Head and Neck Surgery, Department of Otolaryngology, University of Toronto, Toronto, ON, Canada M5G 2N2.
Merkel cell carcinoma (MCC) is a rare and typically aggressive form of skin cancer. It most commonly affects the elderly and has a predilection for the sun-exposed skin of the head and neck region. Other etiological factors include immune suppression, organ transplantation, and polyoma virus infection. MCC has a propensity to spread to regional lymphatics with a high locoregional recurrence rate. Since its discovery in 1972, treatment paradigms have shifted, with no consensus on optimal management strategies. Currently, standard of care includes surgical intervention to the primary and locoregional site with adjuvant radiotherapy for high-risk disease. In this paper, we discuss the history, pathology, and epidemiology of this rare disease with a focus on the evidentiary basis of treatment protocols. The use of sentinel lymph node biopsy as a management option will be the focus of this paper.
INTRODUCTION:Merkel cell carcinoma (MCC) is a rare tumour of skin. This study is a retrospective audit of patients with MCC from St Vincent's and Mater Hospital, Sydney, Australia. The aim of this study was to investigate the influence of radiotherapy (RT) on the local and regional control of MCC lesions and survival of patients with MCC.
The data bases in anatomical pathology, RT and surgery. We searched for patients having a diagnosis of MCC between 1996 and 2007. Patient, tumour and treatment characteristics were collected and analysed. Univariate survival analysis of categorical variables was conducted with the Kaplan-Meier method together with the Log-Rank test for statistical significance. Continuous variables were assessed using the Cox regression method. Multivariate analysis was performed for significant univariate results.
Sixty seven patients were found. Sixty two who were stage I-III and were treated with radical intent were analysed. 68% were male. The median age was 74 years. Forty-two cases - sixty eight percent - were stage I or II, and 20 cases - thirty two percent - were stage III. For the subset of 42 stage I and II patients, those that had RT to their primary site had a 2-year local recurrence free survival of 89% compared with 36% for patients not receiving RT. The cumulative 2-year regional recurrence free survival for patients having adjuvant regional RT was 84% compared with 43% for patients not receiving this treatment . Immune status at initial surgery was a significant predictor for OS and MCCSS. In a multivariate analysis combining macroscopic size (mm) and immune status at initial surgery, only immune status remained a significant predictor of overall survival .
RT is associated with significant improvement in local and regional control in Merkel cell carcinoma. Immunosuppression is an important factor in overall survival.
Department of Pathology and Immunology, Division of Anatomic and Molecular Pathology, Washington University, Saint Louis, MO, United States. Electronic address: PCimino@path.wustl.edu.
Chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL) is the most common B-cell leukemia/lymphoma, effecting >15,000 patients/year. There has been a proposed limited antigenic etiology, at least in some cases, of CLL/SLL based upon immunoglobulin heavy chain stereotypy found across unrelated cases, suggesting viral source may provide such antigenic stimulation. With an established epidemiological link between CLL/SLL and Merkel cell carcinoma (MCC), there has been some interest in investigating a possible leukemogenic role of Merkelcell polyomavirus (MCPyV), which is found in 80% of MCC cases. Recent studies have shown that MCPyV is present in lymphocytes in a small percentage of CLL/SLL cases, but the specific tropism for lymphocytes has not been well-established. In this study, we used quantitative PCR to investigate the presence of MCPyV in fluorescence activated cell sorted purified B- and T-cells from 23 CLL/SLL cases. Three of 23 cases (13%) had detectable MCPyV in T-cells, and none of the cases had detectable MCPyV in B-cells. These findings suggest that MCPyV may have tropism for T-cells in addition to previously reported neoplastic B-cells.
Department of Oral and Maxillofacial Surgery, Helsinki University Central Hospital, P.O. Box 263, Kasarminkatu 11-13, 00029 HUS, Helsinki, Finland, firstname.lastname@example.org.
Merkel cell carcinoma (MCC) is a rare cutaneous neuroendocrine carcinoma. The aims of this study were to investigate the expression of the transcription factors B-lymphoma Moloney murine leukaemia virus insertion (BMI1), myelocytomatosis viral oncogene homologue (c-Myc) and Snail in MCC tumour specimens and to examine the relationship of these markers toMerkelcell polyoma virus (MCV). The study comprised of 133 patients with primary MCC. The expression of BMI1, Snail and c-Myc protein was assessed by immunohistochemistry and compared with clinical parameters, MCV status and patient survival. The presence of MCV was inversely correlated with the expression of BMI1 protein. Tumours expressing BMI1 protein more often presented with lymph node metastases. Snail protein expression was decreased in cases with metastatic dissemination. This study identified two subgroups of MCC: tumours expressing BMI1 but negative for MCV DNA and tumours negative for BMI1 expression but positive for MCV. Importantly, BMI1-positive cases often presented with lymph node metastases. Combined, these results suggest that subtypes of this malignancy exist.
Department of Oral and Maxillofacial Surgery, University Hospital Olomouc, Czech Republic.
BACKGROUND:Merkel cell carcinoma (MCC) is a rare potentially fatal skin tumour affecting older mainly white people and younger immunosuppressed individuals. While uncommon, the incidence is increasing relative to melanoma and with twice the lethality. The benign appearance of the tumour usually on exposed skin parts, contrasting with its extensive microscopic invasion, can delay timely diagnosis. Recurrent MCC is currently attributed to the recently discovered Merkelcellpolyomavirus This brief review of MCC covers the history, epidemiology,etiology,clinical and histological features, treatment and prognosis.
METHODS:Literature search using PubMed and search words Merkel cell carcinoma (MCC), etiology, treatment for the years 1972 to 2010.
Results and conclusion.Merkel cell carcinoma is a rare malignancy with uncertain prognosis. Due to the uncommon occurrence and dearth of randomized studies, there is no agreement on optimal treatment. The tumor has only recently been included in the international classification of tumors (NCCN). The treatment approaches found to be best are radical surgery of primary tumor, drainage of lymph node extension and possibly adjuvant loco-regional radiotherapy. The basis of successful treatment however, remains prevention regular dermatological examination in immunosuppressed patients and early initiation of combination therapy, based on radical surgery supplemented by radiotherapy and palliative chemotherapy in the last resort.
Am old enough to understand the difference between the Bay of Pigs - and roasting a pig at a epicurian feast. Been thru the hippy, yippie and yuppie years - always remaining who I am.
Very much believe in "Sing your own song - weave your own tapestry"
Am young enough to still know the thrill of new discoveries, the beauty of the evening, to celebrate the joy of another tommorow.
Survived these many decades with a severe medical problems. Sorting out the maze of now having two lymphomas and all their nasty little companions, but I continue.
Besides, being a simple iconoclastic eclectic, have been called many things. An incurable romanticist - with a strong touch of reality. Thinker, intellectual (God, how I hate that term) - been told I am a lion with the heart of the poet.
Know how to wage war and conquer my foes - but would rather be known as one who brings hope and life. To bring hope into anothers life is the ultimate of joys.
Life should be about bringing hope, peace, vision... a sense of purpose beyond yourself.