Merkel cell carcinoma with lymph node metastasis in the absence of a primary site: Case report and literature review.

Dec 2012

Zhao M, Meng MB.

Source

Tianjin University of Traditional Chinese Medicine; ; Tianjin Tasly Co. Ltd.;

Abstract 

Merkel cell carcinoma (MCC) is a rare malignant skin neoplasm with the potential for local recurrence, spreading to regional lymph nodes (LNs) and distant metastases. Although it has been identified in various anatomical sites, LN metastatic MCC in the absence of a primary site is extremely rare. The present case reports a 54-year-old male who initially underwent histological examination of a biopsy specimen from the right inguinal LNs. A diagnosis of metastatic small cell carcinoma was made. Nine months later, this diagnosis was changed to MCC with multiple metastases following observation of a tumour mass in the right dorsal thigh. Additionally, in the present study a summary is provided of 23 published cases of MCC with initial LN metastasis in the absence of a primary site, with details of clinical characteristics, natural history and pertinent therapy of this uncommon tumour. The present patient with LN metastatic MCC in the absence of a primary site and the other reported cases demonstrate that although multimodal treatment with surgery, radiotherapy (RT) and chemotherapy provides excellent local control, local recurrence and distant metastases commonly develop in this uncommon tumour. LN metastatic MCC in the absence of a primary site is a highly malignant disease and the role of adjuvant postoperative RT and/or chemotherapy remains to be fully determined.

PubMed

Merkel cell carcinoma.

Dec 2012

Han SY, North JP, Canavan T, Kim N, Yu SS.

Source

Department of Dermatology, University of California, San Francisco, 1701 Divisadero Street, 3rd Floor, San Francisco, CA 94115, USA.

Abstract

Merkel cell carcinoma (MCC) is a rare but aggressive carcinoma of the skin, arising most commonly in sun-exposed sites of elderly patients. The diagnosis is based on characteristic histopathologic features. In 2008, the discovery of the Merkel cell polyomavirus led to intensified research into the viral pathogenesisis of MCC. MCC staging guidelines were established in 2010, and it demonstrated the importance of distinguishing clinical vs. pathologic evaluation of lymph nodes in MCC. Surgery and/or radiation is of the mainstay of therapy for early disease, while chemotherapy is reserved for more advanced disease. Treatments based on immunologic mechanisms are currently in development.

SciVerse

Arm and Leg Swelling After Merkel Cell Cancer

Arm and Leg Swelling After Merkel Cell Cancer

With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.

Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.

This swelling occurs because of one of several factors.

First, the swelling begins after lymph nodes have been removed for cancer biopsies.

Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.

Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling begins.

This swelling is called lymphedema and it can effect either your leg or your arm. The swelling that occurs is permanent, and while it is not curable it is treatable.

Hopefully, in the future with radiological scans becoming more sensitive and with increased use of such techniques such as the small needle biopsy, we can put an end to this epidemic of secondary lymphedema from cancer biopsy.

Permanent Leg or Arm Swelling

In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered

There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.

Group One

This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.

Group Two

Another extremely large group that experiences permanent leg or arm swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid.

In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries.

This is also referred to as secondary lymphedema.

Group Three

Group three consists of people who have leg or arm swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.

The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later.

This type of leg or arm swelling is called primary lymphedema. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system.

This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.

WHAT IS LYMPHEDEMA?

Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.

A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.

WHAT ARE THE SYMPTOMS OF LYMPHEDEMA?

If you are an at risk person for arm lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.

1.) Unexplained aching, hurting or pain in the arm or leg

2.) Experiencing “fleeting lymphedema.” This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown arm lymphedema or leg lymphedema.

3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand. This is an indication of early lymphatic malfunction.

4.) Any arm inflammation, redness or infection.

5.) You may experience a feeling of tightness, heaviness or weakness of the arm.

The symptoms for leg lymphedema are very similar to ones for the arm.

HOW IS LYMPHEDEMA TREATED?

The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MLD), there are variances, but most involve these two type of treatment.

It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally.

With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.

WHAT ARE SOME OF THE COMPLICATIONS OF LYMPHEDEMA?

1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immunocompromised.

2. Draining wounds that leak lymphorrhea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.

3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.

4. Loss of Function due to the swelling and limb changes.

5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.

6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.

7. Sepsis, Gangrene are possibilities as a result of the infections.

8. Possible amputation of the limb.

9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.

10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.

11. Chronic localized inflammations.

CAN LYMPHEDEMA BE CURED?

No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.

For extensive information on lymphedema, please visit our home page:

Lymphedema People

Quantitation of human seroresponsiveness to Merkel cell polyomavirus.

Quantitation of human seroresponsiveness to Merkel cell polyomavirus.
PLoS Pathog. 2009 Sep

Pastrana DV, Tolstov YL, Becker JC, Moore PS, Chang Y, Buck CB.
Laboratory of Cellular Oncology, National Cancer Institute, Bethesda, Maryland, USA.

Merkel cell carcinoma (MCC) is a relatively uncommon but highly lethal form of skin cancer. A majority of MCC tumors carry DNA sequences derived from a newly identified virus called Merkel cell polyomavirus (MCV or MCPyV), a candidate etiologic agent underlying the development of MCC. To further investigate the role of MCV infection in the development of MCC, we developed a reporter vector-based neutralization assay to quantitate MCV-specific serum antibody responses in human subjects. Our results showed that 21 MCC patients whose tumors harbored MCV DNA all displayed vigorous MCV-specific antibody responses. Although 88% (42/48) of adult subjects without MCC were MCV seropositive, the geometric mean titer of the control group was 59-fold lower than the MCC patient group (p less then 0.0001). protein, Only 4% (2/48) of control subjects displayed neutralizing titers greater than the mean titer of the MCV-positive MCC patient population. MCC tumors were found not to express detectable amounts of MCV VP1 capsid protein, suggesting that the strong humoral responses observed in MCC patients were primed by an unusually immunogenic MCV infection, and not by viral antigen expressed by the MCC tumor itself. The occurrence of highly immunogenic MCV infection in MCC patients is unlikely to reflect a failure to control polyomavirus infections in general, as seroreactivity to BK polyomavirus was similar among MCC patients and control subjects. The results support the concept that MCV infection is a causative factor in the development of most cases of MCC. Although MCC tumorigenesis can evidently proceed in the face of effective MCV-specific antibody responses, a small pilot animal immunization study revealed that a candidate vaccine based on MCV virus-like particles (VLPs) elicits antibody responses that robustly neutralize MCV reporter vectors in vitro. This suggests that a VLP-based vaccine could be effective for preventing the initial establishment of MCV infection.

PubMed

The Interdisciplinary Treatment of Merkel Cell Carcinoma: A Retrospective Case Analysis and Review of the Current Literature.

The Interdisciplinary Treatment of Merkel Cell Carcinoma: A Retrospective Case Analysis and Review of the Current Literature.
Zentralbl Chir. 2009 Sept

Strub B, Weindel S, Witt P, Grünert J.
Klinik für Hand-, Plastische und Wiederherstellungschirurgie Kantonsspital St. Gallen, Schweiz.

BACKGROUND: Merkel cell carcinoma is a rare, -aggressive, neuroendocrine malignancy of the skin. Over the period from June 2005 to January 2007 (18 months) 7 Merkel cell carcinomas in different tumour stages and localisations were -treated in our department for hand, plastic and reconstructive surgery. Given an incidence rate of approximately 0.1-0.4 per 100 000 habitants, this accumulation of cases is considerably higher than would be statistically expected. This led us to analyse our cases retrospectively and provided the opportunity to discuss the treatment modalities on the basis of the current literature.

PATIENTS AND METHODS: All patients were referred to our clinic after incomplete tumour excision and histopathological diagnosis elsewhere, for fur-ther surgical treatment. Two female and 5 male patients, aged 63 to 83 years, were treated. The patients' data were collected and analysed retrospectively.

RESULTS: In all cases an R0 resection could be achieved, with safety resection margins between 1.5 cm and 4.5 cm. The reconstruction of the resulting defects was achieved by skin transplants in six cases and a primary suture in one case. All patients received adjuvant radiotherapy. Furthermore, three patients received adjuvant chemotherapy due to metastases of the progressed Merkel cell carcinoma. Three patients remained free of recurrence and in two patients a progression of the tumour disease could be impeded, whilst two patients succumbed to their disease.

CONCLUSIONS: In the case of an early diagnosis, an R0 resection can usually be achieved. A safety -resection margin of a minimum of 1.5 cm in the face and 3 cm for localisation on the extremities should be obtained. To reduce the local recurrence rate, adjuvant radiotherapy should be conducted. The benefit from chemotherapy still -remains unclear. © Georg Thieme Verlag Stuttgart ˙ New York.

PubMed

Treatment Option Overview by Stage for Merkel Cell Carcinoma

Treatment Option Overview by Stage for Merkel Cell Carcinoma

-

How Merkel cell carcinoma is treated

-

There are treatments for all patients with Merkel cell carcinoma. Three kinds of treatment are used:

-

surgery (taking out the cancer)

-

radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)

-

chemotherapy (using drugs to kill cancer cells)

-

There are several different types of surgery that may be used to remove the tumor. These include:

-

Wide surgical excision takes out the cancer and some of the skin around the tumor.

-

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.

-

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.

-

STAGE I MERKEL CELL CARCINOMA

-

Treatment may be one of the following:

-

1. Surgery alone.

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2. Surgery followed by radiation therapy to the tumor site and regional lymph nodes.

-

STAGE II MERKEL CELL CARCINOMA

-

Treatment may be one of the following:

-

1. Surgery alone.

-

2. Surgery followed by radiation therapy to the tumor site and regional lymph nodes.

-

3. Surgery with or without radiation therapy followed by adjuvant chemotherapy.

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STAGE III MERKEL CELL CARCINOMA

-

Treatment will probably be chemotherapy.

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RECURRENT MERKEL CELL CARCINOMA

-

Treatment may be one of the following:

-

1. Surgery alone.

-

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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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.

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