title: Primary salivary clear cell tumors--a diagnostic approach: a clinicopathologic and immunohistochemical study of 20 patients with clear cell carcinoma, clear cell myoepithelial carcinoma, and epithelial-myoepithelial carcinoma.
abstract: BACKGROUND
  Primary salivary clear cell tumors comprise an uncommonly encountered subgroup of salivary neoplasia. We hypothesize that clear cell carcinoma does not represent a "monomorphic" variant of epithelial-myoepithelial carcinoma, but is distinct in terms of histogenesis and tumor biology.
  
  
  OBJECTIVE
  To compare the clinicopathologic features of 20 cases of salivary primary clear cell tumors, including 12 clear cell carcinomas (CCCs), 7 epithelial-myoepithelial carcinomas (EMECs), and 1 clear cell myoepithelial carcinoma (CCMEC); to investigate their interrelationship with regard to myoepithelial differentiation; and to offer a diagnostic approach for distinguishing between these entities.
  
  
  METHODS
  Retrospective and prospective identification and review of patients diagnosed with primary salivary clear cell neoplasia and review of the English language literature.
  
  
  METHODS
  Three academic tertiary-care hospitals.
  
  
  METHODS
  We identified 12 patients with CCC, 7 with EMEC, and 1 with CCMEC. Patients included 11 men and 9 women, aged 30 to 88 years (median 72.5 years).
  
  
  METHODS
  Immunohistochemical reactivity for S100, muscle-specific actin, and calponin; ultrastructural examination when feasible; review of patient charts; and telephone interviews to establish clinical outcome.
  
  
  RESULTS
  Clear cell carcinoma has a predilection for intraoral sites, whereas EMEC has a predilection for the parotid. All 3 of the tumor types studied have a propensity for locoregional recurrence, which can manifest decades after initial surgery. There were no mortalities among patients with CCC, even after pulmonary metastasis in 2 patients, confirming the indolent nature of this group of clear cell tumors. A meta-analysis of reported cases of CCC, EMEC, and CCMEC confirmed that EMEC and CCMEC have a much greater propensity for locoregional recurrence than CCC, despite the predilection of both for a more surgically accessible site (parotid). We found no definitive evidence of myoepithelial differentiation in CCC, indicating that it is probably morphogenetically distinct from EMEC and CCMEC, both tumors with diagnostically requisite myoepithelial differentiation.
  
  
  CONCLUSIONS
  The initial treatment of choice for CCC, CCMEC, and EMEC is surgical resection with negative margins. Locoregional recurrence should be treated aggressively, as it is still consistent with long disease-free intervals. The lack of myoepithelial differentiation in CCC is consistent with the concept that this tumor is histomorphogenically distinct from EMEC and that it is not merely a monomorphic variant.
authors.affiliation: Lillian and Henry M. Stratton-Hans Popper Department of Pathology, The Mount Sinai School of Medicine, New York, NY 10021, USA.
authors.forename: Beverly
authors.initials: B
authors.lastname: Wang
authors.forename: Margaret
authors.initials: M
authors.lastname: Brandwein
authors.forename: Ronald
authors.initials: R
authors.lastname: Gordon
authors.forename: Robert
authors.initials: R
authors.lastname: Robinson
authors.forename: Mark
authors.initials: M
authors.lastname: Urken
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Zarbo
mesh_terms: D018262:Adenocarcinoma, Clear Cell / Q000737:chemistry / Q000175:diagnosis* / Q000556:secondary; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D015201:Meta-Analysis as Topic; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000032:analysis; D009364:Neoplasm Recurrence, Local; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000737:chemistry / Q000145:classification / Q000175:diagnosis*
chemical_list: D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins