Merkel-cell carcinoma (MCC) is a rare and highly aggressive skin cancer, which, in most cases, is caused by the Merkel cell polyomavirus (MCPyV or MCV). Merkel cell carcinoma, also called neuroendocrine carcinoma of the skin or trabecular cancer, is a very rare type of skin cancer that forms when. Los hallazgos clínicos más característicos son el rápido crecimiento y la ausencia de dolor. Aunque el carcinoma de células de Merkel puede afectar cualquier.
|Published (Last):||7 October 2009|
|PDF File Size:||9.29 Mb|
|ePub File Size:||19.95 Mb|
|Price:||Free* [*Free Regsitration Required]|
Cutaneous Merkel cell carcinoma. Case report and literature review. Palacios 1J. Hospital Universitario La Paz. Merkel-cell carcinoma is a rare skin cancer of neuroendocrine origin, which has been described as the most aggressive skin malignancy.
The tumor arises from the Merkel cells, or skin pressure receptors. It has an infiltrative growth pattern and spreads by the lymphatic vessels delulas blood. It is similar to small cell lung carcinoma, with an intrinsic sensitivity to chemo-radiotherapy and a remarkable tendency to metastasize. The best treatment cagcinoma are obtained with early diagnosis and a combination of surgery, chemotherapy, and radiotherapy.
A clinical case of Merkel cell carcinoma of the face treated with surgery and radiotherapy is reported and the literature is reviewed. Merkel cell carcinoma is an infrequent variety of skin cancers of neuroendocrine origin, which traditionally is mfrkel as the skin malignancy with the worst prognosis.
It originates from Merkel cells, or cutaneous pressure receptors. These cells were described for the first time in dde They are located in the epidermal basement layer and are said to be slow action mechanoreceptors.
Merkel cell carcinoma was first described in by Toker, 1 who defined it as a carcinpma cell carcinoma. Immunofluorescence studies and electronic microscopy allowed the dr of origin of this pathology to be identified in Barely 2, cases have been reported since the initial description. It occurs in older people, the mean age of diagnosis being about 69 years; 3 it is times more frequent in men. The etiological factors include, above all, sun exposure, as demonstrated by the fact that most lesions occur in exposed areas, especially the periorbital region.
Other associated factors include: Clinically it usually presents as an erythematous-purplish nodule with a shiny surface and, typically, telangiectasia.
Confusion with other pathologies, like basocellular carcinoma, amelanotic melanoma or cutaneous lymphomas, is common. It has a pattern of lymphatic dissemination that favors the appearance of numerous satellite lesions. Lymph node involvement is present in one-third of cases. Regression of the main lesion has been documented. It is generally located in the dermis and can extend to the epidermis or subcutaneous tissue.
The typical cytological features include the triad: Three histological variants have been described intermediate, small cell, and trabecularbut they do not seem to have clinical relevance.
No specific staging system for Merkel cell carcinoma exists, 7 but the tumor is generally classified as: Treatment depends on the disease stage. The treatment most generally accepted is surgical excision of the primary lesion with margins of about 2. A 72 year-old woman was seen in our clinic for a rapidly growing, nodular, erythematous skin lesion with a shiny surface Figs.
No cervical lymph nodes were palpated. The preoperative blood tests, including tumor markers, was normal except for blood glucose Cervical CT did not disclose cervical lymph node involvement. A skin lesion in the right malar region, coinciding with the external location of the lesion, was the only finding.
Bone involvement was not observed. A whole-body scan with thoraco-abdominal CT did not reveal systemic invasion Fig.
Carcinoma cutáneo de células de Merkel: Presentación de un caso y revisión de la literatura
The lesion was excised under general anesthesia with oncological margins and the skin was reconstructed with a cervicofacial rotation-sliding skin flap Fig. Twelve months after surgery, a submandibular tumor was found during a scheduled follow- up visit; the FNAB revealed cervical metastasis of cutaneous Merkel cell carcinoma. Radical cervical dissection disclosed disease in two lymph nodes, neither of which presented extracapsular involvement Fig. The case of our patient was classified initially as stage I.
Given the merlel evolution between the date of intervention with radiation therapy and the appearance of cervical metastasis, the disease probably was initially stage II with microscopic lymph node involvement. The most unfavorable prognostic factor with respect to the clinical evolution is the existence of lymph node involvement.
For that reason, some authors recommend initial surgical treatment of cervical lymph nodes due to the risk of cervical metastasis and the poor prognosis. We chose postoperative irradiation for our patient because of the absence of clinical lymph node involvement. The authors believe that the proper approach to this type of cases should include carcinkma study by the sentinel ee node technique to detect micrometastases.
Other factors of poor prognosis are: It is important to study the extension carcimoma the disease when the diagnosis is made.
The existence of satellite lesions and cutaneous spread should be investigated and lymphatic drainage chains should be palpated. Systemic involvement was not demonstrated before the first intervention or at the time of cervical metastatic recurrence in our patient.
The primary objective of treatment must be the local and regional control of the primary lesion and associated lymph nodes, which improves the quality of life and reduces the risk of disease dissemination. Secondary objectives include preservation of the aesthetic appearance and function.
In our patient, because of the absence of skeletal involvement, a broad excision was carcinima with 3-cm margins except for the edge carcinoam the eyelid, where the margin was 2 cmincluding underlying periostium to rule out the presence of bone involvement.
Surgery and irradiation are recommended.
Safety margins of 2. Many authors recommend postoperative irradiation based on retrospective studies that compare patients treated with surgery alone versus patients treated with surgery and irradiation. Our patient underwent surgery followed by radiation therapy approximately one month after the intervention.
Radiotherapy at a dose of 60 Gy also has been used alone, obtaining adequate levels of local control, which supports the characterization of this tumor as radiosensitive. Management of the primary site is similar to stage I.
El Carcinoma de Células de Merkel
It is not certain whether surgical excision of the affected lymph nodes is better celulxs radiation treatment alone. Lymph node involvement frequently appears as large lymph node clumps that may be inoperable. Lately it has become increasingly evident that chemotherapy should be added to the treatment of high risk patients, either as a radiosensitizer or as coadjuvant treatment.
The existence of disseminated disease reduces mean survival to less than 9 months. In consequence, if metastasis is diagnosed, treatment is merely palliative. The most frequent locations include: The response to chemotherapy generally is of short duration and patients ultimately die from the disease.
Merkel-cell carcinoma – Wikipedia
Trabecular carcinoma of the skin. Tang C, Toker C. Trabecular carcinoma of the skin: J Clin Oncol ; Inmunosupression and Merkel cell cancer. A imaging of Merkel Cell Carcinoma.
Br J Surg ; Radiotherapy as an adjunct dee management of Merkel Cell carcinoma. Early aggressive treatment for Merkel cell carcinoma improves outcome.
Am J Surg ; Multimodality treatment of Merkel cell carcinoma: Ann Surg Oncol ;8: Radiotherapy alone for primary Merkel cell carcinoma. Primary cutaneous neuroendocrine Merkel cell or trabecular carcinoma tumour of the skin: Merkel cells; Merkel cancer; Skin cancer. Introduction Merkel cell carcinoma is an infrequent variety of skin cancers of neuroendocrine origin, which traditionally is described as the skin malignancy with the worst prognosis.
Case description A 72 year-old woman was seen in our clinic for a rapidly growing, nodular, erythematous skin lesion with a shiny surface Figs. Treatment The primary objective of treatment must be the local and regional control of the primary lesion and associated lymph nodes, which improves the quality of life and reduces the risk of disease dissemination.
Travesera de Gracia,Barcelona, Barcelona, ES, maxilo elsevier.