title: Desmoplastic melanoma of the eyelid and conjunctival melanoma in neurofibromatosis type 1: a clinical pathological correlation.
abstract: A 56-year-old woman with neurofibromatosis type 1 (NF1) presented with a left upper eyelid amelanotic nodule with adjacent eyelid margin hyperpigmentation. Physical examination additionally revealed primary acquired melanosis (PAM) on the palpebral conjunctiva of the same eyelid. Full thickness eyelid excision and conjunctival map biopsy identified desmoplastic melanoma of the eyelid in addition to invasive conjunctival melanoma and conjunctival melanoma in situ. Sentinel lymph node biopsy was negative for metastasis. She was treated with surgical excision for the eyelid melanoma and topical mitomycin C for the conjunctival melanoma. We discuss the rare entity of desmoplastic melanoma of the eyelid and its possible association with NF1.
authors.affiliation: Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, USA.
authors.forename: Tal J
authors.initials: TJ
authors.lastname: Rubinstein
authors.affiliation: Department of Pathology and Laboratory Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Plesec
authors.affiliation: Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, USA. Electronic address: singha@ccf.org.
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D003230:Conjunctival Neoplasms / Q000473:pathology* / Q000628:therapy; D005142:Eyelid Neoplasms / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008545:Melanoma / Q000473:pathology* / Q000628:therapy; D018328:Melanoma, Amelanotic / Q000473:pathology* / Q000628:therapy; D008548:Melanosis / Q000473:pathology; D008875:Middle Aged; D016685:Mitomycin / Q000627:therapeutic use; D009361:Neoplasm Invasiveness; D009456:Neurofibromatosis 1 / Q000473:pathology*; D013508:Ophthalmologic Surgical Procedures; D014792:Visual Acuity
chemical_list: D000903:Antibiotics, Antineoplastic; D016685:Mitomycin
keywords: desmoplastic melanoma; eyelid; neurofibromatosis type 1; primary acquired melanosis; sentinel lymph node biopsy