Epidemiology of primary Merkel cell carcinoma in the United States

Agelli, Maria; Clegg, Limin X.

doi:10.1016/s0190-9622(03)02108-x

Cited by 522 publications

(490 citation statements)

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“…In the analysis of 1034 cases of MCC, Agelli and Clegg [1] found the 5 year relative survival for patients with localized, regional and distant MCC was approximately 75%, 59% and 25% respectively. Treatment of MCC depends on the stage of the disease, and the patient's age and overall condition.…”

Section: Discussionmentioning

confidence: 99%

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Kamath

Venkateswaran²,

Shetty

et al. 2007

Eur Spine J

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We report a rare case of Merkel cell carcinoma with extra-dural spinal metastasis causing paraplegia. There are only four reported cases in literature. A 57-year-old lady presented with a breast lump, multiple truncal skin swellings, low back pain and rapidly progressive paraplegia. MRI showed multiple epidural soft tissue masses causing neural compression. A biopsy from the truncal skin lesion was diagnosed as Merkel cell carcinoma (MCC). Posterior decompression and tumor debulking at all three sites of neural compression was performed. Histopathology of the epidural tumor was consistent with MCC and the diagnosis was confirmed by immuno-histochemistry staining for cytokeratin-20. She was started on chemotherapy and radiotherapy. One month after diagnosis she died due to extensive metastasis. The short term palliative response seen in our patient demonstrates the poor prognosis for patients with spinal metastasis.Keywords Merkel cell carcinoma Á Spinal metastasis Á Secondaries Á Paraplegia Case reportA 57-year-old diabetic lady presented with persistent low back pain and bilateral radiculopathy of 2 months duration followed by rapidly progressive weakness and numbness of the lower limbs. She also had loss of bowel and bladder control since 1 week. Multiple painless skin lumps over the chest and abdomen had appeared spontaneously a few months prior to presentation. There were no constitutional symptoms. General examination revealed multiple firm nontender shiny skin lumps approximately 3-4 cm in diameter on the anterior abdominal wall, chest and on the right breast. There were also firm and non-tender mobile lymph-nodes of 2-3-cm size in the right posterior triangle of the neck (Fig. 1a). There was no tenderness or pain on movements of the spine. She had a flaccid paraplegia, a left extensor plantar response and sensory hypoesthesia below the ninth thoracic dermatome bilaterally. The radiographs and CT scan done prior to admission were normal (Fig. 1b, c). An MRI showed epidural soft tissue masses causing neural compression at the eighth thoracic, fourth lumbar and first sacral levels. There were also with multiple soft tissue lesions in the paraspinal and retroperitoneal space--features suggestive of metastasis. There was no history to suggest immuno-compromised status and human immunodeficiency virus infection was eliminated by the appropriate laboratory tests.An FNAC of the lymph node, anterior abdominal wall mass and right breast was reported as a small cell tumor. An incisional biopsy from a skin lesion was reported as MCC. A laminectomy and tumor debulking at all three sites of compression was performed. Epidural tumor tissue sent for histopathology was reported as a high grade malignancy composed of epitheliod cells with scanty cytoplasm, markedly pleomorphic nulei having a ''salt and pepper'' chromatin (highly reminiscent of a neuro-endocrine origin). The tumor cells with numerous mitosis were predominantly disposed in a trabercular and cribriform pattern and also arranged around necrotic space...

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Section: Discussionmentioning

confidence: 99%

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Kamath

Venkateswaran²,

Shetty

et al. 2007

Eur Spine J

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“…[1][2][3][4] At the time of diagnosis, B30% of cases have metastatic diseases, mostly to regional lymph nodes and skin of other sites. 2 Rare cases of highgrade neuroendocrine tumor have been encountered in the lymph nodes with unknown extranodal primary, and these tumors were often described as 'Merkel cell carcinoma of lymph node with unknown primary' as they exhibit similar morphologic and immunophenotypic features to those in the skin Merkel cell carcinoma.…”

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Merkel cell carcinoma of lymph node with unknown primary has a significantly lower association with Merkel cell polyomavirus than its cutaneous counterpart

Pan

Chen

et al. 2014

Modern Pathology

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Rare cases of Merkel cell carcinoma have been encountered in lymph nodes with unknown extranodal primary, which exhibit similar morphologic and immunophenotypic features to those in primary cutaneous Merkel cell carcinomas. However, it is uncertain whether the nodal Merkel cell carcinoma is a primary tumor of the lymph node or represents a metastasis from an occult or regressed extranodal lesion. To establish an accurate diagnosis of the nodal Merkel cell carcinoma can be challenging because of significant morphologic mimics, including lymphoblastic lymphoma and metastatic small cell carcinoma. Moreover, there is no consensus for a diagnostic term, and many different terms have been used, which can be confusing and may not fully reflect the nature of nodal Merkel cell carcinoma. In this study, we investigated the detailed clinicopathologic features of 22 nodal Merkel cell carcinomas, with comparison to 763 primary cutaneous cases retrieved from the literature. Overall, the nodal and cutaneous Merkel cell carcinomas shared similar clinical presentations, morphologic spectrum, and immunophenotype; both were mostly seen in elderly male with a typical neuroendocrine morphology. Most of cases expressed CK20, synaptophysin, and chromogranin A; and PAX5 and TdT were also positive in majority of cases. However, nodal Merkel cell carcinomas had a significantly lower association with Merkel cell polyomavirus than cutaneous cases (31% vs 76%, P ¼ 0.001). Therefore, these two entities may arise from overlapping but not identical biological pathways. We also recommend the use of the diagnostic term 'Merkel cell carcinoma of lymph node' to replace many other names used.

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“…Patients present with lymph node disease in 19% to 33% 9,13,[16][17][18] of cases. Traditional recommended practice for the regional therapy for lymph node-positive disease is completion lymphadenectomy (CLND) with or without adjuvant radiotherapy.…”

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Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

Fang

Lemos

Douglas

et al. 2010

Cancer

145

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BACKGROUND:Merkel cell carcinoma (MCC) is an aggressive cutaneous malignancy with a high risk of lymph node involvement. To the authors' knowledge, few data have been published to date regarding the optimal regional therapy for lymph node‐positive patients. This cohort study was performed to analyze the outcomes of patients with lymph node‐positive MCC treated with lymph node irradiation as definitive therapy compared with completion lymphadenectomy (CLND).METHODS:Fifty patients with lymph node involvement of MCC at presentation and adequate follow‐up data were included in this analysis. Forty‐three of these patients were enrolled and followed prospectively. Twenty‐six patients presented with microscopic lymph node disease, and 24 patients presented with palpable lymph node involvement.RESULTS:Regional control for patients with microscopically involved lymph nodes was 100% regardless of treatment modality—definitive lymph node irradiation (n = 19) or CLND ± radiotherapy (n = 7) with median follow‐up of 18 months. Patients with clinically positive lymph nodes had 2‐year regional recurrence‐free survival rate of 78% and 73% in the definitive lymph node irradiation (n = 9) and CLND ± radiotherapy (n = 15) groups, respectively (P = .8) with a median follow‐up of 16 months.CONCLUSIONS:To the best of the authors' knowledge, the current study is the largest series published to date of radiation monotherapy as regional treatment for lymph node‐positive MCC. Lymph node irradiation alone to positive regional lymph nodes was found to confer an excellent regional control rate that was comparable to CLND for both microscopic and palpable lymph node disease. There was no difference noted with regard to overall survival. Given their similar efficacy, the choice between these lymph node therapies may be based on the clinical scenario and anticipated side effect profiles. Cancer 2010. © 2010 American Cancer Society.

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Epidemiology of primary Merkel cell carcinoma in the United States

Abstract: The highest incidence of MCC was observed in whites, males, and in people older than 65 years. Only 49% of cases were reported as localized. Better survival was associated with limb localization, early-stage disease, younger age, and female sex.

Cited by 522 publications

References 50 publications

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Merkel cell carcinoma of lymph node with unknown primary has a significantly lower association with Merkel cell polyomavirus than its cutaneous counterpart

Radiation monotherapy as regional treatment for lymph node‐positive Merkel cell carcinoma

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