Merkel Cell Carcinoma of the Skin

2000 ◽  
Vol 4 (4) ◽  
pp. 186-195 ◽  
Author(s):  
Patricia TH Tai ◽  
Edward Yu ◽  
Jon Tonita ◽  
James Gilchrist

Background: Neuroendocrine/Merkel cell carcinoma (MCC) of the skin is an uncommon tumour. Currently, there are only limited data available on the natural history, prognostic factors, and patient management of MCC. Objective: To review our experience and build the largest database from the literature. Methods: Twenty-eight cases from the London Regional Cancer Center were combined with 633 cases obtained from the literature searched in English, French, German, and Chinese for the years 1966 to 1998. The database included age, sex, initial disease status at presentation to the clinic, site of primary, any coexisting disease, any previous irradiation, sizes of primary/nodal/distant metastases, management details, and final disease status. A new modified staging system was used: stage Ia (primary disease only, size < 2 cm), stage Ib (primary disease only, size > 2 cm); stage II (regional nodal disease), and stage III (beyond regional nodes and/or distant disease). Results: Age > 65 years, male sex, size of primary > 2 cm, truncal site, nodal/distant disease at presentation, and duration of disease before presentation (< 3 months) were poor prognostic factors. Surgery was the initial treatment of choice and it significantly improved overall survival (p = .004). Conclusion: We identified poor prognostic factors that may necessitate more aggressive treatment. The suggested staging system, incorporating primary tumour size, accurately predicted outcomes.

2016 ◽  
Vol 23 (11) ◽  
pp. 3564-3571 ◽  
Author(s):  
Kelly L. Harms ◽  
Mark A. Healy ◽  
Paul Nghiem ◽  
Arthur J. Sober ◽  
Timothy M. Johnson ◽  
...  

2021 ◽  
Author(s):  
Miao Wang ◽  
Ye Qiu ◽  
Fang Tang ◽  
Yi-Xin Liu ◽  
Yi Li ◽  
...  

Abstract Background: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin cancer with increasing incidence and poor prognosis. We sought to develop and validate a nomogram to estimate overall survival (OS) of MCC patients. Methods: 1863 MCC patients between 2010-2015 from the Surveillance, Epidemiology and End Results (SEER) database were randomly divided into the training and validation cohort. Independent prognostic factors determined by Cox regression analysis in the training cohort were used to establish a nomogram. We evaluated prognostic performance using the concordance index (C-index), area under receiver operating characteristic curve (AUC) and calibration curves. Decision curve analysis (DCA), net reclassification index (NRI) and integrated discrimination improvement (IDI) were used to compared the the nomogram’s clinical utility with that of the staging system.Results: eight independent prognostic factors were incorporated in the nomogram. The C-index of the nomogram was 0.744, which was superior to the C-index of AJCC TNM Stage (0.659). The AUC was greater than 0.75 and the calibration plots of this model exhibited good performance. Additionally, the positive NRI and IDI of nomogram versus the staging system illustrated that the nomogram had better predictive accuracy than the staging system (P<0.001) and the DCA showed great clinical usefulness of the nomogram. MCC patients were perfectly classified into three risk groups by the nomogram, showing better discrimination than the staging system.Conclusions: We developed and validated a nomogram to assist clinicians in evaluating prognosis of MCC patients.


2019 ◽  
Vol 12 (2) ◽  
pp. e224834
Author(s):  
João Vasco Barreira ◽  
Margarida Moura Valejo Coelho ◽  
Catarina Ribeiro ◽  
Mónica Semedo

The authors present the case of a woman in the seventh decade of life with medical history of: left nephrectomy for renal tuberculosis and non-Hodgkin’s lymphoma treated with chemotherapy (QT) and radiotherapy. She presented with a 2-month history of non-tender, left inguinal lymph node enlargement. Positron Emission Tomography (PET)—CT —scanshowed hypermetabolic inguinal and retroperitoneal lymphadenopathies, no primary tumour. On the second dermatological examination a pink, 2 cm plaque on the anterior left knee was noted. The histopathological analysis revealed Merkel cell carcinoma. The patient underwent two lines of systemic QT, with life-threatening toxicities limiting treatment. Followed overwhelming disease progression with lymphoedema and numerous skin metastases in the left lower limb. The patient received palliative care until death. The rare incidence of such neoplasia and its uncommon clinical presentation justifies reporting this case and highlights the importance of multidisciplinary teams in the management of cancer patients.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e15696-e15696
Author(s):  
Aman Chauhan ◽  
Leaundra Murray ◽  
Arun Kumar Arumugam Raajasekar ◽  
Zin Myint ◽  
Lowell Brian Anthony

e15696 Background: Neuroendocrine tumor of unknown primary constitutes about 10-15 % of all neuroendocrine tumors. Identification of primary site can helps alter the management. Sunitinib is FDA approved for management of pancreatic neuroendocrine tumors, everolimus is approved for gastroenteropancreatic and bronchial NETs, immune checkpoint inhibitors are active in Merkel cell carcinoma and MIBG treatment is standard of care for pheochromocytoma. Methods: Patients with neuroendocrine tumor with unknown primary were identified from Markey Cancer Center database over a five-year period (2012-2016). Patient who underwent 92-gene reverse transcriptase polymerase chain reaction cancer classification assay (BioTheranostics Tissue Type ID) were analyzed. IRB approval was obtained. Results: 56 patients with neuroendocrine tumors with unknown primary were identified. Median age of cohort was 61 years. 28/56 patients were males. 92 gene cancer ID assay was used in 38 out of 56 patients. Primary site of tumor was identified with > 95% certainty in 36 out of 38 patients. The test reported pancreatic NET as the primary site for 10 patients, gastrointestinal NETs for 14 patients, bronchial carcinoid for 5, large call NEC for 3, Merkel cell carcinoma for two and pheochromocytoma in one patient. Conclusions: Tissue type ID was able to identify a primary site in NETs of unknown primary in majority (94.7%) of cases. The result had direct implication in management of patients with regards to FDA approved treatment options in 13/38 patients (pNETs, merkel cell and pheochromocytoma).


2021 ◽  
Vol 11 ◽  
Author(s):  
Marco Rastrelli ◽  
Paolo Del Fiore ◽  
Irene Russo ◽  
Jacopo Tartaglia ◽  
Alessandro Dal Monico ◽  
...  

BackgroundMerkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. The incidence of the disease has undergone a significant increase in recent years, which is caused by an increase in the average age of the population and in the use of immunosuppressive therapies. MCC is an aggressive pathology, which metastasizes early to the lymph nodes. These characteristics impose an accurate diagnostic analysis of the regional lymph node district with radiography, clinical examination and sentinel node biopsy. In recent years, there has been a breakthrough in the treatment of the advanced pathology thanks to the introduction of monoclonal antibodies acting on the PD-1/PD-L1 axis. This study aimed to describe the clinico-pathological characteristics, treatment strategies and prognostic factors of MCC.MethodsA retrospective cohort study was conducted involving 143 consecutive patients who were diagnosed and/or treated for MCC. These patients were referred to the Veneto Institute of Oncology IOV-IRCCS and to the University Hospital of Padua (a third-level center) in the period between December 1991 and January 2020. In the majority of cases, diagnosis took place at the IOV. However, some patients were diagnosed elsewhere and subsequently referred to the IOV for a review of the diagnosis or to begin specific therapeutic regimens.Results143 patients, with an average age of 71 years, were affected mainly with autoimmune and neoplastic comorbidities. Our analysis has shown that age, autoimmune comorbidities and the use of therapy with immunomodulating drugs (which include corticosteroids, statins and beta-blockers) are associated with a negative prognosis. In this sense, male sex is also a negative prognostic factor.ConclusionsAutoimmune and neoplastic comorbidities were frequent in the studied population. The use of drugs with immunomodulatory effects was also found to be a common feature of the population under examination. The use of this type of medication is considered a negative prognostic factor. The relevance of a multidisciplinary approach to the patient with MCC is confirmed, with the aim of assessing the risks and benefits related to the use of immunomodulating therapy in the individual patient.


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