Merkel cell carcinoma is a rare dermal malignant tumor with neuroendocrine elements. 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. Histopathology shows round to oval tumor cells arranged in diffuse sheets. Departments of dermatology and pathology, the johns hopkins univer. Seventy patients with merkel cell carcinoma were treated at memorial sloankettering cancer center between 1969 and 1989. Clinically, mcc typically presents as an asymptomatic violaceous. Merkel cell carcinoma mcc is a rare neuroendocrine tumor that typically arises from the skinspeci.
Treating merkel cell carcinoma based on the extent of the. Merkel cell carcinoma is a rare tumor of the skin found in the dermis of the elderly patient. It starts when cells in the skin called merkel cells start to grow out of control. Haynes, md tumors in the lung small cell carcinoma and other. This tumor continues to pose problems in diagnosis and effective treatment for physicians unfamiliar with its biological characteristics. Analysis of prognostic factors from 9387 merkel cell carcinoma cases forms the basis for the new 8th edition ajcc staging system. Evidence suggests that the pathogenesis of mcc is related to merkel cell polyomavirus infection or ultraviolet mutagenesis. Treatment of merkel cell carcinoma merkel cell carcinoma. Histopathology of the excision demonstrated prominent solar elastosis and a dense, monomorphic, small blue cell tumor throughout the dermis. Merkel cell carcinoma mcc is a rare aggressive cutaneous tumor that commonly occurs in sunexposed sites.
Merkel cell carcinoma mcc is an aggressive neuroendocrine skin cancer, which can be effectively controlled by. Early diagnosis using electron mi croscopy, light microscopy, and immunohisto chemical techniques are useful in establishing a diagnosis of merkel cell tumor. Merkel cell tumor of the thigh university of michigan. Merkel cell carcinoma is a rare, but highly malignant tumor of the skin with high rates of metastasis and poor survival. More than one half of all mccs occur on the head and neck. 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.
To study histopathology of malignant small round cell tumors. The diagnosis is made by histopathology, and an incisional or excisional. 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. Merkel cell carcinoma is an aggressive tumour that usually arises on chronically sun exposed skin of the elderly. Merkel cell carcinoma can be distinguished from metastatic small cell carcinoma using antibodies to cytokeratin 20 and thyroid transcription factor 1. Histologically, it is characterized by a proliferation of small round cells with a granular chromatin pattern and scant cytoplasm arranged in a trabecular. Find out more about risk factors, symptoms, tests to diagnose, prognosis, staging, and treatment for merkel. Protocol for the examination of specimens from patients with. Merkel cell carcinoma is a cutaneous neoplasm most commonly presenting in white males, in their 7th or 8th decade of life. Pdf merkel cell carcinoma is a rare, aggressive cutaneous malignancy with high rates of. Includes ptnm requirements from the 8th edition, ajcc staging manual.
Merkel cell carcinoma mcc of vulva first reported in 1984 by bottles et al. Histological, immunohistological, and clinical features of merkel. Mcc pathogenesis is associated with either the presence of merkel cell polyomavirus or chronic. On the other hand monotherapies using anthracycli nes, liposomal. Two weeks after the first avelumab infusion mcc lesions were inflamed and slightly enlarged, consistent with. Pathologists suggest that this tumor should be called neuroendocrine carcinoma of the skin2 because it is highly analogous to such 8. Merkel cell carcinoma histopathology reporting guide version 1. Cap merkel cell carcinoma protocol summary of changes. Merkel cell carcinoma is a rare type of skin cancer that usually starts in areas of skin exposed to the sun. An update on diagnostic features of merkel cell carcinoma. The cell of origin remains debatable but the immunohistochemical profile and morphology resemble native merkel cells in the skin.
Before skin graft followed by radiation could begin, new tumors started growing all around the original site. Asioli s, righi a, volante m, eusebi v, bussolati g. Merkel cell carcinoma is also called neuroendocrine carcinoma of the skin. 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. Primary merkel cell carcinoma of the parotid gland. Hypothesis merkel cell carcinoma is a rare dermal neuroendocrine carcinoma whose optimal treatment and prognostic factors are poorly defined. Some doctors might suggest treatments other than those listed here. Merkel cell carcinoma mcc is a rare type of skin cancer. We hypothesize that highrisk patients with merkel cell carcinoma are best treated with multimodality therapy. Treatment decisions depend on many issues that are highly variable between patients. Merkel cell carcinoma most often develops in older people. Merkel cell carcinoma is a rare type of skin cancer that usually appears as a fleshcolored or bluishred nodule, often on your face, head or neck. Mcc affects most frequently the elderly white population, only. Primary merkel cell carcinoma mcc is a neuroendocrine tumor that typically affects older whites.
Clinical differential diagnoses include basal cell carcinoma, cyst, amelanotic melanoma, lymphoma and atypical fibroxanthoma. Over 400 cases of neuroendocrine merkel cell carcinoma of the skin ncs have been reported. In some reports, irregular contours and abundant cytoplasm were associated with no detectible mcpyv. A second surgery on march 12 removed the tumor and 17 lymph nodes. Merkel cell carcinoma mcc is an unusual and highly aggressive skin cancer and often appears in the elderly population. Factors predictive of improved survival included head and neck site and negative lymph nodes at. 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.
Table 1 tnm clinical staging for merkel cell carcinoma. Update on eighth edition american joint committee on. 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. 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 cells are small to mediumsized basophilic cells with round nuclei and scant cytoplasm. Granular or stippled chromatin can be seen on histopathology figure 2a. Aggressive merkel cell carcinoma in a liver transplant. Sun exposure and having a weak immune system can affect the risk of merkel cell carcinoma. Pathogenesis, clinical features, and diagnosis of merkel.
Association between neuroendocrine merkel cell carcinoma and squamous carcinoma of the skin. Timely diagnosis and proper staging of this tumour are crucial as mcc has high rates of regional recurrence and lymph node and distant metastasis. The presence of an mcc in the parotid gland is extremely rare. Histopathology and neuronspecific enolase positivity from a wide local exci sion were consistent with a diagnosis of merkel cell tumor. An infrequent but highly malignant type of skin cancer. Merkel cell carcinoma mcc is rare, so it has been hard for doctors to study how best to treat this cancer. Diffuse lichen planuslike keratoses and clinical pseudo. This coordinated approach helps make sure youll be treated by the physicians most expert in the care of your particular tumor. Multiple white arsenical keratoses can also be seen in addition to the red nodule of the merkel cell. 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.
T n m t1 primary tumor 2 cm n1 lymph node positive m1 metastasis presence staging criteria. 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. Histological, immunohistological, and clinical features of. A merkel cell carcinoma arising on the foot of a japanese man exposed to arsenic. Merkel cell carcinoma mcc is a rare primary cutaneous neuroendocrine carcinoma. Histopathology nodular tumor of small blue cells epidermal, dermal, or subcutaneous sheets, nests, or trabeculae scant cytoplasm, high nuclear. Merkel cell carcinoma of the tongue and head and neck oral. Merkel cell carcinoma is a rare, but highly malignant tumor of the skin with.
Risk factors include immunosuppression, advanced age, exposure to uv radiation, and infection with the merkel cell polyomavirus. 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. Merkel cell carcinoma mcc is a rare, aggressive, and often fatal cutaneous. The first surgeon could not remove all of the tumor. 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.
The epidemiology, pathogenesis, clinical features, and diagnosis of mcc are discussed here. Merkel cells are thought to be a type of skin neuroendocrine cell, because they share some features with nerve cells and. With guidance from the cap cancer and cap pathology electronic reporting committees. Merkel cell carcinoma histopathology reporting guide. Mucosal mcc is aggressive, and there is a high risk for local recurrence and regional and distant metastasis. Merkel cell carcinoma mcc is a rare and aggressive skin cancer occurring in about 3 people per 1,000,000 members of the population. We report a case of a 67yearold woman with rapidly. There are multiple treatments used for merkel cell carcinoma, and treatment is generally based on stage of the disease. A rare clinical presentation and diagnostic challenge 17. Factors involved in the development of mcc include the merkel cell polyomavirus mcpyv or mcv, a. It has a tendency for rapid growth, local recurrence, lymph node invasion, and distant metastases. Mcc tends to grow quickly and can be hard to treat if it spreads beyond the skin. Merkel cell carcinoma usually appears as a single painless lump on sunexposed skin. In summary, merkel cell tumor of the skin is a relatively rare neoplasm with a potentially aggres sive behavior.
Association of merkel cell polyomavirus infection with. 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 demographics, morphology, and survival based on 3870 cases. Merkel cell carcinoma mcc is an aggressive primary cutaneous neuroendocrine carcinoma that predominantly affects the elderly and immunosuppressed. Mcc has a high propensity for local recurrence, as well as regional and distant metastases. Merkel cell carcinoma mcc is a malignant, cutaneous neuroendo crine tumour of the elderly with an increasing worldwide incidence. The latter, termed combined merkel cell carcinomas, are usually characterized by admixed neuroendocrine and squamous elements, identifiable on routine microscopy e.
Merkel cell carcinoma mcc is a rare tumour of the skin of neuroendocrine ori. Merkel cell carcinoma has a nonspecific clinical appearance. Merkel cell carcinoma is a neuroendocrine carcinoma composed of densely blue cells. 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 mcc is a rare neuroendocrine carcinoma of the skin. Merkel cell carcinoma mcc is a rare but highly aggressive skin cancer with neuroendocrine features. The annual incidence of mcc is increasing in the usa. 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. Ultraviolet radiation, immunosuppression and the merkel cell polyomavirus mcpyv are thought to be causative factors. The tumour is centered in the dermis with frequent involvement of the overlying epidermis figures 1, 2 and may invade the subcutaneous fat. 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.
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