Primary effusion lymphoma involving cerebrospinal fluid, deep cervical lymph nodes and adenoids. Report of a case supporting the lymphatic connection between brain and lymph nodes.

We describe an unusual presentation of primary effusion lymphoma in CSF of a 45-year-old HIV-positive man, with no evidence of involvement of pleural, peritoneal or pericardial cavities. Cytologic examination and flow cytometric analysis suggested the diagnosis, eventually made in an excised deep cervical lymph node, in which the neoplastic cells involved selectively the sinuses. This case represents the fifth reported example of CSF involvement by this type of lymphoma, and supports the alleged connection between CSF and cervical lymph nodes via lymphatic vessels. Interestingly, review of an adenoidectomy specimen obtained 9 months before presentation for nonspecific complaints showed rare clusters of neoplastic cells involving surface epithelium and chorium, a finding that might represent a homing mechanism and implies an asymptomatic, occult phase of lymphoma development.

Primary effusion lymphoma involving the cerebrospinal fluid.

Primary effusion lymphoma or body cavity based lymphoma is a form of large B-cell lymphoma which usually presents as serous effusions without detectable tumor masses. It is universally associated with human herpesvirus-8 also known as Kaposi sarcoma herpesvirus. This condition, so far, has been reported in the body cavity effusions that include pleura, peritoneum, and pericardium. We report a case of primary effusion lymphoma which has involved the cerebrospinal fluid. To our knowledge, this is the first case of PEL reported in the cerebrospinal fluid.