title: Clear cell colorectal carcinoma: Time to clarify diagnosis.
abstract: Primary clear cell colorectal carcinoma (CCC) is a very rare entity accounting for only 35 cases reported in the Literature. CCC is neither classified as a distinct entity nor is it defined as a CRC variant because its ontogeny remains unclear. Most of the reported CCC were found in the distal colon in patients with a mean age of 56 years. Histologically, clear cell change is the main morphologic feature and may present in a "pure" form, composed exclusively of clear cells, or in a "composite" form, admixed with other morphologically different components. It is possible to distinguish two biologically different types of CCC, with different clinical-pathologic features, therapeutic management and diagnostic criteria: a) Intestinal CCC consisting of an aggressive neoplasm, affecting mainly adult men, characterized by an intestinal-type immunoprofile (CK20+, CK7-, CEA+, CDX-2+) and b) Müllerian CCC consisting of an indolent carcinoma of the sigmoid-rectum, affecting young women, characterized by a different (CK7+, CK20-, CEA-, CA125 +) immunoprofile. Considerable diagnostic difficulties can arise in distinguishing CCC and primary or secondary clear cell neoplasms, such as metastases from renal carcinoma, lower urinary tract, female genital tract, adrenal gland, mesothelioma, melanoma and primary intestinal PEComa. In this paper we review the Literature with two additional cases in order to define the diagnostic criteria of CCC.
authors.affiliation: Pathology Unit, Hospital "Mater Salutis" Legnago (VR), Italy. Electronic address: remino76@yahoo.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Remo
authors.affiliation: Department of Surgical and Diagnostic Sciences (DISC), Pathology Unit, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Grillo
authors.affiliation: Department of Surgical and Diagnostic Sciences (DISC), Pathology Unit, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Mastracci
authors.affiliation: Department of Medicine (DIMED), University of Padua, PD, Italy.
authors.forename: Matteo
authors.initials: M
authors.lastname: Fassan
authors.affiliation: Department of Diagnostics and Public Health, University and Hospital Trust of Verona, ARC-Net Research Center, Verona, Italy.
authors.forename: Sokol
authors.initials: S
authors.lastname: Sina
authors.affiliation: Pathology Unit, Hospital "Mater Salutis" Legnago (VR), Italy.
authors.forename: Caterina
authors.initials: C
authors.lastname: Zanella
authors.affiliation: Department of Diagnostics and Public Health, University and Hospital Trust of Verona, ARC-Net Research Center, Verona, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Parcesepe
authors.affiliation: Istituto Oncologico Veneto, IOV-IRCCS, Padua, PD, Italy.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Damiano Urso
authors.affiliation: Department of Sciences and Technologies, University of Sannio, Via Port'Arsa, 11, 82100, Benevento, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Pancione
authors.affiliation: Pathology Unit, Hospital "Mater Salutis" Legnago (VR), Italy.
authors.forename: Germana
authors.initials: G
authors.lastname: Bortuzzo
authors.affiliation: Department of Diagnostics and Public Health, University and Hospital Trust of Verona, ARC-Net Research Center, Verona, Italy.
authors.forename: Aldo
authors.initials: A
authors.lastname: Scarpa
authors.affiliation: Department of Diagnostics and Public Health, University and Hospital Trust of Verona, ARC-Net Research Center, Verona, Italy.
authors.forename: Erminia
authors.initials: E
authors.lastname: Manfrin
mesh_terms: D018262:Adenocarcinoma, Clear Cell / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism*; D015179:Colorectal Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D014408:Biomarkers, Tumor
keywords: Clear cell carcinoma; Colorectal carcinoma; Endometriosis