Sarcomatoid carcinoma of the lung

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Sarcomatoid carcinoma of the lung
Specialty Oncology/pulmonology

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. [1]

Contents

Genetics

Abnormal duplication of the EGFR gene is a relatively infrequent phenomenon in SCL (>/= 4 copies in >/= 40% of cells in 5/22). [2]

Overexpression of the EGFR protein occurs in nearly all cases (22/22). [2]

Mutations of the EGFR gene are relatively rare (0/23). [2]

K-ras mutations found in 8/22 cases (Gly12Cys in 6 cases and Gly12Val in 2 cases). [2]

SCL show intense immune infiltration which is predominantly neutrophillic. However the tumors evade the immune system by increased expression of a negative regulator of T-cells mainly programmed death ligand-1. [3]

Diagnosis

Classification

Lung cancer is a large and exceptionally heterogeneous family of malignancies. [4] Over 50 different histological variants are explicitly recognized within the 2004 revision of the World Health Organization (WHO) typing system ("WHO-2004"), currently the most widely used lung cancer classification scheme. [1] Many of these entities are rare, recently described, and poorly understood. [5] However, since different forms of malignant tumors generally exhibit diverse genetic, biological, and clinical properties, including response to treatment, accurate classification of lung cancer cases are critical to assuring that patients with lung cancer receive optimum management. [6] [7]

Approximately 98% of lung cancers are carcinoma, 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. [8] Under WHO-2004, lung carcinomas are divided into 8 major taxa: [1]

Sarcomatoid carcinomas are unique among lung carcinomas in that, although they are considered carcinomas, they contain cytological and tissue architectural features that are usually characteristic of sarcoma. [1]

Treatment

Because these tumors are so rare, there have been no randomized clinical trials yet conducted with respect to specific treatment regimens for any subtype. [9] [10]

Very few histospecific studies of individual subtypes SCL have been published in the literature; most data has been small retrospective case series or case reports.

In many cases, SCL are generally treated like other NSCLC. However, as SCL are considered particularly aggressive non-small cell lung cancers, [2] some experts recommend particularly aggressive treatment approach to these tumors.

Little is known about the effects of EGFR inhibitors in SC, although some evidence suggests that these tumors are not likely to be highly responsive. [2]

Prognosis

As a group, SCL prognosis is considered to be worse than that of most types of NSCLC.[ citation needed ]

Related Research Articles

<span class="mw-page-title-main">Non-small-cell lung cancer</span> Any type of epithelial lung cancer other than small-cell lung carcinoma

Non-small-cell lung cancer (NSCLC), or non-small-cell lung carcinoma, is any type of epithelial lung cancer other than small-cell lung cancer (SCLC). NSCLC accounts for about 85% of all lung cancers. As a class, NSCLCs are relatively insensitive to chemotherapy, compared to small-cell carcinoma. When possible, they are primarily treated by surgical resection with curative intent, although chemotherapy has been used increasingly both preoperatively and postoperatively.

<span class="mw-page-title-main">Adenocarcinoma in situ of the lung</span> Medical condition

Adenocarcinoma in situ (AIS) of the lung —previously included in the category of "bronchioloalveolar carcinoma" (BAC)—is a subtype of lung adenocarcinoma. It tends to arise in the distal bronchioles or alveoli and is defined by a non-invasive growth pattern. This small solitary tumor exhibits pure alveolar distribution and lacks any invasion of the surrounding normal lung. If completely removed by surgery, the prognosis is excellent with up to 100% 5-year survival.

Large-cell lung carcinoma (LCLC), or large-cell carcinoma (LCC) in short, is a heterogeneous group of undifferentiated malignant neoplasms that lack the cytologic and architectural features of small cell carcinoma and glandular or squamous differentiation. LCC is categorized as a type of NSCLC which originates from epithelial cells of the lung.

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

Combined small cell lung carcinoma is a form of multiphasic lung cancer that is diagnosed by a pathologist when a malignant tumor, arising from transformed cells originating in lung tissue, contains a component of;small cell lung carcinoma (SCLC), admixed with one components of any histological variant of non-small cell lung carcinoma (NSCLC) in any relative proportion.

Treatment of lung cancer refers to the use of medical therapies, such as surgery, radiation, chemotherapy, immunotherapy, percutaneous ablation, and palliative care, alone or in combination, in an attempt to cure or lessen the adverse impact of malignant neoplasms originating in lung tissue.

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.

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

Targeted therapy of lung cancer refers to using agents specifically designed to selectively target molecular pathways responsible for, or that substantially drive, the malignant phenotype of lung cancer cells, and as a consequence of this (relative) selectivity, cause fewer toxic effects on normal cells.

HOHMS is the medical acronym for "Higher-Order HistoMolecular Stratification", a term and concept which was first applied to lung cancer research and treatment theory.

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.

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

Adenocarcinoma of the lung is the most common type of lung cancer, and like other forms of lung cancer, it is characterized by distinct cellular and molecular features. It is classified as one of several non-small cell lung cancers (NSCLC), to distinguish it from small cell lung cancer which has a different behavior and prognosis. Lung adenocarcinoma is further classified into several subtypes and variants. The signs and symptoms of this specific type of lung cancer are similar to other forms of lung cancer, and patients most commonly complain of persistent cough and shortness of breath.

<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.

Acinar adenocarcinoma is a histological subtype of gland-forming cancer that is diagnosed when cuboidal and/or columnar shaped malignant cells in the neoplastic tissue form acini and tubules. It is a common form of cancer occurring in the lung and prostate gland.

<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.

Basaloid large cell carcinoma of the lung, is a rare histological variant of lung cancer featuring certain distinctive cytological, tissue architectural, and immunohistochemical characteristics and clinical behavior.

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

Squamous-cell carcinoma (SCC) of the lung is a histologic type of non-small-cell lung carcinoma (NSCLC). It is the second most prevalent type of lung cancer after lung adenocarcinoma and it originates in the bronchi. Its tumor cells are characterized by a squamous appearance, similar to the one observed in epidermal cells. Squamous-cell carcinoma of the lung is strongly associated with tobacco smoking, more than any other forms of NSCLC.

References

  1. 1 2 3 4 Travis, William D; Brambilla, Elisabeth; Muller-Hermelink, H Konrad; et al., 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   978-92-832-2418-1 . Retrieved 27 March 2010.
  2. 1 2 3 4 5 6 Italiano A, Cortot AB, Ilie M, et al. (November 2009). "EGFR and KRAS status of primary sarcomatoid carcinomas of the lung: implications for anti-EGFR treatment of a rare lung malignancy". Int. J. Cancer. 125 (10): 2479–82. doi: 10.1002/ijc.24610 . PMID   19681124. S2CID   205938398.
  3. ↑ Velcheti, V.; Rimm, D. L.; Schalper, K. A. (2013). "Sarcomatoid Lung Carcinomas Show High Levels of Programmed Death Ligand-1 (PD-L1)". Journal of Thoracic Oncology. 8 (6): 803–805. doi:10.1097/JTO.0b013e318292be18. PMC   3703468 . PMID   23676558.
  4. ↑ 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". Hum. Pathol. 16 (6): 569–79. doi:10.1016/S0046-8177(85)80106-4. PMID   2987102.
  5. ↑ Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y (December 2001). "The new World Health Organization classification of lung tumours". Eur. Respir. J. 18 (6): 1059–68. doi: 10.1183/09031936.01.00275301 . PMID   11829087.
  6. ↑ Rossi G, Marchioni A, Sartori1 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  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. ↑ 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.
  9. ↑ National Library of Medicine. Available at http://www.pubmed.com Archived 13 February 2015 at the Wayback Machine
  10. ↑ National Institutes of Health. Clinical Trials Search Engine. Available at http://www.clinicaltrials.gov/ct2/results?term=sarcomatoid+carcinoma
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