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==Classification==
==Classification==
Mercell cell carcinoma is classified into 3 subgroups: trabecular, intermediate, and small cell.
Mercell cell carcinoma is classified into 3 subgroups: trabecular, intermediate, and small cell.
==Pathophysiology==
==Pathophysiology==
==Causes==
==Causes==
Line 31: Line 30:
==Diagnosis==
==Diagnosis==
===Staging===
===Staging===
The staging of Merkel cell cancer is based on the TNM staging system.
===History and Symptoms===
===History and Symptoms===
Patients with Merkel cell carcinoma usually present with a rapid growing, painless, small mass that is typically located on sun exposed area of the body.
===Physical Examination===
===Physical Examination===
Physical exam findings of Merkel cell cancer include a firm, dome-shaped or raised, red/violaceous skin mass that appear in sun exposed areas.
===Laboratory Findings===
===Laboratory Findings===
===CT===
===CT===
CT scan is useful in detecting metastasis to organs and regional lymph nodes.
===Other Imaging Findings===
===Other Imaging Findings===
Positron emission tomography maybe used in staging of Merkel cell cancer.
===Other Diagnostic Studies===
===Other Diagnostic Studies===
On biopsy, Merkel cell cancer is characterized by positivity on cytokeratin-20 immunohistochemical staining. On electron microscopy, the presence of neurosecretory granules within cytoplasmic extensions is characteristics.
==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===

Revision as of 02:08, 21 August 2015

Merkel cell cancer Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Merkel Cell Cancer from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

CT

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Merkel cell cancer is a rare and highly aggressive cancer where malignant cancer cells develop on or just beneath the skin and in hair follicles. This cancer is a type of neuroendocrine tumor, like small cell lung cancer. Merkel cell carcinoma is the most serious form of skin cancer. Once it has metastasized to the lymph nodes, the 5-year survival rate for a patient is about 50 percent. A small tumor (less than 2 cm) that has not metastasized to the lymph nodes reported a 5-year survival rate of more than 90 percent; however, at the time of diagnosis of MCC the 5-year survival was 64 percent. Recurrence can occur in up to half of all patients. [1]

It occurs most often on the face, head, and neck. It usually appears as firm, painless, nodules, or tumors. These flesh-colored, red, or blue tumors vary in size from 5 mm (less than a quarter of an inch) to more than 5cm (2 inches). The tumor grows rapidly. About half of all Merkel cell cancers occur on the sun-exposed areas of the head and neck, while one-third begin on the legs, and 15% occur on the arms. The cancer may also begin on other parts of the body, such as the trunk.

From initial onset, Merkel cell cancer metastasizes quickly and spreads to other parts of the body, tending towards the regional lymph nodes. The tumor tends to invade underlying subcutaneous fat, fascia, and muscle. It can also metastasize to the liver, lungs, brain or bones.

Historical Perspective

Merkel cell polyomavirus, the pathogen responsible for Merkel cell cancer, was first discovered in 2008.[2]


Classification

Mercell cell carcinoma is classified into 3 subgroups: trabecular, intermediate, and small cell.

Pathophysiology

Causes

Merkel cell cancer is caused by Merkel cell polyomavirus (MCV) infection.

Differential Diagnosis

Merkel cell cancer must be differentiated from other skin lesions, such as basal cell carcinoma, squamous cell carcinoma, malignant melanoma, lymphoma, vascular tumors, and other benign skin tumors.

Epidemiology and Demographics

The incidence of Merkel cell carcinoma ranges from 0.15 to 0.44 per 100,000 individuals. The median age at diagnosis is approximately 65 years. Merkel cell carcinoma is more common among males and individuals of Caucasian race.

Risk Factors

Risk factors for the development of Merkel cell cancer include old age, male gender, Caucasian race, chronic exposure to sunlight, immunodeficiency, and personal history of cancer.

Screening

Screening for Merkel cell cancer is not recommended.

Natural History, Complications and Prognosis

Merkel cell carcinoma is a highly aggressive tumor with a mortality rate that approaches 30% to 40% within 3 years of diagnosis. If diagnosed early, Merkel cell cancer has a good prognosis with a 5-year survival rate of approximately 90%.

Diagnosis

Staging

The staging of Merkel cell cancer is based on the TNM staging system.

History and Symptoms

Patients with Merkel cell carcinoma usually present with a rapid growing, painless, small mass that is typically located on sun exposed area of the body.

Physical Examination

Physical exam findings of Merkel cell cancer include a firm, dome-shaped or raised, red/violaceous skin mass that appear in sun exposed areas.

Laboratory Findings

CT

CT scan is useful in detecting metastasis to organs and regional lymph nodes.

Other Imaging Findings

Positron emission tomography maybe used in staging of Merkel cell cancer.

Other Diagnostic Studies

On biopsy, Merkel cell cancer is characterized by positivity on cytokeratin-20 immunohistochemical staining. On electron microscopy, the presence of neurosecretory granules within cytoplasmic extensions is characteristics.

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

  1. Allen PJ, Browne WB, Jaques DP; et al. (2005). "Merkel cell carcinoma: Prognosis and treatment of patients from a single institution". Journal of Clinical Oncology. 23 (10): 2300–2309.
  2. Spurgeon ME, Lambert PF (2013). "Merkel cell polyomavirus: a newly discovered human virus with oncogenic potential". Virology. 435 (1): 118–30. doi:10.1016/j.virol.2012.09.029. PMC 3522868. PMID 23217622.


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