Surgical Therapy of Merkel Cell Carcinoma-Single Centre Experience of 157 Patients in Germany.
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ABSTRACT:
SUBMITTER: Heister M
PROVIDER: S-EPMC10908190 | biostudies-literature | 2024 Jan-Feb
REPOSITORIES: biostudies-literature
Publications
Surgical Therapy of Merkel Cell Carcinoma-Single Centre Experience of 157 Patients in Germany.
Heister Martin M Häfner Hans-Martin HM Neuner Julian J Kofler Katrin K Schulz Claudia C Kofler Lukas L
Journal of cutaneous medicine and surgery 20240116 1
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Project description:BackgroundMerkel cell carcinoma (MCC) is a rare but highly aggressive neuroendocrine skin malignancy, with Australia having the highest reported incidence in the world. There is currently a lack of consensus regarding optimal management of this disease.MethodsThis was a retrospective audit conducted by reviewing existing medical records of MCC patients presenting to the Peter MacCallum Cancer Centre (PMCC) between 1980 and 2018. The primary endpoint was locoregional recurrence. The secondary endpoints were distant recurrence, disease-free survival (DFS) and overall survival (OS).ResultsA total of 533 patients were identified. Locoregional recurrence occurring at one, two and 5 years was 24, 31 and 32%, respectively. The estimated 5-year OS and DFS were 46% (95% Confidence Interval [CI] 41-51%) and 34% (95% CI 30-39%) respectively. Older age at diagnosis (hazard ratio [HR] per year = 1.07, 95% CI 1.06-1.07, p < 0.001), and larger primary tumour diameter (HR =1.16, 95% CI 1.03-1.31, p = 0.019) were associated with worse OS on multivariable analysis. Positive or negative histopathological margin status was not associated with OS or DFS differences in patients treated with post-operative radiotherapy.ConclusionsIn our study, about a third of patients developed locoregional recurrence, distal recurrence or both, and there appears to be no change over the last four decades. If treated with adjuvant radiotherapy, there is no difference in OS or DFS with positive surgical margins. Findings should influence future guidelines.
| S-EPMC9826569 | biostudies-literature
Project description:Merkel cell carcinoma (MCC) is an aggressive, polyomavirus-associated cancer with limited therapeutic options for metastatic disease. Cytotoxic chemotherapy is associated with high response rates, but responses are seldom durable and toxicity is considerable. Here, we report our experience with palliative single-fraction radiotherapy (SFRT) in patients with metastatic MCC. We conducted retrospective analyses of safety and efficacy outcomes in patients that received SFRT (8 Gy) to MCC metastases between 2010 and 2013. Twenty-six patients were treated with SFRT to 93 MCC tumors located in diverse sites that included skin, lymph nodes, and visceral organs. Objective responses were observed in 94% of the measurable irradiated tumors (86/92). Complete responses were observed in 45% of tumors (including bulky tumors up to 16 cm). "In field" lesion control was durable with no progression in 77% (69/89) of treated tumors during median follow-up of 277 days among 16 living patients. Clinically significant toxicity was seen in only two patients who had transient side effects. An exploratory analysis suggested a higher rate of in-field progression in patients with an immunosuppressive comorbidity or prior recent chemotherapy versus those without (30% and 9%, respectively; P = 0.03). Use of SFRT in palliating MCC patients was associated with an excellent in field control rate and durable responses at treated sites, and with minimal toxicity. SFRT may represent a convenient and appealing alternative to systemic chemotherapy for palliation, for which most patients with oligometastatic MCC are eligible. SFRT may also synergize with emerging systemic immune stimulants by lowering tumor burden and enhancing presentation of viral/tumor antigens.
| S-EPMC4559027 | biostudies-literature
Project description:Merkel cell carcinoma (MCC) is an aggressive neuroendocrine tumor of the skin with a rising incidence. MCC has metastatic potential regardless the size of the primary tumor and a 5-year disease associated mortality rate is 46 %. Surgery and radiation are the mainstays of management for primary MCC. There is no evidence-based effective chemotherapy for recurrent or metastatic diseases to date. In-depth mechanistic studies in MCC have uncovered important cellular events and the association with a polyomavirus, which has provided direct evidence for molecular targeted and immunotherapy. Further perspective studies and clinical trials are warranted to provide reliable evidence of possible pitfalls and effectiveness of molecular targeted immunotherapy alone or in combination with chemotherapy in MCC.
| S-EPMC3931972 | biostudies-literature
Project description:BackgroundMerkel cell carcinoma (MCC) is an aggressive cutaneous malignancy with poor prognosis. Limited data exists to guide treatment decisions. Here we report on our institutional experience and outcomes treating patients with MCC.MethodsA database search (1984-2014) of patients treated at the University of Wisconsin Hospital and Clinics was used to identify patients with histologically confirmed MCC. Patient, tumor, and treatment characteristics were examined via review of medical records. Statistical analyses were performed to assess outcomes and associated prognostic factors.ResultsA total of 87 patients with MCC were identified with a median follow-up of 17 months (mean: 38, range: 0-210 months). Two and five-year overall survival rates were 53.9% and 32.8%, respectively. Recurrence was documented in 31.0% of patients (85.2% locoregional, 48.1% distant and 33.3% both). Patients with a history of immunosuppression exhibited significantly worse survival (hazard ratio, 2.01; 95% CI, 1.1-3.7) when compared to immune-competent individuals. The head and neck region was the most common location of primary lesion (N=49) followed by the extremities (N=31). Upper extremity primaries predicted significantly better overall survival (hazard ratio, 0.48; 95% CI, 0.23-0.99) while lower extremity primaries did not have significantly better results (hazard ratio, 0.5; 95% CI, 0.21-1.2) in comparison to head and neck site of primary. Nodal involvement (hazard ratio, 2.95; 95% CI, 1.5-5.79) was also a negative prognostic factor associated with poor overall survival when compared with clinically node negative patients. Primary tumor size > 2 cm (hazard ratio, 1.76; 95% CI, 0.91-3.4) was not associated with survival.ConclusionsThis study highlights the role of various factors in determining prognosis of Merkel cell carcinoma; history of immunosuppression, nodal involvement, and head/neck primary predicted worse overall survival. These findings suggest that improvements in both distant and locoregionally directed therapies might play an important role in control of MCC and identify areas for future study.
| S-EPMC4460120 | biostudies-literature
Project description:Several molecular imaging modalities have been evaluated in the management of Merkel cell carcinoma (MCC), a rare and aggressive tumor with a high tendency to metastasize. Continuous progress in the field of molecular imaging might improve management in these patients. The authors review the current modalities and their impact on MCC in this brief review article.
| S-EPMC4074814 | biostudies-literature
Project description:Merkel cell polyomavirus (MCPyV) causes the highly aggressive and relatively rare skin cancer known as Merkel cell carcinoma (MCC). MCPyV also causes a lifelong yet relatively innocuous infection and is one of 14 distinct human polyomaviruses species. Although polyomaviruses typically do not cause illness in healthy individuals, several can cause catastrophic diseases in immunocompromised hosts. MCPyV is the only polyomavirus clearly associated with human cancer. How MCPyV causes MCC and what oncogenic events must transpire to enable this virus to cause MCC is the focus of this essay.This article is part of the themed issue 'Human oncogenic viruses'.
| S-EPMC5597743 | biostudies-literature
Project description:Viruses are the cause of approximately 15% of all human cancers. Both RNA and DNA human tumor viruses have been identified, with Merkel cell polyomavirus being the most recent one to be linked to cancer. This virus is associated with about 80% of Merkel cell carcinomas, a rare, but aggressive cutaneous malignancy. Despite its name, the cells of origin of this tumor may not be Merkel cells. This review provides an update on the structure and life cycle, cell tropism and epidemiology of the virus and its oncogenic properties. Putative strategies to prevent viral infection or treat virus-positive Merkel cell carcinoma patients are discussed.
| S-EPMC7407210 | biostudies-literature
Project description:We conducted this study to evaluate the demography, clinical presentation, management and outcomes of medullary thyroid carcinoma (MTC) from the Indian context. This was a retrospective study of patients with MTC managed between January 2008 and December 2016. All pertinent data was collected and the results were analysed using STATA (v.13.1). MTC accounted for 90/2022 (4.45%) patients managed with thyroid cancer during the study period. The mean age of presentation was 40 years (range 14-70 years) with 47 males and 43 females. The most common presentation included goitre with cervical lymphadenopathy seen in 60 patients (66.7%). There were 11 patients (12.2%) with systemic metastasis at presentation. Rearranged during transfection (RET) testing was performed in 71 patients and was positive in 25 (35.2%). The mutations among these patients were seen in the following codons: 634 (12), 804 (8), 790 (3) and 618 (2). Persistent hypercalcitoninemia (calcitonin >?50 pg/ml) was observed in 62/80 (77.5%) patients. Forty patients underwent a meta-iodo-benzyl-guanidine (MIBG) scan in the postoperative period, 10 were positive. The mean duration of follow-up was 32 months and 10 patients defaulted from follow-up. Sixteen patients developed metastasis during the period of follow-up while eight patients expired. The mean survival was 85.75 months (95% CI 78.7-92.7). MTC accounted for 4.5% of thyroid carcinomas in this cohort among which 35% were hereditary. Persistent hypercalcitoninemia following surgery is seen in more than 70% of patients but this does not affect survival. RET screening should be performed for all patients with MTC as curative surgery can be offered for mutation positive offspring.
| S-EPMC5856701 | biostudies-literature
Project description:IntroductionThe National Comprehensive Cancer Network (NCCN) recommends excision of the primary tumor using 1-2-cm surgical margins and sentinel lymph node biopsy (SLNB) as the initial management of early-stage Merkel cell carcinoma (MCC). However, there is no clear consensus on the appropriate size of the surgical margins and/or the use of Mohs micrographic surgery (MMS). Our aim was to demonstrate that, independent of the type of surgery, obtaining negative surgical margins is associated with enhanced overall survival (OS).MethodsA retrospective study was performed using early-stage MCC patients from the National Cancer Database (NCDB) who were diagnosed between 2004 and 2020 and underwent surgical excision (SE) of their primary tumor. Patients were stratified into three groups based on the surgical treatment they received: < 1 cm excision margin, ≥ 1 cm excision margin, or MMS. OS was assessed with Kaplan-Meier curves, log-rank tests, and multivariable risk-adjusted Cox proportional-hazards regression.ResultsOf the 4,607 patients included in this study; 53% underwent SE of ≥ 1 cm (n = 2,474), 41% had SE < 1 cm (n = 1,905), and the remainder had MMS (n = 228). 75% of patients had an SLNB, and 56% received adjuvant radiation to the primary site and/or nodal basin. While no difference in OS was observed between the three surgical treatments, negative surgical margins (hazard ratio (HR) 0.78; 95% confidence interval (CI) 0.65-0.94) and receipt of radiation to the primary site (HR 0.82; 95% CI 0.73-0.92) were both independently associated with improved OS.ConclusionAchieving negative surgical margins is associated with improved OS in MCC, suggesting that MMS and conventional excision are both suitable approaches for the treatment of primary MCC.
| S-EPMC11909352 | biostudies-literature
Project description: Not available
| S-EPMC2603124 | biostudies-literature