Early Organ Metastasis in Granulomatous Mycosis Fungoides: A Systematic Review.

BACKGROUND: Granulomatous mycosis fungoides (GMF) is a rare form of cutaneous T-cell lymphoma characterized by a granulomatous inflammatory infiltrate.

OBJECTIVE: The impact of granulomatous inflammation on the prognosis of the disease remains controversial as there are both favorable and unfavorable outcomes documented.

METHODS: We performed a systematic review of 116 GMF cases previously described in the literature.

RESULTS: In contrast to the classic Alibert-Bazin type of mycosis fungoides (MF), cutaneous lesions in GMF tend to involve distal extremities (lower legs, feet, hands) early in the disease course. In the literature, 30% of GMF patients developed organ metastasis, most frequently to the lung. The median time to stage progression was 25 months.

CONCLUSION: GMF is an aggressive form of mycosis fungoides. Therefore, screening for distant metastases should be considered at presentation and repeated during follow-up.