A Practical Update of Surgical Management of Merkel Cell Carcinoma of the Skin

The role of surgeons in the treatment of Merkel cell carcinoma (MCC) of the skin is reviewed, with respect to diagnosis and treatment. Most of the data in the literature are case reports. Surgery is the mainstay of treatment. A wide local excision, with sentinel node (SLN) biopsy, is the recommended treatment of choice. If SLN is involved, nodal dissection should be performed; unless patient is unfit, then regional radiotherapy can be given. Surgeons should always refer patients for assessment of the need for adjuvant treatments. Adjuvant radiotherapy is well tolerated and effective to minimize recurrence. Adjuvant chemotherapy may be considered for selected node-positive patients, as per National Comprehensive Cancer Network guideline. Data are insufficient to assess whether adjuvant chemotherapy improves survival. Recurrent disease should be treated by complete surgical resection if possible, followed by radiotherapy and possibly chemotherapy. Generally results of multimodality treatment for recurrent disease are better than lesser treatments. Future research should focus on newer chemotherapy and molecular targeted agents in the adjuvant setting and for gross disease.

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Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis

Case Reports in Oncological Medicine ◽

10.1155/2020/1257587 ◽

2020 ◽

Vol 2020 ◽

pp. 1-6

Author(s):

Sophia Z. Shalhout ◽

Kevin S. Emerick ◽

Peter M. Sadow ◽

Jenny J. Linnoila ◽

David M. Miller

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Psychiatric Symptoms ◽

Checkpoint Inhibitors ◽

Case Reports ◽

Intensity Modulated Radiation Therapy ◽

Sun Exposure ◽

Merkel Cell ◽

Paraneoplastic Encephalitis ◽

Aspartate Receptor

Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine cancer with a high risk of recurrence and metastasis. MCC is generally associated with advanced age, fair skin, sun exposure, immunosuppression, and in the majority of cases, the Merkel cell polyomavirus. Neuroendocrine malignancies are associated with a variety of paraneoplastic neurological syndromes (PNS), characterized as autoimmune responses to malignancy-associated expression of neural antigens. Our literature review underscores previous case reports of MCC-associated PNS with voltage-gated calcium channel (VGCC) and anti-Hu (or ANNA-1) autoantibodies. We present the case of a 59-year-old male with regionally metastatic Merkel cell carcinoma complicated by the paraneoplastic manifestation of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. His primary lower neck subcutaneous MCC and metastasis were initially treated with surgery. Additional recurrent lymph node metastases were successfully treated with definitive intensity-modulated radiation therapy. His PNS improved with rituximab therapy. Although rare, this case highlights that in the setting of seizures and prominent psychiatric symptoms accompanying an MCC diagnosis, evaluation for autoimmune paraneoplastic encephalitis is warranted. Awareness and detection of preexisting PNS are crucial in the era of immune checkpoint inhibitors (ICI) for advanced MCC, where treatment with ICI has the potential to exacerbate preexisting autoimmune PNS and lead to worsened or even lethal neurologic immune-related adverse events (nirAEs).

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Frequency and locations of systemic metastases in Merkel cell carcinoma by imaging

Acta Radiologica Open ◽

10.1177/2058460117700449 ◽

2017 ◽

Vol 6 (3) ◽

pp. 205846011770044 ◽

Cited By ~ 12

Author(s):

Maria Kouzmina ◽

Virve Koljonen ◽

Junnu Leikola ◽

Tom Böhling ◽

Eila Lantto

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Primary Tumor ◽

Case Reports ◽

Tumor Diagnosis ◽

Merkel Cell ◽

Imaging Studies ◽

Organ Systems ◽

Systemic Metastasis ◽

The Mean

Background The primary neuroendocrine skin cancer, Merkel cell carcinoma (MCC), has a well-known predilection to metastasize systemically. However, the experience of systemic metastases in MCC is mainly disseminated through case reports due to the rarity of MCC. Purpose To elucidate the frequency and locations of systemic metastasis in MCC by reviewing the imaging of patients with metastatic MCC in a national cohort. Material and Methods Patients with diagnosed metastatic MCC by imaging studies in Finland during 1999–2012 were included in this study. We reviewed their imaging studies to evaluate the most frequent sites for systemic metastasis and determined the latency between the primary tumor diagnosis and systemic metastasis. The material includes 30 MCC patients with complete imaging series and 187 examinations, of which 102 (54%) were CT images. Results The mean latency from the primary tumor diagnosis to systemic metastasis was 2.1 years and the mean latency between the radiologic diagnosis of the metastases and death was 299 days. Metastases were recorded in several organ systems in most of the cases, and at least two separate metastatic sites in 63% of the cases. Metastatic spread was noted in 60% of the cases in distant lymph nodes. Liver and lungs were the most affected solid organs. Conclusion Systemic metastasis in MCC has no predilection site, basically every organ system can be involved. Most of the systemic metastases were recorded during the first two years after the MCC diagnosis.

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Complete Spontaneous Regression of Merkel Cell Carcinoma

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940511400507 ◽

2005 ◽

Vol 114 (5) ◽

pp. 376-380 ◽

Cited By ~ 32

Author(s):

Luis Junquera ◽

Aintza Torre ◽

Luis García-Consuegra ◽

Juan C. Vicente ◽

Manuel F. Fresno

Keyword(s):

Computed Tomography ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Spontaneous Regression ◽

Case Reports ◽

The Elderly ◽

Merkel Cell ◽

Review Of The Literature ◽

The Right

Merkel cell carcinoma (MCC) is a very aggressive primary cutaneous neoplasm most often occurring on the head and neck of the elderly. Spontaneous regression of MCC was first described in 1986. A 79-year-old woman with MCC on the right cheek underwent spontaneous regression of the malignancy, documented by photographic follow-up, computed tomography, and histologic studies. A review of the literature is presented. Complete clinical and histologic regression of MCC was observed in the present case. Although the literature documents 11 similar cases, only 6 can be regarded as complete spontaneous regressions following exclusive performance of a biopsy (primary complete spontaneous regression). Primary complete spontaneous regression of MCC is infrequent, and most case reports describe this phenomenon in women with MCC on the cheek. The reasons underlying regression are unknown.

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Adjuvant Chemotherapy of Merkel Cell Carcinoma

Merkel Cell Carcinoma - Molecular Medicine and Medicinal Chemistry ◽

10.1142/9781848163140_0015 ◽

2010 ◽

pp. 257-265

Author(s):

Ronald C. DeConti

Keyword(s):

Adjuvant Chemotherapy ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Merkel Cell

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Benefits of combined modality treatment of Merkel cell carcinoma of the head and neck: single institution experience

The Journal of Laryngology & Otology ◽

10.1017/s0022215113001862 ◽

2013 ◽

Vol 127 (9) ◽

pp. 908-916 ◽

Cited By ~ 7

Author(s):

V Balakrishnan ◽

S Berry ◽

B Stew ◽

A Sizeland

Keyword(s):

Cell Carcinoma ◽

Head And Neck ◽

Merkel Cell Carcinoma ◽

Adjuvant Radiotherapy ◽

Recurrent Disease ◽

Clinical Status ◽

Combined Modality Treatment ◽

Merkel Cell ◽

Single Institution ◽

Combined Modality

AbstractBackground:There is ongoing debate surrounding the roles of surgery and adjuvant radiotherapy in the management of primary and recurrent Merkel cell carcinoma of the head and neck. This study assessed the influence of local excision, margin status, adjuvant radiotherapy and chemotherapy on locoregional recurrence and survival.Method:A retrospective review of 54 consecutive cases of head and neck Merkel cell carcinoma at a single institution.Results:Median disease-specific survival time was 120 months. Forty-four per cent of patients developed locoregional recurrence. Combined treatment with surgery and locoregional radiotherapy improved disease-specific survival. Radiotherapy was associated with longer time to recurrence and regional recurrence. Irradiation of the regional nodes improved regional control, irrespective of clinical status. Margin-negative excision was not associated with improved local control. Combined modality treatment of recurrent disease resulted in a four-fold improvement of local control, but small numbers prevented this trend from reaching statistical significance.Conclusion:Surgical excision of the primary disease and clinically involved regional nodes, plus adjuvant radiotherapy to the surgical bed and regional nodes are recommended for all patients with Merkel cell carcinoma of the head and neck, irrespective of clinical status. Recurrent disease should be aggressively treated with combined modality treatment.

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Multimodality Treatment of Merkel Cell Carcinoma: Case Series and Literature Review of 1024 Cases

Annals of Surgical Oncology ◽

10.1007/s10434-001-0204-4 ◽

2001 ◽

Vol 8 (3) ◽

pp. 204-208 ◽

Cited By ~ 293

Author(s):

Heriberto Medina-Franco ◽

Marshall M. Urist ◽

John Fiveash ◽

Martin J. Heslin ◽

Kirby I. Bland ◽

...

Keyword(s):

Literature Review ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Case Series ◽

Multimodality Treatment ◽

Merkel Cell

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Merkel cell carcinoma: an aggressive cutaneous carcinoma with rare metastasis to the thyroid gland

BMJ Case Reports ◽

10.1136/bcr-2018-228273 ◽

2019 ◽

Vol 12 (4) ◽

pp. e228273

Author(s):

Donata Vaiciunaite ◽

Gregory Beddell ◽

Nedyalko Ivanov

Keyword(s):

Thyroid Gland ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Distant Metastasis ◽

Case Reports ◽

Primary Tumour ◽

Merkel Cell ◽

Imaging Studies ◽

Thyroid Metastasis ◽

Rare Site

Merkel cell carcinoma (MCC) is an aggressive and rare neuroendocrine cutaneous carcinoma with poor prognosis and with increasing morbidity and mortality in cases of distant metastasis. Given the rarity of MCC, optimal treatment is not well established. Treatment usually consists of multidisciplinary management with local excision of the primary tumour. Imaging studies are essential for accurate staging and monitoring of disease progression. The incidence of local recurrence is highest in the first 2 years and ranges from 27% to 60%. Distant metastasis is most common in the skin, liver, bone, lung/pleura, brain or distant lymph nodes. The thyroid gland is a rare site of metastasis for MCC with only two case reports published to date. Our patient had a repeat 2-[18F]-fluoro-2-deoxy-d-glucose-postiron emission tomography/CT after the discovery of recurrence of MCC. The high-intensity maximal standardised uptake value of 10.9 in the thyroid isthmus is consistent with malignancy. This case report describes an exceedingly rare thyroid metastasis secondary to MCC.

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Merkel Cell Carcinoma and Diagnostic Experience in a Reference Hospital: A Case Series

Case Reports in Medicine ◽

10.1155/2020/8391510 ◽

2020 ◽

Vol 2020 ◽

pp. 1-4

Author(s):

Rocio del Pilar López Panqueva ◽

David A. Suarez-Zamora ◽

Luis E. Barrera-Herrera ◽

Mariam Rolón Cadena

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Case Reports ◽

Case Series ◽

Recurrence Risk ◽

Cross Sectional Study ◽

University Hospital ◽

Merkel Cell ◽

Cross Sectional ◽

Exposed Skin

Merkel cell carcinoma (MCC) is a rare poorly differentiated neuroendocrine tumor, usually located in sun-exposed skin, with aggressive behavior and with high recurrence risk and metastatic disease. In Latin America, case series have been published, and it does not exceed 32 patients in 10 years, and in Colombia, there are case reports. We present a descriptive retrospective cross-sectional study in patients diagnosed with MCC in the Department of Pathology and Laboratories at the University Hospital Fundación Santa Fe de Bogotá(FSFB) between January 2003 and December 2018. We present the demographic, clinical, and pathological variables of these patients, as well as a literature review.

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