Reactivity with TdT in Merkel cell carcinoma: a potential diagnostic pitfall
Am J Clin Pathol. 2008 Jun
Buresh CJ, Oliai BR, Miller RT.
Immunohistochemistry Division, ProPath Laboratory, Dallas, TX 75247, USA.
Merkel cell carcinoma (MCC) is a high-grade neuroendocrine carcinoma of skin characterized by cells with a "blastic" appearance, scant cytoplasm, and fine, evenly distributed chromatin. Terminal deoxynucleotidyl transferase (TdT) is a DNA polymerase present in thymic T cells, lymphoblastic lymphoma/leukemia, and some cases of acute myeloid leukemia. After observing TdT immunoreactivity in a case of MCC, we analyzed 26 tumors by immunohistochemical analysis to determine their spectrum of reactivity with TdT and identified TdT in 19 (73%) of 26 MCCs. Staining intensity was variable but was often moderate to strong and present in a significant percentage of cells. Because MCC has cytomorphologic features similar to those of lymphoblastic lymphoma and may manifest as metastatic disease, reactivity with TdT in MCC could represent a diagnostic pitfall in the differential diagnosis with lymphoblastic lymphoma, particularly because the latter may lack CD45 and/or CD20, yet both neoplasms may express PAX-5, a B-cell-associated marker.
MetaPress American Journal of Clinical Pathology
Tuesday, December 30, 2008
Merkel cell carcinoma: molecular pathogenesis, clinical features and therapy.
Merkel cell carcinoma: molecular pathogenesis, clinical features and therapy.
J Dtsch Dermatol Ges. 2008 Sep
Becker JC, Kauczok CS, Ugurel S, Eib S, Bröcker EB, Houben R.
Department of Dermatology, Venereology and Allergy, University of Würzburg, Germany. becker_jc@klinik.uni-wuerzburg.de
Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine carcinoma of the skin. The incidence of this rare tumor is increasing rapidly; the American Cancer Society estimates for 2008 almost 1500 new cases in the U.S. Thus, the incidence of MCC will exceed the incidence of cutaneous T-cell lymphoma. Moreover, the mortality rate of MCC with 33% is considerably higher than that of cutaneous melanoma. These clinical observations are especially disturbing as we are only recently beginning to understand the pathogenesis of MCC. For the same reason, the therapeutic approach is often unclear; reliable data are only available for the therapy of locoregional disease.
Wiley InterScience
J Dtsch Dermatol Ges. 2008 Sep
Becker JC, Kauczok CS, Ugurel S, Eib S, Bröcker EB, Houben R.
Department of Dermatology, Venereology and Allergy, University of Würzburg, Germany. becker_jc@klinik.uni-wuerzburg.de
Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine carcinoma of the skin. The incidence of this rare tumor is increasing rapidly; the American Cancer Society estimates for 2008 almost 1500 new cases in the U.S. Thus, the incidence of MCC will exceed the incidence of cutaneous T-cell lymphoma. Moreover, the mortality rate of MCC with 33% is considerably higher than that of cutaneous melanoma. These clinical observations are especially disturbing as we are only recently beginning to understand the pathogenesis of MCC. For the same reason, the therapeutic approach is often unclear; reliable data are only available for the therapy of locoregional disease.
Wiley InterScience
Sunday, November 23, 2008
Treatment Option Overview by Stage for Merkel Cell Carcinoma
Treatment Option Overview by Stage for Merkel Cell Carcinoma
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How Merkel cell carcinoma is treated
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There are treatments for all patients with Merkel cell carcinoma. Three kinds of treatment are used:
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surgery (taking out the cancer)
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radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)
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chemotherapy (using drugs to kill cancer cells)
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There are several different types of surgery that may be used to remove the tumor. These include:
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Wide surgical excision takes out the cancer and some of the skin around the tumor.
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Cryosurgery freezes the tumor and then removes it.
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Micrographic surgery is a tissue-sparing technique that removes only the tumor.
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Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors.
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Radiation may come from a machine outside the body (external radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy).
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Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body.
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If a doctor removes all the cancer that can be seen at the time of the operation, a patient may be given chemotherapy after surgery to kill any cancer cells that are left. Chemotherapy given after an operation to a person who has no cancer cells that can be found is called adjuvant chemotherapy.
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Treatment may be one of the following:
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1. Surgery alone.
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2. Surgery followed by radiation therapy to the tumor site and regional lymph nodes.
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STAGE II MERKEL CELL CARCINOMA
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Treatment may be one of the following:
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1. Surgery alone.
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2. Surgery followed by radiation therapy to the tumor site and regional lymph nodes.
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3. Surgery with or without radiation therapy followed by adjuvant chemotherapy.
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STAGE III MERKEL CELL CARCINOMA
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Treatment will probably be chemotherapy.
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RECURRENT MERKEL CELL CARCINOMA
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Treatment may be one of the following:
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1. Surgery alone.
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2. Surgery followed by radiation therapy to the tumor site and regional lymph nodes.
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3. Surgery with or without radiation therapy followed by adjuvant chemotherapy.
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Stages of Merkel cell carcinoma
Stages of Merkel cell carcinoma
Stage Explanation
After Merkel cell carcinoma has been diagnosed (found), more tests will be done to find out if cancer cells have spread from the place the cancer started to other parts of the body. The process used to find out whether the cancer has spread to other parts of the body is called staging. It is important to know the stage of the disease to plan the best treatment. The following stages are used for Merkel cell carcinoma:
Stage I
The primary tumor has not spread to lymph nodes or other parts of the body. Lymph nodes are small, bean-shaped structures that are found throughout the body. They produce and store infection-fighting cells.
Stage II
The cancer has spread to nearby lymph nodes, but has not spread to other parts of the body.
Stage III
The cancer has spread beyond nearby lymph nodes and to other parts of the body.
Recurrent stage
Recurrent disease means that the cancer has recurred (come back) after it has been treated. It may come back in the same part of the body or in another part of the body.
Cancer Web UK
Stage Explanation
After Merkel cell carcinoma has been diagnosed (found), more tests will be done to find out if cancer cells have spread from the place the cancer started to other parts of the body. The process used to find out whether the cancer has spread to other parts of the body is called staging. It is important to know the stage of the disease to plan the best treatment. The following stages are used for Merkel cell carcinoma:
Stage I
The primary tumor has not spread to lymph nodes or other parts of the body. Lymph nodes are small, bean-shaped structures that are found throughout the body. They produce and store infection-fighting cells.
Stage II
The cancer has spread to nearby lymph nodes, but has not spread to other parts of the body.
Stage III
The cancer has spread beyond nearby lymph nodes and to other parts of the body.
Recurrent stage
Recurrent disease means that the cancer has recurred (come back) after it has been treated. It may come back in the same part of the body or in another part of the body.
Cancer Web UK
Merkel cell carcinoma: outcome and role of radiotherapy
Merkel cell carcinoma: outcome and role of radiotherapy
Cancer Radiother. 2008 Sep
Salvador Alonso R, Lahbabi I, Ben Hassel M, Boisselier P, Chaari N, Lesimple T, Chevrier S, de Crevoisier R.
Département de radiothérapie, centre Eugène-Marquis, avenue Bataille-Flandres-Dunkerque, 35042 Rennes, France.
Merkel cell carcinoma (MCC) are rare neuroendocrine malignant tumor of the skin, occurring in elderly patients. It affects primarily the sun-exposed areas of the skin, with approximately 50% of all tumors occurring in the face and neck and 40% in the extremities. Immunohistochemical markers (CK20+, CK7- and TTF1-) are used to distinguish between MCC and other tumors. MCC have a tendency to rapid local progression, frequent spread to regional lymph nodes and distant metastases. Due to the rarity of the disease, the optimal treatment has not been fully defined. Localized stages (stages I and II) are treated by surgical excision of the primary tumor (with 2 to 3 cm margin) and lymphadenectomy in case of node-positive disease, followed by external beam radiotherapy (EBRT) to a total dose of 50 to 60Gy in the tumor bed. Adjuvant EBRT has been shown to decrease markedly locoregional recurrences and to increase survival in recent studies. Treatment of lymph nodes area is more controversial. Chemotherapy is recommended only for metastatic disease.
Eslevier
Cancer Radiother. 2008 Sep
Salvador Alonso R, Lahbabi I, Ben Hassel M, Boisselier P, Chaari N, Lesimple T, Chevrier S, de Crevoisier R.
Département de radiothérapie, centre Eugène-Marquis, avenue Bataille-Flandres-Dunkerque, 35042 Rennes, France.
Merkel cell carcinoma (MCC) are rare neuroendocrine malignant tumor of the skin, occurring in elderly patients. It affects primarily the sun-exposed areas of the skin, with approximately 50% of all tumors occurring in the face and neck and 40% in the extremities. Immunohistochemical markers (CK20+, CK7- and TTF1-) are used to distinguish between MCC and other tumors. MCC have a tendency to rapid local progression, frequent spread to regional lymph nodes and distant metastases. Due to the rarity of the disease, the optimal treatment has not been fully defined. Localized stages (stages I and II) are treated by surgical excision of the primary tumor (with 2 to 3 cm margin) and lymphadenectomy in case of node-positive disease, followed by external beam radiotherapy (EBRT) to a total dose of 50 to 60Gy in the tumor bed. Adjuvant EBRT has been shown to decrease markedly locoregional recurrences and to increase survival in recent studies. Treatment of lymph nodes area is more controversial. Chemotherapy is recommended only for metastatic disease.
Eslevier
Wednesday, November 12, 2008
Merkel cell carcinoma: histologic features and prognosis.
Merkel cell carcinoma: histologic features and prognosis.
Cancer. 2008 Nov
Andea AA, Coit DG, Amin B, Busam KJ.
Department of Pathology, Memorial Sloan- Kettering Cancer Center, New York, New York, USA. aandea@uab.edu
BACKGROUND: Currently, little is known regarding the potential prognostic value of histologic features in primary cutaneous neuroendocrine (Merkel cell) carcinomas (MCC).
METHODS: In a retrospective review of the tumor histology and clinical outcome data (median follow-up, 51 months; range, 3-224 months) of 156 patients with a diagnosis of MCC, the following histologic features were evaluated: tumor thickness, tumor size (greatest dimension of the tumor), microanatomic compartment involved by tumor (dermis and/or subcutis and/or deeper), tumor growth pattern (nodular circumscribed vs infiltrative), lymphovascular invasion (LVI), tumor-infiltrating lymphocytes, tumor necrosis, ulceration, and solar elastosis.
RESULTS: The overall 5-year survival rate was 67.5%. On univariate analysis, parameters that were associated significantly with survival were tumor thickness (P= .001), tumor size (P= .0002), deepest anatomic compartment involved by tumor (P= .0003), tumor growth pattern (P= .003), LVI (P< .00001), tumor-infiltrating lymphocytes (P= .05), and solar elastosis (P= .04). On multivariate analysis, the presence of a nodular growth pattern, low tumor depth, and absence of LVI were associated with longer survival.
CONCLUSIONS: In addition to the known prognostic value of tumor stage, 3 histologic features were identified to have prognostic significance: tumor thickness (depth of tumor invasion), the presence of LVI, and tumor growth pattern.
Wiley InterScience
Cancer. 2008 Nov
Andea AA, Coit DG, Amin B, Busam KJ.
Department of Pathology, Memorial Sloan- Kettering Cancer Center, New York, New York, USA. aandea@uab.edu
BACKGROUND: Currently, little is known regarding the potential prognostic value of histologic features in primary cutaneous neuroendocrine (Merkel cell) carcinomas (MCC).
METHODS: In a retrospective review of the tumor histology and clinical outcome data (median follow-up, 51 months; range, 3-224 months) of 156 patients with a diagnosis of MCC, the following histologic features were evaluated: tumor thickness, tumor size (greatest dimension of the tumor), microanatomic compartment involved by tumor (dermis and/or subcutis and/or deeper), tumor growth pattern (nodular circumscribed vs infiltrative), lymphovascular invasion (LVI), tumor-infiltrating lymphocytes, tumor necrosis, ulceration, and solar elastosis.
RESULTS: The overall 5-year survival rate was 67.5%. On univariate analysis, parameters that were associated significantly with survival were tumor thickness (P= .001), tumor size (P= .0002), deepest anatomic compartment involved by tumor (P= .0003), tumor growth pattern (P= .003), LVI (P< .00001), tumor-infiltrating lymphocytes (P= .05), and solar elastosis (P= .04). On multivariate analysis, the presence of a nodular growth pattern, low tumor depth, and absence of LVI were associated with longer survival.
CONCLUSIONS: In addition to the known prognostic value of tumor stage, 3 histologic features were identified to have prognostic significance: tumor thickness (depth of tumor invasion), the presence of LVI, and tumor growth pattern.
Wiley InterScience
Merkel cell carcinoma within follicular cysts: report of two cases.
Merkel cell carcinoma within follicular cysts: report of two cases.
Requena L, Jaqueti G, Rütten A, Mentzel T, Kutzner H.
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
Merkel cell carcinoma is a rare cutaneous neoplasm of unknown histogenesis. Several reports have described the association of Merkel cell carcinoma of the skin with other cutaneous neoplasms within the same lesion, and there are also reports describing three examples of Merkel cell carcinoma within follicular cysts. We describe two examples of Merkel cell carcinoma developed within epithelial cysts. Neoplastic cells of Merkel cell tumor expressed immunoreactivity for chromogranin, synaptophysin, neuron-specific enolase, CAM 5.2 and cytokeratin 20, the last two markers showing the characteristic paranuclear dot-like pattern. In contrast, the epithelial wall lining the cyst and surrounding Merkel cell tumor only expressed immunoreactivity for cytokeratin MNF116. The description of five cases of Merkel cell carcinoma within follicular cysts, including the two cases of this report, support some relationship between Merkel cell tumor and the hair follicle.
J Cutan Pathol. 2008 Dec
PMID: 18988316 [PubMed - in process]
Requena L, Jaqueti G, Rütten A, Mentzel T, Kutzner H.
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
Merkel cell carcinoma is a rare cutaneous neoplasm of unknown histogenesis. Several reports have described the association of Merkel cell carcinoma of the skin with other cutaneous neoplasms within the same lesion, and there are also reports describing three examples of Merkel cell carcinoma within follicular cysts. We describe two examples of Merkel cell carcinoma developed within epithelial cysts. Neoplastic cells of Merkel cell tumor expressed immunoreactivity for chromogranin, synaptophysin, neuron-specific enolase, CAM 5.2 and cytokeratin 20, the last two markers showing the characteristic paranuclear dot-like pattern. In contrast, the epithelial wall lining the cyst and surrounding Merkel cell tumor only expressed immunoreactivity for cytokeratin MNF116. The description of five cases of Merkel cell carcinoma within follicular cysts, including the two cases of this report, support some relationship between Merkel cell tumor and the hair follicle.
J Cutan Pathol. 2008 Dec
PMID: 18988316 [PubMed - in process]
Tuesday, November 4, 2008
Merkel Cell Carcinoma
Merkel Cell Carcinoma
MD Anderson Cancer Center
Merkel Cell Carcinoma
Merkel Cell Carcinoma (MCC) is a rare skin malignancy which was first described in 1972 by Cyril Toker. However, the name Merkel cell comes from the anatomist who discovered the normal Merkel cell in pig snouts - Friedreich Sigmund Merkel in 1875. Because of the tumor cell characteristics seen under the microscope, the name neuroendocrine carcinoma of the skin is used synonymously with Merkel cell carcinoma.
The true incidence of this cancer is not known, but 400 - 600 cases have been described in the literature. Merkel cell carcinoma is almost exclusively seen in Caucasian people, and most are more than 65 years old. Some of the literature reports that MCC affects men and women equally, but other studies have shown a male predominance. The etiology of this tumor is not clearly defined, but there has been association of MCC to sun exposure. Many of the patients have a history of other basal or squamous cell cancers of the skin, and approximately 10% of the patients will develop a second primary cancer.
Merkel cell carcinoma most commonly occurs in the skin of the head and neck (50%), followed by the extremities and the trunk. The head and neck, cheek, forehead, temple, nose, and upper neck are the most common sites. This tumor is innocuous in appearance - described as a painless, solitary, raised, pink nodule. Most tumors are less than 2 cm when initially discovered. Biopsy is the only way to diagnose MCC. However, the diagnosis is difficult for most pathologists, and requires both the use of the electron microscope and special staining of the cells to differentiate it from other small cell carcinomas.
Merkel cell carcinoma is a very aggressive cancer. Lymph node metastasis occurs early and often. Some studies report palpable lymph nodes at presentation in 45% of patients. However, distant metastasis is rare at presentation. Local and regional recurrence occurs frequently, and is felt to be related to the adequacy of the initial therapy. Distant metastasis occurs in approximately 25% of patients, regardless of therapy.
The current thoughts on treating Merkel cell cancer include both the primary lesion and the regional lymphatics. It is felt that the primary cancer should be surgically removed with negative margins. Studies have shown that surgical treatment alone has a higher rate of local and regional recurrence that those who receive surgical excision and radiation therapy. Thus, after the tumor is removed, 6 weeks of radiation to the surgical site and to the regional lymphatics is recommended. However, despite this aggressive therapy, approximately 40% still develop locoregional recurrence.
It is felt that regardless of therapy, 25 - 35% of patients may ultimately recur. It is felt that the development of locoregional recurrence is a predictor of multiple recurrence and distant metastasis. Thus, it is implied that early locoregional treatment will improve tumor control. However, it is not proven whether locoregional control will affect the rate of distant metastasis and ultimately affect survival.
MD Anderson Cancer Center
Merkel Cell Carcinoma
Merkel Cell Carcinoma (MCC) is a rare skin malignancy which was first described in 1972 by Cyril Toker. However, the name Merkel cell comes from the anatomist who discovered the normal Merkel cell in pig snouts - Friedreich Sigmund Merkel in 1875. Because of the tumor cell characteristics seen under the microscope, the name neuroendocrine carcinoma of the skin is used synonymously with Merkel cell carcinoma.
The true incidence of this cancer is not known, but 400 - 600 cases have been described in the literature. Merkel cell carcinoma is almost exclusively seen in Caucasian people, and most are more than 65 years old. Some of the literature reports that MCC affects men and women equally, but other studies have shown a male predominance. The etiology of this tumor is not clearly defined, but there has been association of MCC to sun exposure. Many of the patients have a history of other basal or squamous cell cancers of the skin, and approximately 10% of the patients will develop a second primary cancer.
Merkel cell carcinoma most commonly occurs in the skin of the head and neck (50%), followed by the extremities and the trunk. The head and neck, cheek, forehead, temple, nose, and upper neck are the most common sites. This tumor is innocuous in appearance - described as a painless, solitary, raised, pink nodule. Most tumors are less than 2 cm when initially discovered. Biopsy is the only way to diagnose MCC. However, the diagnosis is difficult for most pathologists, and requires both the use of the electron microscope and special staining of the cells to differentiate it from other small cell carcinomas.
Merkel cell carcinoma is a very aggressive cancer. Lymph node metastasis occurs early and often. Some studies report palpable lymph nodes at presentation in 45% of patients. However, distant metastasis is rare at presentation. Local and regional recurrence occurs frequently, and is felt to be related to the adequacy of the initial therapy. Distant metastasis occurs in approximately 25% of patients, regardless of therapy.
The current thoughts on treating Merkel cell cancer include both the primary lesion and the regional lymphatics. It is felt that the primary cancer should be surgically removed with negative margins. Studies have shown that surgical treatment alone has a higher rate of local and regional recurrence that those who receive surgical excision and radiation therapy. Thus, after the tumor is removed, 6 weeks of radiation to the surgical site and to the regional lymphatics is recommended. However, despite this aggressive therapy, approximately 40% still develop locoregional recurrence.
It is felt that regardless of therapy, 25 - 35% of patients may ultimately recur. It is felt that the development of locoregional recurrence is a predictor of multiple recurrence and distant metastasis. Thus, it is implied that early locoregional treatment will improve tumor control. However, it is not proven whether locoregional control will affect the rate of distant metastasis and ultimately affect survival.
Merkel cell carcinoma. On one case
Merkel cell carcinoma. On one case
An Sist Sanit Navar. 2008 May-Aug
Cires Bezanilla M, González C, Jiménez F, Rubio T, Amat I.
Servicio de Cirugía General, Hospital García Orcoyen, Estella, 31200, Spain. mcires@gmail.com.
We present the case of an 86 year old woman who was sent for consultation at General Surgery due to asymptomatic tumouration on the back face of the left thigh whose size had increased during the 2 previous months. Physical exploration revealed tumouration that was painless, excrescent, indurated, mobile and well delimited, with a diameter of 5 cm, a reddish colour and a rough surface. Tumoural extirpation was carried out with broad margins, with a hystopathological result of primary cutaneous neuro-endocrynal carcinoma or Merkel cell carcinoma. Computerised tomography was carried out, observing tumoural adenopathies in the para-aortic spaces, iliac chains and left femorals, as well as edema in the lower left extremity.
The patient was sent to the Oncology service for treatment, without presenting a satisfactory evolution and with a progressive deterioration of her general state, dying within the first year after diagnosis.
Sunday, October 26, 2008
What is Merkel cell carcinoma?
What is Merkel cell carcinoma?
Merkel cell carcinoma (MCC) is a rare, aggressive type of skin cancer that forms on or just under the skin. It is also called primary small cell carcinoma of the skin, trabecular carcinoma, APUDoma, neuroendocrine carcinoma, endocrine carcinoma, or primary undifferentiated tumor of the skin. MCC is believed to start in neuroendocrine cells called Merkel cells. These cells release hormones into the blood when stimulated by the nervous system. They migrate from part of the nervous system called the neural crest to the skin. Merkel cells are believed to play a role in making the skin sensitive to touch.
How often does Merkel cell carcinoma occur?
Approximately 1,200 new cases of MCC are diagnosed in the United States each year, compared with almost 60,000 new cases of melanoma and more than 1 million new cases of nonmelanoma skin cancer. The incidence of MCC has been rising, with a 3-fold increase between 1986 and 2001. Most patients diagnosed with MCC are over age 50 at diagnosis (the average age is 69), with only 5 percent of cases diagnosed in those under age 50. MCC is more common in white people than in other racial/ethnic groups. Some cases have been reported in Japanese people, but very few have been seen in black people.
What are the possible causes of Merkel cell carcinoma?
The exact cause of MCC is unknown, but it appears to be linked to sun exposure and immunosuppression (suppression of the body's immune system and its ability to fight infections or disease). Sun exposure as a risk factor for MCC is supported by data that show a rise in incidence corresponding with the solar UVB index (scale indicating the intensity of solar ultraviolet-B (UVB) radiation at noon for a particular location). MCC has been linked to conditions such as HIV infection, chronic lymphocytic leukemia, Hodgkin lymphoma (cancer of the lymph system), ectodermal dysplasia (a disease involving abnormal tissue development), and Cowden disease (a disease in which masses of abnormal but benign tissues grow in multiple sites in the body). Other possible causes include exposure to arsenic and treatment for psoriasis that uses psoralens (a medication that causes the skin to become sensitive to light) and ultraviolet-A light (PUVA).
What are the symptoms of Merkel cell carcinoma?
The most common symptom of any skin cancer, including MCC, is a change in the skin, especially a change in an existing mole or a new growth. MCC appears as a firm, painless lump within the skin that may resemble a cyst but is fixed; i.e., cannot be moved. The lump is usually less than 2 cm (about ¾ inch) in size and can be red, pink, or blue-violet. MCC is different from other skin cancers in that it grows rapidly over a few weeks or months.
Where does Merkel cell carcinoma develop?
MCC is usually found on sun-exposed areas of the body. Fifty percent of cases occur on the head and neck, especially around the eye and on the eyelid. Forty percent of cases occur on the arms and legs. MCC has also been found on the trunk and other areas of the skin that are not usually exposed to the sun.
How is Merkel cell carcinoma diagnosed and staged?
The doctor may use the following procedures and tests to diagnose MCC. Some of these tests are also used to help determine the stage of the disease. Stage is a description of the extent of cancer.
A biopsy is the removal of cells or tissue from a tumor for examination by a pathologist. The pathologist may study tissue samples under a microscope or perform other tests on the cells or tissue. Biopsies are used for both diagnosis and staging. The surgeon may also remove lymph nodes (small, round organs that trap cancer cells, bacteria, or other harmful substances) to help determine the stage of the disease.
Sentinel lymph node (SLN) biopsy is a procedure in which the sentinel lymph node is removed and examined under a microscope to determine whether cancer cells are present. The sentinel lymph node is the first lymph node to which cancer is likely to spread from the primary tumor. SLN biopsy is used to help determine the stage of the disease. SLN biopsy may cause fewer side effects than standard lymph node removal because fewer lymph nodes are taken out.
Immunohistochemistry (staining of cells with agents that react with antibodies on the surface of cancer cells) is a laboratory technique used to tell the difference between MCC and other types of cancer.
Computed tomography (CT), a procedure that uses special x-ray equipment to obtain cross-sectional pictures of the body, can distinguish MCC from small cell lung cancer and show whether the disease has metastasized (spread) to other parts of the body.
In an octreotide scan (sometimes called Somatostatin Receptor Scintigraphy or SRS), the doctor injects a small amount of a radioactive drug into a vein. The drug travels through the bloodstream and attaches to tumor cells. A machine called a scanner detects the radioactive material and creates scans (pictures) showing where the tumor cells are located in the body. For MCC, this test can be used for both diagnosis and staging.
A PET scan uses radioactive sugar, which is absorbed by cancer cells and appears as dark areas on the scan. It can be used for both diagnosis and staging of MCC.
How is Merkel cell carcinoma treated?
Surgery is the most common treatment for MCC. Surgery with wide margins (a large border of healthy tissue removed with the tumor) is the recommended treatment for MCC. Mohs micrographic surgery, a technique in which individual layers of tissue are removed and examined under a microscope until all cancerous tissue has been removed, may be used instead of traditional surgery with wide margins. Mohs micrographic surgery may be a good alternative for MCC tumors on highly visible areas such as the face, and in areas where the surgeon would not be able to obtain wide margins.
The surgeon may remove lymph nodes to help stage the disease or to prevent recurrence (cancer coming back). The patient may also receive adjuvant radiation therapy (treatment given after the primary therapy) to decrease the chance of recurrence. Chemotherapy is the usual treatment if the disease has spread beyond the lymph nodes to areas that are not treatable by radiation therapy.
Supportive care is treatment given to improve the quality of life of patients who have a serious or life-threatening disease, such as cancer. It prevents or treats as early as possible the symptoms of the disease, side effects caused by treatment of the disease, and psychological, social, and spiritual problems related to the disease or its treatment. For example, anticancer drugs such as carboplatin and etoposide may be given to relieve symptoms in some patients with MCC. Radiation may be used to relieve pain from MCC that has metastasized to the brain or bones, and to reduce discomfort from skin problems associated with MCC (2). Additionally, meeting with a social worker, counselor, or member of the clergy can be helpful to those who want to talk about their feelings or discuss their concerns. A social worker can often suggest resources for help with recovery, emotional support, financial aid, transportation, or home care.
Are clinical trials (research studies) available? Where can people get more information about clinical trials?
Yes. The National Cancer Institute (NCI), a component of the National Institutes of Health, is sponsoring clinical trials that are designed to find new treatments and better ways to use current treatments. Before any new treatment can be recommended for general use, doctors conduct clinical trials to find out whether the treatment is safe for patients and effective against the disease. Participation in clinical trials may be a treatment option for patients with MCC.
What is the prognosis for patients with Merkel cell carcinoma?
Prognosis describes the likely course and outcome of a disease—that is, the chance that a patient will recover or have a recurrence. The prognosis for MCC patients depends greatly on the stage of the disease at the time of diagnosis. If the tumor is small (less than 2 cm or about ¾ inch) and cancer cells have not spread to the lymph nodes, the 5-year survival rate is more than 90 percent. Patients with MCC that has spread to the lymph nodes have a 5-year survival rate of about 50 percent. Overall 5-year survival for patients diagnosed with MCC is 64 percent, but half of patients with advanced MCC will live only 9 months. The disease recurs in about 50 percent of patients. It is important to keep in mind, however, that these statistics are averages based on large numbers of patients. Statistics cannot be used to predict what will happen to a particular patient because each person's situation is unique.
National Cancer Instsitute
Merkel cell carcinoma (MCC) is a rare, aggressive type of skin cancer that forms on or just under the skin. It is also called primary small cell carcinoma of the skin, trabecular carcinoma, APUDoma, neuroendocrine carcinoma, endocrine carcinoma, or primary undifferentiated tumor of the skin. MCC is believed to start in neuroendocrine cells called Merkel cells. These cells release hormones into the blood when stimulated by the nervous system. They migrate from part of the nervous system called the neural crest to the skin. Merkel cells are believed to play a role in making the skin sensitive to touch.
How often does Merkel cell carcinoma occur?
Approximately 1,200 new cases of MCC are diagnosed in the United States each year, compared with almost 60,000 new cases of melanoma and more than 1 million new cases of nonmelanoma skin cancer. The incidence of MCC has been rising, with a 3-fold increase between 1986 and 2001. Most patients diagnosed with MCC are over age 50 at diagnosis (the average age is 69), with only 5 percent of cases diagnosed in those under age 50. MCC is more common in white people than in other racial/ethnic groups. Some cases have been reported in Japanese people, but very few have been seen in black people.
What are the possible causes of Merkel cell carcinoma?
The exact cause of MCC is unknown, but it appears to be linked to sun exposure and immunosuppression (suppression of the body's immune system and its ability to fight infections or disease). Sun exposure as a risk factor for MCC is supported by data that show a rise in incidence corresponding with the solar UVB index (scale indicating the intensity of solar ultraviolet-B (UVB) radiation at noon for a particular location). MCC has been linked to conditions such as HIV infection, chronic lymphocytic leukemia, Hodgkin lymphoma (cancer of the lymph system), ectodermal dysplasia (a disease involving abnormal tissue development), and Cowden disease (a disease in which masses of abnormal but benign tissues grow in multiple sites in the body). Other possible causes include exposure to arsenic and treatment for psoriasis that uses psoralens (a medication that causes the skin to become sensitive to light) and ultraviolet-A light (PUVA).
What are the symptoms of Merkel cell carcinoma?
The most common symptom of any skin cancer, including MCC, is a change in the skin, especially a change in an existing mole or a new growth. MCC appears as a firm, painless lump within the skin that may resemble a cyst but is fixed; i.e., cannot be moved. The lump is usually less than 2 cm (about ¾ inch) in size and can be red, pink, or blue-violet. MCC is different from other skin cancers in that it grows rapidly over a few weeks or months.
Where does Merkel cell carcinoma develop?
MCC is usually found on sun-exposed areas of the body. Fifty percent of cases occur on the head and neck, especially around the eye and on the eyelid. Forty percent of cases occur on the arms and legs. MCC has also been found on the trunk and other areas of the skin that are not usually exposed to the sun.
How is Merkel cell carcinoma diagnosed and staged?
The doctor may use the following procedures and tests to diagnose MCC. Some of these tests are also used to help determine the stage of the disease. Stage is a description of the extent of cancer.
A biopsy is the removal of cells or tissue from a tumor for examination by a pathologist. The pathologist may study tissue samples under a microscope or perform other tests on the cells or tissue. Biopsies are used for both diagnosis and staging. The surgeon may also remove lymph nodes (small, round organs that trap cancer cells, bacteria, or other harmful substances) to help determine the stage of the disease.
Sentinel lymph node (SLN) biopsy is a procedure in which the sentinel lymph node is removed and examined under a microscope to determine whether cancer cells are present. The sentinel lymph node is the first lymph node to which cancer is likely to spread from the primary tumor. SLN biopsy is used to help determine the stage of the disease. SLN biopsy may cause fewer side effects than standard lymph node removal because fewer lymph nodes are taken out.
Immunohistochemistry (staining of cells with agents that react with antibodies on the surface of cancer cells) is a laboratory technique used to tell the difference between MCC and other types of cancer.
Computed tomography (CT), a procedure that uses special x-ray equipment to obtain cross-sectional pictures of the body, can distinguish MCC from small cell lung cancer and show whether the disease has metastasized (spread) to other parts of the body.
In an octreotide scan (sometimes called Somatostatin Receptor Scintigraphy or SRS), the doctor injects a small amount of a radioactive drug into a vein. The drug travels through the bloodstream and attaches to tumor cells. A machine called a scanner detects the radioactive material and creates scans (pictures) showing where the tumor cells are located in the body. For MCC, this test can be used for both diagnosis and staging.
A PET scan uses radioactive sugar, which is absorbed by cancer cells and appears as dark areas on the scan. It can be used for both diagnosis and staging of MCC.
How is Merkel cell carcinoma treated?
Surgery is the most common treatment for MCC. Surgery with wide margins (a large border of healthy tissue removed with the tumor) is the recommended treatment for MCC. Mohs micrographic surgery, a technique in which individual layers of tissue are removed and examined under a microscope until all cancerous tissue has been removed, may be used instead of traditional surgery with wide margins. Mohs micrographic surgery may be a good alternative for MCC tumors on highly visible areas such as the face, and in areas where the surgeon would not be able to obtain wide margins.
The surgeon may remove lymph nodes to help stage the disease or to prevent recurrence (cancer coming back). The patient may also receive adjuvant radiation therapy (treatment given after the primary therapy) to decrease the chance of recurrence. Chemotherapy is the usual treatment if the disease has spread beyond the lymph nodes to areas that are not treatable by radiation therapy.
Supportive care is treatment given to improve the quality of life of patients who have a serious or life-threatening disease, such as cancer. It prevents or treats as early as possible the symptoms of the disease, side effects caused by treatment of the disease, and psychological, social, and spiritual problems related to the disease or its treatment. For example, anticancer drugs such as carboplatin and etoposide may be given to relieve symptoms in some patients with MCC. Radiation may be used to relieve pain from MCC that has metastasized to the brain or bones, and to reduce discomfort from skin problems associated with MCC (2). Additionally, meeting with a social worker, counselor, or member of the clergy can be helpful to those who want to talk about their feelings or discuss their concerns. A social worker can often suggest resources for help with recovery, emotional support, financial aid, transportation, or home care.
Are clinical trials (research studies) available? Where can people get more information about clinical trials?
Yes. The National Cancer Institute (NCI), a component of the National Institutes of Health, is sponsoring clinical trials that are designed to find new treatments and better ways to use current treatments. Before any new treatment can be recommended for general use, doctors conduct clinical trials to find out whether the treatment is safe for patients and effective against the disease. Participation in clinical trials may be a treatment option for patients with MCC.
What is the prognosis for patients with Merkel cell carcinoma?
Prognosis describes the likely course and outcome of a disease—that is, the chance that a patient will recover or have a recurrence. The prognosis for MCC patients depends greatly on the stage of the disease at the time of diagnosis. If the tumor is small (less than 2 cm or about ¾ inch) and cancer cells have not spread to the lymph nodes, the 5-year survival rate is more than 90 percent. Patients with MCC that has spread to the lymph nodes have a 5-year survival rate of about 50 percent. Overall 5-year survival for patients diagnosed with MCC is 64 percent, but half of patients with advanced MCC will live only 9 months. The disease recurs in about 50 percent of patients. It is important to keep in mind, however, that these statistics are averages based on large numbers of patients. Statistics cannot be used to predict what will happen to a particular patient because each person's situation is unique.
National Cancer Instsitute
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