Hyalinizing clear cell carcinoma
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| name = Hyalinizing clear cell carcinoma
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| image = Hyalinizing_clear_cell_carcinoma_-_high_mag.jpg
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| caption = Micrograph of a hyalinizing clear cell carcinoma showing the characteristic clear cells and surrounding hyalinized stroma. H&E stain.
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| field = Oncology, ENT surgery
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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.{{cite journal | vauthors = Kauzman A, Tabet JC, Stiharu TI | title = Hyalinizing clear cell carcinoma: a case report and review of the literature | journal = Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics | volume = 112 | issue = 1 | pages = e26–e34 | date = July 2011 | pmid = 21669357 | doi = 10.1016/j.tripleo.2011.02.041 }}
Signs and symptoms
HCCCs typically present as a painless mass in the mouth.{{cite journal | vauthors = Milchgrub S, Gnepp DR, Vuitch F, Delgado R, Albores-Saavedra J | title = Hyalinizing clear cell carcinoma of salivary gland | journal = The American Journal of Surgical Pathology | volume = 18 | issue = 1 | pages = 74–82 | date = January 1994 | pmid = 7506496 | doi = 10.1097/00000478-199401000-00007 | s2cid = 30519518 }}
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.{{cite journal | vauthors = O'Sullivan-Mejia ED, Massey HD, Faquin WC, Powers CN | title = Hyalinizing clear cell carcinoma: report of eight cases and a review of literature | journal = Head and Neck Pathology | volume = 3 | issue = 3 | pages = 179–185 | date = September 2009 | pmid = 20596970 | pmc = 2811632 | doi = 10.1007/s12105-009-0124-3 }} Nuclear pleomorphism is usually minimal and mitoses are infrequently seen.
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.{{cite journal | vauthors = Antonescu CR, Katabi N, Zhang L, Sung YS, Seethala RR, Jordan RC, Perez-Ordoñez B, Have C, Asa SL, Leong IT, Bradley G, Klieb H, Weinreb I | display-authors = 6 | title = EWSR1-ATF1 fusion is a novel and consistent finding in hyalinizing clear-cell carcinoma of salivary gland | journal = Genes, Chromosomes & Cancer | volume = 50 | issue = 7 | pages = 559–570 | date = July 2011 | pmid = 21484932 | doi = 10.1002/gcc.20881 | s2cid = 20457515 | doi-access = free }} The same translocation is seen in 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.
Image: Hyalinizing clear cell carcinoma - intermed mag.jpg | Intermed. mag.
Image: Hyalinizing clear cell carcinoma - very high mag.jpg | Very high mag.
Prognosis
See also
- {{annotated link|Clear cell renal cell carcinoma}}
References
{{Reflist}}
{{Tumors of lip, oral cavity and pharynx}}