with the acquired immune deficiency syndrome (AIDS). Seventeen patients with AIDS and biopsy-proven CNS lymphoma were treated with whole-brain radiation. At presentation, most patients were severely debilitated from previous AIDS-related illnesses. Patients generally had focal neurologic symptoms such as seizures and paralysis. Headaches and mental status changes, often noticed after hospital admission, seldom brought our patients to seek medical attention. Computed tomography (CT) scan showed low-density, contrast-enhancing, mass lesions with variable amounts of peritumor edema. Size, location, and pattern of contrast enhancement of the lesions varied. No specific pattern was seen that could be used to distinguish between CNS lymphoma, toxoplasmosis, or other CNS diseases that occur in patients with AIDS. Biopsy results showed angiocentric, high-grade, large cell tumors with frequent necrosis. Immunohistochemical analysis showed B-cell phenotype with small amounts of T-cells, presumably reactive. All patients received irradiation to the whole brain with parallel opposed fields. A variety of doses and treatment regimens were used. Mean survival was only 72 days. Survival was longer in patients with higher pretreatment Karnofsky scores. The correlation between dose and survival was not significant. At completion of therapy, most patients showed improvement in Karnofsky score and had partial improvement in neurologic symptoms. CNS lymphomas in patients with AIDS are responsive to radiation. Posttreatment CT scans showed regression of tumors. Autopsy examinations showed regression of tumors, but also showed concurrent CNS infections, AIDS encephalopathy, and radiation-induced changes within the normal CNS tissue. Opportunistic infections rather than cerebral herniation or uncontrolled lymphoma was the most common cause of death.

Human immunodeficiency virus associated central nervous system infections

“…Most patients have constitutional symptoms such as fever, night sweats and weight loss. The mean duration of symptoms before diagnosis is 22-54 days (So et al 1986;Baumgartner et al 1990;Goldstein et al 1991;Fine & Mayer 1993;Chamberlain & Kormanik 1999).…”

Focal Neurological Disease in Patients with Acquired Immunodeficiency Syndrome

“…The majority of patients have B symptoms (e.g., fever, night sweats, weight loss) at the time of presentation. The mean duration of symptoms before diagnosis is 22-54 days [99,106,107].…”

“…In the pre-HAART era, the median duration of survival for untreated patients with primary CNS lymphoma was quite poor (1-2.5 months) [99,106,107,110]. Because, most often, the tumor is multifocal, complete surgical resection is usually not possible and does not improve prognosis.…”

“…For 50%-75% of patients, cranial whole-brain radiation (usual dose, 2000-4500 cGy) effectively shrinks the tumor and improves symptoms; however, most studies have documented a median survival benefit of only 1-3 months [99,100,103,104,106,107,111,112]. In most studies, patients died of other causes related to their severe immunosuppression [105,107].…”

Primary central nervous system lymphoma

“…Clinically, the presenting symptoms in patients with AIDS are more likely than in the immunocompetent population to be generalized, consisting of encephalopathy rather than focal neurological dysfunction (Goldstein et al, 1991a;Forsyth et al, 1994). The radiographic features of AIDS-related PCNSL are also less predictable, and cannot reliably distinguish this tumor from other CNS neoplasms or infections, particularly toxoplasmosis (Dina, 1991;Goldstein et al, 1991b).…”