title: Dedifferentiated adenoid cystic carcinoma of the lacrimal gland.
abstract: A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.
authors.affiliation: Department of Ophthalmology, New York Eye and Ear Infirmary, New York, NY, USA. jpanarelli@nyee.edu
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Panarelli
authors.forename: Christopher I
authors.initials: CI
authors.lastname: Zoumalan
authors.forename: Krishna
authors.initials: K
authors.lastname: Mukkamala
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Maher
authors.forename: Codrin
authors.initials: C
authors.lastname: Iacob
authors.forename: David A
authors.initials: DA
authors.lastname: Della Rocca
mesh_terms: D003528:Carcinoma, Adenoid Cystic / Q000473:pathology*; D054337:Cell Dedifferentiation / Q000502:physiology; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000473:pathology*