ABSTRACT
Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described distinct pattern of lung apical fibrosis involving the upper lobe parenchyma and pleural dome. PPFE has definable and reproducible clinical, radiological and histopathological criteria, which allowed its classification as an independent interstitial lung disease. Several factors have been associated with PPFE, such as chemotherapy, especially with alkylating agents. The authors present a case of a 34-year-old female with previous history of Hodgkin lymphoma treated with first line chemotherapy (doxorubicin, bleomycin, vinblastine and dacarbazine). The patient had no other known comorbidities or relevant exposure to lung irritants. A total of 2 years after completing cancer treatment, the patient developed clinical and radiological features of PPFE. Given their previous history of malignancy, a biopsy of the lesion was obtained, which confirmed the diagnosis of PPFE. The authors present this case to raise awareness of this disease and to demonstrate that PPFE can develop months to years following chemotherapy treatment. Moreover, to date, none of these chemotherapy agents have been associated with the development of PPFE.
ABSTRACT
Synchronous tumours are defined as two or more independent primary neoplasms of different origins diagnosed at the same time in 1 individual. Although rare, its incidence is increasing and the proper diagnosis and staging of each tumour is crucial in defining the patient prognosis and the best therapeutic choice. We present a case of a 56-year-old woman presenting with a lung adenocarcinoma and pulmonary metastases initially diagnosed as stage IV and who was started on a tyrosine kinase inhibitor (erlotinib). In the meantime, she was also diagnosed with papillary thyroid carcinoma and was submitted to complete thyroidectomy. After 6 cycles of erlotinib, thoracic CT showed a decrease in the dimensions of the primary pulmonary tumour, but an increase in the size and number of pulmonary metastases while blood tests showed elevated thyroglobulin. This therefore raised the possibility that the metastases could have originated from the thyroid carcinoma. Anatomo-pathological examination of the lung metastases confirmed this hypothesis. In conclusion, it is important to confirm the origin of metastases in synchronous tumours given this can lead to a re-staging of tumours and a different prognosis, along with other therapeutic options. A multidisciplinary team meeting is crucial to define management and therapeutic approaches for these patients. LEARNING POINTS: Although rare, synchronous primary tumours are a possibility and physicians should be aware of this possibility.Papillary thyroid carcinoma may metastasize to the lungs and its differential diagnosis should be made in the presence of lung carcinoma.When in doubt, anatomo-pathological examination of the metastases should be performed for proper staging of both tumours and to decide on the best therapeutic option.
ABSTRACT
Primary effusion lymphoma is a rare and aggressive large B-cell lymphoma presenting as malignant effusion with poor prognosis. Although it is more prevalent among HIV patients, it has also been described in non-HIV immunocompromised individuals. Given its rarity, there are no large randomized trials regarding the best therapeutic option. The choice of therapy is based on retrospective studies, case reports and preclinical data. We present the case of a non-HIV patient with relapsed disease after treatment with CHOP who was then successfully treated with brentuximab vedotin, achieving complete remission. LEARNING POINTS: Primary effusion lymphoma is a rare entity in non-HIV patients, so there are no clinical trials regarding therapeutic options or management decisions.Targeted therapy with brentuximab vedotin has been used in other CD30 positive malignancies and in HIV-related primary effusion lymphoma with good outcomes.We present the case of a non-HIV patient with primary effusion lymphoma who was successfully treated with brentuximab vedotin, which highlights the potential role of a new therapeutic approach in this subgroup of patients.
ABSTRACT
Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described interstitial pneumonia. It consists of progressive fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes, with defined and reproducible clinical, radiological and histopathological criteria. No effective treatment has yet been shown to modify the natural course of the disease, which vary greatly in the literature. Several conditions have been associated with PPFE, including connective tissue diseases (CTD). The authors present two cases of female patients with a CTD (rheumatoid arthritis and limited cutaneous systemic sclerosis, respectively) who presented with typical bilateral upper lobe thickening in chest-HRCT. In the first case, diagnosis was based on "definite" radiological and histopathological criteria for PPFE, while in the second case diagnosis was established on clinical grounds after discussion in a multidisciplinary team meeting. The authors present these cases of CTD-associated PPFE in order to raise awareness of this entity among clinicians.
ABSTRACT
Analisa dados estatísticos sobre a violência policial no Rio de Janeiro. Em 1997, eram 300 as vítimas fatais de ações policiais no Rio de Janeiro; em 1998, passou 397; em 2001, subiu para 597 e em 2003, foi para 1195, segundo dados do NECVU-UFRJ (Núcleo de Estudos da cidadania, conflito e violência urbana da UFRJ). As áreas onde residem as populações de baixa renda, tais como favelas, conjuntos habitacionais para proletários e posses, concentram um maior número de atingidos por essas ações. Segundo o relatório da Unesco, de 2003, em sua maioria essas vítimas são jovens, entre 18 e 24 anos, pobres e negros. (AU)
