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

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

Publikationsverlauf

Eingereicht: 09. Februar 2020

Angenommen: 24. März 2020

Artikel online veröffentlicht:
31. Juli 2020

• References

• 1 Guode Z, Qi P, Hua G, Shangchen X, Hanbin W. Primary cerebellopontine angle angiosarcoma. J Clin Neurosci 2008; 15 (08) 942-946
• 2 Baldovini C, Martinoni M, Marucci G. Epithelioid angiosarcoma of the septum pellucidum. Case Rep Pathol 2013; 2013: 603671 . Doi: 10.1155/2013/603671
• 3 La Corte E, Acerbi F, Schiariti M. , et al. Primary central nervous system angiosarcoma: a case report and literature review. Neuropathology 2015; 35 (02) 184-191
• 4 Charman HP, Lowenstein DH, Cho KG, DeArmond SJ, Wilson CB. Primary cerebral angiosarcoma. Case report. J Neurosurg 1988; 68 (05) 806-810
• 5 Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991; 35 (04) 305-309
• 6 Mena H, Ribas JL, Enzinger FM, Parisi JE. Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature. J Neurosurg 1991; 75 (01) 73-76
• 7 Folpe AL, Chand EM, Goldblum JR, Weiss SW. Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms from potential mimics. Am J Surg Pathol 2001; 25 (08) 1061-1066
• 8 Miettinen M, Wang ZF, Paetau A. , et al. ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. Am J Surg Pathol 2011; 35 (03) 432-441
• 9 Ritter JH, Mills SE, Nappi O, Wick MR. Angiosarcoma-like neoplasms of epithelial organs: true endothelial tumors or variants of carcinoma?. Semin Diagn Pathol 1995; 12 (03) 270-282
• 10 Zakaria Z, Tambirajoo K, Sattar MT, Farrell MA. Rapid clinical course of multiple metastatic cerebral angiosarcoma. Turk Neurosurg 2015; 25 (04) 643-648
• 11 Berkman RA, Merrill MJ, Reinhold WC. , et al. Expression of the vascular permeability factor/vascular endothelial growth factor gene in central nervous system neoplasms. J Clin Invest 1993; 91 (01) 153-159
• 12 Drosos E, Kalyvas A, Komaitis S. , et al. Angiosarcoma-related cerebral metastases: a systematic review of the literature. Neurosurg Rev 2019 Doi: 10.1007/s10143-019-01127-y
• 13 Hackney JR, Palmer CA, Riley KO, Cure JK, Fathallah-Shaykh HM, Nabors LB. Primary central nervous system angiosarcoma: two case reports. J Med Case Reports 2012; 6: 251 . Doi: 10.1186/1752-1947-6-251
• 14 Balamurali G, du Plessis DG, Wengoy M, Bryan N, Herwadkar A, Richardson PL. Thorotrast-induced primary cerebral angiosarcoma: case report. Neurosurgery 2009; 65 (01) E210-E211 , discussion E211. 10.1227/01.NEU.0000348294.05571.D9
• 15 Akutsu H, Tsuboi K, Sakamoto N, Nose T, Honma S, Jikuya T. Cerebral metastasis from angiosarcoma of the aortic wall: case report. Surg Neurol 2004; 61 (01) 68-71
• 16 Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol 2010; 11 (10) 983-991
• 17 Vaquero J, Martínez R, Coca S, Oya S, Burgos R. Cerebral metastasis from angiosarcoma of the heart. Case report. J Neurosurg 1990; 73 (04) 633-635
• 18 Jerjir N, Lambert J, Vanwalleghem L, Casselman J. Primary angiosarcoma of the central nervous system: Case report and review of the imaging features. J Belg Soc Radiol 2016; 100 (01) 82
• 19 Kirk IR, Dominguez R, Castillo M. Congenital primary cerebral angiosarcoma: CT, US, and MR findings. Pediatr Radiol 1992; 22 (02) 134-135
• 20 Suzuki Y, Yoshida YK, Shirane R, Yoshimoto T, Watanabe M, Moriya T. Congenital primary cerebral angiosarcoma. Case report. J Neurosurg 2000; 92 (03) 466-468
• 21 Kurian KM, Tagkalakis P, Erridge SC, Ironside JW, Whittle IR. Primary intracranial angiosarcoma of the Pineal gland: an unusual cause of recurrent intraventricular haemorrhage and superficial haemosiderosis. Neuropathol Appl Neurobiol 2006; 32 (05) 557-561
• 22 Deyrup AT, Miettinen M, North PE. , et al. Angiosarcomas arising in the viscera and soft tissue of children and young adults: a clinicopathologic study of 15 cases. Am J Surg Pathol 2009; 33 (02) 264-269
• 23 Lin CT, Ducis K, Tucker S, Tranmer B. Metastatic cardiac angiosarcoma to the lung, spine and brain: A case report and review of the literature. World Neurosurg 2017; 107 (1049): 1049.e9-1049.e12
• 24 Kardes O, Aydemir F, Suner HI. , et al. Brain metastasis of penile angiosarcoma. Urol J 2018; 15 (01) 53-55
• 25 Plotnik AN, Schweder P, Tsui A, Kavar B. Splenic angiosarcoma metastasis to the brain. J Clin Neurosci 2008; 15 (08) 927-929