CASE REPORT Hyalinizing clear cell carcinoma of salivary gland: an aggressive variant E. O’Regan a , M. Shandilya b , D.R. Gnepp c , C. Timon b , M. Toner a, * a Department of Oral Surgery, Oral Medicine and Oral Pathology, Dublin Dental School and Hospital, Dublin, Ireland b Department of Otolaryngology and Head and Neck Surgery, St. James’s Hospital, Dublin, Ireland c Department of Pathology, Brown University School of Medicine, Rhode Island Hospital, Rhode Island, USA Received 29 July 2003; accepted 28 August 2003 Summary Although hyalinizing clear cell carcinoma (HCCC) had been previously illustrated by several authors, it was not until 1994 that this tumour was characterized by Milchgrub et al. [Am J Surg Pathol (1994),18,74] and separated from the hetero- geneous group of clear cell carcinomas described in the literature. HCCC is a distinctive infiltrative low-grade, monomorphic, glycogen-rich clear cell carcinoma with prominent stromal hyalinization occurring most often in the minor salivary glands of adult women. A case of hyalinizing clear cell carcinoma arising in the tongue of an adult female is described with special reference to the presence of minor foci of mitotic activity, necrosis and anaplasia in this otherwise typical low-grade carcinoma. Widespread metastases and death within a year of initial presentation in this case suggests that there may be a subset of this indolent tumour in which these features are associated with a poor prognosis. # 2003 Elsevier Ltd. All rights reserved. KEYWORDS Salivary gland; Hyalinizing clear cell carcinoma; Aggressive variant 1. Case report In December 2000, a 57 year-old female pre- sented to the Ear Nose and Throat Department in St. James’s Hospital, Dublin, Ireland, with a 4- month history of severe pain in her right ear and throat along with profound anaesthesia of the right side of her tongue. Coinciding with this history of pain, the patient had a rash affecting her ear and neck on that side, diagnosed as shingles. On examination, there was a large ulcerated mass at the base of her tongue with a palpable cervical lymph node on the right side. A CT scan of the region at this stage revealed a mass in the right side of the tongue extending into the right para- pharyngeal space, along with an enlarged lymph node in the right side of her neck. A chest X-ray at this stage showed no evidence of lung metastases. A biopsy of the tongue lesion revealed nests of cells with bland uniform nuclei, mostly with clear cytoplasm and a minor component with eosino- philic cytoplasm associated with a densely hyali- nized stroma. The appearances, on biopsy, were typical of a hyalinizing clear cell carcinoma (HCCC). The patient underwent a near total glossectomy, along with a right modified radical neck dissection, 1368-8375/$ - see front matter # 2003 Elsevier Ltd. All rights reserved. doi:10.1016/j.oraloncology.2003.08.023 Oral Oncology (2004) 40 348–352 www.elsevier.com/locate/oraloncology * Corresponding author.