Published online Apr 26, 2020. doi: 10.12998/wjcc.v8.i8.1507
Peer-review started: January 18, 2020
First decision: February 26, 2020
Revised: April 3, 2020
Accepted: April 8, 2020
Article in press: April 8, 2020
Published online: April 26, 2020
Processing time: 97 Days and 3.9 Hours
We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma (MCL), an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.
The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body. Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers, sparing the epidermis. Immunohistochemical staining revealed CD20, cyclin-D1, CD5, and SOX-11 expression. Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement. Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved; these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease. In this case, positron emission tomography scanning and clinical staging revealed no systemic involvement, and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease. The prognosis of primary cutaneous MCL is relatively good. Our patient received six cycles of chemotherapy, and the cutaneous manifestations presented almost complete remission.
Primary cutaneous MCL is rare, and its prognosis is relatively favorable. However, correct diagnosis is a prerequisite for proper treatment.
Core tip: Primary cutaneous mantle cell lymphoma (MCL) is rare and has been the subject of isolated case reports. To correctly diagnose primary cutaneous MCL, it must be ensured that no other parts are involved, and these cases require close follow-up to monitor the possible progression to systemic disease and treat relapsed cutaneous disease. Correct diagnosis is crucial for clinical treatment and prognosis. The prognosis of primary cutaneous MCL is relatively favorable, and treatment options should comply with the clinical condition of the patient.