Hyalinizing clear cell carcinoma

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Hyalinizing clear cell carcinoma
Diagnosis in short

Hyalinizing clear cell carcinoma. H&E stain.

LM cells with abundant clear cytoplasm in cords, trabeculae or clusters; minimal nuclear pleomorphism; hyalinized stroma
LM DDx mucoepidermoid carcinoma (clear cell variant), acinic cell carcinoma (clear cell variant), metastatic clear cell renal cell carcinoma, epithelial-myoepithelial carcinoma, other clear cell tumours
Stains PAS +ve
IHC AE1/AE3 +ve, EMA +ve.
Molecular t(12;22) -- EWSR1/ATF1
Site usu. palate or tongue

Prevalence rare
Prognosis good

Hyalinizing clear cell carcinoma, abbreviated HCCC, is a rare malignant head and neck tumour with a good prognosis.

General

  • Rare.
  • Good prognosis.[1]
  • Typically palate or tongue.[2]

Microscopic

Features:[3]

  • Groups of cells with abundant clear cytoplasm.
  • Hyalinized stroma.

Notes:

  • Clear cytoplasm due to glycogen.[4]
  • Low mitotic rate.
  • Should not have epidermoid or mucinous differentiation - seen in clear cell variant of mucoepidermoid carcinoma.

DDx:

Images

www:

Stains

  • PAS +ve.

IHC

  • AE1/AE3 +ve.
  • EMA +ve.[4]

Others:

  • Desmin -ve.
  • SMA -ve.[4]
  • S-100 -ve.[4]

Molecular

Recurrent translocation:[5]

See also

References

  1. Masilamani, S.; Rao, S.; Chirakkal, P.; Kumar, AR.. "Hyalinizing clear cell carcinoma of the base of tongue: a distinct and rare entity.". Indian J Pathol Microbiol 54 (1): 167-9. doi:10.4103/0377-4929.77393. PMID 21393908.
  2. Kauzman, A.; Tabet, JC.; Stiharu, TI. (Jul 2011). "Hyalinizing clear cell carcinoma: a case report and review of the literature.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 112 (1): e26-34. doi:10.1016/j.tripleo.2011.02.041. PMID 21669357.
  3. 3.0 3.1 O'Sullivan-Mejia, ED.; Massey, HD.; Faquin, WC.; Powers, CN. (Sep 2009). "Hyalinizing clear cell carcinoma: report of eight cases and a review of literature.". Head Neck Pathol 3 (3): 179-85. doi:10.1007/s12105-009-0124-3. PMID 20596970.
  4. 4.0 4.1 4.2 4.3 4.4 Milchgrub, S.; Gnepp, DR.; Vuitch, F.; Delgado, R.; Albores-Saavedra, J. (Jan 1994). "Hyalinizing clear cell carcinoma of salivary gland.". Am J Surg Pathol 18 (1): 74-82. PMID 7506496.
  5. Antonescu, CR.; Katabi, N.; Zhang, L.; Sung, YS.; Seethala, RR.; Jordan, RC.; Perez-Ordoñez, B.; Have, C. et al. (Jul 2011). "EWSR1-ATF1 fusion is a novel and consistent finding in hyalinizing clear-cell carcinoma of salivary gland.". Genes Chromosomes Cancer 50 (7): 559-70. doi:10.1002/gcc.20881. PMID 21484932.