Merkel Cell Carcinoma Metastatic to the Heart

Cahill

et al. 2022

Cancers

Merkel cell carcinoma (MCC), an aggressive neuroendocrine skin cancer, has a high rate (20%) of distant metastasis. Within a prospective registry of 582 patients with metastatic MCC (mMCC) diagnosed between 2003–2021, we identified 9 (1.5%) patients who developed cardiac metastatic MCC (mMCC). We compared overall survival (OS) between patients with cardiac and non-cardiac metastases in a matched case–control study. Cardiac metastasis was a late event (median 925 days from initial MCC diagnosis). The right heart was predominantly involved (8 of 9; 89%). Among 7 patients treated with immunotherapy, 6 achieved a complete or partial response of the cardiac lesion. Among these 6 responders, 5 received concurrent cardiac radiotherapy (median 20 Gray) with immunotherapy; 4 of 5 did not have local disease progression or recurrence in the treated cardiac lesion. One-year OS was 44%, which was not significantly different from non-cardiac mMCC patients (45%, p = 0.96). Though it occurs relatively late in the disease course, cardiac mMCC responded to immunotherapy and/or radiotherapy and was not associated with worse prognosis compared to mMCC at other anatomic sites. These results are timely as cardiac mMCC may be increasingly encountered in the era of immunotherapy as patients with metastatic MCC live longer.

Section: Discussionsupporting

confidence: 91%

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Management and Prognosis of Cardiac Metastatic Merkel Cell Carcinoma: A Case–Control Study and Literature Review

Cahill

et al. 2022

Cancers

Australasian Journal of Dermatology

“…MCC can spread to almost any site in the body 6 including the soft tissues 7 and gut. 8 Patients developing visceral sites of recurrence are considered incurable and this in part explains the high (30-40%) cancer specific mortality published in many series.…”

Section: Discussionmentioning

confidence: 99%

Merkel cell carcinoma metastatic to the transverse colon: Disease free after six years - cure or just prolonged remission?

Veness

Howle

2011

Merkel cell carcinoma is an uncommon but highly immunogenic skin malignancy that has the potential to metastasize to any site in the body. Despite treatment many patients experience relapse, often to distant sites beyond the site of initial treatment. The development of distant soft tissue or visceral metastases is considered incurable, despite treatment with prognosis usually being measured in months. We report the case of an elderly man who developed colonic metastases from a head and neck primary and with treatment has survived disease free for over 6 years. Such reports are infrequently documented and highlight the unpredictable nature of this disease.

“…With a 33% mortality rate, MCC is deadlier than other more common forms of skin cancer [ 2 ]. MCC is associated with local recurrence, regional metastasis, and distant metastasis to the brain, bone, liver, lung, and heart [ 3 ]; it is the second most common cause of skin cancer death after melanoma, with an estimated cause-specific death rate of 0.6 per 100,000 persons (in 2006) [ 4 , 5 ]. In addition, the death rate of MCC has been rising rapidly—in the past two decades, deaths due to MCC have increased more than 3-fold [ 6 ].…”

Section: Merkel Cell Carcinoma: Introductionmentioning

confidence: 99%

Merkel Cell Carcinoma in Immunosuppressed Patients

Janice

Brewer

2014

Cancers

Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous malignancy. The infectivity of Merkel cell polyomavirus (MCPyV), an apparent agent in MCC development, may be exacerbated with impaired immune responses. This paper reviews relevant data regarding the role of immunosuppression in the development of MCC and describes modes of immunodeficient states. Because of the inherently low incidence rate of MCC, several case studies and series are also briefly mentioned to provide a more comprehensive summary of MCC in the setting of immunosuppression. We describe immunosuppressed patients who have experienced excessive UV radiation, organ transplantation, human immunodeficiency virus infection/AIDS, autoimmune diseases, and lymphoproliferative disorders. Iatrogenic forms of immunosuppression are also highlighted. Studies that quantify risks consistently report that individuals with a history of solid organ transplantation, autoimmune diseases, AIDS, and/or lymphoproliferative diseases have a significantly elevated risk of developing MCC. Overall, immunocompromised patients also appear to have an early onset and more aggressive course of MCC, with poorer outcomes. Recommendations for multidisciplinary approaches are proposed to effectively prevent and manage MCC in these patients.