Letter to the Editor Merkel cell carcinoma: an illustrative case and review

Marek, Luiza; Grzanka, Alina; Chmielowska, Ewa; Jankowski, Marek; Schwartz, Robert A.; Czajkowski, Rafał

doi:10.5114/pdia.2014.40797

Cited by 7 publications

(7 citation statements)

References 24 publications

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“…The presence of chromogranin A, a neuropeptide precursor, as well as numerous neuropeptides themselves has been confirmed both in primary and metastatic MCC cells (11)(12)(13)(14) supporting the hypothesis of true Merkel cell origin of MCC. However, the expression of chromogranin A, although common, is almost exclusively focal and highly variable ranging from 5% to 80% of positive cells compared with the whole tumor's cellularity (12).…”

Section: Premisesmentioning

confidence: 67%

Merkel cell carcinoma: is this a true carcinoma?

Jankowski

Kopiński

Schwartz

et al. 2014

Experimental Dermatology

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Section: Premisesmentioning

confidence: 67%

Merkel cell carcinoma: is this a true carcinoma?

Jankowski

Kopiński

Schwartz

et al. 2014

Experimental Dermatology

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“…It is a biologically aggressive tumor with a poor prognostic outcome resulting from local, regional and distant recurrences. Older men are more commonly affected and the primary tumor is usually located on sun-exposed skin [ 1 , 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Merkel cell carcinoma usually presents as an asymptomatic rapidly enlarging flesh-colored or blue-red nodule [ 6 , 8 ]. In our patient, the tumor clinically appeared as a firm red nodule below the skin surface.…”

Section: Discussionmentioning

confidence: 99%

“…The tumor shows a positive paranuclear dot-like pattern of staining for cytokeratin 20. In addition, the immunoperoxidase profile of the tumor often includes positive staining for CD56 (neural cell adhesion molecule), chromogranin A, gastrin, and somatostatin [ 6 , 8 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…A positive sentinel lymph node may be followed by a complete lymph node dissection. Adjuvant radiotherapy is often included for patients with positive sentinel lymph nodes and considered in patients with tumors of the head and neck region that have a negative sentinel lymph node because of the higher risk of a false-negative result [ 1 , 6 – 8 ].…”

Section: Discussionmentioning

confidence: 99%

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Merkel Cell Carcinoma with a Suppressor of Fused (SUFU) Mutation: Case Report and Potential Therapeutic Implications

Cohen

Kurzrock

2015

Dermatol Ther (Heidelb)

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IntroductionMerkel cell carcinoma is a neuroendocrine malignancy. Suppressor of fused (SUFU) is a tumor suppressor oncogene that participates in the Hedgehog (Hh) signaling pathway. The aim of the study was to describe a patient whose Merkel cell carcinoma demonstrated a SUFU genomic alteration.Case StudyThe Hh signaling pathway is involved in the pathogenesis of several tumors, including nevoid basal cell carcinoma syndrome that is associated with an alteration of the patched-1 (PTCH1) gene. Targeted molecular therapy against smoothened (SMO) with vismodegib has been shown to be an effective therapeutic intervention for patients with PTCH-1 mutation. The reported patient was presented with metastatic Merkel cell carcinoma. Analysis of his tumor, using a next-generation sequencing-based assay, demonstrated a genomic aberration of SUFU protein, a component of the Hh signaling pathway that acts downstream to SMO and, therefore, is unlikely to be responsive to vismodegib. Of interest, arsenic trioxide or bromo and extra C-terminal inhibitors impact signals downstream to SUFU, making this aberration conceivably druggable. His tumor has initially been managed with chemotherapy (carboplatin and etoposide) and subsequent radiation therapy is planned.ConclusionThe pathogenesis of Merkel cell carcinoma is multifactorial, and related to ultraviolet radiation exposure, immunosuppression, and Merkel cell polyomavirus. We report a patient with a mutation in SUFU, a potentially actionable component of the Hh signaling pathway.Electronic supplementary materialThe online version of this article (doi:10.1007/s13555-015-0074-5) contains supplementary material, which is available to authorized users.

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