[Long-term survival case of primitive neuroectodermal tumor in the chest wall of a young adult].

A very rare case of Askin's tumor is described. A 22-year-old male was admitted to our hospital because of an abnormal shadow on chest roentgenogram. His chest X-ray film showed a tumor shadow (4.0×5.0 cm) of chest wall protruding into the upper right lung field. The tumor shadow had markedly enlarged in size compared to previous examinations of chest X-ray film 1 month before. Chest computed tomography(CT) and magnetic resonance imaging( MRI) revealed an heterogenous tumor fixed on the right 4th rib, which did not infiltrate the surrounding tissues. In the examination of bone scintigram, no abnormal accumulation was seen at the site of the chest wall tumor. Because of the possibility of malignancy, an en bloc excision of the tumor was performed including the surrounding pleura. The histologic examination revealed primitive neuroectodermal tumors of the chest wall (Askin's tumor).The patient underwent both additional adjuvant chemotherapy and radiation therapy postoperatively, being alive and well without any sign of recurrence 10 years after the operation. Askin's tumor is a very rare disease with an extremely poor prognosis, however, the effective treatment has not been established yet. Complete excision at initial operation seemed to be correlated with long-term survival in this case.

Immunoglobulin G4-related lung disease accompanied by organizing pneumonia.

The full picture of immunoglobulin G4-related lung disease (IgG4-RLD) has not yet been elucidated. A 69-year-old man was referred to us with a more than 2-week history of productive cough and fatigue. Chest CT showed an airspace consolidation along the bronchovascular bundles. The pathological findings that were obtained from an open-lung biopsy showed both organizing pneumonia and interstitial pneumonia. Based on the established, comprehensive diagnostic criteria for IgG4-related disease (RD) as of 2011, this patient was given a definitive diagnosis of IgG4-RD. A further accumulation and analysis of those cases that concomitantly present with both IgG4-RLD and organizing pneumonia, like our patient, may contribute to the elucidation of the pathology of IgG4-RLD and the establishment of the disease spectrum.

Pulmonary hypertension confirmed histologically five months prior to scleroderma renal crisis onset.

A 69-year-old man presented shortness of breath and acute renal failure. He had undergone pulmonary partial resection for lung cancer 5 months prior. On examination, severe hypertension, skin sclerosis of his forearms, and anticentromere antibody were observed. A renal biopsy specimen showed characteristic findings for scleroderma renal crisis, and a right heart catheterization revealed severe pulmonary arterial hypertension. Re-examination of the resected lung specimen revealed sclerodermatous vascular involvement was present.

[Sleeveresection of the left main bronchus for bronchogenic carcinoid for preserving lung parenchyma].

UNLABELLED: We performed bronchoplasty for a bronchogenic tumor of low-grade malignancy without lung parenchyma resection. A 69-year-old man visited our hospital in March 2008 because of cough. Chest computed tomography (CT) revealed atelectasis of the entire left upper lobe and a 2-cm mass with strong contrast enhancement in the lumen of the left main bronchus. Bronchoscopy identified a polypoid mass in the left main bronchus, about 3 cm distal to carina, obstructing the lumen. Biopsy led to a diagnosis of typical carcinoid tumor. Surgery : Thoracotomy showed complete atelectasis of the left upper lobe. After lymph node dissection, resection of the left main bronchus including the site of tumor origin was performed. From the extent of expansion, the left upper lobe was decided to be possible to be spared, and end-to-end anastomosis of the bronchus was performed. Postoperative respiratory rehabilitation resulted in improved aeration of the left upper lobe and markedly improved respiratory function. CONCLUSION: The judgment of whether the long-standing atelectatic left upper lobe could be spared or not was a key in choosing this procedure.

[A case of paragonimiasis miyazakii suspected after pathologic examination and subsequently confirmed].

A 58-year-old man presented with right backache and bloody sputum. Chest X-ray revealed a nodular opacity in the right lung. Since could not obtain a diagnosis by bronchoscopy, we performed a lower lobectomy. Histopathologically, there were irregularly shaped necrotizing granulomas and an area of acute hemorrhagic, eosinophilic abscess. We suspected paragonimiasis. The diagnosis of paragonimiasis miyazakii was confirmed by ELISA. This is a valuable case of suspected paragonimiasis confirmed pathologically.

Autopsy case of acute liver failure due to scrub typhus.

Scrub typhus (Tsutsugamushi disease) is an acute febrile disease caused by infection with Orientia tsutsugamushi transmitted by mites. Although patients with scrub typhus commonly display mild liver injury, few die of acute liver failure. We describe herein an autopsy case of acute liver failure due to scrub typhus, which was complicated by disseminated intravascular coagulation and showed rapid progression of liver injury just before death. Histopathological findings revealed submassive hepatocellular necrosis, inflammatory cell infiltration in Glisson's capsules, and sporadic fibrin thrombi in the hepatic sinusoids. Cause of death was primarily associated with acute liver failure related to disseminated intravascular coagulation.