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About This PDQ SummaryGeneral Information About Merkel Cell CarcinomaMerkel cell carcinoma (MCC) was originally described by Toker in 1972 as trabecular carcinoma of the skin. One group has suggested a list of 12 elements that should be described in pathology reports of resected primary lesions and nine elements to be described in pathology reports of sentinel lymph nodes.
Some authors have advocated the use of Mohs micrographic surgery as a tissue-sparing technique. About This PDQ SummaryPurpose of This SummaryThis PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of merkel cell carcinoma. Use these free images for your websites, art projects, reports, and Powerpoint presentations! Kawasaki Disease is a kind of illness involving skin, lymph nodes, mouth and generally affects kids who are under 5.
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There are few cases in patients younger than 50 years, and the median age at diagnosis is about 65 years (see Figure 2). An imaging work-up should be tailored to the clinical presentation as well as any relevant signs and symptoms.
Magnetic resonance imaging has been used to evaluate MCC but has not been studied systematically.
There are no known circulating tumor markers specifically for MCC.Initial Staging ResultsThe results of initial clinical staging of MCC vary widely in the literature, based on retrospective case series reported over decades.
Tumors without an identifiable primary lesion have been attributed to either spontaneous regression of the primary or metastatic neuroendocrine carcinoma from a clinically occult site.
In one series of 237 patients presenting with local or regional disease, the median time-to-recurrence was 9 months (range, 2–70 months). Adoption of these findings into a global prognostic algorithm awaits independent confirmation by adequately powered studies.
For this reason, the relapse and survival rates reported by stage vary widely in the literature. The College of American Pathologists has published a protocol for the examination of specimens from patients with MCC of the skin.
These recurrences have been typically attributed to inadequate surgical margins or possibly a lack of adjuvant radiation therapy. Recommendations about the optimal minimum width and depth of normal tissue margin that should be excised around the primary tumor differ among the various retrospective case series, but this question has not been studied systematically. The relapse rate has been reported to be similar to or better than that of wide excision, but comparatively few cases have been treated in this manner and none in randomized, controlled trials. Nodal basin radiation in contiguity with radiation to the primary site has been considered, especially for patients with larger tumors, locally unresectable tumors, close or positive excision margins that cannot be improved by additional surgery, and those with positive regional nodes, especially after SLND (stage II). When possible, patients should be encouraged to participate in clinical trials.From 1997 to 2001, the Trans-Tasman Radiation Oncology Group performed a phase II evaluation of 53 MCC patients with high-risk, local-regional disease. In one retrospective study of 107 patients, 57% of patients with metastatic disease and 69% with locally advanced disease responded to initial chemotherapy. Given the propensity for local and regional recurrence, clinicians should perform at least a thorough physical examination of the site of initial disease and the regional nodes. Consideration should be given to enrollment in clinical trials.Local RecurrenceTreatment options for patients with local recurrence include wider local surgery if possible, followed by radiation if not previously given.


The cause of the disease is unknown, but if diagnosed at the early stage it can be recovered within few days. While this cancer has a high survival rate, especially when it is caught early, it is critical that you know what the symptoms are.
There has been no systematic study of the optimal imaging work-up for newly diagnosed patients, and it is not clear if all newly diagnosed patients, especially those with the smallest primaries, benefit from a detailed imaging work-up. In addition, 545 of 982 patients (55.5%) had lymph node metastases at diagnosis or during follow-up.
A 2009 study investigated whether the presence of newly identified MCPyV in MCC tumor specimens influenced clinical outcome among 114 Finnish patients with MCC. Furthermore, for MCC sites with indeterminate lymphatic drainage, such as those on the back, SLN biopsy techniques can be used to identify the pertinent lymph node bed(s). Various models of tumor and patient characteristics were studied to predict node positivity.
Should all or just certain patients with negative or omitted nodal work-up receive local or local-regional radiation routinely?Because of the small size of these nonrandomized, retrospective series, the precise benefit from radiation therapy remains unproven.When recommended, the radiation dose given has been at least 50 Gy to the surgical bed with margins and to the draining regional lymphatics, delivered in 2 Gy fractions.
High risk was defined as recurrence after initial therapy, involved lymph nodes, primary tumor greater than 1 cm, gross residual disease after surgery, or occult primary with positive nodes. Imaging studies may be ordered to evaluate signs and symptoms of concern, or they may be performed to identify distant metastases early; but, there are no data suggesting that early detection and treatment of new distant metastases results in improved survival. Regional lymph node dissection (RLND) can also be considered if regional draining nodes have not been previously removed. When left untreated it may lead to complications which will affect the heart.  The disease cannot be prevented but it has symptoms.
If an imaging work-up is performed, it may include a computed tomography (CT) scan of the chest and abdomen to rule out primary small cell lung cancer as well as distant and regional metastases.
In this small study, patients whose tumors were MCPyV+ appeared to have better survival than patients whose tumors were MCPyV-. If performed, SLN biopsy should be done at the time of the wide resection, when the local lymphatic channels are still intact. There was no subgroup of patients predicted to have lower than 15% to 20% likelihood of SLN positivity, suggesting that SLN biopsy may be considered for all curative patients with clinically negative nodes and no distant metastases. For patients with unresected tumors or tumors with microscopic evidence of spread beyond resected margins, higher doses of 56 Gy to 65 Gy to the primary site have been recommended. Given the poor prognosis after recurrence, consideration can also be given to systemic chemotherapy, although there is no evidence that it improves survival.Nodal RecurrenceTreatment options for patients with only regional nodal recurrence include RLND and adjuvant radiation therapy if the regional draining nodes have not been previously treated. A rash on the chest, genitals or stomach, redness in eyes, dry or cracked lips, sore throat, lymph nodes,  though with big red bumps and white coating or the symptoms of the Kawaski Disease.  It should be treated at the early stage to avoid heart problems. Because of this, if you notice any symptoms associated with this cancer, it is important that you make an appointment with your doctor immediately so that you can discuss your symptoms and get a diagnostic plan in place.
Although this rate of increase is faster than any other skin cancer including melanoma, the absolute number of U.S.
Imaging studies designed to evaluate suspicious signs and symptoms may also be recommended. Several reports have found the use of SLN biopsy techniques in MCC to be reliable and reproducible. Surgery was not standardized for either the primary or the nodes, and 12 patients had close margins, positive margins, or gross residual disease.


Given the poor prognosis after recurrence, consideration can also be given to systemic chemotherapy, although there is no evidence that it improves survival.Distant RecurrenceFor patients with distant recurrence only, chemotherapy is an option for patients who have good performance status. Twenty-eight patients had undissected nodal beds, and the remainder had a variety of nodal surgeries. With a median follow-up of 48 months, 3-year OS, local-regional control, and distant control were 76%, 75% and 76%, respectively. Berman, MD (New York University School of Medicine)Scharukh Jalisi, MD, FACS (Boston University Medical Center)Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Radiation reactions in the skin and febrile neutropenia were significant clinical acute toxicities. Do not contact the individual Board Members with questions or comments about the summaries. Given the heterogeneity of the population and the nonstandardized surgery, it is difficult to infer a clear treatment benefit from the chemotherapy. Board members will not respond to individual inquiries.Levels of EvidenceSome of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.Permission to Use This SummaryPDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. Most clinical management recommendations in the literature are based on case series that describe a relatively small number of patients who were not entered on formal clinical trials, evaluated with uniform clinical staging procedures, treated with uniform treatment protocols, or provided with regular, prescribed follow-up.
These reports are also confounded by potential selection bias, referral bias, and short follow-up; and they are underpowered to detect modest differences in outcome. In addition, outcomes of patients with American Joint Committee on Cancer stage IA, stage IB, and stage II are often reported together.
In the absence of results from clinical trials with prescribed work-up, treatments, and follow-up, most MCC patients have been treated using institutional or practitioner preferences that consider the specifics of each case as well as patient preference.Two competing philosophies underlie many of the controversies about the most appropriate method of treating MCC. Lunder EJ, Stern RS: Merkel-cell carcinomas in patients treated with methoxsalen and ultraviolet A radiation. DeCaprio JA: Does detection of Merkel cell polyomavirus in Merkel cell carcinoma provide prognostic information? Buck CB, Lowy DR: Immune readouts may have prognostic value for the course of merkel cell carcinoma, a virally associated disease.
Missotten GS, de Wolff-Rouendaal D, de Keizer RJ: Merkel cell carcinoma of the eyelid review of the literature and report of patients with Merkel cell carcinoma showing spontaneous regression. Senchenkov A, Barnes SA, Moran SL: Predictors of survival and recurrence in the surgical treatment of merkel cell carcinoma of the extremities. Goldberg SR, Neifeld JP, Frable WJ: Prognostic value of tumor thickness in patients with Merkel cell carcinoma.



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