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Cancer and Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. Read Disclaimer
Overview
Angiosarcoma
Autoantibodies
Breast Cancer
Cervical Cancer
Gastrointestinal Tract Cancer
Lymphoma
Malignant Atrophic Papulosis/Degos
MALT Lymphoma
Non-Hodgkin's Lymphoma
Medications and Cancer
Liver Cancer
Lung Cancer
Melanoma
Myeloma (Bone Marrow Cancer)
T Cell Lymphoma
Tongue and Oral Cancer
Tumors
Personal Stories

Overview of Cancer and Scleroderma

Regular cancer screening is recommended for people who have systemic sclerosis due to 75% increased risk.
Systemic sclerosis (scleroderma) may be associated with an increased incidence of cancer, including breast cancer, B-cell lymphoma, lung cancer and tongue cancer. (Also see: Autoimmune Diseases)
Just because someone has cancer does not mean that they also have scleroderma, nor does it mean that someone with scleroderma will inevitably acquire cancer. Scleroderma affects everyone differently and there is no predictable course of the illness, either in its severity, progression or outcome. However, there is an increased risk of cancer in those who have systemic scleroderma, as documented in the research articles below. (Also see: Types of Scleroderma, Associated Conditions, and Causes of Scleroderma)
The war on cancer may NEVER be won: Cure could be impossible because the disease is so highly evolved. Cancer is as old as the earliest life on earth and may never be completely wiped out, scientists have warned. Mail Online, 08/22/2014.
Sitting Can Increase Your Risk of Cancer By Up to 66%. For a change, the cancer-promoter isn't something you eat or breathe, but something you do every day. Time Healthland, 06/17/2014.
New therapies raise hope for a breakthrough in tackling cancer. Immune-checkpoint blockade is a form of immunotherapy, meaning it aims to help the patient's own immune system fight cancer. Washington Post, 02/18/2014.
New evidence on how weight, diet and exercise can help reduce cancer risk. Common-sense lifestyle strategies for lowering the risk of heart disease and diabetes are now being shown to help prevent many types of cancer. Washington Post, 02/18/2014.
Association of the Autoimmune Disease Scleroderma with an Immunologic Response to Cancer. This offers an insight into the pathogenesis of scleroderma and provide support for the idea that acquired immunity helps control naturally occurring cancers. Science Express, 12/05/2013.
Cancer incidence in systemic sclerosis (SSc): Meta-analysis of population-based cohort studies. Systemic sclerosis is associated with an increased risk of cancer, particularly lung, liver, hematological and bladder cancer, though absolute risk is relatively low. Men with SSc are at higher cancer risk than women. PubMed, Arthritis Rheum. 2013 Apr 10.
Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta-analysis of observational studies. 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. PubMed. Rheumatology (Oxford). 2013 Jan;52(1):143-54.
Malignancies associated with systemic sclerosis (SSc). Secondary tumors including lung, skin and breast cancer, as well as lymphomas are more common in SSc than in the general population. Autoimmun Rev. 2012 Mar 5.
Effect of autoimmune diseases on mortality and survival in subsequent digestive tract cancers. Patients with some autoimmune diseases (AIDs) are at increased risk of cancer, possibly a result of an underlying dysregulation of the immune system, medication, or treatment (and not by surveillance bias). Annals of Oncology, 11/22/2011.
Autoimmune disease and subsequent risk of developing alimentary tract cancers among 4.5 million US male veterans. These findings support the importance of localized inflammation in alimentary tract carcinogenesis. Future research is needed to confirm the findings and improve understanding of underlying mechanisms by which autoimmune diseases contribute to alimentary tract carcinogenesis. Landgren AM. Cancer. 2011 Mar 15;117(6):1163-71. (Also see: Autoimmunity)
Cancer and systemic sclerosis: novel insights into pathogenesis and clinical implications. New investigations have demonstrated an association between RNA polymerase III autoantibodies and a close temporal relationship between cancer diagnosis and the development of clinical scleroderma. PubMed, Current Opinion in Rheumatology, 30 August 2011.

Angiosarcoma and Scleroderma

Angiosarcoma: A type of cancer that begins in the lining of blood vessels. This type of tumor tends to be aggressive, recur locally, and spread widely. It can originate anywhere in the body but is well known to arise in skin, soft tissue, liver, breast, spleen, bone, lung and heart. MedicineNet.com.
(Case Report) Angiosarcoma arising in sclerodermatous skin. This is the first report of cutaneous angiosarcoma occurring in sclerodermatous skin. PubMed. Acta Dermatovenerol.

Autoantibodies and Cancer with Systemic Sclerosis

Malignancies in Italian Patients with Systemic Sclerosis (SSc) Positive for Anti-RNA Polymerase III Antibodies. In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc. The Journal of Rheumatology, 101144. (Also see: Antibodies)

Breast Cancer

BMI linked to breast cancer risk after menopause. Overall body size, rather than shape, is a better indicator of breast cancer risk after menopause, according to a recent study. Reuters Health, 04/25/2014.
High-fat diets linked to some types of breast cancer. Women who eat a lot of fat, particularly saturated fat, may be at higher risk of certain types of breast cancer, new research suggests. Chicago Tribune, 04/22/2014.
Half of women have needless operations for early breast cancer. The study found that in 49% of such cases, the mastectomy was either unnecessary or was being carried out because a previous operation had failed. The Telegraph, 03/21/2014.
Even With Metastatic Breast Cancer, Some Patients Should Say No to Surgery and Radiation. When it comes to cancer, sometimes less is more. Times Healthland, 12/11/2013.
Sequential Breast Regimen Passes Clinical Test. A potentially more heart-friendly sequential chemotherapy regimen for breast cancer proved as effective as the standard concurrent approach but with less cardiotoxicity. Med Page Today, 11/16/2013.
Channel Blockers for Blood Pressure Linked to Breast Cancer Risk, Study Finds. Postmenopausal women who use a type of blood pressure-lowering medication called a calcium channel blocker may have increased odds of developing breast cancer, new research suggests. Healthday, 08/05/2013. (Also see: Cancer and Scleroderma and Raynaud's Treatments)
How Exercise May Lower Breast Cancer Risk. Breaking a sweat does more for your body than just trim your waistline. E.xercise may lower a woman's risk for breast cancer and researchers are finding out why. Times Healthline, 05/08/2013.
Alcohol Doesn't Affect Ability to Survive Breast Cancer. Breast cancer patients who raised a glass or two a week may even enjoy slightly longer lives than those who didn't drink. Time Healthland. 04/10/2013. (Also see: Scleroderma Food Guide)
Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta-analysis of observational studies. The relation with breast cancer, suggested in some previous epidemiological studies, was not confirmed. Rheumatology. 2013 Jan;52(1):143-54.
The risk of cancer development in systemic sclerosis: A meta-analysis. This meta-analysis demonstrated an increased risk of lung, non-Hodgkin's lymphoma and hematopoietic cancers among patients with SSc, but not for breast cancer. PubMed. Cancer Epidemiol. 2013 May 28.
Diane M: MCTD, and Surviving Sister of Scleroderma Patient I do not know what lies ahead for me on this path, I only hope that I can be as strong as my sister was...

Cervical Cancer

Reports of abnormal cervical cancer screening tests in systemic sclerosis (SSc). We noted a high prevalence of abnormal Pap tests self-reported in our sample. Increased risk was seen among those with diffuse SSc. Thus, it seems prudent to ensure that adequate attention is paid to cervical cancer screening for women with SSc. S. Bernatsky. Rheumatology. 12/15/2008.

Gastrointestinal Tract Cancer

Autoimmune diseases increase the risk of gastrointestinal tract cancer.
Aspirin May Cut Cancer Risk. A large British study shows that taking small amounts of aspirin over long periods of time reduces the risk of colon cancer and certain other cancers. New York Times, 08/11/2014.
The Dreaded Turning-50 Test. One of the joys of turning 50 is that your doctor will start recommending a colonoscopy, which involves having a tube inserted into the rectum to look for cancers and precancers. New York Times, 03/24/2014.

Liver Cancer

Risk of malignancy in scleroderma: A population-based cohort study. Contrary to previous studies, this study did not find statistical evidence of an increased incidence of cancer in scleroderma patients, except for liver cancer. One possible reason is the high background rates of certain cancers in the metropolitan Detroit area. It may be necessary to consider local cancer rates when comparing different scleroderma cohorts. PubMed. Arthritis Rheum. 2005 Jul 28;52(8):2415-2424. (Also see: Liver Involvement)

Lung Cancer

Solitary organizing pneumonia (SOP) in systemic sclerosis (SSc) mimicking lung adenocarcinoma. SOP is often misdiagnosed and removed surgically as it is rare and difficult to distinguish from lung carcinoma. SOP should be considered when a solitary nodular lung lesion is noted in a patient with SSc. PubMed, Arthritis Rheumatol, 2014 Apr 22. (Also see: Lung Involvement: Pneumonia)
Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta-analysis of observational studies. There was a strong association with lung cancer, and a significant increased risk also for haematological neoplasms. However, the relation with breast cancer was not confirmed. Rheumatology, DOI 10.1093, Nov 2012.
Possible anti-metastatic effect of Iloprost in a patient with systemic sclerosis (SSc) with lung cancer: a case study. Herein, we report iloprost treatment of a 60-year-old-woman with SSc, who lately developed pulmonary hypertension as a complication of her disease and lung adenocarcinoma as a co-incidence simultaneously. Yavuz Pehlivan, Reheumatology International, 30 March 2011 (Also see: Iloprost)

Lymphoma

Incidence of lymphoma associated with underlying lupus (SLE): Lessons learned from observational studies. Although the association of non-Hodgkin's lymphoma in SLE is well established, risk factors attributing to this association are still not understood. PubMed, Curr Opin Rheumatol. 2014 Mar;26(2):111-7. (Also see: Systemic Lupus Erythematosus Symptoms)
Lymphoma risk in systemic lupus: effects of disease activity versus treatment. There was a suggestion of higher lymphoma risk with exposure to cyclophosphamide and high cumulative steroids although disease activity itself was not clearly associated with lymphoma risk. PubMed, Ann Rheum Dis. 2014 Jan 1;73(1):138-42. (Also see: Systemic Lupus Erythematosus Symptoms)
A Clinical Prediction Rule For Lymphoma Development In Primary And Secondary Sjögren's Syndrome. This study allowed us to create a prediction model which might be useful in improving the follow-up management of patients with primary and secondary Sjogren's (pSS and sSS-SSc). C. Baldini. EULAR 2011 OPO039. Ann Rheum Dis 2011;70(Suppl3):80. (Also see: Sjogren's)

Malignant Atrophic Papulosis (MAP) or Degos Syndrome

Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis (scleroderma). Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology (cause). It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. PubMed. Cutis. 2005 Feb;75(2):101-4. (Also see: Types of Scleroderma)

MALT Lymphoma

Systemic sclerosis and gastric MALT lymphoma. MALT (mucosa-associated lymphoid tissue) lymphomas are usually confined to the gastric wall. Gastric MALT lymphomas have a well-established association with H. pylori infection. Eradication of H. pylori is the mainstay of treatment. PubMed. Joint Bone Spine. 2005 Jun 30. (Also see: Gastrointestinal Involvement)

Medications and Cancer

Gene Therapy for Cancer Shows Continued Progress. Researchers from University of Pennsylvania and Children’s Hospital of Philadelphia continue to present hopeful results from gene therapy for leukemia three years after starting clinical trials. Times Healthland, 12/09/2013.

Melanoma

Blood Test Could Show If Skin Cancer Has Spread. A straightforward blood test could identify patients whose skin cancer has spread to other parts of the body. Times Healthland, 11/04/2013.
Jo-1 positive paraneoplastic systemic sclerosis in a patient with metastatic melanoma. This rare case of SSc concurrent with melanoma suggests that besides other possible mechanisms, paraneoplastic etiology (cause) can be responsible for the association between SSc and cancer. PubMed. Eur J Dermatol. 2006 Jul-Aug;16(4):428-30.

Myeloma (Bone Marrow Cancer)

History Of Infection Or Autoimmune Disease May Increase Risk For Myelodysplastic Syndromes. Swedish researchers recently found that having a history of certain infections and autoimmune diseases may increase the risk of developing myelodysplastic syndromes and acute myeloid leukemia. Jessica Langholtz. The MDS Beacon. 07/29/2011.

Non-Hodgkin's Lymphoma

Non-Hodgkin lymphoma and autoimmunity: Does gender matter? Autoimmune disorders are more frequent in women whereas most non-Hodgkin lymphomas (NHLs) are commoner in men, yet sex-specific autoimmune-lymphoma associations are rarely reported. Our results add to the accumulating knowledge on this topic, and suggest that future studies should analyse data for men and women separately. Ansell P. (PubMed) Int J Cancer. 2010 Sep 17.
Laira: MCTD, Scleroderma, SLE, Lupus, CREST, Lymphoma I was just seventeen when I was told I had rheumatoid arthritis...

T Cell Lymphoma

Angioimmunoblastic T cell lymphoma with an unusual proliferation of epstein-barr virus-associated large B cells arising in a patient with progressive systemic sclerosis. The cause was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosis. PubMed. Acta Haematol. 2005;114(2):108-12. (Also see: Causes of Scleroderma: Medications)

Tongue and Oral Cancers

Cancers of the Oral Mucosa. Immune defects may predispose to oral squamous cell carcinoma (OSCC), especially lip cancer. OSCC is also now being reported with increased frequency in association with diabetes and systemic sclerosis. Crispian Scully, MD. eMedicine.04/27/10. (Also see: Dental Involvement: Oral Cancers and Diabetes)
Increased incidence of carcinoma of the tongue in patients with systemic sclerosis. There is a highly significant increase in the incidence of squamous cell carcinoma of the tongue in patients with SSc. A remarkable observation was that all patients within this cohort who developed oral cancer had the diffuse subset of SSc. J Rheumatol. 2005 Apr;32(4):637-41. (Also see: Diffuse Scleroderma and Dental Involvement: Oral Cancer)

Tumors

Rheumatic disorders as paraneoplastic syndromes. The long-established observation that some rheumatologic disorders (RDs) are associated with, or precede, the clinical manifestations of a variety of solid and hematological tumors represents an important clue for the early diagnosis and effective treatment of the cancers. (PubMed) Racanelli V, Autoimmun Rev. 2008 May;7(5):352-8.

Personal Stories of Cancer

Anna B: Morphea My morphea started as bruise-like marks on the backs of my legs about three years ago, after I had just fought cervical cancer and won...
Bri: Morphea Scleroderma I am currently waiting to have plastic surgery. They are going to do reverse liposuction; taking some fat and tissue from my left leg and then inject it into my right leg...
Buggzy: Autoimmune Hepatitis, Fibromyalgia, Undiagnosed I am really desperate to get some answers or to hear from others in the same boat as me...
Carrie: CREST Syndrome All this started in 1995, as far as receiving any diagnosis. First, I was diagnosed with breast cancer. I went through radiation and chemotherapy...
Christin: Linear Scleroderma My name is Christin. I am a twenty-year-old college student. I have had scleroderma for as long as I can remember...
Craig R: CREST Scleroderma I had my first scleroderma symptoms at age twenty-two, which included severe Raynaud's with some ulceration and tissue loss...
Dwight: Surviving Son of Scleroderma Patient My dear mother, Drema L Shaver, passed away one year ago today...
Janice E: CREST/Fibromyalgia At some point I came to the realization that what I was feeling just could not be normal. By this time my hands were swelling...
Kellie: Overlap Scleroderma and Mixed Connective Tissue Disease (MCTD) I am so relieved to read about so many people who have had the same experiences...
Kym: Diffuse, CREST Scleroderma, Lupus and Fibromyalgia It all started with Raynaud's in my fingers and toes when I was about nineteen years old, in 1979...
Marina: Diffuse Scleroderma I was sent to a rheumatologist who spent one and a half hours checking me while explaining this rare and incurable autoimmune connective tissue disease in a nut shell...
Mike Thomas: Diffuse Scleroderma The doctors keep passing me around saying that I am too complicated for them to treat. So nothing gets done...
Rachel: Morphea Scleroderma I have learned to control the use of addicting medication and to manage the pain as well as possible...
 
Go to Associated Conditions: Dermatomyositis/Polymyositis
 

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