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Matsui S.,University of Toyama | Hebisawa A.,NHO Tokyo National Hospital | Sakai F.,Saitama University | Yamamoto H.,Shinshu University | And 15 more authors.
Respirology | Year: 2013

Background and objective Immunoglobulin G4 (IgG4)-related disease is a multi-organ disorder that can include the lungs. IgG4-related lung disease can present in various forms; the clinical, radiological and pathological features of patients with this disease have been assessed. Methods Forty-eight patients suspected of having IgG4-related lung disease, with a high serum concentration of IgG4 and abundant IgG4-positive plasma cell infiltration into the intrathoracic organs, were retrospectively evaluated. Their clinical features, chest imaging findings and pathological findings were examined, with final diagnoses made by an open panel conference. Results Of the 48 patients, 18 with extrathoracic manifestations were diagnosed as having IgG4-related lung disease. Most of these patients were middle-Aged to elderly men. IgG4-related lung disease was characterized by high serum concentrations of IgG and IgG4, normal white blood cell count and serum C-reactive protein concentration and a good response to corticosteroids. Common radiological findings included mediastinal lymphadenopathy and thickening of the perilymphatic interstitium, with or without subpleural and/or peribronchovascular consolidation. Pathological examination showed massive lymphoplasmacytic infiltration with fibrosis in and around the lymphatic routes, with distribution well correlated with radiological manifestations. Conclusions The findings suggest that the intrathoracic manifestations of IgG4-related lung disease develop through lymphatic routes of the lungs and show various clinical characteristics. Because some lymphoproliferative disorders show similar findings, the correlation of clinicoradiological and pathological characteristics is crucial for the diagnosis of IgG4-related lung disease. The clinicoradiological and pathological features of IgG4-RLD were assessed. Clinically, this disease is silent, although radiological and pathological examinations showed that it involves lymphatic routes, with spread observed on imaging and infiltration on pathological examination. Clinicoradiological and pathological correlation is necessary for the diagnosis of this disease. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology. Source


Sekine A.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Kato T.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Hagiwara E.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Shinohara T.,Kanagawa Prefectural Cardiovascular and Respiratory Center | And 9 more authors.
Lung Cancer | Year: 2012

Introduction: Miliary brain metastasis is a rarity and refers to the presence of numerous small tumors in a perivascular distribution without intraparenchymal invasion and focal edema. Although the presence of epidermal growth factor receptor (EGFR) mutation and good response to gefitinib have been reported in non-small cell lung cancer (NSCLC) patients with miliary brain metastases, the influence of the EGFR mutations on the radiographic features remains unclear. Patients and methods: All NSCLC patients with synchronous brain metastases detected at the time of a new diagnosis of NSCLC from March 2005 through May 2011 were divided according to EGFR mutation status. The number of brain tumors, size of the largest brain tumors, and size of peritumoral brain edema were compared among the groups. Results: Fifty-seven patients who met the criteria were divided into three groups: wild-type EGFR group (31 patients), exon 19 deletion group (18 patients), and exon 21 point mutation group (8 patients). The exon 19 deletion group had more multiple and smaller brain tumors with smaller peritumoral brain edema than did the wild-type group (P= 0.024, P= 0.0016, and P= 0.0036, respectively). The exon 21 point mutation group showed no significant difference in any of the radiographic values when compared with the wild-type group. Conclusion: Our results indicate that NSCLC patients with the exon 19 deletion have such a peculiar pattern of brain metastases as multiple small metastases with small brain edema. This metastatic pattern may be similar to that of miliary brain metastases. Because it is unclear whether or not severe neurologic symptoms develop during their clinical courses like miliary brain metastases, regular evaluation with brain magnetic resonance imaging (MRI) should be considered, regardless of the presence of neurologic symptoms. Accumulation of knowledge about specific pattern of brain metastasis will help approach to "individual" management. © 2012 Elsevier Ireland Ltd. Source


Samejima J.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Takahashi K.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Omori T.,Kanagawa Prefectural Cardiovascular and Respiratory Center | Okudela K.,Yokohama City University | And 2 more authors.
Annals of Thoracic Surgery | Year: 2010

A 46-year-old man was referred to our department complaining of anterior chest pain. A chest computed tomographic scan revealed an anterior mediastinal tumor measuring 38 × 35 × 50 mm. Suspecting a thymoma, we performed extended thymectomy through a median sternotomy under general anesthesia. Pathologically, the tumor was composed of pleomorphic spindle-shaped cells arranged in a storiform pattern, with marked inflammatory cell infiltration. The tumor was contiguous with the thymus tissue. Based on these findings, we made the diagnosis of inflammatory malignant fibrous histiocytoma originating from the thymus. Until now, 12 months later, the patient has shown no evidence of tumor recurrence. © 2010 The Society of Thoracic Surgeons. Source

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