First description of Merkel Cell polyomavirus DNA detection in a patient with Stevens-Johnson syndrome.

May 2013

Maximova N, Granzotto M, Kiren V, Zanon D, Comar M.

Source

Institute for Maternal and Child Health, IRCCS Burlo Garofolo, University of Trieste, Trieste, Italy.

Abstract

Merkel Cell polyomavirus (MCPyV), a ubiquitous DNA tumor virus, has been found to be associated with Merkel cell carcinoma and chronic lymphocytic leukaemia while other associations are still being explored. MCPyV sequences have also been detected in normal tissues of tumor patients and in the blood of healthy donors. This report documents a new MCPyV association with the Stevens-Johnson syndrome, a rare immune-modulated mucocutaneous process particularly associated with specific drugs and infective agents. A high MCPyV viral load was detected simultaneously in fluid from skin lesions (2.0 × 10(4) copies/ml) and in matched blood (7.4 × 10(5) copies/ml) from a young adult patient after bone marrow transplant for a relapsed T-cell acute lymphatic leukaemia. MCPyV clearance concurred with the complete resolution of skin lesions after 5 days of cidofovir treatment. DNA sequencing classified the amplicons as the European/Italian MKL-1 strain. Given its ubiquitous nature, MCPyV could account for part of Stevens-Johnson syndrome idiopathic cases. J. Med. Virol. 85:918-923, 2013.

Wiley Online

Merkel cell carcinoma with divergent differentiation: histopathological and immunohistochemical study of 15 cases with PCR analysis for Merkel cell polyomavirus.

Apr 2013

Martin B, Poblet E, Rios JJ, Kazakov D, Kutzner H, Brenn T, Calonje E.

Source

Dermatopathology Department, St John's Institute of Dermatology, St Thomas' Hospital, London, UK.

Abstract

AIMS:

To report on 15 cases of Merkel cell carcinoma (MCC) with divergent differentiation, to characterize its clinicopathological spectrum and its relationship with Merkel cell polyomavirus (MCV).

METHODS AND RESULTS: Fifteen patients with a mean age of 81 years were included. Follow-up was available for 13 cases (range 12 days to 6 years; median 6 months). Recurrence, metastasis and mortality rates were 15.4%, 53.8% and 61.5%, respectively. All tumours showed the typical histological and immunohistochemical features of MCC, with at least one additional divergent component. Eight cases had a single aberrant component (squamous in six cases, follicular in one case, and porocarcinoma in one case), six cases had two aberrant components (squamous and sarcomatous in three cases, glandular and squamous in two cases, and sarcomatous and neuroblastic in one case), and one case had three aberrant components (glandular, squamous, and sarcomatous). All cases had dysplastic changes in the overlying epithelium, and four of 15 showed epidermotropism. PCR analysis for Merkel cell polyomavirus (MCV) gave negative results in all 12 cases tested.

CONCLUSIONS:

Merkel cell carcinoma with divergent differentiation is a highly aggressive tumour that might be difficult to recognize, owing to its wide histological variability. Negativity for MCV suggests that the virus is not implicated in the development of this subtype of MCC.

Wiley Online Library

Sentinel lymph node in Merkel cell carcinoma: To biopsy or not to biopsy?

Feb 2013

Sattler E, Geimer T, Sick I, Flaig MJ, Ruzicka T, Berking C, Kunte C.

Source

Department of Dermatology and Allergology, Ludwig-Maximilian University of Munich, Munich, Germany.

Abstract

Sentinel lymph node biopsy (SLNB) is commonly recommended for patients with primary Merkel cell carcinoma (MCC). However, it is critically discussed whether survival rates improve by SLNB in MCC patients in general or in subgroups of higher risk (e.g. with primary tumor size >1 cm). The present study correlates clinical data, histology and lymph node status with follow-up and survival data to see if subgroups can be identified for modification of the current recommendations. The medical records of 47 patients with histologically confirmed MCC treated between 1995 and 2010 at a German dermatosurgery department were reviewed. Nineteen patients with excision of the primary tumor and SLNB were compared to 28 patients with excision of the primary tumor but without SLNB. End-points of this study were disease-free survival (DFS) and overall survival (OS). In addition, clinical course was correlated with tumor size and size of safety margin. The group of patients who received SLNB showed a significant advantage in terms of OS (P < 0.05), but not in terms of DFS. Tumors of smaller size were associated with a significantly better DFS and a trend towards better OS. Comparing the groups with different safety margins (1-3 cm), no differences in DFS and OS could be found. Our data support the current recommendation for SLNB in all MCC patients and question the use of extensive safety margins in MCC surgery. Larger prospective multicenter studies with multivariate analysis are needed to confirm whether a prolonged OS is really due to the SLNB procedure or biased by other factors.

PubMed

Merkel cell carcinoma: interdisciplinary management of a rare disease.

2013

Schneider S, Thurnher D, Erovic BM.

Source

Department of Otolaryngology-Head and Neck Surgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria.

Abstract

Background. The goal of this paper is to review contemporary multidisciplinary treatment with reference to Merkel cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations. 

Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient's treatment. 

Conclusion. This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma.

Full Text Article

Journal of Skin Cancer

Neuroembolization of metastatic Merkel cell cancer to the face for treatment of Kasabach-Merritt syndrome.

Feb 2013

Lee JH, Roychowdhury S, Nissenblatt MJ.

Source

Department of Radiology, UMDNJ Robert Wood Johnson University Hospital, New Brunswick, New Jersey, USA.

Abstract

Kasabach-Merritt syndrome is defined as a consumptive thrombocytopenia in the presence of a highly vascular tumor. Multiple treatment options, including transarterial embolization, have been described. We demonstrate that transarterial embolization is a viable option in the treatment of a rapidly progressive and debilitating Merkel cell tumor metastasizing to the head and neck presenting with Kasabach-Merritt syndrome.

BMJ - NeuroInterventional Surgery

A rare case of merkel cell carcinoma metastasis to the adrenal resected robotically.

Feb 2013

Alguraan Z, Agcaoglu O, Aliyev S, Berber E.

Source

Endocrinology and Metabolism Institute, Division of Endocrine Surgery, Cleveland Clinic, Cleveland, OH.

Abstract

INTRODUCTION:

 Merkel cell carcinoma (MCC) is an uncommon skin malignancy that has a high propensity for metastatic spread and there is no consensus regarding the optimal therapeutic approach. The relative roles of surgery, radiotherapy, and chemotherapy are still controversial. As the robotic approaches are gaining more popularity over the recent years, herein we report our result for MCC using robotics.

METHODS:

We herein describe a patient with MCC metastases to the left adrenal gland, which was resected robotically. The procedure was performed under general anesthesia using the robotic camera and 2 robotic working ports.

RESULTS:

A 59-year-old woman, who was diagnosed to have MCC of the left forearm 1 year ago with stage T2 N1 and removed by wide excision, underwent left robotic lateral transabdominal adrenalectomy because of MCC metastasis to the left adrenal gland. She was discharged home on postoperative day 2 uneventfully and is currently alive at 18 months with no evidence of recurrent disease on imaging studies.

CONCLUSIONS:

 This case report demonstrates that minimally invasive adrenalectomy may be performed for isolated MCC metastasis.

PubMed

Vascular E-selectin expression correlates with CD8 lymphocyte infiltration and improved outcome in Merkel cell carcinoma.

Jan 2013

Afanasiev OK, Nagase K, Simonson W, Vandeven N, Blom A, Koelle DM, Clark R, Nghiem P.

Source

1] Department of Medicine/Dermatology, University of Washington, Seattle Washington, USA [2] Department of Pathology, University of Washington, Seattle Washington, USA.

Abstract

Merkel cell carcinoma (MCC) is an aggressive, polyomavirus-linked skin cancer. While CD8 lymphocyte infiltration into the tumor is strongly correlated with improved survival, these cells are absent or sparse in most MCCs. We investigated whether specific mechanisms of T-cell migration may be commonly disrupted in MCC tumors with poor CD8 lymphocyte infiltration. Intratumoral vascular E-selectin, critical for T-cell entry into skin, was downregulated in the majority - 52% - of MCCs , and its loss was associated with poor intratumoral CD8 lymphocyte infiltration). Importantly, survival was improved in MCC patients whose tumors had higher vascular E-selectin expression. 

Local nitric oxide production is one mechanism of E-selectin downregulation and it can be tracked by quantifying nitrotyrosine, a stable biomarker of NO-induced reactive nitrogen species (RNS). Indeed, increasing levels of nitrotyrosine within MCC tumors were associated with low E-selectin expression and decreased CD8 lymphocyte infiltration. These data suggest that one mechanism of immune evasion in MCC may be restriction of T cell entry into the tumor. 

Existing therapeutic agents that modulate E-selectin expression and/or RNS generation may restore T cell entry and could potentially synergize with other immune-stimulating therapies.Journal of Investigative Dermatology accepted article preview online, 25 January 2013

Nature