Primary angiosarcomas of the chest wall and pleura

Abstract

Primary angiosarcomas of the chest wall and pleura are extremely rare and carry a dismal prognosis. Two cases are reported. One patient (case 1), presented with massive recurrent haemothorax, was found to have multifocal angiosarcoma of the pleura, treated with surgical de-bulking, chemical pleurodesis and chemotherapy, achieving control of the bleeding. She died 10 months later from complications related to chemotherapy. A full post-mortem examination confirmed this was a primary pleural angiosarcoma with no evidence of disease elsewhere. Another patient (case 2) with a large solitary angiosarcoma of the chest wall, discovered incidentally on a routine physical examination, was successfully treated with surgical excision and subsequent radical radiotherapy, remaining well 15 years post-operatively.

Diffuse Primary Angiosarcoma of the Pleura: A Case Report and Review of the Literature

“…7 Patients with primary chest wall/lung angiosarcoma are often diagnosed in the setting of massive, recurrent hemothorax, although pain, chest wall mass, swelling or hemoptysis may represent the clinical picture. [1][2][3]8,9 However, as opposed to tumors arising from the chest wall, pleuropulmonary angiosarcoma patients usually present with a massive hemothorax, as in our case.…”

“…Surgical debulking, pneumonectomy at the most, sometimes with rib excision in the case of chest wall tumors must be undertaken whenever possible. [1][2][3]9,[14][15][16] Radiation therapy may have a role in the adjuvant setting for angiosarcoma 1,2,16 but it has little interest in patients with distant metastasis or diffuse pleural angiosarcoma, as in our case. Combined modality treatment has been reported in the neoadjuvant setting in a patient who secondarily underwent successful surgery of a left lung inferior lobe angiosarcoma.…”

Hemoptysis in a Patient with Multifocal Primary Pulmonary Angiosarcoma

“…Similarly, almost one third of patients reviewed by Shimabukuro et al died within the first two months after establishing the diagnosis [6]. There have been only few reports of survival time longer than 12 months [5,11,23].…”

“…The early diagnosis is very difficult due to non-specific signs and symptoms presented by the patients. The majority of reports emphasize the following symptoms: (1) hemoptysis (the most common sign related to the vessel injury [7,9], (2) cough, dyspnea (related to local growth) [7], (3) chest pain [1], (4) non-specific symptoms: weight loss, malaise, fatigue, fever [5,7,10]. Nevertheless, some patients may present the tration [6,11,12].…”

“…Due to multifocal lesions and/or poor clinical status the majority of patients are not good candidates for surgery. The literature brings evidence of using some chemotherapeutic regimens [11,13,22,23], radiotherapy [5,16], immunotherapy [16], or combination of methods [5,16,17,19]. The chemotherapy regimens include gemcitabine, docetaxel [23], ifosfamide and adriamycin [12].…”