Mantle cell lymphoma manifesting neurological symptoms similar to those of hypertrophic cranial pachymeningitis

The patient was a 58-year-old man, who presented with headache and myodesopsia. He demonstrated papilledema and hemorrhage in the fundus of the left eye and MRI findings showed localized hypertrophic dura mater. He was diagnosed as having hypertrophic cranial pachymeningitis and treated with antibiotics and prednisolone. However, the patient complained of persistent headache. Therefore, CT scans of the chest and abdomen were obtained. These images demonstrated superficial and intraabdominal lymphadenopathy and a histological diagnosis of mantle cell lymphoma was made on biopsy of an inguinal mass. Specimen obtained at craniotomy also showed the same lymphoma cells diffusely infiltrating the dura mater. Complete remission of the lymphoma including disappearance of hypertrophic dura mater was obtained after 4 courses of rituximab plus hyper-CVAD alternating with high-dose methotrexate and cytarabine, and the neurological manifestation improved thereafter. Subsequently, he underwent autologous peripheral blood stem cell transplantation. Hypertrophic pachymeningitis is a chronic progressive inflammatory disorder with hypertrophic dura mater of brain and spinal cord, caused by diverse illnesses. Mantle cell lymphoma infiltrating the dura mater is extremely rare, and has not been reported previously. In some cases of hematological malignancy with hypertrophic pachymeningitis, dural biopsy is required to differentiate the etiology.