Merkel Cell Carcinoma: An Update of Key Imaging Techniques, Prognostic Factors, Treatment, and Follow-up

Llombart, B.; Kindem, S.; Chust, M.

doi:10.1016/j.adengl.2016.12.003

Cited by 20 publications

(29 citation statements)

References 70 publications

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“…While current data calls for using 18 F‐FDG PET/CT as primary modality , it is important to ascertain in advance whether this procedure will be covered by health insurance funds in an outpatient setting. Should this imaging technique not be available, the initial diagnostic workup shall include CT of the thorax and abdomen as well as cranial MRI .…”

Section: Diagnosismentioning

confidence: 99%

“…Apart from skin and soft tissues, distant hematogenous spread primarily affects bones, lungs, liver, and brain . Given the markedly higher sensitivity for cerebral metastasis, the workup should, if possible, include magnetic resonance imaging of the neurocranium; if this is not feasible, computed tomography is to be used . Given the higher sensitivity for bone metastases and the frequent association of MCC with osseous spread, the workup should, if possible, include 18 F‐FDG PET/CT .…”

Section: Diagnosismentioning

confidence: 99%

“…Die aktuelle Datenlage spricht für den primären Einsatz der 18 F-FDG-PET/CT [8,[50][51][52][53][54][55][56], allerdings sollte die Kostenübernahme im ambulanten Bereich vorab geklärt werden. Sofern dies nicht zur Verfügung steht, soll die initiale Ausbreitungsdiagnostik ein CT-Thorax/Abdomen und MRT-Schädel beinhalten [57].…”

Section: Ausbreitungsdiagnostik Bei Erstdiagnoseunclassified

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S2k guidelines for Merkel cell carcinoma (MCC, neuroendocrine carcinoma of the skin) – update 2018

Becker

Eigentler

Frerich

et al. 2019

J Deutsche Derma Gesell

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Summary Merkel cell carcinoma (MCC, ICD‐O M8247 / 3) is a rare malignant primary skin tumor with epithelial and neuroendocrine differentiation. The neoplastic cells share many morphological, immunohistochemical and ultrastructural characteristics with Merkel cells of the skin. The diagnosis of MCC is rarely made on clinical grounds. Histological and immunohistochemical studies are usually required to confirm the clinical suspicion. Given the frequent occurrence of occult lymph node metastasis, sentinel lymph node biopsy should be performed once distant metastasis has been ruled out by cross‐sectional imaging. Primary tumors without evidence of organ metastases are treated with complete surgical excision with appropriate surgical margins. Radiation therapy should be considered at all stages of the disease. For advanced MCC that is no longer amenable to curative treatment by surgery or radiation therapy, there is currently no established systemic therapy for which an improvement in recurrence‐free survival or overall survival has been demonstrated in a prospective randomized trial. However, immunotherapy using PD‐1/PD‐L1 blockade seems to be superior to chemotherapy. Various factors warrant that further diagnostic and therapeutic interventions be determined by an interdisciplinary tumor board. These factors include the tumor's aggressiveness, the frequent indication for sentinel lymph node biopsy along with the frequent occurrence in the head and neck region, the potential indication for adjuvant radiation therapy as well as the complexity of the required diagnostic workup.

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Section: Diagnosismentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Ausbreitungsdiagnostik Bei Erstdiagnoseunclassified

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S2k guidelines for Merkel cell carcinoma (MCC, neuroendocrine carcinoma of the skin) – update 2018

Becker

Eigentler

Frerich

et al. 2019

J Deutsche Derma Gesell

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“…Die aktuelle Datenlage spricht für den primären Einsatz der 18 F‐FDG‐PET/CT , allerdings sollte die Kostenübernahme im ambulanten Bereich vorab geklärt werden. Sofern dies nicht zur Verfügung steht, soll die initiale Ausbreitungsdiagnostik ein CT‐Thorax/Abdomen und MRT‐Schädel beinhalten .…”

Section: Diagnostikunclassified

“…Bei einer hämatogenen Fernmetastasierung sind neben Haut und Weichteilen vorwiegend Knochen, Lunge, Leber, und Gehirn betroffen . Aufgrund der deutlich höheren Sensitivität für eine zerebrale Metastasierung sollte die Ausbreitungsdiagnostik wenn möglich mittels Magnetresonanztomographie des Neurocraniums erfolgen; falls dies nicht möglich ist, ist sie mittels Computertomographie durchzuführen . Aufgrund der höheren Sensitivität für Knochenmetastasen bei häufiger ossärer Metastasierung des MCC sollte die Ausbreitungsdiagnostik möglichst mittels 18 F‐FDG PET/CT erfolgen .…”

Section: Diagnostikunclassified

S2k‐Leitlinie Merkelzellkarzinom (MZK, MCC, neuroendokrines Karzinom der Haut) – Update 2018

Becker

Eigentler

Frerich

et al. 2019

J Deutsche Derma Gesell

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Zusammenfassung Das Merkelzellkarzinom (MCC, ICD‐O M8247/3) ist ein seltener, maligner, primärer Hauttumor mit epithelialer und neuroendokriner Differenzierung. Die Tumorzellen teilen viele morphologische, immunhistochemische und ultrastrukturelle Eigenschaften mit den Merkelzellen der Haut. Die Diagnose MCC wird selten klinisch gestellt. Erst histologisch und immunhistochemisch wird der Verdacht auf ein MCC bestätigt. Nachdem eine Fernmetastasierung durch Bildgebung mit Schnittbildverfahren ausgeschlossen worden ist, sollte aufgrund der hohen Zahl von okkulten Lymphknotenmetastasen eine Schildwächterlymphknotenbiopsie erfolgen. Bei Primärtumoren ohne Hinweise auf das Vorliegen von Organmetastasen soll eine vollständige chirurgische Exzision mit Sicherheitsabstand erfolgen. Die strahlentherapeutische Behandlung sollte in allen Stadien der Tumorerkrankung in Betracht gezogen werden. Für das fortgeschrittene, operative oder strahlentherapeutische nicht mehr kurativ behandelbare MCC gibt es bisher keine etablierte systemische Therapie, für die in einer prospektiv‐randomisierten Studie eine Verbesserung des rezidivfreien Überlebens oder des Gesamtüberlebens demonstriert werden konnte. Eine Immuntherapie mittels PD‐1/PD‐L1‐Blockade erscheint aber einer Chemotherapie überlegen zu sein. Aufgrund seiner Aggressivität, der in der Regel gegebenen Indikation zur Schildwächterlymphknotenbiopsie bei gleichzeitig häufiger Lokalisation im Kopf‐Hals‐Bereich, der zu evaluierenden Indikation einer adjuvanten Radiotherapie sowie der Komplexität der notwendigen Diagnostik zur korrekten Prognoseabschätzung soll das Vorgehen im Rahmen einer interdisziplinären Zusammenarbeit (interdisziplinäres Tumorboard) festgelegt werden.

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Merkel cell carcinoma

Mistry

Levell

Craig

et al. 2021

Skin Health and Disease

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Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma. The cellular origin of MCC may include Merkel cell precursors. The incidence of MCC has increased significantly however trends may have been confounded by evolving diagnostic criteria. The two key aetiologies of MCC are ultraviolet radiation and Merkel cell polyoma virus (MCPyV). Both have unique mechanisms of carcinogenesis. MCC presents non-specifically as a rapidly growing, red-to-violet nodule on sun-exposed areas. Diagnostic accuracy has improved through immunohistochemical markers such as CK-20. Lymph nodes should be evaluated in MCC through examination and sentinel biopsy. USS, CT, MRI and CT-PET may be useful in staging. Management depends on tumour location, stage and comorbidities. MCPyV status may guide treatment strategy in the future. Treatment for the primary MCC is commonly wide local excision followed by radiotherapy, guided by anatomical constraints. There is uncertainty about surgical margins. Treatments for nodal disease have not been determined through trials. They include nodal dissection or radiotherapy for clinically or radiologically apparent disease, and adjuvant nodal irradiation for negative nodes, microscopic disease or following nodal dissection for definite disease. Patients with loco-regional advanced inoperable disease should be considered for combination therapy including chemotherapy, radiotherapy, surgery and immunotherapy. Systemic therapy for advanced disease includes immune checkpoint inhibitors targeting the PD-1/PD-L1 pathway. Avelumab can improve survival in metastatic MCC. Immunotherapy may result in longer disease control. Various other immunotherapeutic and molecular agents are undergoing trials. MCC continues to have a high mortality characterized by high recurrence and early metastases. | INTRODUCTIONThis review article presents a concise update of knowledge in these areas relevant to the clinician. This review will discuss the pathogenesis, epidemiology, diagnosis, staging, treatment and prognosis of Merkel cell carcinoma (MCC). | PATHOGENESISMCC is a rare, aggressive neuroendocrine carcinoma first described in Baltimore, USA by Cyril Toker in 1972. 1 Merkel cells are postmitotic neuroendocrine cells in the basal epidermis which secrete amine and polypeptide hormones. Despite the name, it is unlikelyThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Merkel Cell Carcinoma: An Update of Key Imaging Techniques, Prognostic Factors, Treatment, and Follow-up

Cited by 20 publications

References 70 publications

S2k guidelines for Merkel cell carcinoma (MCC, neuroendocrine carcinoma of the skin) – update 2018

S2k guidelines for Merkel cell carcinoma (MCC, neuroendocrine carcinoma of the skin) – update 2018

S2k‐Leitlinie Merkelzellkarzinom (MZK, MCC, neuroendokrines Karzinom der Haut) – Update 2018

Merkel cell carcinoma

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