Extrapulmonary small cell carcinoma

Our plain-English summary of Extrapulmonary small cell carcinoma, by H.F. van der Heijden, Y.F. Heijdra (Southern Medical Journal, 2005).

The study

• Authors: H.F. van der Heijden, Y.F. Heijdra
• Journal: Southern Medical Journal
• Published: 2005;98(3):345-9
• Type of article: Review
• Cited by: 65 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

This article reviews the recent literature on extrapulmonary small cell carcinomas. Until now, only four cases have been published in the English literature, two of those in the Southern Medical Journal. Sharing the information on diagnosis and treatment of these cases is important for better understanding and treatment of these patients. The differential diagnosis of pleural masses and the relation to asbestos exposure is briefly discussed. In addition, the available literature on other extrapulmonary localizations of small cell carcinoma is reviewed. The focus is on the available larger series of patients. Small cell carcinoma of the skin is included. This form of small cell carcinoma is often excluded from the larger review series, but can be an important alternative in the diagnostic process of patient evaluation.

In plain English

This article reviewed published research on small cell carcinoma arising outside the lung. It focused on the pleura (the lining around the lungs) and on skin small cell carcinoma (Merkel cell carcinoma), illustrated with two cases from the authors' clinic. The authors reported that only four cases of pleural small cell carcinoma had been published. All four were in heavy smokers (30 to 70 pack-years), and two had a history of asbestos exposure. One was the authors' own earlier case, a 66-year-old man whose chest radiographs had shown pleural plaques dating back 17 years before diagnosis. Another case had no detectable asbestos exposure. No fiber type was named for either asbestos-exposed case. In a cited Mayo Clinic series of 81 patients, which excluded Merkel cell carcinoma and well-differentiated neuroendocrine cell carcinomas, overall 5-year survival was 13%. They named malignant mesothelioma as the most important alternative diagnosis for pleural masses. They stated that their case underlines the need for careful analysis of radiologic changes in people with asbestos-related pleural abnormalities. They noted that this pleural literature consists entirely of case reports.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Literature review (narrative review of case series and case reports), with two illustrative cases of skin small cell carcinoma from the authors' clinic
• Who was studied: The review covered three groups: published series of patients with small cell carcinoma outside the lung (6 to 600 cases each, mostly published after 1982, plus two 1981 papers); four published case reports of pleural small cell carcinoma; and two patients with skin small cell carcinoma seen at the authors' clinic in Nijmegen, The Netherlands.
• Exposure: Two of the four published cases of pleural small cell carcinoma had asbestos exposure. No fiber type was stated for these cases. All four cases were heavy smokers (30 to 70 pack-years).
• Main results:
  • Only four cases of small cell carcinoma originating in the pleura had been reported in the literature.
  • All four reported pleural cases were heavy smokers (range 30 to 70 pack-years). Two of the four had a history of asbestos exposure.
  • The first published case (Bouvier and Bell, 1989) was a 67-year-old man with a history of heavy smoking and asbestos exposure. He survived at least 10 months.
  • A third case (Schinkewitch et al, 1996) was a 60-year-old man with a 40 pack-year smoking history and no detectable asbestos exposure. He survived more than 26 months.
  • The fourth case was published by the authors' group in 2002. He was a 66-year-old man with a 40 pack-year smoking history and asbestos exposure. Pleural plaques were present on his chest radiographs dating back 17 years before the diagnosis. He died 11 days after starting chemotherapy.
  • A cited review of 500 cases of malignant mesothelioma (Attanoos et al) found other cancers occurring at the same time in nine patients (1.8%). Six of these nine had lung carcinomas, including one small cell carcinoma.
  • The cited Mayo Clinic series (Galanis et al, 81 patients) excluded Merkel cell carcinoma and well-differentiated neuroendocrine cell carcinomas. In that series, overall 5-year survival for small cell carcinoma outside the lung was 13%.
• The authors' conclusion: The authors concluded that small cell carcinoma starting in the pleura is extremely rare. They named malignant mesothelioma as the most important alternative diagnosis for pleural masses. They stated that their case report underlines the need for careful analysis of radiologic changes in patients with asbestos-related pleural abnormalities.
• Limitations the authors state:
  • The literature on small cell carcinoma starting in the pleura is based entirely on case reports.
  • In the pathology-based series, the relative frequency of the different sites of origin is likely influenced by the study design. It therefore differs from data from clinical studies.

Topics

Medical terms in this study, explained in our Lexicon: Carcinoma, Differential Diagnosis, Asbestos Exposure.

Related studies

• The importance of retaining post mortem tissue--'pseudomesotheliomatous' Merkel cell carcinoma of the pleura — G.D. Leopold, R.L. Attanoos (2011)
• Extrapulmonary small cell carcinoma mimicking malignant pleural mesothelioma — K. Noguchi, N. Fujimoto, et al. (2013)
• Pleural small cell carcinoma in pre-existent asbestos related pleural disease — H.F. van der Heijden, Y.F. Heijdra, et al. (2002)
• Nuclear Brachyury Expression Is Consistent in Chordoma, Common in Germ Cell Tumors and Small Cell Carcinomas, and Rare in Other Carcinomas and Sarcomas: An Immunohistochemical Study of 5229 Cases — M. Miettinen, Z. Wang, et al. (2015)
• Collision tumour of the pleura composed of small cell carcinoma and malignant mesothelioma — A.H. Lee, I.N. Soomro (2004)
• Expression of the small cell carcinoma antigens of cluster-5 and cluster-5A in primary lung tumours — A. Maier, U. Schmidt, et al. (1989)

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