This article has Open Peer Review reports available.
Primary myoepithelial carcinoma of the lung: a rare entity treated with parenchymal sparing resection
© Sarkaria et al; licensee BioMed Central Ltd. 2011
Received: 21 October 2010
Accepted: 8 March 2011
Published: 8 March 2011
Primary lung myoepithelial carcinomas are rare neoplasms arising from the salivary glands of the respiratory epithelium. Given the rare occurrences and reports of these tumors, appropriate recommendations for resection are difficult to formulate. Although classified as low-grade neoplasms, these tumors have a significant rate of recurrence and distant metastasis.
Primary salivary gland-type tumors of the lung are rare and include mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma, oncocytoma, epithelial-myoepithelial carcinoma, benign myoepithelioma, and mixed tumors of both benign and malignant nature [1–16]. Primary pulmonary myoepithelial carcinomas are exceedingly rare, with only five known prior cases reported in the English literature to date [17–20]. We report a case of primary myoepithelial carcinoma of the lung and a review of the literature.
The patient developed a biopsy proven solitary liver/diaphragmatic metastasis diagnosed on routine follow-up at 36 months post-resection.
Gross pathologic examination revealed the mass to be a 13 × 8 × 8 centimeter lower lobe carcinoma of myoepithelial origin involving the visceral pleura (Figure 1B).
Histologically, the mass was consistent with a malignant myoepithelial neoplasm with a fibrous capsule and 20% necrosis. The mass was thought to arise from the right lower lobe and involved the visceral pleura. The margin was focally within 0.1 cm of the tumor, but otherwise grossly free of invasion. The separate nodule was 1 cm in size and histologically similar to the primary tumor.
Immunohistochemical stains were performed and were focally positive for AE1/AE3, Bcl2, Cam5.2, S100p, GFAP, 4A4, SMA, and CD99. Stains for EMA, desmin, CD34, calponin, FLI1, myogenin, and synaptophysin were negative.
Myoepithelial carcinomas primarily arise from the salivary glands, the parotid, or the breast . Rarely, they may arise in soft tissues, most often in the lower and upper limbs, occurring equally in men and women . These soft-tissue tumors, distinguished from benign myoepitheliomas by their moderate or severe cytologic atypia or invasive growth pattern, recur locally in 42% of patients and metastasize to distant sites in 32%.
Clinical characteristics of reported cases of myoepithelial carcinoma of the lung.
Pre-op Bx Diagnosis
RLL, Pleural & parenchymal
Low grade spindle cell neoplasm
Yes, same lobe/pleura at time of resection, liver at 36 months
AWD at 36 months
LLL, Parenchymal & Endobronchial
Sarcoma, sarcomatoid carcinoma, carcinosarcoma
NED 15 months
Right hilum, Right Main Stem, Endobronchial
No atypical cells
AWD at 7 months
Sleeve bilobectomy (RUL/RML)
Yes, soft tissue left arm and hip
DOC at 14 months (metastatic synchronous adenocarcinoma)
Squamous cell carcinoma
Sleeve lobectomy (LUL)
DOD, 60 months
Given the available reported data, there are a number of unique characteristics of the current case when compared to the previous five. This case represents the eldest and only female patient, as well as the only known never-smoker. This patient's tumor is also the greatest in size within this series, more than doubling the previous known largest of these tumors. All other patients in the series presented with a major component of endobronchial disease, whereas the current tumor was primarily pleural/parenchymal based. Finally, the current case represents the only patient treated with a limited sub-anatomic resection.
Given the relatively high rates of recurrence, low-grade malignant status, and the propensity for recurrence at distant sites, it is reasonable to consider limited sub-anatomic, parenchymal sparing resections for these patients, especially if pneumonectomy is contemplated. While this may not be adequate for endobronchial lesions involving major pulmonary segments, it is feasible for lesions presenting with primarily parenchymal or pleural based disease, as in the current case. Given the rarity of these tumors, recommendations regarding chemotherapy or radiation, either pre- or postoperatively, are difficult to formulate.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Doganay L, Bilgi S, Ozdil A, Yoruk Y, Altaner S, Kutlu K: Epithelial-myoepithelial carcinoma of the lung. A case report and review of the literature. Arch Pathol Lab Med. 2003, 127: e177-180.PubMedGoogle Scholar
- Cagirici U, Sayiner A, Inci I, Veral A: Myoepithelioma of the lung. Eur J Cardiothorac Surg. 2000, 17: 187-189. 10.1016/S1010-7940(00)00338-9.View ArticlePubMedGoogle Scholar
- Fulford LG, Kamata Y, Okudera K, Dawson A, Corrin B, Sheppard MN, Ibrahim NB, Nicholson AG: Epithelial-myoepithelial carcinomas of the bronchus. Am J Surg Pathol. 2001, 25: 1508-1514. 10.1097/00000478-200112000-00006.View ArticlePubMedGoogle Scholar
- Cho KJ, el-Naggar AK, Ordonez NG, Luna MA, Austin J, Batsakis JG: Epithelial-myoepithelial carcinoma of salivary glands. A clinicopathologic, DNA flow cytometric, and immunohistochemical study of Ki-67 and HER-2/neu oncogene. Am J Clin Pathol. 1995, 103: 432-437.View ArticlePubMedGoogle Scholar
- Davis PW, Briggs JC, Seal RM, Storring FK: Benign and malignant mixed tumours of the lung. Thorax. 1972, 27: 657-673. 10.1136/thx.27.6.657.View ArticlePubMedPubMed CentralGoogle Scholar
- Fechner RE, Bentinck BR, Askew JB: Acinic cell tumor of the lung. A histologic and ultrastructural study. Cancer. 1972, 29: 501-508. 10.1002/1097-0142(197202)29:2<501::AID-CNCR2820290241>3.0.CO;2-B.View ArticlePubMedGoogle Scholar
- Horinouchi H, Ishihara T, Kawamura M, Kato R, Kikuchi K, Kobayashi K, Maenaka Y, Torikata C: Epithelial myoepithelial tumour of the tracheal gland. J Clin Pathol. 1993, 46: 185-187. 10.1136/jcp.46.2.185.View ArticlePubMedPubMed CentralGoogle Scholar
- Moran CA, Suster S, Askin FB, Koss MN: Benign and malignant salivary gland-type mixed tumors of the lung. Clinicopathologic and immunohistochemical study of eight cases. Cancer. 1994, 73: 2481-2490. 10.1002/1097-0142(19940515)73:10<2481::AID-CNCR2820731006>3.0.CO;2-A.View ArticlePubMedGoogle Scholar
- Nistal M, Garcia-Viera M, Martinez-Garcia C, Paniagua R: Epithelial-myoepithelial tumor of the bronchus. Am J Surg Pathol. 1994, 18: 421-425. 10.1097/00000478-199404000-00010.View ArticlePubMedGoogle Scholar
- Pelosi G, Fraggetta F, Maffini F, Solli P, Cavallon A, Viale G: Pulmonary epithelial-myoepithelial tumor of unproven malignant potential: report of a case and review of the literature. Mod Pathol. 2001, 14: 521-526. 10.1038/modpathol.3880341.View ArticlePubMedGoogle Scholar
- Ryska A, Kerekes Z, Hovorkova E, Barton P: Epithelial-myoepithelial carcinoma of the bronchus. Pathol Res Pract. 1998, 194: 431-435. discussion 436-438View ArticlePubMedGoogle Scholar
- Santos-Briz A, Terron J, Sastre R, Romero L, Valle A: Oncocytoma of the lung. Cancer. 1977, 40: 1330-1336. 10.1002/1097-0142(197709)40:3<1330::AID-CNCR2820400350>3.0.CO;2-K.View ArticlePubMedGoogle Scholar
- Spencer H: Bronchial mucous gland tumours. Virchows Arch A Pathol Anat Histol. 1979, 383: 101-115. 10.1007/BF00427013.View ArticlePubMedGoogle Scholar
- Strickler JG, Hegstrom J, Thomas MJ, Yousem SA: Myoepithelioma of the lung. Arch Pathol Lab Med. 1987, 111: 1082-1085.PubMedGoogle Scholar
- Tsuji N, Tateishi R, Ishiguro S, Terao T, Higashiyama M: Adenomyoepithelioma of the lung. Am J Surg Pathol. 1995, 19: 956-962. 10.1097/00000478-199508000-00012.View ArticlePubMedGoogle Scholar
- Wilson RW, Moran CA: Epithelial-myoepithelial carcinoma of the lung: immunohistochemical and ultrastructural observations and review of the literature. Hum Pathol. 1997, 28: 631-635. 10.1016/S0046-8177(97)90088-5.View ArticlePubMedGoogle Scholar
- Higashiyama M, Kodama K, Yokouchi H, Takami K, Kabuto T, Tsuji N, Mano M, Ishiguro S, Ueda T, Yoshikawa H, Tatsuta M: Myoepithelioma of the lung: report of two cases and review of the literature. Lung Cancer. 1998, 20: 47-56. 10.1016/S0169-5002(98)00006-3.View ArticlePubMedGoogle Scholar
- Masuya D, Haba R, Huang CL, Yokomise H: Myoepithelial carcinoma of the lung. Eur J Cardiothorac Surg. 2005, 28: 775-777. 10.1016/j.ejcts.2005.08.003.View ArticlePubMedGoogle Scholar
- Miura K, Harada H, Aiba S, Tsutsui Y: Myoepithelial carcinoma of the lung arising from bronchial submucosa. Am J Surg Pathol. 2000, 24: 1300-1304. 10.1097/00000478-200009000-00016.View ArticlePubMedGoogle Scholar
- Sekine I, Kodama T, Yokose T, Nishiwaki Y, Suzuki K, Goto K, Nagai K, Kuriyama T: Rare pulmonary tumors - a review of 32 cases. Oncology. 1998, 55: 431-434. 10.1159/000011891.View ArticlePubMedGoogle Scholar
- Colby TV, Koss MN, Travis WD: Tumors of salivary gland type. Atlas of tumor pathology Tumors of the lower respiratory tract. Edited by: Colby TV, Koss MN, travis WD. 1995, Washington, D.C.: Armed Forces Institute of Pathology, 65-89.Google Scholar
- Hornick JL, Fletcher CD: Myoepithelial tumors of soft tissue: a clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters. Am J Surg Pathol. 2003, 27: 1183-1196. 10.1097/00000478-200309000-00001.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. 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.