ABSTRACT
The first case report concerns a 59-year-old man presenting a chronic cough and the second a 23-year-old woman farmer presenting with worsening dyspnoea associated with cough, expectoration and haemoptysis. In the second case, the woman died 7 months after the onset of the respiratory symptoms. In both cases, chest radiography and thoracic CT scans showed multiple, bilateral pulmonary nodules, bronchial endoscopy was normal and surgical biopsy revealed epithelioid haemangioendothelioma. It is a rare primary pulmonary tumour which is usually found in soft tissue, bone or liver. Epithelioid haemangioendothelioma is a vascular tumour usually affecting women less than 40 years of age. The histological appearance is characteristic and may be confirmed by immunohistochemistry. Chemotherapy and radiotherapy are generally ineffective and surgery is sometimes impossible because of the multifocal lesions. Epithelioid haemangioendothelioma is considered a sarcoma without precise histological prognostic criteria. In its pulmonary location it is responsible for death due to respiratory failure in 50% of cases.
Subject(s)
Hemangioendothelioma, Epithelioid , Lung Neoplasms , Biopsy , Female , Follow-Up Studies , Hemangioendothelioma, Epithelioid/diagnostic imaging , Hemangioendothelioma, Epithelioid/mortality , Hemangioendothelioma, Epithelioid/pathology , Hemangioendothelioma, Epithelioid/surgery , Humans , Lung/pathology , Lung Neoplasms/diagnostic imaging , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Lung Neoplasms/surgery , Male , Middle Aged , Radiography, Thoracic , Time Factors , Tomography, X-Ray Computed , Treatment Outcome , Young AdultABSTRACT
Epithelial tumours of the thymus include thymomas, thymic carcinomas and neuro-endocrine tumours. Rare, they nevertheless represent 20% of all mediastinal tumours and 50% of those located in the anterior mediastinum. Thymomas, in particular, can be associated to auto-immune disorders, among which predominates myasthenia gravis. Their clinical behaviour varies widely, from a relative indolence to the potential of lymph node and/or systematic metastases. However, even patients with an invasive disease may have a long clinical history, explaining that a 10-year or 20-year survival from diagnosis does not imply a definitive cure. In daily practice, both the clinical Masaoka's staging system and the WHO histological classification condition the treatment strategies and allow to anticipate the prognosis. The initial treatment, as well as that of the recurrence, is based mainly on a complete resection. Postoperative radiotherapy is systematically added to the treatment of invasive tumours and/or to those with an aggressive histological subtype. Inoperable or metastatic tumours require a cisplatine and anthracyclin-based chemotherapy, followed by radical surgery and/or radiotherapy.