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

Abul-Kasim K, Söderström K, Hallsten L - J Med Case Rep (2011)

Bottom Line: Magnetic resonance imaging revealed a large meningeal metastasis.The radiologic workup showed retroperitoneal and inguinal lymph node metastases.This indicates the aggressive nature of the disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Faculty of Medicine, Lund University, Diagnostic Centre for Imaging and Functional Medicine, Skåne University Hospital, 205 02 Malmö, Sweden. Kasim.Abul-Kasim@med.lu.se.

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 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.

No MeSH data available.


Related in: MedlinePlus

Magnetic resonance imaging (MRI) scan of the lumbar spine eight months after the onset of symptoms. T2-weighted (A) and T1-weighted (B-C) images before and after contrast administration show mild contrast enhancing multilobular tumors in the epidural fat behind the dural sac at the level of L3-L4 (black arrows) and a dural sac filled with intradural tumors (white arrows). Note the absence of the normal cerebrospinal fluid signal in the dural sac below the medullary conus. Axial T1-weighted images before (D) and after (E) contrast administration show the epidural metastases lateral and dorsal to the dural sac (black arrows in D; arrowheads in E). The white arrow in E shows the dorsal limit of the dural sac. F) Axial T1-weighted image after contrast administration shows a tumor-filled dural sac (white arrows).
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Figure 2: Magnetic resonance imaging (MRI) scan of the lumbar spine eight months after the onset of symptoms. T2-weighted (A) and T1-weighted (B-C) images before and after contrast administration show mild contrast enhancing multilobular tumors in the epidural fat behind the dural sac at the level of L3-L4 (black arrows) and a dural sac filled with intradural tumors (white arrows). Note the absence of the normal cerebrospinal fluid signal in the dural sac below the medullary conus. Axial T1-weighted images before (D) and after (E) contrast administration show the epidural metastases lateral and dorsal to the dural sac (black arrows in D; arrowheads in E). The white arrow in E shows the dorsal limit of the dural sac. F) Axial T1-weighted image after contrast administration shows a tumor-filled dural sac (white arrows).

Mentions: About three months after the retroperitoneal and inguinal lymph node dissection, the patient received 40 Gy of external radiation for the paraaortal and iliac retroperitoneal lymph node metastasis and 50.9 Gy for the metastasis in the left inguinal region. Thereafter, FDG-PET showed total regression of the FDG uptake in the lymph nodes, which previously had shown increased uptake. A planned MRI of the brain about six months after the onset of symptoms showed evidence of cerebellar leptomeningeal enhancement (Figures 1E and 1F), which was immediately treated with 30 Gy of palliative radiation therapy. FDG-PET study approximately seven months after the onset of symptoms showed a new enlarged left-sided axillary lymph node with increased FDG uptake. Approximately eight months after the onset of symptoms, the patient was admitted for increasing back pain and a four-day history of rapidly progressing weakness of the lower limbs that required the patient to start using a wheelchair. On examination, lower limb weakness, hyporeflexia and a positive Babinski sign were found. Emergency MRI of the spine and the spinal canal showed that the dural sac between the first and fifth lumbar vertebra was filled with intradural tumor masses with mild contrast enhancement (Figures 2A to 2F). There were multiple tumor masses in the epidural space with extension into several lumbar and lower thoracic neural foramens. Because the patient's general condition deteriorated rapidly, further radiation therapy against the intraspinal tumors was not possible. The patient died two weeks after the detection of the intraspinal tumors. At autopsy, lung metastases were found, but there was no evidence of residual macroscopic intracranial tumor and no metastases to the vertebral column. Unfortunately, some technical difficulties restrained the examination of the spinal canal.


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

Abul-Kasim K, Söderström K, Hallsten L - J Med Case Rep (2011)

Magnetic resonance imaging (MRI) scan of the lumbar spine eight months after the onset of symptoms. T2-weighted (A) and T1-weighted (B-C) images before and after contrast administration show mild contrast enhancing multilobular tumors in the epidural fat behind the dural sac at the level of L3-L4 (black arrows) and a dural sac filled with intradural tumors (white arrows). Note the absence of the normal cerebrospinal fluid signal in the dural sac below the medullary conus. Axial T1-weighted images before (D) and after (E) contrast administration show the epidural metastases lateral and dorsal to the dural sac (black arrows in D; arrowheads in E). The white arrow in E shows the dorsal limit of the dural sac. F) Axial T1-weighted image after contrast administration shows a tumor-filled dural sac (white arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3037324&req=5

Figure 2: Magnetic resonance imaging (MRI) scan of the lumbar spine eight months after the onset of symptoms. T2-weighted (A) and T1-weighted (B-C) images before and after contrast administration show mild contrast enhancing multilobular tumors in the epidural fat behind the dural sac at the level of L3-L4 (black arrows) and a dural sac filled with intradural tumors (white arrows). Note the absence of the normal cerebrospinal fluid signal in the dural sac below the medullary conus. Axial T1-weighted images before (D) and after (E) contrast administration show the epidural metastases lateral and dorsal to the dural sac (black arrows in D; arrowheads in E). The white arrow in E shows the dorsal limit of the dural sac. F) Axial T1-weighted image after contrast administration shows a tumor-filled dural sac (white arrows).
Mentions: About three months after the retroperitoneal and inguinal lymph node dissection, the patient received 40 Gy of external radiation for the paraaortal and iliac retroperitoneal lymph node metastasis and 50.9 Gy for the metastasis in the left inguinal region. Thereafter, FDG-PET showed total regression of the FDG uptake in the lymph nodes, which previously had shown increased uptake. A planned MRI of the brain about six months after the onset of symptoms showed evidence of cerebellar leptomeningeal enhancement (Figures 1E and 1F), which was immediately treated with 30 Gy of palliative radiation therapy. FDG-PET study approximately seven months after the onset of symptoms showed a new enlarged left-sided axillary lymph node with increased FDG uptake. Approximately eight months after the onset of symptoms, the patient was admitted for increasing back pain and a four-day history of rapidly progressing weakness of the lower limbs that required the patient to start using a wheelchair. On examination, lower limb weakness, hyporeflexia and a positive Babinski sign were found. Emergency MRI of the spine and the spinal canal showed that the dural sac between the first and fifth lumbar vertebra was filled with intradural tumor masses with mild contrast enhancement (Figures 2A to 2F). There were multiple tumor masses in the epidural space with extension into several lumbar and lower thoracic neural foramens. Because the patient's general condition deteriorated rapidly, further radiation therapy against the intraspinal tumors was not possible. The patient died two weeks after the detection of the intraspinal tumors. At autopsy, lung metastases were found, but there was no evidence of residual macroscopic intracranial tumor and no metastases to the vertebral column. Unfortunately, some technical difficulties restrained the examination of the spinal canal.

Bottom Line: Magnetic resonance imaging revealed a large meningeal metastasis.The radiologic workup showed retroperitoneal and inguinal lymph node metastases.This indicates the aggressive nature of the disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Faculty of Medicine, Lund University, Diagnostic Centre for Imaging and Functional Medicine, Skåne University Hospital, 205 02 Malmö, Sweden. Kasim.Abul-Kasim@med.lu.se.

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 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.

No MeSH data available.


Related in: MedlinePlus