Cervical extramedullary lymphomatoid granulomatosis

Abstract

Lymphomatoid granulomatosis (LYG) is a rare multisystem disease involving most frequently the lung, kidney and skin. LYG is characterized by an infiltration of atypical lymphocytoid and plasmocytoid cells, with granulomatous inflammation in an angiocentric and angiodestructive pattern. There have been only a few reports of spinal involvement of LYG. To our knowledge, we report the first patient with cervical extramedullary LYG undergoing surgical removal of a lesion causing spinal cord compression, and review the pertinent literature. The patient underwent C5-partial T1 laminectomy with partial removal of the lesion and decompression of the spinal cord. A diagnosis of a grade III LYG was made and chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) was administered. The patient was in good neurological condition at the 6-month follow-up but died 5 months later because of systemic progression of the disease. Although prognosis of LYG remains poor, surgery can be considered to improve pain relief and neurological morbidity of spinal LYG.