Clinical and Pathological Features of Breast Cancer in Systemic Sclerosis: Results from the Sclero-Breast Study.

Angela Toss,Guido Ficarra,Antonino Maiorana,Amelia Spinella,Anna Iannone,Simonetta Piana,Luca Magnani,Luca Fabbiani,Paola Castrignanò,Chrystel Isca,Dilia Giuggioli,Caterina Vacchi,Elisa Gasparini,Carlo Salvarani,Laura Cortesi,Pierluca Macripò,Massimo Dominici

doi:10.3390/jpm11060580

Abstract

Systemic Sclerosis (SSc) is a chronic disease associated with a 1.5-fold increase in cancer risk, including lung cancer, hematological malignancies, and breast cancer (BC). This is a retrospective study aiming to explore the clinical and pathological features of BC developed by SSc patients. A total of 54.5% of patients developed BC before SSc (median interval: 5 years), whereas 45.5% of patients developed BC after SSc (median delay: 8 years). A total of 93.1% of patients were diagnosed with an early stage tumor. Among invasive carcinomas, 70.8% presented with a low Mib1, 8.3% with a tubular histotype, and 42.8% with a Luminal A-like phenotype. A total of 66.6% of patients underwent breast-conserving surgery and 55.5% RT. A total of 40% of patients developed interstitial lung disease after RT and 20% diffuse cutaneous SSc. The cause of death of the six deceased patients was PAH. A significant association was observed between the use of immunosuppressive therapy and diffuse skin extension, negative ACA, positive Anti-Scl-70, and interstitial lung disease, but not BC status. SSc patients developed BC at a good prognosis, suggesting a de-escalation strategy of cancer therapies. In particular, ionizing radiation and chemotherapeuticals should be limited to higher-risk cases. Finally, proper screening is mandatory in order to allow for early cancer detection in SSc patients.

Highlights

Systemic Sclerosis (SSc) is a chronic disease characterized by vascular dysfunction, specific autoimmune alterations, and fibrosis of the skin and internal organs, such as lungs and heart
Clinical–rheumatological features related to SSc were collected: age at disease onset; smoking habits; skin extension of the disease; presence of skin ulcers, calcinosis, and telangiectasia; presence of gastrointestinal and kidney involvement; interstitial lung disease; forced vital capacity (FVC) evaluation at pulmonary function tests and diffusing capacity of the lungs for carbon monoxide through the single-breath technique (DLCO SB); ECG abnormalities; echocardiographic assessment of pulmonary arterial hypertension (PAH) with measurement of pulmonary artery systolic pressure (PAPs); SSc pattern at videocapillaroscopy; autoantibody profile; exposure to immunosuppressive and vasoactive therapies; status at last
In a retrospective cohort of 65 SSc patients affected by breast cancer (BC), Scope et al reported that 75% of scleroderma and BC cases were diagnosed within 3 years of each other and in 44% of cases, scleroderma appeared before or simultaneously with the BC diagnosis [16]

Summary

Introduction

Systemic Sclerosis (SSc) is a chronic disease characterized by vascular dysfunction, specific autoimmune alterations, and fibrosis of the skin and internal organs, such as lungs and heart. In 2008, Lu et al described 21 cases of BC in SSc patients and analyzed their exposure to BC risk factors through standardized self-administered questionnaires and case note reviews. In this analysis, scleroderma patients with BC were found to have a higher incidence of a positive family history of BC and a lower incidence of hormone-replacement therapy use [11]. In 2014, Colaci et al observed a significant increase in BC incidence compared with the general population from the same geographic area with a close temporal relationship between SSc and BC onset [6]. This close temporal relationship suggests that early onset of BC might trigger the immune system, leading to an excessive immune response and the onset of SSc [13]

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