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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2020; 39(03): 222-227
DOI: 10.1055/s-0040-1712108
Case Report | Relato de Caso

Intra-Axial Metastatic Angiosarcoma of the Central Nervous System Associated with Anemia, Pulmonary Tuberculosis and Short Survival

Angiossarcoma metastático intra-axial do sistema nervoso central associado a anemia, tuberculose pulmonar e sobrevivência curta
Eduardo Cambruzzi 
1   Department of Histology and Pathology, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
2   Department of Pathology, Complexo Hospitalar Santa Casa, Hospital Santa Rita, Porto Alegre, RS, Brazil
3   Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
4   Universidade Luterana do Brasil, Canoas, RS, Brazil
5   Instituto de Cardiologia, Fundação Universitária de Cardiologia, Porto Alegre, RS, Brazil
,
Júlia Rispoli dos Santos 
4   Universidade Luterana do Brasil, Canoas, RS, Brazil
,
Karla Lais Pêgas 
2   Department of Pathology, Complexo Hospitalar Santa Casa, Hospital Santa Rita, Porto Alegre, RS, Brazil
3   Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
,
William Mazzuco Nesi 
6   Department of Pathology, Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
,
William Pegoraro Kus 
6   Department of Pathology, Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
,
Felipe Lourezon Schiavo 
6   Department of Pathology, Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
,
Samir Cezimbra dos Santos 
6   Department of Pathology, Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil
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Abstract

Introduction Angiosarcoma (AG) is a malignant mesenchymal neoplasm that predominantly affects the soft tissues and, to variable degrees, expresses the morphological and functional characteristics of the endothelium. The incidence of sarcomas of the central nervous system (CNS) is low (0.5% to 2.7%), and AGs involving the brain are even rarer.

Case Description A 45-year-old male patient presented with complaints of headache, nausea, and vomiting. An examination showed bilateral papilledema and a right lung pleurotomy. The patient's previous history included drug addiction, pulmonary tuberculosis, lung abscess, pleural empyema, and pulmonary artery embolization for severe hemoptysis. Computed tomography/magnetic resonance imaging scans revealed a large intra-axial lesion extending into the right parietal and temporal lobes, with hemorrhagic zones. The patient underwent surgical resection of the lesion. Microscopy showed a poorly-differentiated, high-grade malignant tumor composed of plump/epithelioid cells forming small vascular spaces and solid nests, compatible with AG.In the postoperative period, the patient developed recurrent hemoptysis. A biopsy of the tissues adjacent to the pleurotomy determined the diagnosis of pulmonary AG. At 30 days after the resection, the patient died from hemoptysis, hemothorax, lung atelectasis, and intracranial hypertension related to the recurrence of the brain tumor.

Conclusion Angiosarcoma is a rare neoplasia related to short survival due to the high proliferative index, which must be considered in patients presenting hemorrhagic tumors. No specific genetic abnormalities have been described for this neoplasia.

Resumo

Introdução O angiossarcoma (AG) é uma neoplasia mesenquimal maligna que afeta predominantemente os tecidos moles e, em graus variáveis, recapitula as características morfológicas e funcionais do endotélio. A incidência de sarcomas do sistema nervoso central (SNC) é baixa (0,5% a 2,7%), e os AGs envolvendo o cérebro são ainda mais raros.

Descrição do Caso Paciente masculino, 45 anos, apresentou queixa de dor de cabeça, náusea e vômitos. O exame físico mostrou papiledema bilateral e pleurostomia à direita. A história prévia incluía drogadição, tuberculose pulmonar, abscesso pulmonar, empiema pleural e embolização da artéria pulmonar por hemoptise grave. A tomografia computadorizada / ressonância magnética revelou uma grande lesão intra-axial com zonas hemorrágicas que se estendia para os lobos parietal e temporal direitos. O paciente foi submetido à ressecção cirúrgica da lesão. A microscopia mostrou um tumor maligno de alto grau, pouco diferenciado, composto por células fusiformes / epitelioides, formando pequenos espaços vasculares e ninhos sólidos, compatíveis com AG. No pós-operatório, o paciente desenvolveu hemoptise recorrente. A biópsia dos tecidos adjacentes à pleurostomia determinou o diagnóstico de AG pulmonar. Após 30 dias da ressecção, o paciente faleceu por hemoptise, hemotórax, atelectasia pulmonar e hipertensão intracraniana relacionada à recorrência do tumor cerebral.

Conclusão A AG é uma neoplasia rara relacionada à curta sobrevida devido ao alto índice proliferativo, que deve ser considerada em pacientes com tumores hemorrágicos. Nenhuma anormalidade genética específica foi descrita para esta neoplasia.

Keywords

angiosarcoma - brain sarcoma - central nervous system - pathology - immunohistochemistry - prognosis

Palavras-chave

angiossarcoma - sarcoma cerebral - sistema nervoso central - patologia - imuno-histoquímica - prognóstico


Publication History

Received: 09 February 2020

Accepted: 24 March 2020

Article published online:
31 July 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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