Epithelial-myoepithelial carcinoma of the lung

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Epithelial-myoepithelial carcinoma of the lung
Other namesEMECL Adenomyoepithelioma, [1] Myoepithelioma, Epithelial-myoepithelial tumor, Epimyoepithelial carcinoma, Malignant mixed tumor containing epithelial and myoepithelial cells. [2]
Specialty Oncology

Epithelial-myoepithelial carcinoma of the lung is a very rare histologic form of malignant epithelial neoplasm ("carcinoma") arising from lung tissue. [2]

Contents

Genetics

The epithelial component in EMECL's typically strongly express cytokeratins, but are negative for actin and S-100 protein, while the myoepithelial component stains strongly for actin and S-100 protein, and only focally weakly for cytokeratins. [3]

Diagnosis

Microscopically, EMECL features bi-layered glandular or ductular structures consisting of inner cuboidal cells and outer multipolar cells. [4]

The histologic appearance of these tumors varied, but all shared the common feature of a biphasic proliferation of epithelial (strong cytokeratin-positive; actin and S-100-negative) and myoepithelial (strong actin and S-100 and focal weak cytokeratin-positive) cells with formation of bilayered ductlike structures. The focal resemblance to other salivary gland-type tumors may cause diagnostic difficulties, particularly in small endobronchial biopsies. Although little is known about their biologic potential due to limited follow-up data, these tumors when in the lung clearly have the capacity to infiltrate and metastasize and therefore should be designated as epithelial-myoepithelial carcinoma. At present, it appears that treatment by complete surgical resection with negative margins alone is appropriate and adequate. [3]

Classification

Lung cancer is an extremely heterogeneous family of malignant neoplasms, [5] with well over 50 different histological variants recognized under the 4th revision of the World Health Organization (WHO) typing system ("WHO-2004"), [2] currently the most widely used lung cancer classification scheme. Because these variants have differing genetic, biological, and clinical properties, including response to treatment, correct classification of lung cancer cases are necessary to assure that lung cancer patients receive optimum management. [2] [6] [7]

The WHO-2004 scheme groups lung carcinomas into 8 major types: [2]

EMECL is considered a subtype of salivary gland-like carcinoma, tumors so named because their histological appearance and characteristics closely resemble malignant neoplasms arising in the major and minor salivary glands.[ citation needed ]

Staging

EMECL is staged in the same manner as other non-small cell lung carcinomas, based on the TNM (Tumor-Node-Metastasis) staging system. [8]

Treatment

Complete radical surgical resection is the treatment of choice for EMECL, and in most cases, results in long-term survival or cure. [9] [10]

Prognosis

The prognosis of EMECL is relatively good, and considerably better than most other forms of NSCLC. The skull and dura are possible sites for metastasis from pulmonary EMC. The MIB-1 index is a predictive marker of malignant potential.[ citation needed ]

Incidence

EMECL is extremely rare, with only a handful of cases reported in the literature. [11]

In the lung, two salivary gland-like carcinomas, mucoepidermoid carcinoma and adenoid cystic carcinoma, while extremely uncommon, occur far more often than does EMECL. [2] [12]

Related Research Articles

<span class="mw-page-title-main">Carcinoma</span> Malignancy that develops from epithelial cells

Carcinoma is a malignancy that develops from epithelial cells. Specifically, a carcinoma is a cancer that begins in a tissue that lines the inner or outer surfaces of the body, and that arises from cells originating in the endodermal, mesodermal or ectodermal germ layer during embryogenesis.

<span class="mw-page-title-main">Pleomorphic adenoma</span> Medical condition

Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.

<span class="mw-page-title-main">Myoepithelial cell</span>

Myoepithelial cells are cells usually found in glandular epithelium as a thin layer above the basement membrane but generally beneath the luminal cells. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. Myoepithelial cells in these cases constitute the basal cell layer of an epithelium that harbors the epithelial progenitor. In the case of wound healing, myoepithelial cells reactively proliferate. Presence of myoepithelial cells in a hyperplastic tissue proves the benignity of the gland and, when absent, indicates cancer. Only rare cancers like adenoid cystic carcinomas contains myoepithelial cells as one of the malignant components.

<span class="mw-page-title-main">Acinic cell carcinoma</span> Medical condition

Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. 90% of the time found in the parotid gland, 10% intraorally on buccal mucosa or palate. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Often appears pseudoencapsulated.

<span class="mw-page-title-main">Carcinosarcoma</span> Medical condition

Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma and sarcoma. Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary glands, lungs, the esophagus, pancreas, colon, uterus and ovaries.

<span class="mw-page-title-main">Salivary gland tumour</span> Medical condition

Salivary gland tumours, also known as mucous gland adenomas or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800 to 1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.

<span class="mw-page-title-main">Malignant chondroid syringoma</span> Type of skin cancer

A malignant chondroid syringoma is a very uncommon cutaneous (skin) condition characterised by an adnexal eccrine tumour.

Large cell lung carcinoma with rhabdoid phenotype (LCLC-RP) is a rare histological form of lung cancer, currently classified as a variant of large cell lung carcinoma (LCLC). In order for a LCLC to be subclassified as the rhabdoid phenotype variant, at least 10% of the malignant tumor cells must contain distinctive structures composed of tangled intermediate filaments that displace the cell nucleus outward toward the cell membrane. The whorled eosinophilic inclusions in LCLC-RP cells give it a microscopic resemblance to malignant cells found in rhabdomyosarcoma (RMS), a rare neoplasm arising from transformed skeletal muscle. Despite their microscopic similarities, LCLC-RP is not associated with rhabdomyosarcoma.

Mucinous cystadenocarcinoma of the lung (MCACL) is a very rare malignant mucus-producing neoplasm arising from the uncontrolled growth of transformed epithelial cells originating in lung tissue.

Acinic cell carcinoma of the lung is a very rare malignant neoplasm originating from bronchial glands. It is classified as a salivary gland-like carcinoma under the most widely used lung cancer classification system.

<span class="mw-page-title-main">Sarcomatoid carcinoma</span> Medical condition

Sarcomatoid carcinoma, sometimes referred to as pleomorphic carcinoma, is a relatively uncommon form of cancer whose malignant cells have histological, cytological, or molecular properties of both epithelial tumors ("carcinoma") and mesenchymal tumors ("sarcoma"). It is believed that sarcomatoid carcinomas develop from more common forms of epithelial tumors.

Sarcomatoid carcinoma of the lung is a term that encompasses five distinct histological subtypes of lung cancer, including (1) pleomorphic carcinoma, (2) spindle cell carcinoma, (3) giant cell carcinoma, (4) carcinosarcoma, or (5) pulmonary blastoma.

<span class="mw-page-title-main">Giant-cell carcinoma of the lung</span> Medical condition

Giant-cell carcinoma of the lung (GCCL) is a rare histological form of large-cell lung carcinoma, a subtype of undifferentiated lung cancer, traditionally classified within the non-small-cell lung carcinomas (NSCLC).

Adenosquamous lung carcinoma (AdSqLC) is a biphasic malignant tumor arising from lung tissue that is composed of at least 10% by volume each of squamous cell carcinoma (SqCC) and adenocarcinoma (AdC) cells.

Salivary gland–like carcinomas of the lung generally refers a class of rare cancers that arise from the uncontrolled cell division (mitosis) of mutated cancer stem cells in lung tissue. They take their name partly from the appearance of their abnormal cells, whose structure and features closely resemble those of cancers that form in the major salivary glands of the head and neck. Carcinoma is a term for malignant neoplasms derived from cells of epithelial lineage, and/or that exhibit cytological or tissue architectural features characteristically found in epithelial cells.

Basaloid squamous cell carcinoma (Bas-SqCC) is an uncommon histological variant of lung cancer composed of cells exhibiting cytological and tissue architectural features of both squamous cell lung carcinoma and basal cell carcinoma.

Large-cell neuroendocrine carcinoma of the lung, or pulmonary large-cell neuroendocrine carcinoma (PLCNC), is a highly malignant neoplasm arising from transformed epithelial cells originating in tissues within the pulmonary tree. It is currently considered to be a subtype of large-cell lung carcinoma.

<span class="mw-page-title-main">Epithelial-myoepithelial carcinoma</span> Medical condition

Epithelial-myoepithelial carcinoma (EMCa) is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours.

<span class="mw-page-title-main">Hyalinizing clear cell carcinoma</span> Medical condition

Hyalinizing clear cell carcinoma (HCCC) is a rare malignant salivary gland tumour, with a good prognosis, that is usually found on the tongue or palate.

A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.

References

  1. ↑ Tsuji N, Tateishi R, Ishiguro S, Terao T, Higashiyama M (August 1995). "Adenomyoepithelioma of the lung". The American Journal of Surgical Pathology. 19 (8): 956–62. doi:10.1097/00000478-199508000-00012. PMID   7611543.
  2. 1 2 3 4 5 6 Travis, William D; Brambilla, Elisabeth; Muller-Hermelink, H Konrad; Harris, Curtis C, eds. (2004). Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (PDF). World Health Organization Classification of Tumours. Lyon: IARC Press. ISBN   92-832-2418-3. Archived from the original (PDF) on 23 August 2009. Retrieved 27 March 2010.
  3. 1 2 Nguyen CV, Suster S, Moran CA (March 2009). "Pulmonary epithelial-myoepithelial carcinoma: a clinicopathologic and immunohistochemical study of 5 cases". Human Pathology. 40 (3): 366–73. doi:10.1016/j.humpath.2008.08.009. PMID   18973918.
  4. ↑ Okudela K; Yazawa T; Tajiri M; et al. (February 2010). "A case of epithelial-myoepithelial carcinoma of the bronchus - a review of reported cases and a comparison with other salivary gland-type carcinomas of the bronchus". Pathol. Res. Pract. 206 (2): 121–9. doi:10.1016/j.prp.2009.03.003. PMID   19369010.
  5. ↑ Roggli VL, Vollmer RT, Greenberg SD, McGavran MH, Spjut HJ, Yesner R (June 1985). "Lung cancer heterogeneity: a blinded and randomized study of 100 consecutive cases". Human Pathology. 16 (6): 569–79. doi:10.1016/S0046-8177(85)80106-4. PMID   2987102.
  6. ↑ Rossi G, Marchioni A, Sartoril G, Longo L, Piccinini S, Cavazza A (2007). "Histotype in non-small cell lung cancer therapy and staging: The emerging role of an old and underrated factor". Curr Respir Med Rev. 3: 69–77. doi:10.2174/157339807779941820. S2CID   52904357.
  7. ↑ Vincent MD (August 2009). "Optimizing the management of advanced non-small-cell lung cancer: a personal view". Curr Oncol. 16 (4): 9–21. doi:10.3747/co.v16i4.465. PMC   2722061 . PMID   19672420.
  8. ↑ Rami-Porta R, Crowley JJ, Goldstraw P (February 2009). "The revised TNM staging system for lung cancer". Annals of Thoracic and Cardiovascular Surgery. 15 (1): 4–9. PMID   19262443.
  9. ↑ Muro M; Yoshioka T; Idani H; et al. (March 2010). "肺に発生した上皮-筋上皮癌" [Epithelial-myoepithelial carcinoma of the lung]. Kyobu Geka (in Japanese). 63 (3): 220–3. PMID   20214352.
  10. ↑ Nishihara M, Takeda N, Tatsumi S, Kidoguchi K, Hayashi S, Sasayama T, Kohmura E, Hashimoto K (2011). "Skull metastasis as initial manifestation of pulmonary epithelial-myoepithelial carcinoma: a case report of an unusual case". Case Reports in Oncological Medicine. 2011: 610383. doi: 10.1155/2011/610383 . PMC   3352177 . PMID   22629483.
  11. ↑ Doganay L, Bilgi S, Ozdil A, Yoruk Y, Altaner S, Kutlu K (April 2003). "Epithelial-myoepithelial carcinoma of the lung. A case report and review of the literature". Archives of Pathology & Laboratory Medicine. 127 (4): e177–80. doi:10.5858/2003-127-e177-ECOTL. PMID   12683896.
  12. ↑ Travis WD, Travis LB, Devesa SS (January 1995). "Lung cancer". Cancer. 75 (1 Suppl): 191–202. doi:10.1002/1097-0142(19950101)75:1+<191::AID-CNCR2820751307>3.0.CO;2-Y. PMID   8000996. S2CID   34718856.
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