Merkel Cell Carcinoma: A Population Analysis on Survival

Sridharan, Vishwajith; Muralidhar, Vinayak; Margalit, Danielle N.; Tishler, Roy B.; DeCaprio, James A.; Thakuria, Manisha; Rabinowits, Guilherme; Schoenfeld, Jonathan D.

doi:10.6004/jnccn.2016.0134

Cited by 63 publications

(77 citation statements)

References 21 publications

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“…At the time of initial diagnosis, up to 20 % of patients present with clinically manifest lymphogenic metastases and thus have a poor prognosis . In addition, almost one‐third of the patients have lymphogenic, clinically inapparent micrometastases . It is therefore imperative that patients be worked up for locoregional spread at the time of initial diagnosis, either by lymph node ultrasound of the draining lymph node basins and/or cross‐sectional imaging.…”

Section: Diagnosismentioning

confidence: 99%

“…It is currently unclear whether therapeutic lymphadenectomy following detection of micrometastases in the sentinel node will prolong overall survival . Until the answer to this question has been further investigated in controlled prospective trials, physicians will have to advise their patients on a case‐by‐case basis.…”

Section: Surgical Proceduresmentioning

confidence: 99%

“…Distant metastasis, too, is usually observed within the first two years of diagnosis. As the majority of MCC‐specific deaths occur within the first five years after the diagnosis, patients are usually followed up for a period of five years .…”

Section: Follow‐upmentioning

confidence: 99%

“…While the prognosis of patients with MCC strongly depends on the disease stage, large epidemiological studies have also shown significant regional differences. Five‐year survival rates of patients with primary tumors < 2 cm are between 66 % and 75 %; for primary tumors > 2 cm, between 50 % and 60 %; for individuals with nodal involvement, between 42 % and 52 %; and for patients with distant metastatic disease, between 17 % and 18 % .…”

Section: Introductionmentioning

confidence: 99%

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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: Surgical Proceduresmentioning

confidence: 99%

Section: Follow‐upmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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 Prognose des MCC hängt stark vom Stadium der Erkrankung ab; allerdings zeigen größere epidemiologische Studien auch starke regionale Unterschiede. Die Fünfjahresüberlebensraten für Primärtumoren mit einem Durchmesser < 2 cm liegt zwischen 66 % und 75 %, für Primärtumoren mit einem Durchmesser > 2 cm zwischen 50 % und 60 %, für Patienten mit Lymphknotenmetastasen zwischen 42 % und 52 %, und für Patienten mit Fernmetastasen zwischen 17 % und 18 % .…”

Section: Allgemeinesunclassified

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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Identifying an Optimal Adjuvant Radiotherapy Dose for Extremity and Trunk Merkel Cell Carcinoma Following Resection

Patel¹,

Qureshi

Sahni

et al. 2017

JAMA Dermatol

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IMPORTANCE Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine neoplasm with a high risk of recurrence following resection. Despite a rising incidence over the past 3 decades, there is a paucity of prospective data owing to the rarity of this disease.OBJECTIVE To determine the optimal adjuvant radiation therapy (RT) dose following resection of localized MCC of the extremities or trunk. DESIGN, SETTING, AND PARTICIPANTSUsing the National Cancer Database, a large national database consisting of a heterogeneous population and treatment settings, we retrospectively analyzed a cohort of 2093 patients 18 years or older with stage I to III MCC of the extremities and/or trunk treated with definitive surgery and adjuvant RT between 1998 and 2011. Exclusion criteria included receiving treatment with palliative intent, preoperative RT, non-external-beam RT, and radiation dose of 30 Gy or lower or 70 Gy or higher. Cox proportional hazards regression model was used to compare overall survival (OS) between RT dose groups, accounting for age, sex, race, stage, surgery type, margin status, comorbidities, and use of chemotherapy.EXPOSURES Radiation therapy dose was categorized into 4 groups: group 1 received the lowest dose (>30 to <40 Gy); group 2, the next lowest (40 to <50 Gy); group 3, the second highest dose (50 to 55 Gy); and group 4, the highest dose (>55 to 70 Gy). MAIN OUTCOME AND MEASURE Overall survival.RESULTS Data from 2093 patients were analyzed; there were 1293 men (61.8%) and 800 women (38.2%) (median age, 73 years). After a median follow-up of 37 months for the entire cohort, 904 deaths were reported. The 3-year OS rates for groups 1, 2, 3, and 4 were 41.8%, 69%, 69.2%, and 66%, respectively (omnibus P < .001). Compared with group 3 (50 to 55 Gy), equivalent OS was seen in group 2 (40 to <50 Gy; adjusted hazard ratio [AHR], 0.89; 95% CI, 0.63-1.27; P = .52) and group 4 (>55 to 70 Gy; AHR, 1.18; 95% CI, 0.93-1.48; P = .17), but worse OS was found in group 1 (>30 to <40 Gy; AHR, 2.63; 95% CI, 1.44-4.80; P < .001).

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Merkel Cell Carcinoma: A Population Analysis on Survival

Cited by 63 publications

References 21 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

Identifying an Optimal Adjuvant Radiotherapy Dose for Extremity and Trunk Merkel Cell Carcinoma Following Resection

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