Primary Cutaneous MZL with Amyloid Deposition, Initially Misdiagnosed as Amyloidosis Mimics Primary Dural MZL upon Dural Dissemination

Primary cutaneous marginal zone B-cell lymphoma (MZL) is considered a cutaneous counterpart of extranodal MZL of mucosa-associated lymphoid tissue and an indolent lymphoma. Amyloid deposition in this tumor is very rare. We report here a case of primary cutaneous MZL with massive amyloid deposition. The tumor disseminated to the dura and mimicked primary dural MZL. The patient had a four year history of the recurrence cutaneous lesions with a misdiagnosis of amyloidosis. The correct diagnosis was made after the metastatic dural lesion was confirmed by radiology, pathology and molecular biology approaches. It is of crucial importance for differential diagnosis to avoid misdiagnosis and the patients are indeed required long-term regular examinations and treatment because of the possibility of recurrence or dissemination.