Resumen

ANTECDENTES: Los angiosarcomas son tumores mesenquimatosos malignos, raros. Los menos diferenciados, como los epitelioides, representan un reto diagnóstico para el patólogo. Los angiosarcomas epitelioides pueden expresar algunos inmunomarcadores de forma aberrante, como citoqueratinas, p63 y sinaptofisina.

CASO CLÍNICO: Paciente masculino de 65 años, con una lesión indurada en la zona lumbar derecha, diagnosticada por ultrasonido como hematoma. Seis meses después acudió al servicio de urgencias de un hospital por dolor abdominal agudo. En la laparotomía exploradora se identificó y resecó un segmento de íleon con una perforación. Se tomó una biopsia de piel de la región lumbar. El primer diagnóstico fue de carcinoma neuroendocrino primario del intestino con metástasis a piel porque el tumor fue positivo para CKAE1/AE3 y sinaptofisina.

CONCLUSIÓN: El diagnóstico correcto es de angiosarcoma epitelioide primario de la piel con metástasis al íleon, que expresó de forma aberrante sinaptofisina. En este informe se subraya la importancia de conocer las variantes morfológicas de los angiosarcomas, el inmunofenotipo habitual y la expresión aberrante de algunos inmunomarcadores.

PALABRAS CLAVE: Angiosarcoma; tumores mesenquimatosos malignos; angiosarcomas epitelioides; sinaptofisina; dolor abdominal agudo.

Abstract

BACKGROUND: Angiosarcomas are rare malignant mesenchymal tumors. The less differentiated, as epithelioids, represent a diagnostic challenge for the pathologist. In addition, it has been reported that epithelioid angiosarcomas can express some immunomarkers in aberrant ways such as cytokeratins, p63 and synaptophysin.

CLINICAL CASE: This paper presents a case of a 65-year-old man who presented an indurated lesion in the right lumbar area, diagnosed by ultrasound as a hematoma. Six months later he went to the emergency department of a hospital for acute abdominal pain. An exploratory laparotomy was performed in which a segment of ileum with a perforation was identified and resected. At the same time a skin biopsy of the lumbar region was taken. The first diagnosis the patient received was of “primary neuroendocrine carcinoma of the intestine with skin metastasis” since the tumor was positive for CKAE1 / AE3 and synaptophysin.

CONCLUSION: In our institution the case of revision was received, and it was concluded that the correct diagnosis is of primary epithelioid angiosarcoma of the skin with metastasis to the ileum, which expressed aberrant synaptophysin. This report highlights the importance of knowing the morphological variants of angiosarcomas, the usual immunophenotype and the aberrant expression of some immunomarkers.

KEYWORDS: Angiosarcoma; Malignant mesenchymal tumors; Epithelioid angiosarcomas; Synaptophysin; Acute abdominal pain.