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Background Primary central nervous system non-Hodgkin's lymphoma (NHL-CNS) is an enigmatic disease of uncertain origin. At the Massachusetts General Hospital, 104 patients with NHL-CNS were seen from 1958 through 1989. An impression of changes in the frequency of diagnosis, character of the tumors, and therapy for this disease prompted this study of the pathologic features, clinical data, and natural history of this tumor in these 104 patients. Methods. Histologic slides (neurosurgical specimens and autopsy tissues) were available for 99 patients. The tumors were classified by the Working Formulation classification. Immunostaining data and all clinical data were retrieved from the relevant offices and hospital charts. Results. Primary central nervous system non-Hodgkin's lymphoma tripled in frequency (5.66 cases per year in 1978-89 versus 1.75 cases per year in 1958-77) and now represents 6.6% of all primary brain neoplasms (versus 3.3% before 1978; [[chi].sup.2] = 17.52, P < 0.01). For the 99 tumors histologically classified, 89% were high grade. Intermediate grade lymphomas, once the second most common subtype, have disappeared since 1983. All tumors had diffuse architecture; 77% (including all 11 patients with acquired immune deficiency syndrome) were large cell subtypes. Two cases were intravascular lymphoma. With one exception, all of the 41 tumors evaluated were B-cell types; 32 of 40 had monotypic surface immunoglobulin. There was 1 T-cell lymphoma. Of 64 tumor recurrences, 29 were at the initially defined site; 12 were in the leptomeninges, 29 were in other sites in the neuraxis, and 8 were in systemic sites. Systemic metastases have not occurred since 1984. Median survival for the 68 patients who survived after diagnostic surgery and for whom follow-up information could be obtained was 19 months; 9 months for those with high grade tumors and 30.5 months for those with intermediate grade tumors. This difference was not significant (P = 0.13). A separate set of seven patients had focal tumorlike lymphoid infiltrates composed of benign-appearing lymphocytes, which were associated with good long term survival. The differential histologic diagnosis of NHL-CNS was occasionally difficult, and the spectrum of this differential was broader than generally stated. Conclusions. Primary central nervous system non-Hodgkin's lymphoma has increased in frequency even in nonimunocompromised patient populations. This increase has been accompanied by the disappearance of intermediate grade histologic types, suggesting a fundamental shift in the biology of the neoplasms. The introduction of chemotherapeutic regimens appears to have altered the natural history such that systemic metastases outside the central nervous system no longer occur, and there are now some long term survivors of this formerly uniformly fatal disease. Cancer 1994; 74:1383-97.

Background. Peripheral nerve tumors (PNT) containing glands are uncommon. Which types of PNT contain glands is a matter of controversy, a factor bearing on the prognosis of these tumors. Methods, The authors reviewed the files of 11 patients with glandular PNT seen in their laboratory and 27 patients reported in the literature. Results. The 11 instances of glandular PNT seen in their laboratory affected male and female patients equally; patient ages ranged from 8 to 68 years (mean, 28 years). Six patients had neurofibromatosis-1 (NF-1). Eleven of the tumors were histologically malignant PNT, and one was a benign neurofibroma. There were no schwannomas (neurilemomas). The glands were discrete, usually localized to a few areas and in every patient were lined by a keratin-positive epithelium, which in two patients was malignant. One tumor also contained areas of rhabdomyosarcoma, chondrosarcoma, and osteosarcoma (a pluridirectional malignant PNT). Treatment in all patients was some form of surgical resection, followed by radiation in three and chemotherapy in two. Follow-up data were available for nine patients; six of eight patients died with disease. Review of the literature revealed two purported glandular schwannomas (neurilemomas). The authors think these patients had schwannomas containing trapped skin adnexa. Overall, 74% of the patients bad NF-1. Ninety-two percent of the tumors were histologically malignant, and 23% of the malignant tumors were pluridirectional malignant PNT. Of the 21 patients for whom follow-up was available, 71% died with tumor. Conclusion. Most glandular PNT are histologically malignant and often are fatal.

Background. Intradural parenchymal involvement (IPI) in the spinal subarachnoid space associated with primary lung cancer is rare. A retrospective study was undertaken to investigate the clinical and pathologic features of IPI. Method. A total of 1215 cases of primary lung cancer were studied at autopsy; the results were reviewed retrospectively. Results. Twenty (1.65%) of the cases revealed IPI in the spinal subarachnoid space. The histologic diagnoses were small cell carcinoma in ten cases, adenocarcinoma in eight cases, and squamous cell carcinoma in two cases. In 14 (70%) cases, the IPI was located between the lumbar and cauda equina of the spinal cord. However, no metastases were observed in the cervical spinal cord. Brain metastasis, vertebral metastasis, and meningeal carcinomatosis were seen in 70%, 60%, and 40% of the 20 cases, respectively, suggesting that these metastases may be related to the metastatic pathway to the spinal cord. Most patients had neurologic symptoms or signs referable to IPI; IPI could be diagnosed before death in only one patient by magnetic resonance imaging. The median interval between diagnosis of lung cancer and development of IPI and median survival after the onset of neurologic symptoms referable to IPI were 415 days and 110 days, respectively. Conclusion. The authors retrospectively received 1215 autopsies of patients with primary lung cancer and found 20 (1.65%) with IPI.

Patients with cancer are at increased risk for the development of some other cancer later in life. These secondary neoplasms are often leukemias or lymphomas, but it is not known to what degree these conditions arise as a response to the treatment of the first cancer, or to what degree they represent a predisposition towards cancer. Lymphoma that arises as a secondary neoplasm, the primary lymphoma of the central nervous system is considered uncommon. The author reports the cases of seven patients who developed a primary lymphoma of the central nervous system as a secondary tumor. The lymphoma occurred an average of 10 years after the diagnosis of the original tumor, and an average of six years after the last evidence of disease in the original tumor. Alkylating agents are chemotherapeutic drugs that are associated with a high rate of secondary cancers. However, four of the seven patients did not receive any chemotherapy, and two did not receive either chemotherapy or radiotherapy. Therefore, the brain lymphomas cannot be attributed to previous treatment in all cases. Review of these cases revealed that there tends to be a delay in the diagnosis of brain lymphoma due to confusion of the lymphoma with a brain metastasis of the original cancer. In one case, the patient's history suggested that the primary central nervous system lymphoma actually had begun at least five years prior to its diagnosis. Since brain lymphoma is more readily treatable before dissemination, such delays may have unfortunate clinical consequences. In the present series, three patients were alive at the time of this report, and of these, two have no evidence of disease 12 and 75 months after diagnosis of lymphoma. (Consumer Summary produced by Reliance Medical Information, Inc.)