Case reportPleuropulmonary angiosarcoma involving the liver, the jejunum and the spine, developed from chronic tuberculosis pyothorax: Multidisciplinary approach and review of literature
Introduction
Vascular sarcomas are considered an uncommon type of sarcomas, representing overall 2 to 3% of all soft-tissue tumours [1], [2]. Under this heading, the latest WHO classification includes up to 14 different histological subtypes, categorizing them according to their degree of malignancy, from benign tumours, such as haemangiomas, to highly aggressive neoplasms, such as high-grade angiosarcomas [3].
With regards to the latter, more than half of angiosarcomas are cutaneous while the rest arise in deep soft tissues, breast, bones and solid organs, particularly the liver, spleen and heart. There are a few well-documented general predisposing factors, such as previous radiation-exposure and chronic lymphedema [4], [5]. Some associations have been described with specific-site angiosarcomas, such as the link between hepatic angiosarcoma and a previous exposure to vinyl chloride, thorium dioxide or arsenic. However, in most cases, the exact mechanisms which drive the development of angiosarcomas remain obscure [5].
In the case of primary pleuropulmonary vascular sarcomas in general [6], and specifically of angiosarcomas, they are exceedingly rare, with fewer than 50 case reports described in the literature. With regards to their pathogenesis, several series of cases from Japan have reported an association with chronic tuberculosis pyothorax or other sequels of tuberculosis [7], [8], [9], [10], [11], [12], [13]. Interestingly, in Western patients, no history of tuberculosis has been described in the reported cases, possibly because of a lower incidence of mycobacterial infections in the West and/or a better antitubercular treatment compliance [14], [15]; in some cases, asbestos and radiation exposure have been proposed as causal factors [16] but most angiosarcomas are described as de novo [16].
Section snippets
Case report
Our patient was a 68-year man who presented in March 2012 with hemoptysis, pleuritic pain and occasional chills. No fever, weight loss or dyspnea were reported. The patient had a previous history of childhood pulmonary tuberculosis. He did not receive antitubercular treatment at that time and he had developed a chronic calcified pyothorax in the lower right chest that had remained stable since then. He had stopped smoking 15 years ago. There was no other personal history of interest. His family
Discussion
There is a definite relationship between tuberculosis and cancer [17], [18], which can manifest itself in three different ways: first, tuberculosis increases the risk of cancer. Second, immunosuppression due to cancer and oncologic therapy can reactivate any latent tuberculosis infection. Lastly, tuberculosis can mimick on occasions malignancies, posing a challenging differential diagnosis. It is noteworthy that all these three situations occurred in our patient's history.
The quintessential
Conflicts of interest statement
None declared.
References (32)
- et al.
Angiosarcomas and other sarcomas of endothelial origin
Hematol Oncol Clin N Am
(2013) - et al.
Rare pleural tumors
Clin Chest Med
(2013) - et al.
Malignant epithelioid vascular tumors of the pleura: report of a series and literature review
Hum Pathol
(2000) - et al.
Primary pleural epithelioid angiosarcoma. A case report and review of the literature
Pathol Res Pract
(2010) - et al.
Increased lung cancer risk among patients with pulmonary tuberculosis: a population cohort study
J Thorac Oncol
(2011) Histology-driven chemotherapy of soft-tissue sarcoma
Ann Oncol
(2010)- et al.
Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial
Lancet Oncol
(2014) - et al.
Management of soft tissue sarcoma
(2013) - et al.
Primary angiosarcomas of the chest wall and pleura
Eur J Cardiothorac Surg
(1998) - et al.
WHO classification of tumours of soft tissue and bone
(2013)