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Extensive central nervous system involvement in Merkel cell carcinoma : a case report and review of the literature

Abul-Kasim, Kasim LU ; Söderström, Kristina LU and Hallsten, Lennart (2011) In Journal of Medical Case Reports 5.
Abstract

INTRODUCTION: Merkel cell carcinoma is a rare malignant cutaneous neoplasm that is locally invasive and frequently metastasizes to lymph nodes, liver, lungs, bone and brain. The incidence of Merkel cell carcinoma has increased in the past three decades.

CASE PRESENTATION: A 65-year-old Caucasian man presented with a sudden onset of severe headache and a three-month history of balance disturbance. Magnetic resonance imaging revealed a large meningeal metastasis. The radiologic workup showed retroperitoneal and inguinal lymph node metastases. Biopsy of the inguinal lymph nodes showed metastases of Merkel cell carcinoma. Biopsy from three different suspected skin lesions revealed no Merkel cell carcinoma, and the primary site of... (More)

INTRODUCTION: Merkel cell carcinoma is a rare malignant cutaneous neoplasm that is locally invasive and frequently metastasizes to lymph nodes, liver, lungs, bone and brain. The incidence of Merkel cell carcinoma has increased in the past three decades.

CASE PRESENTATION: A 65-year-old Caucasian man presented with a sudden onset of severe headache and a three-month history of balance disturbance. Magnetic resonance imaging revealed a large meningeal metastasis. The radiologic workup showed retroperitoneal and inguinal lymph node metastases. Biopsy of the inguinal lymph nodes showed metastases of Merkel cell carcinoma. Biopsy from three different suspected skin lesions revealed no Merkel cell carcinoma, and the primary site of Merkel cell carcinoma remained unknown. Leptomeningeal metastases, new axillary lymph node metastases, and intraspinal (epidural and intradural) metastases were detected within six, seven and eight months, respectively, from the start of symptoms despite treating the intracranial metastasis with gamma knife and the abdominal metastases with surgical dissection and external radiotherapy. This indicates the aggressive nature of the disease.

CONCLUSION: To the best of our knowledge, this is the first report in the literature of an intracranial meningeal metastasis of Merkel cell carcinoma treated with gamma knife and of intraspinal intradural metastases of Merkel cell carcinoma. Despite good initial response to radiotherapy, recurrence and occurrence of new metastases are common in Merkel cell carcinoma.

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Contribution to journal
publication status
published
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in
Journal of Medical Case Reports
volume
5
article number
35
publisher
BioMed Central (BMC)
external identifiers
  • scopus:78951480698
  • pmid:21269448
ISSN
1752-1947
DOI
10.1186/1752-1947-5-35
language
English
LU publication?
yes
id
0b78c3d9-ad2f-428a-accd-448e68b278ed
date added to LUP
2016-04-19 09:06:39
date last changed
2024-06-14 01:30:06
@article{0b78c3d9-ad2f-428a-accd-448e68b278ed,
  abstract     = {{<p>INTRODUCTION: Merkel cell carcinoma is a rare malignant cutaneous neoplasm that is locally invasive and frequently metastasizes to lymph nodes, liver, lungs, bone and brain. The incidence of Merkel cell carcinoma has increased in the past three decades.</p><p>CASE PRESENTATION: A 65-year-old Caucasian man presented with a sudden onset of severe headache and a three-month history of balance disturbance. Magnetic resonance imaging revealed a large meningeal metastasis. The radiologic workup showed retroperitoneal and inguinal lymph node metastases. Biopsy of the inguinal lymph nodes showed metastases of Merkel cell carcinoma. Biopsy from three different suspected skin lesions revealed no Merkel cell carcinoma, and the primary site of Merkel cell carcinoma remained unknown. Leptomeningeal metastases, new axillary lymph node metastases, and intraspinal (epidural and intradural) metastases were detected within six, seven and eight months, respectively, from the start of symptoms despite treating the intracranial metastasis with gamma knife and the abdominal metastases with surgical dissection and external radiotherapy. This indicates the aggressive nature of the disease.</p><p>CONCLUSION: To the best of our knowledge, this is the first report in the literature of an intracranial meningeal metastasis of Merkel cell carcinoma treated with gamma knife and of intraspinal intradural metastases of Merkel cell carcinoma. Despite good initial response to radiotherapy, recurrence and occurrence of new metastases are common in Merkel cell carcinoma.</p>}},
  author       = {{Abul-Kasim, Kasim and Söderström, Kristina and Hallsten, Lennart}},
  issn         = {{1752-1947}},
  language     = {{eng}},
  publisher    = {{BioMed Central (BMC)}},
  series       = {{Journal of Medical Case Reports}},
  title        = {{Extensive central nervous system involvement in Merkel cell carcinoma : a case report and review of the literature}},
  url          = {{http://dx.doi.org/10.1186/1752-1947-5-35}},
  doi          = {{10.1186/1752-1947-5-35}},
  volume       = {{5}},
  year         = {{2011}},
}