Title: Primary Pulmonary Epithelial- Myoepithelial Tumour - Report of an Aggressive Clinical Case
Authors: Dr Geethi M H, Dr Sajeed Abdul Rahuman, Dr Anila.K.R
DOI: http://dx.doi.org/10.18535/jmscr/v3i10.22
Abstract
Primary epithelial-myoepithelial tumor is usually a salivary gland neoplasm and extremely rare in the respiratory tract. These tumors are thought to arise from tracheo-bronchial submucosal glands. They usually present as early lesions due to the endobronchial location and have a benign course after surgical resection. In literature about twenty five cases have been reported so far and almost all of them had small tumors with a mean size of 2.5 cm with good prognosis after radical resection. We report a case with large unresectable primary pulmonary epithelial-myoepithelial tumour. The patient was a fifty eight year old gentleman who presented with a three week history of cough, chest pain, and breathlessness. CT scan of thorax showed a large heterogeneous mass lesion occupying upper right hemithorax infiltrating the great vessels. Trucut biopsy showed a neoplasm composed of cells arranged in diffuse sheets and glandular pattern with interspersed hyalinised material. Immunohistochemistry showed epithelial cells strongly positive for cytokeratin and myoepithelial cells positive for p63. There was myoepithelial cell predominance and MIB 1 index was 7/10 HPF. Our case had an aggressive clinical course with poor response to chemotherapy and expired after nine months of diagnosis due to progressive disease. None of the reported cases had an extensive tumour with aggressive clinical behaviour as in our case.
Key words: Primary epithelial-myoepithelial tumor, aggressive
References
1. Fonseca and J. Soares. “Epithelial myoepithelial carcinoma,”in World Health Organization Classification of Tumors, Pathology and Genetics of Head and Neck Tumors. L. Barnes, J. W. Eveson, P. Reichart, and D. Sidransky, Eds. pp. 225–226, IARC Press, Lyon, France, 2005
2. G. Munoz, F. Felipo, I. Marquina, and C. Del Agua. Epithelial-myoepithelial tumour of the lung: a case report referring to its molecular histogenesis. Diagnostic Pathology, vol. 6, article 71, 2011.
3. Donath K, Seifert G, Schmitz R. Diagnosis and ultrastructure of the tubular carcinoma of salivary gland ducts: epithelial-myoepithelial carcinoma of the intercalated ducts [in German]. Virchows Arch A Pathol Pathol Anat. 1972; 356:16–31.
4. Moran CA. Primary salivary gland-type tumors of the lung. Semin Diagn Pathol. 1995.12:106-22.
5. Farzana Arif, Susan Wu, Shahriyour Andaz, and Stewart Fox. “Primary Epithelial Myoepithelial Carcinoma of Lung, Reporting of a Rare Entity, Its Molecular Histogenesis and Review of the Literature,” Case Reports in Pathology. vol. 2012, Article ID 319434, 6 pages, 2012. doi:10.1155/2012/319434.
6. L. G. Fulford, Y. Kamata, K. Okudera et al. “Epithelial myoepithelial carcinomas of the bronchus,” The American Journal of Surgical Pathology. vol. 25, no. 12, pp. 1508–1514,2001.
7. Ru Kun, Srivastava A, Tischler, AS. Bronchial epithelial myoepithelial carcinoma. Arch Pathol Lab Med. 2004.128:92–4.
8. Wilson RW, Moran CA. Epithelial-myoepithelial carcinoma of the lung: immunohistochemical and ultrastructural observations and review of the literature. Hum Pathol. 1997;28:631–5.
9. Doganay L., Bilgi S., Ozdil A., Yoruk Y., Altaner S., Kutlu K. Epithelial-myoepithelial carcinoma of the lung: a case report and review of literature. Arch Pathol Lab Med. 2003;127(4):177–180.
10. C. V. Nguyen, S. Suster, and C. A. Moran, “Pulmonary epithelial-myoepithelial carcinoma: a clinicopathologic and immunohistochemical study of 5 cases,” Human Pathology, vol. 40, no. 3, pp. 366–373, 2009.