Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

Treatment and Outcome of Merkel Cell Carcinoma in A Single Center / 肿瘤防治研究

Objective To investigate the clinical features, treatment, and outcome characteristics of patients with Merkel cell carcinoma. Methods The clinical manifestations, laboratory tests, diagnosis and treatment, and follow-up data of six patients with Merkel cell carcinoma were retrospectively analyzed. Results Among the six patients with Merkel cell carcinoma, four were males and two were females, with a median age of 66 years old (57-76 years old). All six patients presented with skin swelling, and the clinical stages were as follows: stageⅠ in three patients, stage Ⅲ in one patient, and stage IV in two patients. Two patients were treated with surgery alone, three patients with surgery combined with radiotherapy and/or chemotherapy, and one patient with immunotherapy combined with chemotherapy. Until the follow-up time, four patients had no disease progression, one patient died because of disease progression, and one patient remained under treatment. Conclusion Limited-stage Merkel cell carcinoma is primarily treated with surgery and radiotherapy, meanwhile, metastatic Merkel cell carcinoma needs systemic therapy, and first-line immune checkpoint inhibitors targeting PD-1/ PD-L1 pathway can achieve better therapeutic results.

Merkel cell polyomavirus and its etiological relationship with skin tumors

Abstract Viruses have been frequently identified in several human neoplasms, but the etiological role of these viruses in some tumors is still a matter of controversy. Polyomaviruses stand out among the main viruses with oncogenic capacity, specifically the Merkel cell polyomavirus (MCPyV). Recent revisions in the taxonomy of polyomaviruses have divided the Polyomaviridae family into six genera, including 117 species, with a total of 14 currently known human-infecting species. Although the oncogenicity of polyomaviruses has been widely reported in the literature since 1950, the first description of a polyomavirus as an etiological agent of a neoplasm in humans was made only in 2008 with the description of MCPyV, present in approximately 80% of cases of Merkel cell carcinoma (MCC), with the integration of its genome to that of the tumor cells and tumor-specific mutations, and it is considered the etiological agent of this neoplasm since then. MCPyV has also been detected in keratinocyte carcinomas, such as basal cell carcinoma and squamous cell carcinoma of the skin in individuals with and without immunosuppression. Data on the occurrence of oncogenic viruses potentially involved in oncogenesis, which cause persistence and tissue injury, related to the Merkel cell polyomavirus are still scarce, and the hypothesis that the Merkel cell polyomavirus may play a relevant role in the genesis of other cutaneous carcinomas in addition to MCC remains debatable. Therefore, the present study proposes to explore the current knowledge about the presence of MCPyV in keratinocyte carcinomas.

Evidence and Considerations on Treatment of Small Size Merkel Cell Head and Neck Carcinoma

Abstract Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors.

Merkel cell carcinoma: Preparing to go the distance

@#Merkel cell carcinoma (MCC) is a rare and aggressive malignancy of the skin, with poor clinical outcomes. Typical conditions include a rapidly growing, solitary dome-shaped, violaceous nodule. Several root causes have been identified - sun exposure, age, lighter skin, immunocompromised state, and polyomavirus infection. Wide local excision is the best treatment. The tumour is radiotherapy-responsive. However, the success rate of the treatment with chemotherapy is rather limited. Immunotherapy has shown promising results. Early detection is important to prevent morbidity and mortality. Case Report: In this literature work, we reported on a particular case of MCC, as exhibited by an 84-year-old Chinese woman, and discussed the clinical features and management of MCC. Discussion: We highlighted that MCC cases have a link to the polyomavirus 5. Patients who were identified with the Polyomavirus 5, and underwent immunotherapy, were seen to depict much better prognosis.

Virus polioma en carcinoma de células de Merkel / Polyoma virus in Merkel cell carcinoma

Introducción: El carcinoma de células de Merkel (MCC) es un tumor cutáneo maligno agresivo y de mal pronóstico. La incidencia es mayor en adultos hombres, caucásicos, con edad promedio de 70 años. Feng et al, lograron aislar un nuevo virus en muestras de este tumor, que denominaron virus polioma de células de Merkel (MCPyV). Se ha intentado establecer una relación causal entre el virus y MCC. El virus está integrado al genoma y produciría mutaciones específicas. En muestras de MCC, se ha detectado expresión de oncoproteinas virales (antígenos T) que promueven la replicación viral y tumorogénesis

Introduction: Merkel cell carcinoma (MCC) is an aggressive malignant cutaneous tumor with poor prognosis. Most cases affect elder patient with an average of 70 years of age. Feng et al isolated a new virus, the Merkel cell carcinoma polyoma virus (MCPyV). A causal relationship between MCPyV y MCC has been established. The virus is integrated in the genome and pro-duces specific mutations. MCC samples show ex-pression of viral oncoproteins (T antigens) that promote viral replication and tumorogenesis.

Poorly Differentiated Neuroendocrine Carcinoma of the Eyelid

PURPOSE: To report a case of poorly differentiated neuroendocrine carcinoma of the eyelid. CASE SUMMARY: A 70-year-old male presented with a 5-month history of a right upper eyelid mass. The mass appeared as 1.2 × 1.2 cm on the right upper eyelid. A mass excision was performed under frozen section control. The tumor was completely excised with a safety margin clearance and an upper eyelid reconstruction was performed. Histopathological examination revealed a tumor composed of small atypical cells which showed a high nuclear/cytoplasm ratio, nuclear molding, and increased mitotic activity. Immunohistochemical examination revealed positive reactivity for Ki-67, synaptophysin, CD56, and negative reactivity for chromogranin, cytokeratin 20, and thyroid transcription factor-1. CONCLUSIONS: Primary neuroendocrine carcinoma of the eyelid is extremely rare, but the tumor has high malignancy and readily metastasizes. Poorly differentiated neuroendocrine carcinoma should be considered in the differential diagnosis of a rapidly growing eyelid mass.

Merkel cell carcinoma in the community setting: a case report

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin initially believed to arise from the Merkel cells. In the community setting a general radiation oncologist may only encounter this pathology in a handful of cases over the course of their career. Due to the low incidence of this malignancy, few prospective randomized controlled trials have ever been conducted and therefore guidelines are based on relatively lower levels of evidence upon which the clinical recommendations are made. We discuss the case of a female in her 90s presenting with a classic MCC primary lesion, as well as satellite lesions proximal to both the primary and the draining regional lymph nodes with no evidence of nodal involvement. Here we discuss the presentation, management, treatment planning, underlying pathology, results and sequelae of treatment. We also review new treatment modalities, and the most current staging systems and guidelines.

Merkel Cell Carcinoma of Cheek: 2 Case Reports / 대한이비인후과학회지

Merkel cell carcinoma (MCC) is a rare aggressive skin cancer with the mortality rate of 33%. MCC was first described by Toker in 1972 as a trabecular cell carcinoma and is classified as a neuroendocrine tumor similar to small cell lung carcinoma and melanoma. The pathogenesis of MCC remains largely unknown. However, ultraviolet radiation and immunosuppression are likely to play a significant pathogenic role. The primary skin lesion is usually asymptomatic and it typically presents as a red or purple dome-shaped nodule. The management of MCC is controversial, however, local wide excision followed by radiotherapy is accepted as the primary treatment modality. The regional draining nodal basin is the most common site for recurrence. Therefore, sentinel lymph node biopsy is recommended in all cases, except for the clinically node-negative cases. We herein report two cases of MCC on the left cheek with different clinical manifestations.

Merkel Cell Carcinoma Originating in a Setting of Pre-existing Bowen's Disease / 대한피부과학회지

No abstract available.

Primary Merkel cell carcinoma of the earlobe in a young healthy man

Merkel cell carcinoma (MCC) is an uncommon neuroendocrine cutaneous tumor with poor prognosis. It has the high rate of recurrence, mortality, regional nodal involvement, and distant metastases. It is difficult to diagnose MCC because of its non-specific clinical findings. It usually occurs on sun-exposed areas of the skin, mostly at head and neck. There is a difference in the incidence and prognosis according to site in the head and neck. However, there is no consented site-specific diagnosis, treatment or follow-up protocol for MCC at the head and neck. We herein report a case of MCC arising in the right earlobe of an otherwise healthy young man who has been diagnosed early, thereby successfully treated. With our closed follow-up, there was no tumor recurrence or complication at 33 months after diagnosis.

Cytokeratin 20 negative Merkel cell carcinoma consistent with negative Merkel cell polyomavirus / 영남의대학술지

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor that is highly aggressive in nature and indolent in progression. The common risk factors for MCC are senility, prolonged exposure to sunlight, and immune deficient states. Moreover, Merkel cell polyomavirus has recently been characterized to be significantly associated with pathogenesis of MCC, including the expression of Cytokeratin 20 (CK20). Diagnosis is often difficult since histopathological results require a number of differential diagnoses through immunohistochemical (IHC) stains with other cutaneous malignancies. A 67-year-old man presented with a solitary domeshaped erythematous round mass on the left upper arm for 2 months. Biopsy and IHC studies revealed findings consistent with Merkel Cell Carcinoma of neuroendocrine origin. Common IHC stains usually confirm positive findings for CK20, which is also recognized as the key component in making the diagnosis. We present a CK20 negative MCC in light of expanding the knowledge of unusually stained IHC results in MCC.

Cytokeratin 20 negative Merkel cell carcinoma consistent with negative Merkel cell polyomavirus / 영남의대학술지

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor that is highly aggressive in nature and indolent in progression. The common risk factors for MCC are senility, prolonged exposure to sunlight, and immune deficient states. Moreover, Merkel cell polyomavirus has recently been characterized to be significantly associated with pathogenesis of MCC, including the expression of Cytokeratin 20 (CK20). Diagnosis is often difficult since histopathological results require a number of differential diagnoses through immunohistochemical (IHC) stains with other cutaneous malignancies. A 67-year-old man presented with a solitary domeshaped erythematous round mass on the left upper arm for 2 months. Biopsy and IHC studies revealed findings consistent with Merkel Cell Carcinoma of neuroendocrine origin. Common IHC stains usually confirm positive findings for CK20, which is also recognized as the key component in making the diagnosis. We present a CK20 negative MCC in light of expanding the knowledge of unusually stained IHC results in MCC.

Carcinoma de células de Merkel: estudio de 3 casos / Merkel cell carcinoma: report on 3 patients

Introducción: El objetivo del trabajo es exponer 3 casos de carcinoma de células de Merkel (CCM), carcinoma trabecular o carcinoma indiferenciado de células pequeñas de la piel. Se trata de un tumor poco frecuente y con características bien definidas, asociado a la radiación, la inmunosupresión y recientemente a polyomavirus. Material y método: Estudio clínico descriptivo de 3 adultos intervenidos entre 2011 y 2013 en los servicios de cirugía general y maxilofacial de nuestro centro. Se trata de 2 varones (50 y 79 años) y una mujer (79 años). Resultados: El varón más joven presentó una adenopatía inguinal derecha, sin que pudiera localizarse la lesión primaria; el otro paciente estaba siendo tratado con infliximab por enfermedad inflamatoria intestinal y presentó una lesión en el labio inferior. La paciente presentó una lesión pretibial derecha. El diagnóstico se realizó mediante estudio inmunohistoquímico de las biopsias (CK-20 y CAM 5.2 positivas; TTF-1 negativo; cromogranina y NSE positivas 2 y una intermedia). En los 3 casos se realizó cirugía y la técnica del ganglio centinela. El tratamiento se continuó con quimioterapia adyuvante en 2 pacientes y radioterapia en el paciente que había sido tratado con infliximab. Este paciente falleció. Conclusiones: El CCM es raro, se presenta en adultos con unas manifestaciones clínicas no definidas; su diagnóstico temprano se realiza por sospecha y la biopsia se confirma mediante técnicas de inmunohistoquímica. El tratamiento es quirúrgico, con técnica del ganglio centinela, y quimio-radioterapia adyuvante.

Aim: Our purpose was to report on three patients having Merkel cell carcinoma (MCC). This tumor is a trabecular carcinoma or undifferentiated small cell carcinoma of the skin. It represents a rare and well-defined characteristic neoplasm, associated with radiation, immunosuppression and recently with the polyomavirus. Material and methods: Descriptive clinical study of three adult patients treated between 2011 and 2013 in our center either in the General Surgery Department or Oral Surgery Department. There were two men (50 and 79 years) and one woman (79 years). Results: The youngest man presented with a right inguinal lymph node. The primary lesion was not found. The second male patient had been on infliximab therapy due inflammatory bowel disease and had a lesion on the inferior lip. The single female patient had a right pretibial lesion. Diagnosis was made by means of immunohistochemical analysis of biopsies (CAM 5.2 and CK-20 positive, TTF-1 negative, chromogranin and NSE positive intermediate 2 and 1). In all three cases surgery and sentinel node technique was performed. After surgery, treatment was continued with adjuvant chemotherapy in two patients. Radiotherapy was applied to the patient who had been on infliximab therapy. This patient ultimately died. Conclusions: MCC is a rare tumor. It occurs in adult patients with distinct clinical features. It has to be suspected to allow an early diagnosis. Diagnosis is made by biopsy and it is confirmed by immunohistochemistry. Surgery with the sentinel node technique is the usual treatment, besides adjuvant chemoradiotherapy it is also applied.

Dosimetric comparison of axilla and groin radiotherapy techniques for high-risk and locally advanced skin cancer

PURPOSE: Radiation therapy targeting axilla and groin lymph nodes improves regional disease control in locally advanced and high-risk skin cancers. However, trials generally used conventional two-dimensional radiotherapy (2D-RT), contributing towards relatively high rates of side effects from treatment. The goal of this study is to determine if three-dimensional conformal radiation therapy (3D-CRT), intensity-modulated radiation therapy (IMRT), or volumetric-modulated arc therapy (VMAT) may improve radiation delivery to the target while avoiding organs at risk in the clinical context of skin cancer regional nodal irradiation. MATERIALS AND METHODS: Twenty patients with locally advanced/high-risk skin cancers underwent computed tomography simulation. The relevant axilla or groin planning target volumes and organs at risk were delineated using standard definitions. Paired t-tests were used to compare the mean values of several dose-volumetric parameters for each of the 4 techniques. RESULTS: In the axilla, the largest improvement for 3D-CRT compared to 2D-RT was for homogeneity index (13.9 vs. 54.3), at the expense of higher lung V₂₀ (28.0% vs. 12.6%). In the groin, the largest improvements for 3D-CRT compared to 2D-RT were for anorectum Dmax (13.6 vs. 38.9 Gy), bowel D200cc (7.3 vs. 23.1 Gy), femur D₅₀ (34.6 vs. 57.2 Gy), and genitalia Dmax (37.6 vs. 51.1 Gy). IMRT had further improvements compared to 3D-CRT for humerus Dmean (16.9 vs. 22.4 Gy), brachial plexus D₅ (57.4 vs. 61.3 Gy), bladder D₅ (26.8 vs. 36.5 Gy), and femur D₅₀ (18.7 vs. 34.6 Gy). Fewer differences were observed between IMRT and VMAT. CONCLUSION: Compared to 2D-RT and 3D-CRT, IMRT and VMAT had dosimetric advantages in the treatment of nodal regions of skin cancer patients.

Carcinoma de células de merkel: Presentación de un caso con localización y tamaño atípicos / Merkel cell carcinoma: A case with atypical location and size

El carcinoma de células de Merkel (CCM) es un tumor cutáneo maligno poco frecuente, de rápido crecimiento, localmente agresivo, con tendencia a dar metástasis ganglionares, a distancia y con alta tasa de recidiva local. Su etiopatogenia se desconoce, pero recientemente se ha demostrado la replicación de un virus en su interior (poliomavirus de células de Merkel), lo que ha supuesto un nuevo campo de investigación. Requiere diagnóstico precoz, tratamiento quirúrgico y radiante para mejorar la sobrevida de los pacientes. Se comunica un caso de CCM en una mujer de 72 años, a la que se le confirma el diagnóstico por las características histopatológicas e inmunohistoquímicas, debido a la forma de presentación atípica de gran tamaño, rápido crecimiento y localización infrecuente. Se efectúa una revisión de la entidad, además se describen la evolución clínica y el tratamiento instaurado, sin recidiva a siete meses de seguimiento.

The Merkel Cell Carcinoma (MCC) is a rare malignant skin tumor, fast-growing, locally aggressive, prone to lymph node and distant metastases and high rate of local recurrence. Its pathogenesis is unknown, but has recently shown a virus replication inside (Merkel cell polyomavirus) which has led to a new field of research. It requires early diagnosis and surgical treatment and radiotherapy to improve the survival of patients. CCM case is reported in a 72 years at diagnosis by the histopathologic and immunohistochemical characteristics are confirmed, due to the atypical presentation of large, fast-growing and unusual location. A review of the peculiar disease and also the clinical course and treatment instituted, without recurrence at seven months of follow-up are described.

Spontaneous regression in an ulcerated CK7 positive Merkel cell carcinoma.

Merkel cell carcinoma is an aggressive and frequently lethal tumor of the elderly, associated with sun exposure and immunosuppression which is less common in the dark-skinned. We report the case of a 40-year-old woman who presented with multiple slowly progressive, mildly itchy ulcerated plaques of size ranging from 2 × 3 cm to 5 × 7 cm on the left knee of 1 year duration. Skin biopsy showed diffuse dermal infi ltration by small round cells with molding of cells and lymphocyte infi ltration. The cells stained positive for cytokeratin (CK) 20, CK7, neuron-specifi c enolase, and chromogranin. The skin lesions underwent spontaneous regression within 1 month of skin biopsy and have not recurred during the past 2 years. The immune mechanisms triggered by biopsy possibly explain the spontaneous regression.

A Case of Merkel Cell Carcinoma Concurrent with Bowen's Disease / 대한피부과학회지

No abstract available.

Ocular Flutter associated with Merkel Cell Carcinoma

Ocular flutter is a relatively rare eye movement disorder that refers to occasional bursts of involuntary horizontal oscillation around the point of fixation, characterized by rapid, repetitive, horizontal, symmetrical and sinusoidal oscillation without inter- saccadic interval. It is known to be associated with disorders of the cerebellum and brainstem resulting from various pathological conditions, but it's anatomical and pathophysiological basis remains unclear. Here, we present the case of a patient who had severe ocular flutter associated with Merkel cell carcinoma.

Carcinoma de Merkel (carcinoma neuroendocrino) / Merkel cell carcinoma (neuroendocrine carcinoma)

Desde que el carcinoma de Merkel fue descrito por primera vez, hace poco más de cuatro décadas por Cyril Toker en el año de 1972; ha despertado el interés de los colegas médicos. Factores de esta patología como: su célula de origen, etiología, evolución y tratamiento al día de hoy se mantienen en discusión y se modifican con el paso del tiempo. El advenimiento de la microscopía electrónica y la inmunohistoquímica han colaborado en la definición etiológica de esta neoplasia y otros descubrimientos como el del poliomavirus de células de Merkel, han brindado información importante sobre la génesis de este proceso. El tratamiento del carcinoma de células de Merkel sigue sufriendo leves variaciones con el paso del tiempo y se mantiene en discusión a la fecha. Presentamos el caso de una paciente con un carcinoma de células de Merkel con revisión de la literatura actual.

Since Merkel carcinoma was first described more than four decades ago by Cyril Toker little in the year 1972; has attracted interest from medical colleagues. Factors of this disease as their cell of origin, etiology, course and treatment today remain under discussion and change over time. The advent of electron microscopy and immunohistochemistry have collaborated in the etiological definition of this neoplasm and other discoveries such as the Merkel cell polyomavirus, have provided important information on the genesis of this process. The treatment of Merkel cell carcinoma is still suffering slight variations over time and remains in discussion to date. We report a patient with Merkel cell carcinoma with review of the current literature.