A case of fulminant Type 1 diabetes following anti-PD1 immunotherapy in a genetically susceptible patient.

Programmed cell death-1 protein (PD-1) is an immune checkpoint that has gained popularity in the treatment of several advanced cancers. Inhibiting this checkpoint is known to enhance immune response, but is also known to diminish immune tolerance and to increase autoimmune toxicity. We discuss a case of rapid onset fulminant Type 1 diabetes induced by treatment with anti-programmed cell death-1 monoclonal antibody, nivolumab, in a patient with late-stage non-small-cell lung adenocarcinoma. The patient had no history of previous diabetes but did reveal a high-risk genotype for Type 1 diabetes development (DR3-DQ2; DR4-DQ8). This finding supports that acute Type 1 diabetes can be an important adverse effect of immunotherapies targeting T-cell activation regulation. Because of the severity of this adverse effect, physicians should be aware of it, and studies directed to the detection of new biomarkers for early risk stratification (e.g., HLA) should be sought.

Carcinoma Mucoepidermóide do Pulmão ­ estado da arte a propósito de um caso clínico / Mucoepidermoid carcinoma of the lung ­ state of art with regard to a case report

O Carcinoma Mucoepidermóide do Pulmão (CMP) é um tumor com baixo potencial de malignidade; acredita-se que é indolente, mas pouco se sabe sobre as suas características dada a sua baixa incidência (0,2% de todos os tumores pulmonares). Os autores apresentam um caso clínico de um Carcinoma Mucoepidermóide do Pulmão de alto grau, a que se associa, habitualmente, metastização à distância, recorrência do tumor e mau prognóstico. Destaca-se a exuberante metastização (pleural, pericárdica, ganglionar e cerebral), que contrasta com o curso da doença, relativamente indolente, volvidos 12 meses do diagnóstico. Dado não ter indicação cirúrgica, a doente foi tratada com radioterapia e quimioterapia, apesar de ainda não estar definido um tratamento standard para este tipo histológico. A raridade do diagnóstico e os escassos estudos da literatura condicionam a abordagem terapêutica sistémica destes doentes, constituindo um desafio para a comunidade médica. O possível papel da terapêutica dirigida, como os inibidores da tirosina cinase do receptor do fator de crescimento epidérmico (EGFR) ou a terapêutica contra o oncogene de fusão CRTC1-MAML2, tem sido investigado, em doentes com Carcinoma Mucoepidermóide do Pulmão de alto grau.

Mucoepidermoid Carcinoma of the Lung (MECL) is a tumor of low malignant potential; we believe it is indolent, but little is known about its clinical features because of the low incidence rate (incidence of 0.2% of all lung cancers). We present a clinical case of a high degree Mucoepidermoid carcinoma of the lung, which frequently have distant metastasis, tumor recurrence and a bad prognosis. It is highlighted in this case the exuberant metastases (pleural, pericardial, cerebral, lymph nodes), contrasting with the relatively indolent course of the disease since the diagnosis (10 months ago). Our patient was treated with radiotherapy and chemotherapy, although effective treatment measures for high-grade tumors have not been established. The rarity of the diagnosis and the rare studies in the literature affect the systemic approach to these patients, becoming a challenge for the scientific community. The role of targeted therapy directed against the epidermal growth factor receptor (EGFR) and the novel fusion oncogene CRTC1-MAML2, is being investigated in high-grade tumors.

[Recurrent adenoid cystic carcinoma. Review based on a case report]. / Carcinoma adenóide quístico recorrente. Revisão a propósito de um caso clínico.

The adenoid cystic carcinoma is a malignant epithelial glandular type neoplasia, occurring frequently in the salivary and mammary glands, and rarely in the lung, and is responsible for 0.2% of lung tumours. These tumours present a slow growth and prolonged clinical course, and are characterised by their infiltrative nature and tendency towards late local recurrence. The authors present a case of a woman with adenoid cystic lung carcinoma diagnosed after investigation of a lung nodule, submitted to surgical resection with curative intention, whose follow -up identified late recurrence, 9 years after surgery. The present case emphasises the need for a prolonged surveillance, due to the potential late recurrence of this kind of tumour. The article reviews clinical and pathological features of lung adenoid cystic carcinoma, as well as therapeutic options, namely for prevention of recurrence.

[Emphysema and lung volume reduction surgery--a case report]. / Enfisema pulmonar e cirurgia de redução de volume - a propósito de um caso clínico.

Lung volume reduction surgery (LVRS) has been proposed as a palliative therapy in severe emphysema, with good results in lung function improvement, exercise capacity and quality of life. The authors present a case report of a 34 years old man with severe, heterogeneous lung emphysema submit to bilateral LVRS in 2000 January. Pre-surgical conditions, clinical and radiological evolution after surgery and for a 30 months follow up were analysed. Based on a literature revision, some technical aspects of LVRS, selection criteria and comparative study of medical and surgical therapy were discussed.

[Wegener's granulomatosis - a case report]. / granulomatose de Wegener-a propósito de um caso clínico.

The Wegener granulomatosis (WG) is a rare vasculite which involves preferentially the upper airways, lungs and kidneys, although it can presents in a multissistemic form. The authors present a clinical case of WG, its diagnostic methodology and respective follow-up from de last two years and half. There are discussed the most often presentation forms of the disease, the importance of histologic results and the contribution of ANCA's determination for disease diagnosis and follow-up. Although important, the histological results, are not essential for WG diagnosis, since clinic presentation and immunological data (ANCA's) are suggestive of the disease. Airways infections and ANCA's presence seems to be important in the etiopathogenesis of WG.