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Carcinoma de células de Merkel é um tumor neuroendócrino raro e agressivo de pele que usualmente apresenta-se como lesão única na região de cabeça ou pescoço. Relata-se um caso de topografia e apresentação atípicas, com presença de múltiplos e simultâneos tumores na perna esquerda de rápida evolução, associados à linfonodomegalia inguinal palpável, com diagnóstico confirmado por meio de histopatologia e imuno-histoquímica. Realizada exérese de linfonodo inguinal esquerdo e das lesões cutâneas com margem de segurança
Merkel cell carcinoma is a rare and aggressive neuroendocrine skin tumor usually presenting as a single lesion in the head or neck region. We report a case of atypical topography and presentation, with multiple and simultaneous tumors on the left leg of rapid progression associated with palpable inguinal lymphadenopathy and diagnostic confirmation by histopathology and immunohistochemistry. Exeresis of the left inguinal lymph node and skin lesions with a safety margin was performed
ABSTRACT
Introdução: O carcinoma de células de Merkel é um raro tumor neuroendócrino cutâneo, que se origina das células responsáveis pela sensibilidade tátil, possui caráter agressivo, evolução rápida e difícil tratamento. Relato do caso: Paciente do sexo masculino, 49 anos, caucasiano, que, ao atendimento dermatológico, apresentou nódulo indolor, infiltrando tecidos profundos, não ulcerado e localizado na região do braço esquerdo. O resultado da biópsia incisional foi positivo para carcinoma de células de Merkel. Após ressecção da lesão, os exames complementares evidenciaram doença metastática na axila e parede torácica. Com o tratamento quimioterápico, houve um benefício inicial com redução tumoral, porém, não durável, uma vez que foram reveladas novas áreas com metástases tumorais em regiões superiores do corpo, sendo submetido a novo procedimento cirúrgico, o qual, após novo regime quimioterápico, não obteve sucesso. Conclusão: Na ocasião do tratamento desse paciente, os anticorpos monoclonais, como o avelumab, não estavam disponíveis. O diagnóstico precoce com cirurgia de exérese da lesão imediata, antes do acometimento de outras regiões, permanece sendo a melhor opção para um prognóstico favorável ao paciente. Contudo, a despeito disso, com as limitações à época do tratamento, o paciente evoluiu a óbito.
Introduction: The Merkel cell carcinoma is a rare cutaneous neuroendocrine tumor that originates from cells responsible for tactile sensitivity, it has an aggressive character, fast evolution and difficult treatment. Case report: 49 years Caucasian male patient, with a painless nodule, infiltrating deep tissue, not ulcerated and located in left arm identified during the dermatological consultation. The result of the incisional biopsy was positive for Merkel cell carcinoma. After resection of the lesion, complementary exams revealed metastatic disease in the axilla and chest wall. The chemotherapy treatment brought an initial improvement with tumor reduction, however, it was not durable, because new areas with tumor metastases in upper regions of the body were revealed, the patient was submitted to an another surgical procedure, after which a new chemotherapy regimen failed. Conclusion:At the time of the treatment of this patient, monoclonal antibodies, such as avelumab, were not available. Early diagnosis with immediate lesion excision surgery, before the involvement of other regions, remains the best option for a better prognosis. However, regardless of this, because of the limitations at the time of the treatment, the patient died.
Introducción: El carcinoma de células de Merkel es un tumor neuroendocrino cutáneo raro, que se origina en células responsables de la sensibilidad táctil, tiene un carácter agresivo, una evolución rápida y un tratamiento difícil. Relato del caso: Paciente masculino, de 49 años, caucásico, que en atención dermatológica encontró nódulo indoloro, infiltrando tejidos profundos, no ulcerados y ubicados en la región del brazo izquierdo. El resultado de la biopsia incisional fue positivo para el carcinoma de células de Merkel. Después de la resección de la lesión, los exámenes complementarios mostraron enfermedad metastásica en la axila y la pared torácica. Con el tratamiento de quimioterapia, hubo un beneficio inicial con la reducción del tumor, sin embargo, no es duradero, ya que se revelaron nuevas áreas con metástasis tumorales en las regiones superiores del cuerpo, que se sometieron a un nuevo procedimiento quirúrgico, que después de un nuevo régimen de quimioterapia no tuvo éxito. Conclusión: En el momento del tratamiento de este paciente, los anticuerpos monoclonales, como avelumab, no estaban disponibles. El diagnóstico temprano con cirugía para la escisión de la lesión inmediata, antes de la participación de otras regiones, sigue siendo la mejor opción para un pronóstico favorable para el paciente. Sin embargo, a pesar de esto, con las limitaciones al momento del tratamiento, el paciente falleció.
Subject(s)
Humans , Male , Middle Aged , Skin Neoplasms , Carcinoma, Merkel Cell/diagnosis , Merkel Cells , Neoplasm MetastasisABSTRACT
Merkel cell carcinoma (MCC) is a relatively rare and aggressive cutaneous neuroendocrine malignancy. It is characterized by high rates of recurrence and metastasis, both to regional lymph nodes and to distant locations. Its characteristic clinical manifestation is a single, painless, hard, erythematous nodule on a sun-exposed area, particularly in older men. Surgical management of both the primary site and the sentinel lymph node is the standard of care. In this article, we describe the diagnosis and treatment of a case of MCC in the left cheek.
Subject(s)
Humans , Male , Carcinoma, Merkel Cell , Cheek , Diagnosis , Head , Lymph Nodes , Merkel Cells , Neck , Neoplasm Metastasis , Recurrence , Skin Neoplasms , Standard of CareABSTRACT
Skin is the largest sense organ of human, with many mechanoreceptor cells under epidermis or dermis of skin and Merkel cell is one of them. It has been confirmed that Merkel cells play an important role in the process of mechanical transmission of mammalian soft tactile stimulation. Researches showed that Merkel cells had close relation to tactile formation and functioned by Merkel cell-neurite complexes and ion channels Piezo2. This article reviews Merkel cells and the function, problem and prospect of Merkel cells in tactile formation.
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Objective To explore the expressions of CK20, S100A7 and substance P (SP) in different stages of psoriatic lesions and their relationship. Methods A total of 19 patients, who had received irregular treatment for psoriasis and had both progressive and healed psoriatic lesions, were enrolled in this study. Skin tissue specimens were obtained from perilesional normal skin, progressive lesions and healed lesions of these patients and subjected to immunohistochemical analysis of expressions of CK20, S100A7 and SP. Results The relative expression level (absorbance value obtained from immunohistochemical analysis) was 7683.80 ± 6134.55,18305.04 ± 13171.30, 7257.53 ± 4417.75 for CK20, 8789.05 ± 6240.91, 18058.01 ± 16537.18, 9295.65 ±9310.02 for S100A7, 3242.51 ± 3775.41, 9364.98 ± 7596.64, 2910.85 ± 3349.46 for SP, respectively, in normal skin, progressive lesions and healed psoriatic lesions. A significant increase was observed in the expressions of CK20, S100A7 and SP in progressive lesions compared with normal skin and healed lesions, whereas no statistical difference was noted in those between normal skin and healed lesions (P ＞ 0.05 ). The expression of CK20 was positively correlated with that of S100A7 and SP (r = 0.779, 0.876, both P ＜ 0.05 ). Conclusion The pathogenesis of psoriasis seems to be associated with the changes in the number of Merkel cells.
ABSTRACT
El carcinoma de células de Merkel es un raro carcinoma neuroendocrino primario de la piel que generalmente se presenta como un nódulo redondeado, violáceo, solitario o como una placa indurada en áreas fotoexpuestas de pacientes añosos. El diagnóstico histopatológico puede ser difícil debido a su semejanza con el carcinoma metastásico de células pequeñas de pulmón, linfomas y otros tumores cutáneos. Para la distinción de tumores primarios pobremente diferenciados o metástasis se requiere de análisis inmunohistoquímico. La citoqueratina 20 constituye un marcador sensible y específico para este tumor y es útil para distinguirlo de otras neoplasias, malignas o benignas. Presentamos dos casos vistos en nuestro Servicio con este diagnóstico y una breve revisión de la literatura.
Merkel cell carcinoma is a rare primary neuroendocrine carcinoma of the skin that usually appears as a solitary, violaceous, dome-shaped nodule or indurated plaque in a sun exposed area of elderly patients. Histopathologic diagnosis may be difficult because of histologic and immunohistochemical similarities with metastatic small cell lung carcinomas, lymphomas, and other cutaneous neoplasms. Distinction from poorly differentiated oat cell primary tumors or metastasis requires immunohistochemical analysis. Cytokeratin 20 is a sensitive and specific marker for Merkel cell carcinoma and is helpful in distinguishing this tumor among other malignant and benign neoplasms. We report two cases with this diagnosis seen at our department and a brief report of the literature.
Subject(s)
Humans , Female , Aged , Carcinoma, Merkel Cell/surgery , Carcinoma, Merkel Cell/diagnosis , Skin Neoplasms/surgery , Skin Neoplasms/diagnosis , Carcinoma, Merkel Cell/pathology , Diagnosis, Differential , Immunohistochemistry , Keratins , Biomarkers, Tumor , Skin Neoplasms/pathologyABSTRACT
Objectives To report the first case of multiple trichodis coma in China.A 31-year-old man presented with multiple,broomcorn grain to r ed bean-sized,skin-colored,firm papules on the right lower extremity for 15 years,and similar lesions on the left knee and lower leg for about one year.T he lesions were asymptomatic.Methods and Results Histopathology showed acanth osis of epidermis,proliferation of fine reticulate collagen fibers and depositi on of focal mucinous proteins in upper dermis.Small blood vessels and nerve fib ers were increased,and proliferation of elastic fibers was localized in the hai r follicles and around some rete ridges.Proliferated hair follicles were seen i n the margins of the lesions and extended down in a collar-like pattern.Ultra structurally,Merkel cell-axon complex was located in the overlying basal lami na of the epidermis.Myelinated nerve fibers were seen in the upper dermis.Bloo d vessel alterations were found under electron microscopy,some basal laminae of blood vessels exhibited laminated structure,proliferation of fibrous component,and thickened wall of blood vessels were observed.Conclusions The disease is rare.It is a hamartoma originated from hair disk.