RESUMO
Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d-dimer, troponin T or NT-proBNP, as well as characteristic findings on CT pulmonary angiography such as the 'wall eclipsing sign' and an non-dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2-year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.
RESUMO
Pulmonary lymphomatoid granulomatosis (PLG) is a rare multisystem Epstein-Barr virus (EBV)-associated lymphoproliferative disorder. Exact incidence is unknown and, with its variable clinical presentation, making an accurate diagnosis of PLG can be difficult. We present two distinct cases at our tertiary centre that underline PLG's non-specific clinical presentations. This resulted in the failure of recognizing PLG early with consequently progressive fatal outcomes. The rationale is to enlighten us concisely the knowledge surrounding PLG and consider it as a potential differential diagnosis, particularly in those immunosuppressed patients with radiological evidence of worsening pulmonary infiltrates not responding to customary treatment for common diagnoses. Having a high degree of suspicion for PLG in the right setting and pursuing lung biopsy early if appropriate for histopathology examination would be justified. This is essential to correctly diagnose PLG up-front and subsequently utilize best management approach for a better survival and mortality risk outlook.
