Merkel cell carcinoma from 2008 to 2012: Reaching a new level of understanding (original) (raw)
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Merkel cell carcinoma of skin – current controversies and recommendations
Rare Tumors, 2011
The review covers the current recommendations for Merkel cell carcinoma (MCC), with detailed discussion of many controversies. The 2010 AJCC staging system is more in-line with other skin malignancies although more complicated to use. The changes in staging system over time make comparison of studies difficult. A wide excision with margins of 2.5-3 cm is generally recommended. Even for primary </= 1 cm, there is a significant risk of nodal and distant metastases and hence sentinel node biopsy should be done if possible; otherwise adjuvant radiotherapy to the primary and nodal region should be given. Difficulties of setting up trials owing to the rarity of the disease and the mean age of the patient population result in infrequent reports of adjuvant or concurrent chemotherapy in the literature. The benefit, if any, is not great from published studies so far. However, there may be a subgroup of patients with high-risk features, e.g. node-positive and excellent performance status, for whom adjuvant or concurrent chemotherapy may be considered. Since local recurrence and metastases generally occur within 2 years of the initial diagnosis, patients should be followed more frequently in the first 2 years. However delayed recurrence can still occur in a small proportion of patients and long-term follow-up by a specialist is recommended provided that the general condition of the patient allows it. In summary, physician judgment in individual cases of MCC is advisable, to balance the risk of recurrence versus the complications of treatment.
Merkel Cell Carcinoma: Diagnosis, Management and Controversies
2000
Merkel cell carcinoma (MCC) is a frequently lethal skin cancer with a higher mortality (33%) than melanoma (15%) and evidence of rapidly increasing incidence. Management of MCC is challenging, as therapy is different in nature than for other skin malignancies and is controversial within the literature. Proper care requires coordination between dermatologists, radiation and medical oncologists, and surgeons. In this session, speakers will present the most current data on the clinical presentation, staging, pathology, and management of MCC. Representative and challenging cases will be presented to highlight treatment options and relevant data.
Latest update on the clinical features and management of Merkel cell carcinoma
Journal of the Pakistan Medical Association, 2012
Merkel cell carcinoma (MCC), is a rare, highly malignant skin tumour, with a poor prognosis. Though the aetiology of MCC is not known, but there are several features that it shares with melanoma. These include the natural history, clinical features and behaviour, e.g. an early spread to nodal sites, high local recurrence rate and early metastasis. Incidence of MCC is seen to be increased in immunosuppressed transplant patients, in patients with rheumatoid arthritis and in B cell malignancies, with a strong male predominance. Despite the ongoing research and advancement, MCC yet poses a challenge to the clinicians because of its rarity. The purpose of this paper is to review the most salient and clinically relevant updates of MCC since its first publication in July 2007 in JPMA. In order to expedite an improved understanding of the new diagnostic modalities, treatment and preventive measures, along with the new staging system established in 2009 after an extensive literature review, and an analysis of over 5,000 patients using the National Cancer Database has all been included in our article.
Merkel cell carcinoma: Critical review with guidelines for multidisciplinary management
Cancer, 2007
Merkel cell carcinoma (MCC) is a relatively rare cutaneous malignancy that occurs predominantly in the older white population. The incidence of MCC appears to have tripled during the past 20 years; an increase that is likely to continue because of the growing number of older Americans. The pathogenesis of MCC remains largely unknown. However, ultraviolet radiation and immunosuppression are likely to play a significant pathogenetic role. Many questions currently remain unanswered regarding the biologic behavior and optimal treatment of MCC. Large, prospective, randomized studies are not available and are unlikely to be performed because of the rarity of the disease. The objective of this review was to provide a comprehensive reference for MCC based on a critical evaluation of the current data. The authors investigated the importance of sentinel lymph node biopsy as a staging tool for MCC to assess the status of the regional lymph node basin and to determine the need for additional therapy to the lymph node basin. In an attempt to standardize prospective data collection with the intention to define prognostic indicators, the authors also present histopathologic profiles for primary MCC and sentinel lymph nodes. The controversies regarding the appropriate surgical approach to primary MCC, the use of adjuvant radiation therapy, and the effectiveness of adjuvant chemotherapy were examined critically. Finally, the authors have provided treatment guidelines based on the available evidence and their multidisci
A practical update of surgical management of merkel cell carcinoma of the skin
ISRN surgery, 2013
The role of surgeons in the treatment of Merkel cell carcinoma (MCC) of the skin is reviewed, with respect to diagnosis and treatment. Most of the data in the literature are case reports. Surgery is the mainstay of treatment. A wide local excision, with sentinel node (SLN) biopsy, is the recommended treatment of choice. If SLN is involved, nodal dissection should be performed; unless patient is unfit, then regional radiotherapy can be given. Surgeons should always refer patients for assessment of the need for adjuvant treatments. Adjuvant radiotherapy is well tolerated and effective to minimize recurrence. Adjuvant chemotherapy may be considered for selected node-positive patients, as per National Comprehensive Cancer Network guideline. Data are insufficient to assess whether adjuvant chemotherapy improves survival. Recurrent disease should be treated by complete surgical resection if possible, followed by radiotherapy and possibly chemotherapy. Generally results of multimodality tr...
Treatment of Locally Advanced Merkel Cell Carcinoma—A Multi-Center Study
Cancers, 2022
Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer with a high risk of recurrence and poor prognosis. The treatment of locally advanced disease involves surgery and radiotherapy. To analyze real-life treatment patterns and clinical outcomes, we conducted a retrospective analysis of data from 161 MCC patients treated with curative intent in four oncological centers in Poland. The median age at diagnosis was 72 years (30–94); 49.7% were male. Lymph node (LN) involvement at diagnosis was found in 26.9% of patients. Sentinel lymph node biopsy (SLNB) was performed in 36.5% of patients (positive in 10.5%), and 51.9% of patients received perioperative treatment. The relapse rate was 38.3%. With the median follow-up of 2.3 years, the median disease-free survival (DFS) was not reached, and the 1-year rate was 65%. The negative independent risk factors for DFS were male gender, metastases in LN at diagnosis, no SLNB in patients without clinical nodal metastases, and no perioperativ...
Merkel Cell Carcinoma: From Pathobiology to Clinical Management
Biology
Merkel cell carcinoma (MCC) is an infrequent, rapidly growing skin neoplasm that carries a greater probability of regional lymph node involvement, and a grim prognosis in advanced cases. While it is seen predominantly in old age in sun-exposed body parts, the prevalence varies among different races and geographical regions. Merkel cell polyomavirus and UV radiation-induced mutations contribute to its etiopathogenesis. The clinical presentation of MCC lacks pathognomonic features and is rarely considered highly at the time of presentation. Histopathological examination frequently reveals hyperchromatic nuclei with high mitotic activity, but immunohistochemistry is required to confirm the diagnosis. Sentinel lymph node biopsy (SLNB) and imaging are advised for effective staging of the disease. Multimodal management including surgery, radiation therapy, and/or immunotherapy are deployed. Traditional cytotoxic chemotherapies may result in an initial response, but do not result in a sign...
European Journal of Cancer, 2015
Merkel cell carcinoma (MCC) is a rare tumour of the skin of neuro-endocrine origin probably developing from neuronal mechanoreceptors. A collaborative group of multidisciplinary experts form the European Dermatology Forum (EDF), The European Association of Dermato-Oncology (EADO) and the European Organization of Research and Treatment of Cancer (EORTC) was formed to make recommendations on MCC diagnosis and management, based on a critical review of the literature, existing guidelines and expert's experience. Clinical features of the cutaneous/subcutaneous nodules hardly contribute to the diagnosis of MCC. The diagnosis is made by histopathology, and an incisional or excisional biopsy is
Clinical features and management of Merkel cell carcinoma
JPMA. The Journal of the Pakistan Medical Association, 2007
Merkel cell carcinoma (MCC) is an aggressive dermal tumour of neuroendocrine origin. MCC is a rare tumour and all information pertaining to its behaviour, therapy and prognosis is based on retrospective reports. The two potentially curative treatment modalities are surgery and radiotherapy. It is a rare, highly malignant primary skin tumour, originally called "trabecular carcinoma" of the skin. MCC poses a challenge to the clinician because of its rarity and poor prognosis. The optimal therapy is customised and tailored for each individual patient with the appropriate use of operative resection and radiation therapy. This review covers reports from several authors regarding the rationale of using postoperative radiotherapy to the primary tumour and regional lymphatics. Although MCC is classified as a type of neuroendocrine carcinoma, it is less likely to be controlled by systemic chemotherapy. Management of primary lesion with clinically localised disease is wide excision ...
Merkel cell carcinoma: Preparing to go the distance
The Malaysian journal of pathology, 2020
INTRODUCTION Merkel cell carcinoma (MCC) is a rare and aggressive malignancy of the skin, with poor clinical outcomes. Typical conditions include a rapidly growing, solitary dome-shaped, violaceous nodule. Several root causes have been identified - sun exposure, age, lighter skin, immunocompromised state, and polyomavirus infection. Wide local excision is the best treatment. The tumour is radiotherapy-responsive. However, the success rate of the treatment with chemotherapy is rather limited. Immunotherapy has shown promising results. Early detection is important to prevent morbidity and mortality. CASE REPORT In this literature work, we reported on a particular case of MCC, as exhibited by an 84-year-old Chinese woman, and discussed the clinical features and management of MCC. DISCUSSION We highlighted that MCC cases have a link to the polyomavirus 5. Patients who were identified with the Polyomavirus 5, and underwent immunotherapy, were seen to depict much better prognosis.