Primary Cutaneous Follicular Helper T-cell Lymphoma in a Patient With Neurofibromatosis Type 1: Case Report and Review of the Literature

Abstract

Patients with neurofibromatosis type 1 (NF-1) have a well-known predisposition for certain types of malignancies, including lymphoproliferative disorders. Cutaneous T-cell lymphoma (CTCL) has been reported in patients with NF-1, although it is considered a rare entity in this subset of patients. Cutaneous follicular helper T-cell lymphoma (CTFHCL) is a recently emerged rare subtype of CTCL with peculiar clinical and histopathological features and represents a diagnostic and therapeutic challenge. Only a few cases of CTFHCL have been reported in the literature. We report a case of CTFHCL in a patient with NF-1 and compare our findings with previously reported cases. We aim to raise awareness among pathologists regarding this rare subtype of CTCL and emphasize characteristic histological features of CTFHCL, which can be confused with B-cell lymphomas and lead to mismanagement.

Primary Cutaneous Peripheral T-Cell Lymphoma With Follicular Helper T-Cell Phenotype: Report of 2 Epstein–Barr Virus–Positive Cases

“…16 In the literature, CD7 expression is variable in ENKTL and PC-TFHL. 3,4,8,10,16 In our case no. 1, CD7 expression was downregulated while CD7 was positive in case no.…”

“…PC-TFHL is rare with only sporadic case reports. 3–10 Most cases present as multiple skin lesions. Le Tourneau et al 10 reported 2 such cases presenting as multiple primary skin lesions.…”

“…In recent years, there are sporadic reports of primary cutaneous TFH lymphoma (PC-TFHL). 3–10 These cases usually present with multiple skin lesions with a relatively indolent clinical course. These tumors are not Epstein–Barr virus (EBV)–related, in contrast to extranodal NK/T-cell lymphoma (ENKTL), which is universally associated with EBV.…”

Dermatologic Manifestations of Neurofibromatosis Type 1 and Emerging Treatments

“…Although no distinct relationship has been established, there have been multiple case reports of CTCL in patients with NF1 [87][88][89]. In these three case reports, two patients were diagnosed with mycosis fungoides [88,89] and one with a rare subtype of cutaneous follicular helper T-cell lymphoma [87]. While patients with NF1 are at higher risk of malignancies, there is no known predilection for CTCL specifically.…”