Elsevier

Lung Cancer

Volume 86, Issue 1, October 2014, Pages 105-111
Lung Cancer

Case report
Pleuropulmonary angiosarcoma involving the liver, the jejunum and the spine, developed from chronic tuberculosis pyothorax: Multidisciplinary approach and review of literature

https://doi.org/10.1016/j.lungcan.2014.07.012Get rights and content

Highlights

  • Pleuropulmonary angiosarcomas are rare tumours with a poor prognosis.

  • Most cases arise de novo, but chronic tuberculosis pyothorax, asbestos or previous radiation therapy are possible risk factors.

  • Anthracycline-based chemotherapy can play a part in the management of these patients, usually in the palliative setting.

Abstract

Pleuropulmonary angiosarcomas are very rare, with less than fifty cases reported in the literature. In most cases, the etiology is unknown but the presence of a chronic tuberculous pyothorax has been reported in several Asian case reports as a possible risk factor.

We report the case of a Caucasian 68-year old man who presented with a pleuropulmonary angiosarcoma that arose from a chronic tuberculous pyothorax and which involved the ribs and the vertebrae, the psoas muscle, and the jejunum. The patient received adapted anti-tuberculosis treatment, embolization of the mass in the small bowel, palliative external beam radiotherapy on the spine and systemic chemotherapy with liposomal non-pegylated doxorubicin and ifosfamide. With this multidisciplinary approach the patient‘s symptoms were well controlled and he achieved a complete metabolic response after six cycles of chemotherapy. Unfortunately, the patient died after eight months from the beginning of chemotherapy due to an acute lung injury secondary to extensive bilateral interstitial infiltrates. Opportunistic pathogens or drug-induced lung toxicity were the most probable causes.

Treatment with liposomal non-pegylated doxorubicin and ifosfamide could be a reasonable option in pleuropulmonary angiosarcoma but it should be validated in clinical trials. Chronic pyothorax seems to be a predisposing factor for the development of pleural angiosarcoma but further investigations are required to assess a causal association.

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.

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