Rapid Progression of Primary Cutaneous Gamma–Delta T-Cell Lymphoma With an Initial Indolent Clinical Presentation

Abstract

Primary cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is a rare cutaneous T-cell lymphoma characterized by a rapidly progressive clinical course and a poor prognosis. We report a case of a 52-year-old man with a 10-year history of erythematous nodules and a rapid terminal progression diagnosed as CGD-TCL. Biopsies taken at the time of progression showed a dense lymphocytic infiltrate involving the subcutaneous adipose tissue and deep dermis. One of the biopsies displayed much more limited involvement by CGD-TCL that was nearly identical to the biopsies of the erythematous lesions 10 years before. In conclusion, this case demonstrates a case of CGD-TCL presenting as a longstanding indolent disease with a rapid terminal progression. The indolent clinical course and histological heterogeneity make diagnosing this entity during the initial stage extremely challenging. This case underscores a diverse clinical presentations and a need to consider CGD-TCL in patients showing subcutaneous lesions with an indolent clinical course.

Primary Cutaneous Gamma-Delta T-Cell Lymphoma Initially Diagnosed as Subcutaneous Panniculitis-like T-Cell Lymphoma with Dermatomyositis

“…Although CGD-TCL typically has an aggressive course and a poor prognosis, with a median survival of approximately 12 months [ 1 ], indolent cases of CGD-TCL have also been reported [ 6 , 7 , 8 , 9 ]. Indolent CGD-TCL cases displayed recurrent self-remitting nodules, which mimicked lupus panniculitis or erythema nodosum, and lasted for several years.…”

“…Similar to our case, those cases responded very well to treatment with either multiagent chemotherapy or prednisone [ 6 , 7 ]. Although some patients with indolent courses survived disease-free after treatment, others rapidly progressed and had unfortunate outcomes [ 8 , 9 ]. In our patient, the disease appeared to be indolent, but the prognosis will not be clear for several years.…”

Primary Cutaneous Gamma-Delta T-cell Lymphoma: Not an Aggressive Disease in All Cases

“…However, an increasing corpus of reports suggests the existence of a less frequently encountered subset with an allegedly more "indolent" or "smouldering" clinical course, variable clinical and histological presentations (4)(5)(6)(7)(8)(9)(10)(11)(12) and an overall survival that may range from 2 (4) to more than 15 years (10). Of note, the accurate nature of this "indolent" behaviour appears relatively ambiguous in earlier reports, since it may reflect variable underlying considerations including a less-than-expected clinical aggressiveness, a protracted overall survival (usually estimated since first occurrence of skin lesions) and/or a more favourable response to treatment.…”

Cutaneous Gamma-delta T-cell Lymphoma with an Initially Indolent Course Mimicking Lupus Panniculitis

“…There are several publications emphasizing that CGD-TCL can have an indolent course for years, before it shows the typical rapid disease progression (5)(6)(7)(8)(9)(10). Still, it is crucial to exclude other CTCLs, such as mycosis fungoides or lymphomatoid papulosis with a γ-δ phenotype that, in general, show a far more indolent course (1).…”