Angiosarcoma de aorta diagnosticado mediante ecoendoscopia / Angiosarcoma of the aorta diagnosed via endoscopic ultrasound

Varón de 54 años que consulta por lumbalgia de 5 semanas de evolución refractaria a analgésicos habituales y pérdida de peso significativa. El PET-TAC evidenció una masa retroperitoneal en contacto con la pared anterior de la aorta abdominal. Tras comentarlo con la Unidad de Endoscopias, se decide realizar ecoendosocopia y PAAF por la accesibilidad y el carácter menos invasivo con resultado anatomopatológico de angiosarcoma de aorta. (AU)

Angiosarcoma esplénico primario. Una entidad agresiva e infrecuente / Primary splenic angiosarcoma. An aggressive and infrequent pathology

El angiosarcoma esplénico es una neoplasia muy infrecuente y agresiva proveniente del endotelio vascular esplénico cuyo pronóstico es funesto a pesar del tratamiento quirúrgico. Su diagnóstico preoperatorio mediante pruebas de imagen supone un desafío por su similitud con otros tumores esplénicos vasculares. El tratamiento es la esplenectomía aunque rara vez es curativo dada la naturaleza agresiva y metastásica de la enfermedad. (AU)

Primary central nervous system angiosarcoma with recurrent acute subdural hematoma / Angiosarcoma cerebral primario del sistema nervioso central con hematoma subdural agudo recurrente

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma.This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice (AU)

El angiosarcoma es un tumor infrecuente entre los sarcomas, especialmente cuando se trata de una lesión primaria en el sistema nervioso central, que puede conducir a un rápido deterioro neurológico y a la muerte en pocos meses. Se presenta a un varón de 41 años con una lesión hemorrágica frontal derecha. Se realiza cirugía con resultados histopatológicos que sugieren un angiosarcoma cerebral primario. El paciente recibió tratamiento complementario con quimioterapia y radioterapia. A los cinco meses, el seguimiento con RM mostró dos lesiones y un hematoma subdural agudo, sugiriendo recaída. Se realizó una nueva cirugía hallando membranas tumorales unidas a la cara interna de la duramadre, alrededor del hemisferio derecho. El paciente acabó falleciendo debido a la recurrencia del hematoma subdural. Este caso ilustra una complicación rara y letal de un tumor infrecuente. La literatura revisada muestra que su tratamiento de elección es la resección completa y la radioterapia adyuvante (AU)

Angiosarcoma cardíaco: una causa rara de insuficiencia cardíaca / Cardiac angiosarcoma: a rare cause of heart failure

Se presenta el caso de un hombre, de 76 años, con un angiosarcoma cardíaco que debutó con insuficiencia cardíaca y taponamiento pericárdico. Se trata de un tumor muy raro, esto hace que el diagnóstico sea difícil y en ocasiones tardío. Es más frecuente en hombres de mediana edad y suele debutar con clínica de insuficiencia cardíaca. Se debe diagnosticar con pruebas de imagen y el tratamiento de elección es el quirúrgico, siempre que sea posible, aunque las recidivas locales y las metástasis son frecuentes, lo que lo convierte en un tipo de tumor con mal pronóstico.(AU)

We report the case of a 76-year-old man with a cardiac angiosarcoma that commenced with heart failure and pericardial tamponade. This is an extremely rare tumour and often overlooked as an initial diagnosis. It is more common in middle-aged men and usually presents with clinical symptoms of heart failure. It must be diagnosed with imaging tests and the treatment of choice is surgery whenever possible, although local recurrence and metastases are common. This makes it a tumour with a poor prognosis.(AU)

Tumor microenvironment and its clinicopathological and prognostic associations in surgically resected cutaneous angiosarcoma

PurposeCutaneous angiosarcoma (CAS) is a rare but typically aggressive malignant vascular neoplasm of the skin. Tumor microenvironment (TME) of CAS and its associations with baseline clinicopathological features and patient outcomes are very important, especially when considering the recent advances in understanding of the tumor biology.Methods/patientsWe retrospectively reviewed medical records of patients who underwent surgical resection for CAS at a tertiary Hospital. The pretreated specimens were evaluated by immunohistochemistry for programmed cell death protein 1 (PD-1) and its ligand (PD-L1), densities of tumor infiltrative lymphocytes (TILs) (CD3+, CD4+, CD8+, CD45RO+, FoxP3+), as well as c-MYC and Ki-67 expressions. Overall survival (OS) was estimated by Kaplan–Meier method and compared with Log-rank test.ResultsA total of 21 CAS patients were identified. Median age was 67 (ranges: 20–81) years, 14 (66.7%) were male, and over 50% had lesions of scalp. Histopathological examination showed a predominantly spindle cell type (57.1%). All patients underwent surgery, 16 (76.2%) were treated further. PD-L1 was positively stained (> 1%) in tumor cells (42.9%) and TILs (23.8%). PD-1 expression (> 1%) was identified in TILs of 11 (52.4%) cases. PD-1/PD-L1 expressions were significantly associated with the higher densities of CD3+, CD4+, CD8+, CD45RO+, and Foxp3+ TILs, but not with patient characteristics or c-MYC or Ki-67 expression. Median OS was 18.5 months (95% CI 6.0–35.9), although no prognostic significance was observed with respect to any clinicopathological features.ConclusionWe characterized TME and its clinical and prognostic association in CAS. PD-1/PD-L1 expressions were significantly associated with TILs subtypes but not with OS.

Angiosarcoma metastásico de la cavidad oral: a propósito de un caso / Metastatic angiosarcoma of the oral cavity: report of a case

El angiosarcoma oral es un tumor endotelial raro que, debido a su presentación clínica, requiere ser comparada con varias lesiones similares como diagnóstico diferencial. Presentamos un paciente de 66 años en tratamiento por múltiples episodios hemorrágicos digestivos, derivada al Servicio de Cirugía Oral y Maxilofacial del Hospital de Carabineros de Santiago de Chile debido a la presencia de lesiones vasculares intraorales. Se diagnostican inicialmente como lesiones metastásicas de un tumor estromal gastrointestinal, siendo el diagnóstico histopatológico final de un angiosarcoma agresivo y diseminado. La presentación clínica de este tipo de lesiones dificulta el diagnóstico definitivo y, en consecuencia, el manejo de este tipo de pacientes. (AU)

Oral angiosarcoma is a rare endothelial tumour that requires to be included as a differential diagnosis of several similar lesions due to its clinical presentation. We present a 66-year-old patient undergoing treatment for multiple digestive bleeding episodes, referred to the oral and maxillofacial surgery service of the Hospital de Carabineros de Santiago de Chile due to the presence of intraoral vascular lesions initially diagnosed as metastatic lesions of a gastrointestinal stromal tumour, with the final histopathological diagnosis being an aggressive and disseminated angiosarcoma. The clinical presentation of these lesions makes the diagnosis and, consequently, the management of this type of patient difficult. (AU)

Angiosarcoma primario de mama / Primary angiosarcoma of the breast

El angiosarcoma de mama es un tumor muy poco frecuente pero de comportamiento muy agresivo. Es importante el diagnóstico precoz y diferencial entre su etiología primaria y secundaria. Se caracteriza por su inespecificidad clínica y radiológica, por lo que el anatomopatólogo tiene un papel fundamental para llegar al diagnóstico. La cirugía es el tratamiento fundamental, combinada con quimioterapia y radioterapia. Con un pronóstico desfavorable y la falta de tratamiento definido en las guías clínicas, es un desafío tanto para los cirujanos como para los oncólogos.(AU)

Angiosarcoma of the breast is a very rare tumour, but very aggressive in its behaviour. Early and differential diagnosis between its primary or secondary aetiology is important. It is characterised by being clinically and radiologically unspecific, and the histopathologist has a fundamental role to reach the diagnosis. Surgery is the basic treatment combined with chemotherapy and radiotherapy. Due to its unfavourable prognosis and the lack of treatment defined in the clinical guidelines it is a challenge for both surgeons and oncologists.(AU)

Angiosarcoma radioinducido de mama. Revisión bibliográfica a propósito de dos casos en nuestro centro / Radiotherapy-related angiosarcoma of the breast. A literature review apropos of two cases treated in our hospital

Objetivo Revisión bibliográfica del diagnóstico y tratamiento actual del angiosarcoma radioinducido de mama. Pacientes Presentamos dos casos de angiosarcoma radioinducido de mama comentándose los aspectos clínicos, diagnósticos y terapéuticos de interés. Conclusión El angiosarcoma de mama radioinducido es un tumor infrecuente que se presenta como complicación tardía en pacientes sometidas a tratamiento conservador del cáncer de mama mediante cirugía y radioterapia. La cirugía es el único tratamiento curativo, aunque su elevada tasa de recaída local requiere de tratamiento multimodal para optimizar los resultados de control local. (AU)

Objective Literature review of the diagnosis and treatment of radiotherapy related angiosarcoma of the breast. Case reportWe present two cases of radiotherapy-related angiosarcoma of the breast treated in our hospital and describe the clinical, diagnostic, and therapeutic features. Conclusion Radiotherapy-related angiosarcoma of the breast is an uncommon tumour that usually presents as a late complication in patients who have undergone surgery and radiotherapy as conservative treatment for breast cancer. Surgery is the only curative treatment. However, because of its high rate of local recurrence, multimodal therapy is required to optimise prevention of recurrence. (AU)

Angiosarcoma cardiaco en imágenes / Cardiac angiosarcoma in images

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Edema facial con fóvea, un importante diagnóstico diferencial / Pitting Edema of the Face, An Important Differential Diagnosis

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