title: 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.
authors.affiliation: *Sunnybrook Health Science Centre, Division of Dermatology, University of Toronto, Toronto, Ontario; and †University Health Network, Toronto General Hospital, University of Toronto, Department of Pathology, Toronto, Ontario.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Shamsuyarova
authors.forename: Zaid
authors.initials: Z
authors.lastname: Kamil
authors.forename: Jan
authors.initials: J
authors.lastname: Delabie
authors.forename: Nadya
authors.initials: N
authors.lastname: Al-Faraidy
authors.forename: Danny
authors.initials: D
authors.lastname: Ghazarian
mesh_terms: D000328:Adult; D006801:Humans; D008224:Lymphoma, Follicular / Q000150:complications / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications / Q000473:pathology*; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology*; D006377:T-Lymphocytes, Helper-Inducer / Q000473:pathology*