scholarly journals Merkel Cell Carcinoma: A Retrospective Study on 48 Cases and Review of Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Fernando Cirillo ◽  
Marco Vismarra ◽  
Ines Cafaro ◽  
Mario Martinotti

Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor of the skin. Fourty-eight patients with MCC were observed at the Rare Hormonal Tumors Group of Cremona Hospital, 15 of these with unknown primary site. Due to rarity of Merkel cell carcinoma, clinical experience is generally limited. Data from our series confirm the current recommendations. Wide surgical excision must be associated with radiotherapy also in early stages in order to avoid local relapse and the rapid progression of disease. In advanced stages chemotherapy is the standard despite the short duration of responses and poor quality of life. The data of our series, characterized by a high demand for second opinion, offer some insight about the real rarity of the tumor, the difficulty of managing of disease in our country secondary to a wrong cultural approach to the problem, the indiscriminate use of molecules unnecessary and often expensive, the lack of protocols, and the presence of guidelines often ignored. This results in very poor survival associated with a very low quality of life, requiring to find the right direction towards a correct management of disease.

2017 ◽  
Vol 28 ◽  
pp. xi23
Author(s):  
M. Bharmal ◽  
P. Williams ◽  
M. Hennessy ◽  
M. Schlichting ◽  
M. Hunger ◽  
...  

2003 ◽  
Vol 89 (2) ◽  
pp. 146-151 ◽  
Author(s):  
Fernando Cirillo ◽  
Massimo Buononato ◽  
Gianfranco Lima ◽  
Ines Cafaro ◽  
Palmiro Alquati

Merkel cell carcinoma is a rare neuroendocrine neoplasm of the skin. The tumor most frequently affects elderly patients, with a preference for the head and neck. Eight patients affected by Merkel cell carcinoma have been observed at the General Surgery Unit II of the “Istituti Ospitalieri” hospital in Cremona, each in different stages of the disease; 75% of the cases involved the extremities, and in nearly all of the cases the tumor was nodular in appearance, with an average diameter of 2.2 cm. In 2 cases, the tumor was associated with rheumatoid arthritis, suggesting a dependency on the part of the neoplasm on the immune disorder and on steroid treatment. The available data confirm that in stage I of the disease, surgical treatment should be associated with radiotherapy in order to control the development of local relapses or metastases over time. In this stage, we observed a survival of 34 months (range, 24-48). In stages II and III, survival time falls, with very short duration of responses and poor quality of life as a result of the administration of cytotoxic molecules. Bearing in mind that any local relapse tends to appear within 12 months of the removal of the primitive tumor, that lymph node metastases appear in almost half of the patients, and that metastases over time are manifested in over a third of patients, it is essential to adopt a treatment capable of balancing the demand for longer remissions with a better quality of life. In this situation, we observed that treatment with somatostatin analogues achieves interesting responses without side effects, which suggests a close biological relationship between the tumor and somatostatin and that making a careful assessment of the prognostic factors of the disease can guarantee a correct therapeutic choice.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e21070-e21070
Author(s):  
Murtuza Bharmal ◽  
Fatoumata Fofana ◽  
Lisa Mahnke ◽  
Michael Schlichting ◽  
Howard Kaufman

e21070 Background: There is little data on quality of life (QoL) for patients (pts) with Merkel cell carcinoma (MCC). To better understand how new treatments might impact QoL in MCC, pts with stage IV chemotherapy-treated MCC from a single-arm, open-label, multicenter, international phase 2 trial (NCT02155647) of a novel immunotherapy were followed for QoL and clinical outcomes. Methods: Because there is no MCC-specific QoL instrument, the FACT-Melanoma (FACT-M), a validated QoL questionnaire including multiple subscales and summary scores (Trial Outcome Index [TOI], FACT-G total and FACT-M total) was used. Pts completed the FACT-M at baseline (BL), week 7, every 6 weeks until disease progression (PD) and at end of treatment (EOT). Linear mixed models (LMM) were fitted for change from BL for each scale including the time-varying covariate PD vs non-PD. Established minimal important differences (MID) were used to interpret meaningfulness of changes. Sensitivity analyses explored the effects of missing data. Results: 70 pts were analyzed. Overall, no meaningful changes from BL were observed for each scale during treatment. Moderate correlations between reduction in tumor size and improvements in FACT-M were observed at week 7 for Functional Well-being (-0.47), TOI (-0.36), FACT-M total (-0.36), and FACT-G total (-0.34), suggesting improvements in QoL with tumor shrinkage. LMM showed differences between PD vs non-PD groups in the range of MIDs for Functional Well-being (2.23, p = 0.018), Melanoma (3.19, p = 0.012), Surgery (1.95, p = 0.109), TOI (6.36, p = 0.012) and FACT-G total (3.89, p = 0.105). These models showed improvement for the non-PD group in Emotional Well-being (1.55, p = 0.004) and worsening for the PD group in Physical Well-being (-1.63, p = 0.041), Surgery (-2.09, p = 0.044) and TOI (-4.56, p = 0.036). As expected, all scales worsened in the range of MIDs at EOT, mostly due to PD. Sensitivity analyses were consistent, with modest increases in estimated differences. Conclusions: In pts with metastatic MCC, non-progression during avelumab treatment contributed to statistically and clinically meaningful improvements in health-related QoL. Clinical trial information: NCT02155647.


2016 ◽  
Vol 15 (2) ◽  
pp. 189-195 ◽  
Author(s):  
Bharthi Kanagaratnam ◽  
Aadarsh Shah ◽  
Girija Anand

AbstractBackground and purposeMerkel cell carcinoma is a rare and aggressive primary cutaneous neuroendocrine carcinoma with a high risk of loco-regional and distant metastasis. It is predominantly seen in the elderly, on the head and neck or extremities. Although treated primarily with surgery, some patients are too frail. A World Health Organization performance status of two or more with co-existing medical co-morbidities, or the site of the disease adjacent to a critical structure, can prevent surgical management. In this cohort of patients, primary palliative radiotherapy has been found to achieve excellent tumour regression and improve quality of life. A new palliative split-course hypofractionated regime has been used in North Middlesex University Hospital in this cohort of patients. The purpose of this case series was to provide supporting evidence on the efficacy of this dose and fractionation regime and review the literature for the palliative management of Merkel cell carcinoma.Materials and methodsIn total, four patients were treated with the palliative split-course hypofractionated regime. The regime consisted of an initial 20 Gray in 5 fractions over 1 week, a 2-week gap and then a further 20 Gray in 5 fractions over 1 week. Tolerability and response to treatment were evaluated by history and clinical examination.Results and conclusionThe split-course hypofractionated regime was well tolerated, achieved excellent tumour regression and improved quality of life in all four patients. Since then, a further three patients have been successfully treated with the above regime. This case series demonstrates the efficacy of this dose and fractionation in a select group of patients too frail for radical management and adds to the evidence base for the optimal palliative management of Merkel cell carcinoma.


2018 ◽  
Vol 14 (3) ◽  
pp. 255-266 ◽  
Author(s):  
Howard L Kaufman ◽  
Matthias Hunger ◽  
Meliessa Hennessy ◽  
Michael Schlichting ◽  
Murtuza Bharmal

2014 ◽  
Vol 104 (4) ◽  
pp. 422-425 ◽  
Author(s):  
Dominic J. Roda ◽  
Brian Albano ◽  
Bharti Rathore ◽  
Linda Zhou

Merkel cell carcinoma is a rare, often fatal, neuroendocrine cutaneous malignancy with a highly variable clinical presentation. Due to the rapid progression of the disease, early detection and treatment is vital to survival. Here, we report the case of a 45-year-old woman whose podiatrist noticed an unusual lesion on her lower left leg and referred her to a dermatologist for work-up. A diagnostic excisional biopsy confirmed the diagnosis and was followed by treatment that included wide local excision of the primary lesion with subsequent chemotherapy and radiation. At the time of diagnosis, sentinel lymph node biopsy was positive. Due to the increasing prevalence of Merkel cell carcinoma in the past decade and its propensity to present on the lower extremity, podiatric physicians need to be aware of the clinical presentation and treatment guidelines for this elusive disease.


2019 ◽  
Vol 46 (8) ◽  
Author(s):  
Shiho Hanai ◽  
Takatoshi Shimauchi ◽  
Reiko Kageyama ◽  
Masahiro Aoshima ◽  
Taisuke Ito ◽  
...  

2014 ◽  
Vol 27 (9) ◽  
pp. 1182-1192 ◽  
Author(s):  
Zenggang Pan ◽  
Yuan-Yuan Chen ◽  
Xiaojun Wu ◽  
Vijay Trisal ◽  
Sharon P Wilczynski ◽  
...  

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