Mesothelioma

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Characteristic malignant tumour of the mesothelium as a result of asbestos exposure. Smoking exacerbates risk. Notifiable disease. Appears in the pleural cavity, produces a thick, locally infiltrating tumour which may encase the lung and restrict respiratory function. The disease is unfortunately exceptionally lethal with survival on the order of 1-2 years (slightly better in the epitheloid variant).

Contents

Clinical Features

Dyspnoea or chest pain, particularly around the lower ribs. It may present with pleural effusion or be noted incidentally on chest radiograph. In later stages, it may produce severe pain from infiltration of the intercostal nerves.

Epidemiology

The disease has a latency of at least 15-20 years, but more commonly presents between 30-40 years after first exposure. The incidence in the UK is expected to peak around 2015. The Peto model based on cohort exposure predicts 90,000 deaths in the UK by 2050 (250,000 on Europe), at which point, it will all but have disappeared in the West but still be a ever bigger problem in China etc. [1] The disease incidence in the US is thought to have peaked as the pattern of asbestos use was thought to include less crocidolite, the form of asbestos with the most carcinogenic properties.

One of the particular sources of asbestos exposure is the basement and sub-basement works of old hospitals, thus healthcare workers may have an excess risk.

Investigations

Radiological features combined with pleural biopsy is the usual means of diagnosis. Pleural aspiration if pleural effusion present.

There is interest in developing a non-invasive test by measuring mesothelin-related peptides.[2][3][4]. Fibulin-3 is a likely candidate[5] and serum mesothelin level is a prognostic indicator[6].

Histology

Broadly classified into sarcomatoid, epitheloid or a combination of both variants (biphasic).

Treatment

Limited in advanced disease. Prophylactic pleurodesis may avoid lung entrapment. It is now definitely known after years of inadequate evidence base and controversy that thoracoscopic partial pleurectomy is inferior to talc pleurodesis[7]. Extrapleural pneumonectomy has been been reported to be successful in case series[8] which are suspected to be subject to bias. Palliative radiotherapy directed at catheter or biopsy needle tracks does work[9] but the benefit of palliative chemotherapy is doubtful. As of 2014 phase 1 trials continue[10].

The pain from advanced pleural mesothelioma can be exceptionally severe and unremitting, and requires doses of analgesics which often cause inexperienced medical and nursing attendants and pharmacists to make incorrect decisions and treat inadequately.

References

  1. Hodgson JT, McElvenny DM, Darnton AJ, Price MJ, Peto J. The expected burden of mesothelioma mortality in Great Britain from 2002 to 2050. British journal of cancer 2005;92:587-93. (Direct link – subscription may be required.)
  2. Robinson BW, Creaney J, Lake R, Nowak A, Musk AW, de Klerk N, et al. Mesothelin-family proteins and diagnosis of mesothelioma. Lancet. 2003;362:1612-6.
  3. Robinson BW, Creaney J, Lake R, Nowak A, Musk AW, de Klerk N, et al. Soluble mesothelin-related protein--a blood test for mesothelioma. Lung cancer (Amsterdam, Netherlands) 2005;49 Suppl 1:S109-11. (Direct link – subscription may be required.)
  4. Scherpereel A, Grigoriu B, Conti M, Gey T, Gragoire M, Copin MC, et al. Soluble mesothelin-related peptides in the diagnosis of malignant pleural mesothelioma. American journal of respiratory and critical care medicine. 2006;173:1155-60. (Direct link – subscription may be required.)
  5. Pass HI, Levin SM, Harbut MR, Melamed J, Chiriboga L, Donington J, Huflejt M, Carbone M, Chia D, Goodglick L, Goodman GE, Thornquist MD, Liu G, de Perrot M, Tsao MS, Goparaju C. Fibulin-3 as a blood and effusion biomarker for pleural mesothelioma. The New England journal of medicine. 2012 Oct 11; 367(15):1417-27.(Link to article – subscription may be required.)
  6. Linch M, Gennatas S, Kazikin S, Iqbal J, Gunapala R, Priest K, Severn J, Norton A, Ayite B, Bhosle J, O Brien M, Popat S. A serum mesothelin level is a prognostic indicator for patients with malignant mesothelioma in routine clinical practice. BMC cancer. 2014 Sep 17; 14(1):674.(Epub ahead of print) (Link to article – subscription may be required.)
  7. Rintoul RC, Ritchie AJ, Edwards JG, Waller DA, Coonar AS, Bennett M, Lovato E, Hughes V, Fox-Rushby JA, Sharples LD. Efficacy and cost of video-assisted thoracoscopic partial pleurectomy versus talc pleurodesis in patients with malignant pleural mesothelioma (MesoVATS): an open-label, randomised, controlled trial. Lancet. 2014 Jun 14.(Epub ahead of print) (Link to article – subscription may be required.)
  8. Spaggiari L, Marulli G, Bovolato P, Alloisio M, Pagan V, Oliaro A, Ratto GB, Facciolo F, Sacco R, Brambilla D, Maisonneuve P, Mucilli F, Alessandrini G, Leoncini G, Ruffini E, Fontana P, Infante M, Pariscenti GL, Casiraghi M, Rea F. Extrapleural pneumonectomy for malignant mesothelioma: an Italian multicenter retrospective study. The Annals of thoracic surgery. 2014 Jun; 97(6):1859-65.(Link to article – subscription may be required.)
  9. Thomas R, Budgeon CA, Kuok YJ, Read C, Fysh ET, Bydder S, Lee YC. Catheter tract metastasis associated with indwelling pleural catheters. Chest. 2014 Sep 1; 146(3):557-62.(Link to article – subscription may be required.)
  10. Kanteti R, Dhanasingh I, Kawada I, Lennon FE, Arif Q, Bueno R, Hasina R, Husain AN, Vigneswaran W, Seiwert T, Kindler HL, Salgia R. MET and PI3K/mTOR as a Potential Combinatorial Therapeutic Target in Malignant Pleural Mesothelioma. PloS one. 2014; 9(9):e105919.(Epub) (Link to article – subscription may be required.)