Neuroendocrine (Merkel cell) carcinoma of the skin

Pilotti, Silvana; Rilke, F; Lombardi, Luciano

doi:10.1097/00000478-198204000-00006

Cited by 125 publications

(41 citation statements)

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“…Both patients were free of disease at 38 months and 45 months, respectively. Other case reports of patients treated with radiation as monotherapy have been published with varying results, 12,[30][31][32][33][34] and small numbers have precluded the ability to draw any conclusions. In this series, 28 patients who received lymph node irradiation without CLND had control rates comparable to the surgical cohort for both microscopic and palpable lymph node presentations.…”

Section: Discussionmentioning

confidence: 99%

Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

Fang

Lemos

Douglas

et al. 2010

Cancer

145

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BACKGROUND:Merkel cell carcinoma (MCC) is an aggressive cutaneous malignancy with a high risk of lymph node involvement. To the authors' knowledge, few data have been published to date regarding the optimal regional therapy for lymph node‐positive patients. This cohort study was performed to analyze the outcomes of patients with lymph node‐positive MCC treated with lymph node irradiation as definitive therapy compared with completion lymphadenectomy (CLND).METHODS:Fifty patients with lymph node involvement of MCC at presentation and adequate follow‐up data were included in this analysis. Forty‐three of these patients were enrolled and followed prospectively. Twenty‐six patients presented with microscopic lymph node disease, and 24 patients presented with palpable lymph node involvement.RESULTS:Regional control for patients with microscopically involved lymph nodes was 100% regardless of treatment modality—definitive lymph node irradiation (n = 19) or CLND ± radiotherapy (n = 7) with median follow‐up of 18 months. Patients with clinically positive lymph nodes had 2‐year regional recurrence‐free survival rate of 78% and 73% in the definitive lymph node irradiation (n = 9) and CLND ± radiotherapy (n = 15) groups, respectively (P = .8) with a median follow‐up of 16 months.CONCLUSIONS:To the best of the authors' knowledge, the current study is the largest series published to date of radiation monotherapy as regional treatment for lymph node‐positive MCC. Lymph node irradiation alone to positive regional lymph nodes was found to confer an excellent regional control rate that was comparable to CLND for both microscopic and palpable lymph node disease. There was no difference noted with regard to overall survival. Given their similar efficacy, the choice between these lymph node therapies may be based on the clinical scenario and anticipated side effect profiles. Cancer 2010. © 2010 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

Fang

Lemos

Douglas

et al. 2010

Cancer

145

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“…Most authors now believe that Merkel cell carcinoma originate from pluripotent stem cells within the epidermis or adnexa. [9][10][11][12][13][14] Histologically, Merkel cell carcinomas are dermal and/or subcutaneous tumors with a typical 'undifferentiated' appearance. The tumor consists of small round basophilic cells that is arranged in syncytial sheets with high nuclear to cytoplasmic ratios, prominent mitotic activity and scattered apoptosis.…”

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confidence: 99%

Silent mutations in KIT and PDGFRA and coexpression of receptors with SCF and PDGFA in Merkel cell carcinoma: implications for tyrosine kinase-based tumorigenesis

Kartha

Sundram

2008

Modern Pathology

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Merkel cell carcinoma is a rare and aggressive form of skin cancer of neuroendocrine origin. Its treatment involves wide excision and radiotherapy but no effective therapy exists for advanced disease. Upregulation of the platelet-derived growth factor receptor family of tyrosine kinases, PDGFRA and KIT, has a crucial role in cancer development. Several studies have shown expression of the tyrosine kinase receptor KIT (CD117) in Merkel cell carcinoma. In this study, we examined the expression and mutational status of KIT and PDGFRA in 14 primary and 18 metastatic Merkel cell carcinoma. The expression of KIT and PDGFRA and their respective ligands, stem cell factor (SCF) and PDGFA, was assessed by immunohistochemistry. In addition, we analyzed KIT exons 9, 11, 13 and 17, and PDGFRA exons 10, 12 and 18 for the presence of activating mutations. We found that only 53% of cases of Merkel cell carcinoma expressed KIT, which was mostly seen as diffuse weak staining, and SCF expression was observed only in 31% of cases. In contrast, 87 and 81% of cases expressed PDGFRA and PDGFA, respectively. We observed coexpression of SCF and KIT in only 5 of 32 cases (16%) whereas 25 of 31 cases (81%) showed coexpression of PDGFRA and its ligand PDGFA. While we documented silent mutations in exon 17 of KIT and exons 10, 12 and 18 of PDGFRA, we were not able to identify any known activating mutations. Our results indicate that there is no correlation between positive immunostaining and occurrence of activating mutations in KIT and PDGFRA. Moreover, the presence of KIT/SCF and PDGFRA/PDGFA coexpression in a proportion of cases may indicate an autocrine/paracrine stimulation loop. We think therefore that imatinib mesylate is less likely to be an effective therapy for Merkel cell carcinoma, unless activating mutations exist in other exons of these receptor kinases.

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“…1,2 It was suggested originally that MCCs are derived from neural crest cells. 3 Other studies, however, favor an adnexal 4,5 or epidermal cell 4,6,7 origin, which acquires neuroendocrine features during malignant transformation.…”

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confidence: 99%

Genetic Changes Associated With Primary Merkel Cell Carcinoma

et al. 1998

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Merkel cell carcinoma (MCC) isMerkel cell carcinoma (MCC) is an uncommon malignant neoplasm of the skin with potentially aggressive biologic behavior. Histologic analysis of these tumors alone is a poor predictor of disease progression, and only clinical staging allows a prognostic assessment of the biologic behavior of this neoplasm. Although immunohistochemical and ultrastructural data show evidence of neuroendocrine differentiation, the histogenesis of this tumor remains controversial.1,2 It was suggested originally that MCCs are derived from neural crest cells.3 Other studies, however, favor an adnexal 4,5 or epidermal cell 4,6,7 origin, which acquires neuroendocrine features during malignant transformation.Cytogenetic and molecular studies have shown evidence for genetic changes on chromosome l p in different tumors with well-established neuroendocrine origins, specifically n e u r o b l a s t o m a s , 8 , 9 m a l i g n a n t

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Neuroendocrine (Merkel cell) carcinoma of the skin

Cited by 125 publications

References 0 publications

Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

Silent mutations in KIT and PDGFRA and coexpression of receptors with SCF and PDGFA in Merkel cell carcinoma: implications for tyrosine kinase-based tumorigenesis

Genetic Changes Associated With Primary Merkel Cell Carcinoma

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