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      Carcinoma ex pleomorphic adenoma of soft palate with cavernous sinus invasion

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          Abstract

          Background

          Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive salivary gland malignancy and rare in minor salivary gland. A soft palate CXPA initially presenting as direct cavernous sinus (CS) invasion is very rare.

          Case Presentation

          A 60-year-old male had a 3-month history of a small soft palatal mass with progressing left cheek numbness, proptosis, and disturbed vision. Biopsy of soft palatal tumor showed pleomorphic adenoma. Magnetic resonance imaging showed a tumor involving left maxilla, and extended from pterygopalatine fossa, inferior orbital fissure to CS. Excision of tumor revealed CXPA. Adjuvant concomitant chemo-radiation therapy (CCRT) was given. The tumor recurred 5 months later in left CS which was re-treated with CCRT. The disease status was stable at 2 years after the diagnosis of CXPA.

          Conclusion

          We present this case to emphasize that patients with symptoms such as facial numbness, proptosis and disturbed vision should be carefully investigated for lesions invading CS by perineural spread.

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          Most cited references14

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          Carcinoma ex pleomorphic adenoma: a clinicopathologic review.

          Helen J Lewis, K Olsen (2001)
          Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive, poorly understood salivary gland malignancy. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtypes are possible. We retrospectively reviewed the medical records of 73 patients with major salivary gland CXPA treated at our institution from 1960 to 1994. Of the 73 patients, 66 had primary tumors and 7 had recurrent tumors; 47 were men, and 26 were women; the mean age was 61 years. Adenocarcinoma (31 cases) and salivary duct carcinoma (24 cases) were the most common malignant subtypes. All patients were treated surgically, and 32 also had radiation therapy. Of 66 patients with primary tumors, 23% had local recurrence. Metastasis (either initial or delayed) occurred regionally in 56% and distantly in 44%. Thirty-six patients (55%) died of the disease. At 3 years, overall survival was 39% and at 5 years, 30%. Important prognostic factors include tumor size, grade, and clinical and pathologic stage. Patients with minimally invasive tumors (<5 mm) should do well with appropriate surgical treatment. Copyright 2001 John Wiley & Sons, Inc.
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            Carcinoma ex pleomorphic adenoma: diagnostic difficulty and outcome.

            Sibylle Zbären, Edouard Stauffer, P Zbären … (2008)
            To analyze a series of carcinoma ex pleomorphic adenoma (CXPA) and to assess the diagnostic difficulties. The clinical presentation of 24 CXPAs was compared with 300 pleomorphic adenomas (PAs). Furthermore, pathohistological findings and follow-up results of CXPAs were evaluated. Eight of 24 (33%) CXPAs versus 41 of 300 (14%) PAs were localized in the deep lobe (P < 0.05). Forty-two percent of CXPAs versus 6 percent of PAs, respectively, were greater than 4 cm (P < 0.05). The sensitivity in detecting CXPA by fine-needle aspiration cytology (FNAC) was 47 percent. The tumor was known to be malignant preoperatively in 10 (42%) patients. Six of 24 (25%) patients with CXPA developed a tumor recurrence. The overall 5-year survival rate of CXPA was 76 percent. CXPAs are difficult to identify preoperatively. FNAC has a low accuracy and sensitivity. CXPAs versus PAs are significantly more frequently localized in the deep lobe and are significantly greater in size.
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              Perineural spread of head and neck tumors: how accurate is MR imaging?

              Steven P Donald, Stephen S. Hecht, K McKennan … (1998)
              Our aim was to determine the precision of MR imaging evaluation of perineural spread of head and neck tumors. Nineteen patients had complete extirpation of head and neck tumors (10 squamous cell carcinomas, four adenoid cystic carcinomas, one poorly differentiated carcinoma, one salivary duct carcinoma, one mucoepidermoid carcinoma, one chordoma, and one meningioma) with histologic confirmation of perineural spread. Findings at presurgical contrast-enhanced MR imaging were compared with findings at pathologic examination. The sensitivity of MR imaging for detection of perineural spread was 95%; however, the sensitivity for mapping the entire extent of perineural spread fell to 63%. MR imaging may fail to depict microscopic foci of perineural tumor infiltration, leading to underestimation of the extent of perineural spread. Nevertheless, with careful analysis of foraminal architecture and MR enhancement patterns, one can reliably identify the presence if not the extent of perineural spread.
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                Author and article information

                Journal
                Journal ID (nlm-ta): World J Surg Oncol
                Title: World Journal of Surgical Oncology
                Publisher: BioMed Central
                ISSN (Electronic): 1477-7819
                Publication date Collection: 2010
                Publication date (Electronic): 30 March 2010
                Volume: 8
                Page: 24
                Affiliations
                [1 ]Department of Otolaryngology, Chang Gung Memorial Hospital and Chang Gung University, Taiwan
                [2 ]Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Taiwan
                [3 ]Department of Radiology, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan
                Article
                Publisher ID: 1477-7819-8-24
                DOI: 10.1186/1477-7819-8-24
                PMC ID: 2860350
                PubMed ID: 20353573
                SO-VID: 9aa200bf-cc3c-41e2-a41f-f147ef54b04a
                Copyright © Copyright ©2010 Chen et al; licensee BioMed Central Ltd.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 7 January 2010
                Date accepted : 30 March 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Surgery
                Data availability:
                ScienceOpen disciplines: Surgery

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