Merkel cell carcinoma is a cutaneous neoplasm most commonly presenting in white males, in their 7th or 8th decade of life. Multiple white arsenical keratoses can also be seen in addition to the red nodule of the merkel cell. Aggressive merkel cell carcinoma in a liver transplant. Merkel cell carcinoma mcc is a rare neuroendocrine tumor that typically arises from the skinspeci.
Merkel cell carcinoma of the tongue and head and neck oral. The annual incidence of mcc is increasing in the usa. Asioli s, righi a, volante m, eusebi v, bussolati g. Merkel cell carcinoma mcc is a rare aggressive cutaneous tumor that commonly occurs in sunexposed sites. Mcc affects most frequently the elderly white population, only. Merkel cell carcinoma is a rare, but highly malignant tumor of the skin with. It has received limited coverage in the radiology literature with small case series and case reports often focused on metastatic disease and the role of imaging largely limited to staging. Merkel cell carcinoma mcc is a rare, aggressive, and often fatal cutaneous. Merkel cell carcinoma is also called neuroendocrine carcinoma of the skin. We hypothesize that highrisk patients with merkel cell carcinoma are best treated with multimodality therapy. Merkel cell carcinoma mcc is a malignant, cutaneous neuroendo crine tumour of the elderly with an increasing worldwide incidence. In summary, merkel cell tumor of the skin is a relatively rare neoplasm with a potentially aggres sive behavior. Merkel cell carcinoma mcc of vulva first reported in 1984 by bottles et al.
It usually originates in the dermis, but in rare instances it has arisen in other primary sites, including the oral mucosa and the parotid gland. Before skin graft followed by radiation could begin, new tumors started growing all around the original site. The diagnosis is made by histopathology, and an incisional or excisional. Merkel cell carcinoma mcc is a rare type of skin cancer. Merkel cell carcinoma histopathology reporting guide version 1. Frigerio b, capella c, eusebi v, tenti p, azzopardi jg. Abstract merkel cell carcinoma mcc is a rare skin cancer that is associated with merkel. The presence of an mcc in the parotid gland is extremely rare. The epidemiology, pathogenesis, clinical features, and diagnosis of mcc are discussed here. Merkel cell carcinoma is a rare type of skin cancer that usually starts in areas of skin exposed to the sun.
This is very important for patients with merkel cell carcinoma, as each persons experience of the disease and treatment can be highly variable depending on the specifics of the tumor. Merkel cell carcinoma is a neuroendocrine carcinoma composed of densely blue cells. Mcc tends to grow quickly and can be hard to treat if it spreads beyond the skin. Protocol for the examination of specimens from patients with. Find out more about risk factors, symptoms, tests to diagnose, prognosis, staging, and treatment for merkel. Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases. Merkel cell carcinoma mcc is a rare primary cutaneous neuroendocrine carcinoma. Merkel cell carcinoma see synoptic report refer to the international collaboration on cancer reporting iccr histopathology reporting guide 1st edition, 2019 and cap reporting guidelines for merkel cell carcinoma and merkel cell carcinoma staging. Histopathology nodular tumor of small blue cells epidermal, dermal, or subcutaneous sheets, nests, or trabeculae scant cytoplasm, high nuclear.
Merkel cell carcinoma is a rare tumor of the skin found in the dermis of the elderly patient. Merkel cell carcinoma mcc is an aggressive neuroendocrine skin cancer, which can be effectively controlled by. Malignant small round cell tumors msrct is a term used for tumors composed of malignant round cells that are slightly larger or double the size of red blood cells in airdried smears. It is best to seek at least one consultation from a multidisciplinary team of. An update on diagnostic features of merkel cell carcinoma. This coordinated approach helps make sure youll be treated by the physicians most expert in the care of your particular tumor. Merkel cells are small to mediumsized basophilic cells with round nuclei and scant cytoplasm. In some reports, irregular contours and abundant cytoplasm were associated with no detectible mcpyv.
The cell morphology and aggressive behavior of the neoplasm were compatible with malignant small round cell tumor msrct in humans, a term used for a group of neoplasms characterized by small. Merkel cell carcinoma symptoms and causes mayo clinic. On the other hand monotherapies using anthracycli nes, liposomal. Hypothesis merkel cell carcinoma is a rare dermal neuroendocrine carcinoma whose optimal treatment and prognostic factors are poorly defined. Evidence suggests that the pathogenesis of mcc is related to merkel cell polyomavirus infection or ultraviolet mutagenesis. Sun exposure and having a weak immune system can affect the risk of merkel cell carcinoma. The cell of origin remains debatable but the immunohistochemical profile and morphology resemble native merkel cells in the skin. The tumour is centered in the dermis with frequent involvement of the overlying epidermis figures 1, 2 and may invade the subcutaneous fat. Granular or stippled chromatin can be seen on histopathology figure 2a.
Merkel cell carcinoma mcc is an aggressive primary cutaneous neuroendocrine carcinoma that predominantly affects the elderly and immunosuppressed. Treating merkel cell carcinoma based on the extent of the. Haynes, md tumors in the lung small cell carcinoma and other. Mcc pathogenesis is associated with either the presence of merkel cell polyomavirus or chronic. Merkel cells are thought to be a type of skin neuroendocrine cell, because they share some features with nerve cells and. Histological, immunohistological, and clinical features of. Merkel cell carcinoma mcc is an unusual and highly aggressive skin cancer and often appears in the elderly population. The latter, termed combined merkel cell carcinomas, are usually characterized by admixed neuroendocrine and squamous elements, identifiable on routine microscopy e. Clinical differential diagnoses include basal cell carcinoma, cyst, amelanotic melanoma, lymphoma and atypical fibroxanthoma. Merkel cell carcinoma should be included in the differential diagnosis of head and neck mucosal lesions, especially if the tumor is submucosal, and mcc may involve the tongue. Table 1 tnm clinical staging for merkel cell carcinoma. Merkel cell tumor of the thigh university of michigan. Merkel cell carcinoma is a rare dermal malignant tumor with neuroendocrine elements. Merkel cell carcinoma mcc is a rare and aggressive skin cancer occurring in about 3 people per 1,000,000 members of the population.
Pathogenesis, clinical features, and diagnosis of merkel. Merkel cell carcinoma most often develops in older people. Histopathology shows round to oval tumor cells arranged in diffuse sheets. Seventy patients with merkel cell carcinoma were treated at memorial sloankettering cancer center between 1969 and 1989. Mucosal mcc is aggressive, and there is a high risk for local recurrence and regional and distant metastasis. Merkel cell carcinoma is a rare, but highly malignant tumor of the skin with high rates of metastasis and poor survival.
There are multiple treatments used for merkel cell carcinoma, and treatment is generally based on stage of the disease. It is also known as cutaneous apudoma, primary neuroendocrine carcinoma of the skin, primary small cell carcinoma of the skin, and trabecular carcinoma of the skin. Analysis of prognostic factors from 9387 merkel cell carcinoma cases forms the basis for the new 8th edition ajcc staging system. More than one half of all mccs occur on the head and neck. A merkel cell carcinoma arising on the foot of a japanese man exposed to arsenic. Merkel cell carcinoma is an aggressive tumour that usually arises on chronically sun exposed skin of the elderly. Early diagnosis using electron mi croscopy, light microscopy, and immunohisto chemical techniques are useful in establishing a diagnosis of merkel cell tumor. Pathologists suggest that this tumor should be called neuroendocrine carcinoma of the skin2 because it is highly analogous to such 8. Pdf on sep 1, 2018, iuliana giorgiana spiridon and others published merkel cell carcinoma of the upper extremity find, read and cite all the research you need on researchgate.
To study histopathology of malignant small round cell tumors. Update on eighth edition american joint committee on. Histologically, it is characterized by a proliferation of small round cells with a granular chromatin pattern and scant cytoplasm arranged in a trabecular. Two weeks after the first avelumab infusion mcc lesions were inflamed and slightly enlarged, consistent with. Histopathology and neuronspecific enolase positivity from a wide local exci sion were consistent with a diagnosis of merkel cell tumor. Departments of dermatology and pathology, the johns hopkins univer. Histological, immunohistological, and clinical features of merkel. Merkel cell carcinoma mcc is a rare but highly aggressive skin cancer with neuroendocrine features. The term round cell tumor describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with increased nuclearcytoplasmic ratio.
A rare clinical presentation and diagnostic challenge 17. Includes ptnm requirements from the 8th edition, ajcc staging manual. T n m t1 primary tumor 2 cm n1 lymph node positive m1 metastasis presence staging criteria. Primary merkel cell carcinoma of the parotid gland. We report a 64yearold man with a past history of rectal carcinoma and cutaneous squamous cell carcinoma and with a new onset scalp nodule clinically felt to represent a pilar cyst. Merkel cell carcinoma has a nonspecific clinical appearance.
Factors involved in the development of mcc include the merkel cell polyomavirus mcpyv or mcv, a. Some doctors might suggest treatments other than those listed here. Association between neuroendocrine merkel cell carcinoma and squamous carcinoma of the skin. Merkel cell carcinoma mcc is rare, so it has been hard for doctors to study how best to treat this cancer. It has a tendency for rapid growth, local recurrence, lymph node invasion, and distant metastases. Characteristically starts in a sunexposed area of the head, neck, arms or legs in whites 6080 years of age as a firm, painless, shiny lump that can be red, pink, or blue in color and vary in size from less than a quarter of an inch a half cm to more than two inches 5 cm in diameter. Histopathology of the excision demonstrated prominent solar elastosis and a dense, monomorphic, small blue cell tumor throughout the dermis. Treatment of merkel cell carcinoma merkel cell carcinoma.
Merkel cell carcinoma mcc of the skin is a rare, aggressive cutaneous malignancy that predominantly affects older adults with light skin types and has a propensity for local recurrence and regional lymph node metastases. Treatment decisions depend on many issues that are highly variable between patients. Factors predictive of improved survival included head and neck site and negative lymph nodes at. Primary merkel cell carcinoma mcc is a neuroendocrine tumor that typically affects older whites. A second surgery on march 12 removed the tumor and 17 lymph nodes. Merkel cell carcinoma histopathology reporting guide.
With guidance from the cap cancer and cap pathology electronic reporting committees. Timely diagnosis and proper staging of this tumour are crucial as mcc has high rates of regional recurrence and lymph node and distant metastasis. Clinically, mcc typically presents as an asymptomatic violaceous. Risk factors include immunosuppression, advanced age, exposure to uv radiation, and infection with the merkel cell polyomavirus. Merkel cell carcinoma is a rare and highly aggressive cutaneous neuroendocrine carcinoma that, in most cases, is caused by merkel cell polyomavirus. Merkel cell carcinoma mcc is a rare neuroendocrine carcinoma of the skin. Diffuse lichen planuslike keratoses and clinical pseudo. Mcc has a high propensity for local recurrence, as well as regional and distant metastases. It starts when cells in the skin called merkel cells start to grow out of control. Merkel cell carcinoma can be distinguished from metastatic small cell carcinoma using antibodies to cytokeratin 20 and thyroid transcription factor 1. Association of merkel cell polyomavirus infection with. An infrequent but highly malignant type of skin cancer. Treatment depends mainly on how far the cancer has spread, so having the right tests done to determine the extent of the cancer such as a sentinel lymph node biopsy or. Pdf merkel cell carcinoma is a rare, aggressive cutaneous malignancy with high rates of.
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