Complete Spontaneous Regression of Merkel Cell Carcinoma: A Review of the 10 Reported Cases

Connelly, Thomas J.; Cribier, B.; Brown, Tricia J.; Yanguas, I.

doi:10.1046/j.1524-4725.2000.00054.x

Cited by 83 publications

(41 citation statements)

References 18 publications

(23 reference statements)

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“…Regression of cutaneous Merkel cell carcinoma and nodal metastasis has rarely been reported, mostly after either surgical excision or needle biopsy of the primary lesion; and it was thought to be due to the procedures, which stimulated cell-mediated immune clearance of the residual tumor cells. [36][37][38][39][40][41][42][43][44] It is difficult to envision the spontaneous resolution of cutaneous Merkel cell carcinoma without prior medical intervention in all of our cases; moreover, it is very unlikely that all the primary tumors would have regressed without leaving any visible remnants. Therefore, we speculate that some of our nodal cases may represent primary nodal neoplasms.…”

Section: Possible Histogenesis Of Nodal Merkel Cell Carcinomamentioning

confidence: 99%

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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Section: Possible Histogenesis Of Nodal Merkel Cell Carcinomamentioning

confidence: 99%

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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“…20,33 Spontaneous regression of cutaneous MCC has been described. [35][36][37][38] In 10% to 20% of patients with metastatic MCC, the primary tumor is not identified. Differentiating MCC from primary Merkel cell-like small cell carcinoma of the salivary gland in this scenario can be almost impossible.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…24 Spontaneous regression has been noted in some patients with MCC. [35][36][37][38] Apoptosis and T-cell immune response involvement has been suggested as the mechanism behind tumor regression. 37 In conclusion, the incidence of MCC is rising: It is diagnosed most commonly in elderly or immunocompromised patients, and it has higher mortality than melanoma.…”

Section: Staging and Treatmentmentioning

confidence: 99%

The Merkel cell carcinoma challenge

Bechert

Schnadig

Nawgiri

2012

Cancer Cytopathology

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Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine carcinoma of the skin that occurs primarily in elderly or immunocompromised patients. For this report, the authors reviewed the diagnostic challenges associated with MCC encountered on their fine-needle aspiration (FNA) service and also conducted an in-depth review of the literature on MCC. A computer search for patients who were diagnosed with MCC by FNA at the authors' institution from 2006 to 2010 was conducted, and 5 patients were selected for cytologic and immunochemical analyses based on their varied and diagnostically challenging clinical presentations. The 5 selected patients had clinical findings commonly associated with MCC, including advanced age (4 of the 5 patients were ages 75-85 years) and a history of previous malignancies (3 of the 5 patients had a history of previous malignancy), and 1 patient was diagnosed with a concomitant low-grade lymphoma.The patients and their disease illustrated the protean clinical presentation of MCC and the clinical and cytologic challenges associated with this neoplasm. The current findings indicate the need for cytopathologists to be aware of the deceptive presentation of this neoplasm and its cytologic and immunochemical features to correctly diagnose this insidious neoplasm.

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“…Even though the general nature of the tumor is rapid growth and early metastases, there are published data on spontaneous regression 49 . Mori et al 50 observed high apopoptic tumor activity in 8 cases of patients treated for MCC.…”

Section: Discusionmentioning

confidence: 99%

Merkel cell carcinoma. A review

Pink¹,

Ehrmann²,

Molitor³

et al. 2012

BIOMED PAP

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Background. Merkel cell carcinoma (MCC) is a rare potentially fatal skin tumour affecting older mainly white people and younger immunosuppressed individuals. While uncommon, the incidence is increasing relative to melanoma and with twice the lethality. The benign appearance of the tumour usually on exposed skin parts, contrasting with its extensive microscopic invasion, can delay timely diagnosis. Recurrent MCC is currently attributed to the recently discovered Merkel cell polyomavirus This brief review of MCC covers the history, epidemiology,etiology,clinical and histological features, treatment and prognosis. Methods. Literature search using PubMed and search words Merkel cell carcinoma (MCC), etiology, treatment for the years 1972 to 2010. Results and conclusion. Merkel cell carcinoma is a rare malignancy with uncertain prognosis. Due to the uncommon occurrence and dearth of randomized studies, there is no agreement on optimal treatment. The tumor has only recently been included in the international classification of tumors (NCCN). The treatment approaches found to be best are radical surgery of primary tumor, drainage of lymph node extension and possibly adjuvant loco-regional radiotherapy. The basis of successful treatment however, remains prevention regular dermatological examination in immunosuppressed patients and early initiation of combination therapy, based on radical surgery supplemented by radiotherapy and palliative chemotherapy in the last resort.

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Complete Spontaneous Regression of Merkel Cell Carcinoma: A Review of the 10 Reported Cases

Cited by 83 publications

References 18 publications

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

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

The Merkel cell carcinoma challenge

Merkel cell carcinoma. A review

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