Primary central nervous system lymphoma (PCNSL) is an uncommon neoplasm, historically representing approximately 1% of primary brain tumors. However, its incidence has more than tripled in the United States and United Kingdom since the early 1980s. PCNSL represents 3% of intracranial neoplasms in patients with AIDS. Simultaneously, the incidence of description in the literature of pituitary lymphoma has been increasing and emerging clinical entity has been standing. The aim of this paper will be to review the clinical case reports so far available of pituitary lymphomas in order to depict with the most updated information the clomical syndrome correlated. The total number of cases reported is 17 cases of primary pituitary lymphoma (PPL) and 12 cases of secondary localization (male/female ratio is 2/1; the peak incidence occurs around the 5th and 6th decade of age; the mean age (mean ± SD) of the patients is 61 ± 15.8 years). The clinical presentation of PPL may be similar to that of pituitary invasive macroadenomas. As many as 50% of patients showed at the time of diagnosis clinical and/or laboratory evidence of anterior pituitary hypofunction. Of the patients with anterior pituitary failure, 5 had diabetes insipidus; 3 had hyperprolactinemia; 50% of patients had visual field defects; 40% presented cranial nerve involvement; 15 patients underwent transphenoidal surgery; and 63% had diffuse B cell-large lymphoma. Approximately half of the patients received radiation therapy and chemotherapy in different regimens. PPL seems to have a better prognosis with respect to all PCNSLs (70% of the examined patients had survived quite long). The data so far reported suggest that PPLs are not a unique clinical entity but that they may represent the final presentation of different processes with either hypophyseal or extrahypophyseal origin. Neurosurgical intervention or biopsy are at this point to be considered crucial for early diagnosis of the tumor. Disease treatment cannot be difined as peculiar based on as-yet reported experiences but should follow the most updated standards currently used for cerebral lymphomas.
*Assistant Professor, †Attending Physician, Endocrine Unit, Carlo Poma Hospital, Mantova; ‡Postgraduate Fellow of Endocrinology, ∥Associate Professor of Medicine, Department of Internal Medicine, University of Brescia; and §Associate Professor of Endocrinology, University of Parma, Parma, Italy.
The authors have disclosed that they have no significant relationships with or financial interests in any commercial company that pertains to this educational activity.
Reprints: Prof. Andrea Giustina, Endocrine Section c/o 2a Medicina, Spedali Civili, 25125 Brescia, Italy. E-mail: email@example.com.
Chief Editor’s Note: This article is the 28th of 36 that will be published in 2004 for which a total of up to 36 Category 1 CME credits can be earned. Instructions for how credits can be earned precede the CME Examination at the back of this issue.