title: Primary cutaneous adenoid cystic carcinoma: a clinicopathologic and immunohistochemical study of 27 cases.
abstract: This study examines clinical and pathologic features of primary cutaneous adenoid cystic carcinoma (ACC), with emphasis on biological behavior of these tumors. A total of 27 cases of primary cutaneous ACC with detailed follow-up information were evaluated. Clinically, these were solitary, slow-growing lesions, half of which were in the head and neck area. The median age was 62 years with a male predilection. Surgical excision was the treatment of choice. Histologically, the lesions were similar to those seen in the salivary glands. Tumors were classified as grade 1 (17), grade 2 (3), and grade 3 (7). The mitotic count was generally low (mean=1.9/mm), except in 2 high-grade tumors (>10 mitotic figures/mm). Sixteen cases showed perineural invasion. Immunohistochemically, cytokeratin positivity was noted in 13/13 cases, and CD117 was observed in 10/10 cases, with luminal/cytoplasmic staining for epithelial membrane antigen (14/16) and at least focal luminal expression for carcinoembryonic antigen (11/16), smooth muscle actin (10/13), and S100 staining (9/13). Eighteen cases had follow-up data (median 54 mo), 9 of which had local recurrences (50%). Three cases showed metastatic disease. No statistical difference was noted between tumor grade and local recurrence (P=0.77). Primary cutaneous ACC is a distinct entity with a more indolent behavior compared with its salivary counterpart. The cutaneous lesions tend to recur locally but have a low metastatic potential.
authors.affiliation: *Department of Dermatopathology, St John's Institute of Dermatology, St Thomas' Hospital, London †Department of Pathology, Manchester Royal Infirmary, Manchester #Department of Pathology, Western General Hospital and The University of Edinburgh, Edinburgh, UK ‡Nelson R. Mandela School of Medicine and Central Hospital, Durban, South Africa §Sections of Dermatopathology and Soft Tissue/Sarcoma Pathology, MD Anderson Cancer Center, University of Texas, Houston, TX ∥Department of Pathology, Dublin Hospital, Dublin, Ireland ¶Charles University Medical Faculty Hospital, Pilsen, Czech Republic.
authors.forename: Rathi
authors.initials: R
authors.lastname: Ramakrishnan
authors.forename: Iskander H
authors.initials: IH
authors.lastname: Chaudhry
authors.forename: Pratistadevi
authors.initials: P
authors.lastname: Ramdial
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Lazar
authors.forename: Mairin E
authors.initials: ME
authors.lastname: McMenamin
authors.forename: Dmitry
authors.initials: D
authors.lastname: Kazakov
authors.forename: Thomas
authors.initials: T
authors.lastname: Brenn
authors.forename: Eduardo
authors.initials: E
authors.lastname: Calonje
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D003528:Carcinoma, Adenoid Cystic / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor