MAPK/ERK and PI3K/AKT signaling pathways are activated in adolescent and adult acute lymphoblastic leukemia.

BACKGROUND: The mitogen-activated protein kinase (MAPK)/ERK signaling cascade and the phosphoinosytol-3 phosphate/Akt (PI3K/Akt) pathways are involved in proliferation and differentiation of hematopoietic cells. The frequency of PI3K/Akt and MAPK pathway activation in adult acute lymphoblastic leukemia (ALL) still need to be elucidated. AIMS: To assess the activity and prognostic implications of MAPK/ERK and PI3K/Akt pathways in adult (ALL). METHODS: We examined 28 precursor-B-cell ALL and 6 T-cell primary ALL samples. Flow cytometry was employed to analyze the expression levels of phosphorylated ERK and phosphorylated Akt. RESULTS: Ten out of 15 (67%) ALL fresh samples (7 B-cell, 3 T-cell) showed constitutive p-ERK expression. The p-ERK mean fluorescent index ratio (MFI (R)) showed a tendency to be higher in ALL than in normal T lymphocytes (1.26 [0.74-3.10] vs. 1.08 [1.02-1.21], respectively [p = .069]) and was significantly lower than in leukemic cell lines (median MFI (R) 3.83 [3.71-5.97] [p < .001]). Expression of p-Akt was found in 35% (12/34) (10 B-cell, 2 T-cell). The median MFI (R) expression for p-Akt in primary blast cell was 1.13 (0.48-9.90) compared to 1.01 (1.00-1.20) in normal T lymphocytes (p = ns) and lower than in leukemic cell lines (median MFI (R) 2.10 [1.77-3.40] [p = .037]). Moreover, expression of p-ERK was negatively associated with the expression of CD34 (1.22 [0.74-1.33] vs. 1.52 [1.15-3.10] for CD34(+) and CD34(-) group, respectively, p = .009). CONCLUSION: Our findings suggest that both MAPK/ERK and PI3K/Akt are constitutively activated in adult ALL, indicating a targeted therapy potential for ALL by using inhibitors of these pathways.

Complex pulmonary aspergilloma treated by cavernostomy / Tratamento do aspergiloma pulmonar complexo por cavernostomia

Objective: To evaluate the effectiveness of cavernostomy in patients with complex fungal balls. Methods: We analyzed the medical records of patients undergoing cavernostomy between January 2005 and May 2013, evaluating: age, gender, preoperative signs and symptoms, predisposing disease, preoperative tests, location of the aspergilloma, etiologic agent, cavernostomy indication, postoperative outcome. Results: Ten patients were male. The mean age was 42.9 years (34-56). The most frequent symptom was repeated pulmonary bleeding. Cavernostomy was proposed for patients at high risk for lung resection. It was performed in 17 patients and all of them had pulmonary tuberculosis sequelae, with cavitations. The indication in all cases was hemoptysis and elimination of phlegm. The cavernostomies were performed in a single surgical procedure. In all 17 patients the cavity was left open after the withdrawal of the mycetoma. In all patients hemoptysis ceased immediately. Operative mortality was 9.5% (1). Conclusion: cavernostomy is an effective treatment alternative in patients at high risk. It may be useful in some patients with complex aspergilloma, irrespective of lung function or bilateral disease. It is technically easy, has low-risk, saves parenchyma, and may be performed in a single operative time. .

Objetivo: avaliar a efetividade da cavernostomia nos pacientes com bola fúngica complexa. Métodos: foram analisados os prontuários de pacientes submetidos à cavernostomia entre janeiro de 2005 e maio de 2013. Foram avaliados: idade, sexo, sinais e sintomas pré-operatórios, doença predisponente, exames pré-operatórios, localização do aspergiloma, agente etiológico, indicação da cavernostomia, evolução pós-operatória. Resultados: dez pacientes eram do sexo masculino. A média de idade foi 42,9 anos (34-56). O sintoma mais frequente foi o sangramento pulmonar repetido. A cavernostomia foi proposta para os pacientes com risco elevado para ressecção pulmonar, foi realizada em 17 pacientes e todos eles apresentavam sequelas de tuberculose pulmonar com lesões cavitárias. A indicação em todos os casos foi hemoptise e eliminação de catarro. As cavernostomias foram realizadas em tempo cirúrgico único. Nos 17 pacientes a caverna foi deixada aberta após a retirada do micetoma. Em todos os pacientes a hemoptise cessou imediatamente. A mortalidade operatória foi 1 (9.5%). Conclusão: a cavernostomia é uma alternativa de tratamento efetivo em pacientes com risco elevado. Pode ser útil em alguns pacientes com aspergiloma complexo, independentemente da função pulmonar ou doença bilateral. É tecnicamente fácil, de baixo risco, poupa parênquima, e realizada em tempo operatório único. .

Complex pulmonary aspergilloma treated by cavernostomy.

OBJECTIVE: To evaluate the effectiveness of cavernostomy in patients with complex fungal balls. METHODS: We analyzed the medical records of patients undergoing cavernostomy between January 2005 and May 2013, evaluating: age, gender, preoperative signs and symptoms, predisposing disease, preoperative tests, location of the aspergilloma, etiologic agent, cavernostomy indication, postoperative outcome. RESULTS: Ten patients were male. The mean age was 42.9 years (34-56). The most frequent symptom was repeated pulmonary bleeding. Cavernostomy was proposed for patients at high risk for lung resection. It was performed in 17 patients and all of them had pulmonary tuberculosis sequelae, with cavitations. The indication in all cases was hemoptysis and elimination of phlegm. The cavernostomies were performed in a single surgical procedure. In all 17 patients the cavity was left open after the withdrawal of the mycetoma. In all patients hemoptysis ceased immediately. Operative mortality was 9.5% (1). CONCLUSION: cavernostomy is an effective treatment alternative in patients at high risk. It may be useful in some patients with complex aspergilloma, irrespective of lung function or bilateral disease. It is technically easy, has low-risk, saves parenchyma, and may be performed in a single operative time.

[Traumatic lung hernia]. / Hérnia traumática do pulmão.

Traumatic lung herniation is an unusual clinical problem. We present a case of a large left post-traumatic lung hernia on the left, anterior, second intercostal space following blunt chest trauma. An important factor in the etiology of these lesions is the relative lack of muscular support of the anterior part of the chest. This report describes the diagnosis and management of a post-traumatic lung hernia.

Hérnia traumática do pulmão / Traumatic lung hernia

Traumatic lung herniation is an unusual clinical problem. We present a case of a large left post-traumatic lung hernia on the left, anterior, second intercostal space following blunt chest trauma. An important factor in the etiology of these lesions is the relative lack of muscular support of the anterior part of the chest. This report describes the diagnosis and management of a post-traumatic lung hernia.

Doença de Madelung: relato de caso / Madelung's disease: a case report

Doença de Madelung ou lipomatose benigna múltipla simétrica é uma doença rara, caracterizada pelo acúmulo de gordura não encapsulada, geralmente localizada simetricamente ao redor do pescoço, ombros, região subociptal e no tórax. A etiologia é desconhecida, entretanto, freqüentemente é observada em pacientes com história de alcoolismo crônico acompanhada de alterações metabólicas. Os autores apresentam um caso de lipomatose cervical associada a polineuropatia e ao alcoolismo. A remoção cirúrgica do tecido adiposo por lipectomia ou lipoaspiração é terapia paliativa, pois a recorrência é comum.

Madelung’s disease or multiple symmetric lipomatosis is a rare disease characterized by non-encapsulated accumulation of fat, generally located symmetrically on cervical area, shoulders, suboccipital area and trunk. The etiology is unknown, but is often observed in patients with a history of chronic alcoholism and it is accompanied by metabolic abnormalities. The authors present a case of lipomatosis. The treatment is palliative associated with the removal of excessive fat tissue by lipectomy or liposuction, and the recurrence is common.

Hemangioma pulmonar esclerosante / Pulmonary sclerosing hemangioma

O hemangioma pulmonar esclerosante é um tumor pouco freqüente, que se manifesta como nódulo pulmonar solitário. Entretanto, o seu comportamento biológico ainda não está definido adequadamente. O diagnóstico e a evolução dessas lesões são dificultados pelos achados radiológicos inespecíficos e a dificuldade de interpretação das biópsias de congelação. Os autores descrevem um caso em que o tumor foi completamente retirado com a lobectomia, e o diagnóstico histopatológico definitivo foi de hemangioma esclerosante de pulmão. A ressecção em cunha é realizada na maioria dos casos; porém, quando o diagnóstico não é definido no intra-operatório, é recomendada ressecção mais extensa e linfadenectomia sistemática. Mais estudos são necessários para avaliar a extensão da doença, apesar das metástases não alterarem o excelente prognóstico.

Lung sclerosing hemangioma is an uncommon tumor that presents as a solitary asymptomatic nodule. However, the biologic behavior of this tumor has not yet been characterized adequately. Diagnostic evaluation of this lesion is a diagnostic challenge due to the nonspecific radiological findings, potential intraoperative dilemmas like difficult frozen section interpretation.We describe a case of lung sclerosing hemangioma. The tumour was completely removed by lobectomy and the definitive biopsy was informed as a lung sclerosing hemangioma. Generally, wedge resection is justified in the majority of cases, but in cases of uncertain intraoperative diagnosis, extensive resection with systematic lymphadenectomy is recommended.More studies are needed to evaluate extension of this disease, despite metastasis does not seem to change theexcellent prognosis.

[Pulmonary blastoma]. / Blastoma pulmonar.

Pulmonary blastoma is a rare primary lung tumor with poor prognosis that commonly presents at a younger age than the non-small cell lung cancer. Classically they are large, symptomatic tumors with lymph nodal metastasis and carry poor prognosis. Pathological examination revealed features suggesting a biphasic tumor with mesenchymal and epithelial components. Over 200 cases have been reported so far worldwide since the first description of the tumor in 1945. Authors present a case of pulmonary blastoma with literature revision.