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    Disseminated Merkel Cell Tumor Treatment with Systemic Chemotherapy
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    Abstract:
    Four patients with disseminated Merkel cell tumors were treated with cyclophosphamide (C), doxorubicin (A), and vincristine (V). Two partial responses (PR) were noted and in one case disease remained stable. One of the patients who achieved a PR achieved a second PR when treated with VP-16 (E) and cis-platin (P) after progressive disease developed. Systemic chemotherapy appears capable of inducing objective responses in some patients with disseminated Merkel cell tumors.
    Keywords:
    Merkel cell
    Skin tumor
    Abstract Merkel cells form part of the peripheral neuroendocrine system of the skin and act as mechanoreceptors in touch response. Merkel cell carcinoma (MCC) is a rare, aggressive disease with similarities to small cell lung cancer (SCLC), which is also of neuroendocrine origin. We previously identified a novel DNA binding protein complex specific for MCC suspension cell lines, termed Merkel nuclear factor (MNF) by its binding to the POU‐IV family DNA binding consensus sequence. Here we report that MNF contains the POU‐IV family member Brn‐3c and that Brn‐3c is expressed in normal Merkel cells. Additionally, Brn‐3c protein reactivity is restricted to a subset of MCC biopsies and is not seen in biopsies revealing adherent, variant cell lines lacking neuroendocrine markers. Recently, proper development of murine Merkel cells was shown to require the proneural basic helix‐loop‐helix transcription factor, atonal family member, MATH1. We demonstrate a correlation between Brn‐3c and HATH1 reactivity in MCC biopsies and cell lines with retention of neuroendocrine phenotype. In SCLC, the related basic helix‐loop‐helix transcription factor HASH1 is responsible for neuroendocrine phenotype, but HASH1 transcripts were not detected in MCC cell lines. We propose that HATH1 and Brn‐3c may form a transcriptional hierarchy responsible for determining neuroendocrine phenotype in Merkel cells and that lack of Brn‐3c and/or HATH1 in MCC may indicate a more aggressive disease requiring closer patient follow‐up. © 2002 Wiley‐Liss, Inc.
    Merkel cell
    Mechanoreceptor
    Citations (77)
    Malignant neuroendocrine carcinoma of the skin (Merkel cell tumor) was diagnosed in an 18-year-old spayed female Maine Coon Cat. The diagnosis was made on the basis of morphologic and electron microscopic findings. The cat was euthanatized 321 days after surgical excision of the tumor. The tumor's malignancy contrasted with the benign nature of Merkel cell tumors reported in dogs and was consistent with the malignancy of Merkel cell tumors reported in humans.
    Merkel cell
    Neuroendocrine carcinoma
    Skin tumor
    Citations (39)
    Aims: To evaluate the monoclonal antibody MOC‐31 in Merkel cell carcinomas and normal Merkel cells. Merkel cell carcinoma is a rare and aggressive tumour that occurs mainly in elderly individuals. The histological diagnosis of Merkel cell carcinoma can be difficult because it looks similar to other small blue cell tumours, particularly skin metastases of small‐cell lung carcinomas. This antibody recognizes the epithelial cell adhesion molecule (Ep‐CAM), that has been assigned to the small cell lung cancer cluster 2 of antibodies. To the best of our knowledge, immunostaining for MOC‐31/Ep‐CAM has not been previously described in Merkel cells or Merkel cell carcinomas. Methods and results: Thirty‐one cases of Merkel cell carcinoma and three samples of normal human fingertip were selected to analyse the expression of MOC‐31/Ep‐CAM by immunohistochemistry. A high number of Merkel cell carcinomas (21/31, 67.7%) showed intense and readily interpretable positivity. Immunostaining was diffuse or focal and always localized to the plasma membrane. Normal Merkel cells of human fingertip also showed plasma membrane immunoreactivity for MOC‐31/Ep‐CAM. Conclusion: The demonstration of positivity for MOC‐31/Ep‐CAM in Merkel cell carcinomas precludes the use of this immunohistochemical marker to distinguish between tumours and skin metastases of small‐cell lung carcinoma.
    Merkel cell
    Immunostaining
    Merkel cell polyomavirus
    Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine tumor, most often occurring in the head and neck region in the elderly; it is a highly aggressive tumor, with a high frequency of recurrence. Despite the highly malignant course of MCC, there have been a few reports of spontaneous regression of this tumor.
    Merkel cell
    Skin tumor
    Primary tumor
    Citations (0)
    Merkel cell carcinomas (MCCs) are uncommon, highly malignant skin tumors that develop in sun-exposed areas of the skin. Most of the MCCs are CK 20-positive and CK 7-negative such as our case. About 80% of Merkel cell carcinoma is associated with Merkel cell polyomavirus.
    Merkel cell polyomavirus
    Merkel cell
    Cheek
    Skin tumor
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