Medical condition
Hyalinizing clear cell carcinoma (HCCC ) is a rare malignant salivary gland tumour , with a good prognosis, that is usually found on the tongue or palate .[ 1]
Signs and symptoms
HCCCs typically present as a painless mass in the mouth .[ 2]
Diagnosis
HCCCs are diagnosed by examination of tissue, e.g. a biopsy .
Pathology
HCCC consist of cells with abundant clear cytoplasm , arranged in cords, trabeculae or clusters in a hyalinized stroma .[ 3] Nuclear pleomorphism is usually minimal and mitoses are infrequently seen.[ 2]
Owing to their glycogen content, which explains the "clear" appearance under the microscope , tumour cells stain with PAS . Immunostains for S100 and smooth muscle actin (SMA) are typically negative, but positive for cytokeratins and epithelial membrane antigen (EMA).
HCCCs typically have a recurrent chromosomal translocation , t(12;22), involving the genes EWSR1 and ATF1 .[ 4] clear cell sarcoma .
The histologic differential diagnosis includes mucoepidermoid carcinoma (clear cell variant), acinic cell carcinoma (clear cell variant), epithelial-myoepithelial carcinoma and metastatic clear cell carcinoma .
Intermed. mag.
Very high mag.
Prognosis
They generally have a good prognosis.[ 5]
See also
References
^ Kauzman A, Tabet JC, Stiharu TI (July 2011). "Hyalinizing clear cell carcinoma: a case report and review of the literature". Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics . 112 (1): e26 – e34 . doi :10.1016/j.tripleo.2011.02.041 . PMID 21669357 . ^ a b Milchgrub S, Gnepp DR, Vuitch F, Delgado R, Albores-Saavedra J (January 1994). "Hyalinizing clear cell carcinoma of salivary gland". The American Journal of Surgical Pathology . 18 (1): 74– 82. doi :10.1097/00000478-199401000-00007 . PMID 7506496 . S2CID 30519518 . ^ O'Sullivan-Mejia ED, Massey HD, Faquin WC, Powers CN (September 2009). "Hyalinizing clear cell carcinoma: report of eight cases and a review of literature" . Head and Neck Pathology . 3 (3): 179– 185. doi :10.1007/s12105-009-0124-3 . PMC 2811632 PMID 20596970 . ^ Antonescu CR, Katabi N, Zhang L, Sung YS, Seethala RR, Jordan RC, et al. (July 2011). "EWSR1-ATF1 fusion is a novel and consistent finding in hyalinizing clear-cell carcinoma of salivary gland" . Genes, Chromosomes & Cancer . 50 (7): 559– 570. doi :10.1002/gcc.20881 PMID 21484932 . S2CID 20457515 . ^ Masilamani S, Rao S, Chirakkal P, Kumar AR (2011). "Hyalinizing clear cell carcinoma of the base of tongue: a distinct and rare entity" . Indian Journal of Pathology & Microbiology . 54 (1): 167– 169. doi :10.4103/0377-4929.77393 PMID 21393908 .
