Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta-analysis of observational studies.

Istituto di Ricerche Farmacologiche 'Mario Negri', Via Giuseppe La Masa 19, Milan, Dipartimento di Scienze Cliniche e Molecolari, Istituto di Clinica Medica, Università Politecnica delle Marche, Via Tronto 10/A, Ancona, Dipartimento di Medicina del Lavoro, Università degli Studi di Milano, Via Vanzetti 4, Milan, Clinica Medica, Dipartimento di Medicina Interna e Specialistica, Ospedali Riuniti, Via Conca 71, Ancona and Dipartimento di Biologia e Patologia Cellulare e Molecolare, Università 'Federico II', Via S. Pansini 5, Naples, Italy.
Rheumatology (Oxford, England) (Impact Factor: 4.44). 11/2012; DOI: 10.1093/rheumatology/kes303
Source: PubMed

ABSTRACT Objectives. A higher incidence of cancer in scleroderma patients compared with the general population has been suggested by several observational studies, reporting, however, different estimates. Therefore, we aimed to perform a systematic review and meta-analysis to definitely assess this association.Methods. We searched MEDLINE and Embase for all original articles of observational studies on cancer incidence in scleroderma patients without language restriction published up to December 2011. Two independent authors reviewed all titles/abstracts and retrieved detailed full-text of potentially relevant articles to identify studies according to predefined selection criteria. Summary estimates were derived using random-effects model and reported as relative risk (RR). Publication bias was evaluated by trim and fill analysis.Results. From articles initially identified, 16 original studies, involving more than 7000 patients, were included in the present review. Compared with the general population, the summary RR to develop all invasive cancers in scleroderma patients was 1.75 (95% CI 1.41, 2.18). The results for selected cancer sites indicated a strong association with lung cancer (RR 4.35; 95% CI 2.08, 9.09), and a significant increased risk also for haematological neoplasms (RR 2.24; 95% CI 1.53, 3.29). The relation with breast cancer, suggested in some previous epidemiological studies, was not confirmed (RR 1.05; 95% CI 0.86, 1.29).Conclusion. The present meta-analysis, the first on scleroderma and cancer risk, provides definite estimates on the association between scleroderma and cancer.

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    ABSTRACT: Objective: Systemic sclerosis (SS) is a chronic autoimmune disease of connective tissue, which involves skin and internal organs, and results in collagen deposition and fibroblasts activation. Studies have reported a higher risk of malignancy in patients with progressive systemic sclerosis (PSS).The aim of this study is to report a case of tongue squamous cell carcinoma (SCC) in a patient with PSS. Case: A 46-year-old woman with SS presented to the Department of Oral Medicine of Shahid Beheshti University of Medical Sciences Dental School with chief complaint of an ulcer on her tongue. During intraoral examination, an asymptomatic ulcer measuring 2.0 x1.5 cm was discovered on the right lateral border of the tongue. Incisional biopsy under local anesthesia was performed and histopathological report confirmed presence of squamous cell carcinoma. After further evaluation, surgery was performed, followed by three sessions of brachytherapy and six sessions of chemotherapy. After 26 months of follow-up, there was no evidence of recurrence. Conclusion: Periodic screening examinations are necessary to discover possible malignancies in primary stages in patients with SS. Key words: Systemic sclerosis, Squamous Cell Carcinoma, Tongue.
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    ABSTRACT: Systemic Sclerosis (SSc) is a rare connective tissue disorder associated with an increased risk of malignancy including lung cancer. A single center review of all cases of lung cancer in patients with SSc was conducted. Clinical, radiographic, and detailed pathologic data was collected. Risk factors were compared with our center's SSc Registry. Cancer characteristics were compared with the National Cancer Institute SEER Cancer Statistics (NCI SEER) data. 17 cases were identified; the majority were females (82%) with the lung cancers diagnosed after the onset of SSc (88%). Tobacco use was identified in 65% of cases. Serologic testing showed 50% of cases were Scl-70 positive. Twelve cases had radiographic evidence of SSc lung involvement, however only 6 had restrictive physiology on pulmonary function testing. Thirteen cases had pulmonary nodules preceding lung cancer. Thirteen of the cancers were adenocarcinoma. Ten underwent molecular mutational profiling: 2/8 had KRAS mutation and 1/10 had EGFR mutation. More of the non-small cell lung cancers were diagnosed at localized disease (56%) than in the NCI SEER database. However, 5 years survival among the stage I cases was 25% versus an expected survival of 54%. The high proportion of adenocarcinomas seen in our study is different from that reported in the literature. Lung cancers were diagnosed at an early stage, likely due to our center's practice of radiographic screening for SSc associated lung involvement, however this did not confer a survival advantage. A high proportion of patients who developed lung cancer had interstitial lung disease.
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