A case of Sjögren's syndrome in which diffuse cystic lung lesions led to an accurate diagnosis.

Key Clinical Message: Even in the absence of other symptoms or other pulmonary manifestations suggesting Sjögren's syndrome (SS), it is necessary to include SS in the differential diagnosis of diffuse cystic lung disease (CLD). Abstract: A case of SS that presented initially with diffuse CLD is reported. This case is considered rare because diffuse pulmonary cysts were observed in the early stage with few symptoms, only cysts were observed without other lung lesions on imaging, cyst formation was histologically considered to be alveolar loss, and airway lesions not observed on imaging were suspected based on lung function testing. The details of this case provide extremely important information to consider for the diagnosis and management of CLD and SS.

Surgical Resection to Treat a Japanese Patient with Pulmonary Coccidioidomycosis.

Coccidioidomycosis is an endemic disease that is particularly prevalent in the United States. However, its geographic distribution is becoming widespread. Here, we present a Japanese male who resided in the United States for 1 year, where he was diagnosed with pulmonary coccidioidomycosis that was accompanied by cavity formation. He did not tolerate antifungal therapy and consequently underwent partial resection of the upper lobe of his left lung upon his return to Japan. The patient's symptoms improved after surgery. The trend toward global networking and logistics means that a diagnosis of coccidioidomycosis should be considered in routine practice in nonendemic areas. Due to the rarity of surgical treatment for this disease, prolonged follow-up is necessary. During the last follow-up, the patient was symptom-free.

A Case of Lymphangiomatosis With Infected Lymphangiomas Effectively Treated by Thoracoscopic Debridement and Drainage.

A 40-year-old woman with lymphangiomatosis also had an intrathoracic lymphangioma infection. Since the age of 8 years, the patient had undergone repeated abdominal and mediastinal cyst surgeries and had received a diagnosis of lymphangiomatosis. At this time, she showed a high fever of 38.5°C. Cultures of both blood and fluid aspirated from the cyst were positive for Staphylococcus aureus. Chest CT imaging revealed an enlarged right-sided mediastinal cystic lymphangioma with new septa in it. A chest tube was inserted into the cyst to remove effusion. The patient was then started with the antibacterial drug cefazolin at 3 g/d. But effective drainage was difficult because of the high viscosity of the effusion and septa working as barriers. We removed these components with endoscopic surgical instruments and via a pulsed-lavage system under general anesthesia. Postoperative CT images showed reexpanded lung structure and reduced cyst size. The patient has taken a favorable course for 2 years.

Expression of PTEN and its pseudogene PTENP1, and promoter methylation of PTEN in non-tumourous thymus and thymic tumours.

AIMS: Mutation or promoter methylation of the phosphatase tensin homologue deleted on chromosome 10 tumour suppressor gene (PTEN) promotes some cancers. Moreover, PTENP1 (PTEN pseudogene) transcript regulates PTEN expression and is thought to be associated with tumourigenesis in some cancers. Here, we investigated PTEN expression in thymic epithelium and thymic epithelial tumours. METHODS: Immunohistochemical analysis of PTEN was performed on two non-tumourous thymus (NT) samples, 33 thymomas (three type A, eight type AB, 11 type B1, six type B2, and five type B3), and four thymic carcinomas (TCs). In 16 cases (two NT, three A, five B1, two B2, one B3 and three TC), analyses of mutations, promoter methylation and comparisons of PTEN mRNA and PTENP1 transcripts were undertaken using PCR-direct sequencing, methylation-specific PCR, and reverse-transcription real-time PCR after target cell collection with laser microdissection. RESULTS: PTEN protein was not immunohistochemically detected in NT epithelium or types B1 or B2 thymoma cells, but was expressed in type A thymoma and carcinoma cells. Neither PTEN mutations nor promoter methylation were detected in any samples. Statistical analysis revealed that PTEN mRNA expression was highest in NT epithelium and lowest in type A thymoma cells. PTENP1 transcript expression did not significantly differ among NT, thymoma and TC samples. CONCLUSIONS: We speculated that NT epithelium and types B1/B2 thymoma cells have a mechanism of PTEN translation repression and/or acceleration of protein degradation, whereas type A thymoma cells exhibit transcriptional repression of PTEN mRNA and accelerated translation and/or protein accumulation.

Correlation between histological invasiveness and the computed tomography value in pure ground-glass nodules.

PURPOSE: The purpose of this study was to evaluate the correlation between histological invasiveness and the computed tomography (CT) value and size in pure ground-glass nodules (GGNs) to determine optimal "follow-up or resection" strategies. METHODS: Between 2001 and 2014, 78 resected, pure GGNs were retrospectively evaluated. The maximum diameter and CT value of pure GGNs were measured using a computer graphics support system. RESULTS: All GGNs with a maximum diameter ≤10 mm and CT value ≤-600 Hounsfield units (HU) were considered to be noninvasive lesions, while 21 of 26 (81 %) with a maximum diameter >10 mm and CT value >-600 HU were considered to be invasive lesions. With respect to the correlation between each histological type and pure GGN with a maximum diameter ≤10 mm and CT value ≤-600 HU, the specificity was 90 % and the sensitivity and negative predictive value were both 100 % in atypical adenomatous hyperplasia (AAH), while the specificity was 58 % and the sensitivity and positive predictive value were 0 % in minimally invasive and invasive adenocarcinoma. CONCLUSION: Pure GGNs with a maximum diameter of ≤10 mm and CT value of ≤-600 HU are nearly always pre-invasive lesions; therefore, surgery should be carefully selected in such patients.

[Surgically treated desmoid tumor of the chest wall which located at the previous thoracotomy site].

Desmoid tumor is a soft-tissue tumor of unknown cause. Since recurrence sometimes occurs even with complete resection, careful consideration of which portions to resect and close postoperative followup are recommended. Seventeen months after undergoing a right upper lobectomy for primary lung adenocarcinoma, a 65-year-old female patient experienced pleural tumor which located at the previous thoracotomy site, as revealed by chest X-ray and computed tomography (CT). While needle aspiration biopsy revealed no malignancy, recurrence of the cancer could not be ruled out clinically. The tumor was resected with chest wall and lung and the histopathological diagnosis was desmoid tumor. This case demonstrates the importance of conducting differential diagnosis with recurrence or desmoid tumor after operation to treat lung cancer. Five years after resection of the desmoid tumor, no recurrence is observed.

One-dimensional mean computed tomography value evaluation of ground-glass opacity on high-resolution images.

OBJECTIVE: Differentiation of atypical adenomatous hyperplasia (AAH), bronchioloalveolar carcinoma (BAC), and invasive carcinoma on computed tomography (CT) is useful for determining "follow-up or resection" strategies for lesions displaying ground-glass opacity (GGO). The purpose of this study is to evaluate one-dimensional quantitative CT values of GGO on high-resolution CT (HRCT) images using computer-aided diagnosis. METHODS: Between April 2001 and March 2010, a total of 44 nodules in 42 patients with pure or mixed GGOs ≤2 cm were retrospectively evaluated. Maximum diameter and one-dimensional mean CT (m-CT) value of the diameter were measured using a computer graphics support system (HOPE/DrABLE-EX, Fujitsu, Tokyo, Japan) that displays a CT density profile across the tumor. RESULTS: m-CT values were -682 ± 64 HU (range) for AAH lesions, -544 ± 179 (range) for Type A lesions, -496 ± 147 (range) for Type B lesions, and -371 ± 142 (range) for invasive lesions. AAH lesions had a significantly lower m-CT value than Type B lesions. AAH, Type A, and Type B lesions had significantly lower m-CT values than invasive lesions (p < 0.05). All seven GGO lesions with a maximum diameter ≤1 cm and m-CT value ≤-600 HU were pre-invasive lesions, while 16 of 22 (73 %) cases with maximum diameter >1 cm and m-CT value >-600 HU were invasive lesions. CONCLUSION: Observation may be indicated for GGO lesions with a maximum diameter ≤1 cm and m-CT value ≤-600 HU.

Thymoma with intracystic dissemination arising in a unilocular thymic cyst.

A 33-year-old asymptomatic woman was referred to our hospital for evaluation of an abnormal shadow on a chest radiograph. A chest-computed tomogram revealed a cystic, partially solid tumor in the right inferior mediastinum. Following an initial diagnosis of cystic teratoma, surgery was performed. The tumor, a well-encapsulated, thin-walled cyst located within the right inferior lobe of the thymus, was extirpated by partial resection of the thymus. The cut surface revealed a unilocular cyst containing multiple grayish-white tumors on the inner cyst walls. Some tumors detached from the wall owing to loose connections. Microscopic findings revealed that the cyst was lined with squamous epithelium. The tumor on the cyst wall was diagnosed to be a type B1 thymoma. Based on these findings, we diagnosed this case as a thymoma originating from the wall of a thymic cyst, accompanied by intracystic dissemination from the tumor.

[Case of primary cavitary sarcoidosis: exacerbation after 16 years from onset].

A 46-year-old man presented with the complaint of cough, fever and dyspnea on exercise. Sixteen years previously stage II sarcoidosis was diagnosed, with an ocular lesion. So far, he had had no respiratory symptoms and was followed up without medication. Two months before his visit, he had a cough. He gradually developed a fever and felt dyspnea on exercise. Chest X-ray showed an infiltrative shadow with a cavity in the right upper lobe. Bronchofiberscopy was performed and the biopsy specimen from the cavity wall revealed noncaseating epitheloid cell granulomas, suggesting primary cavitary sarcoidosis. Corticosterid therapy was started. His symptoms improved rapidly, and the infiltrative shadow on the right upper lobe decreased within one month. The cavity wall was thin on the following computed tomography. Thirty-two cases of primary cavity sarcoidosis were reported in Japan from 1975-2005. The mean age of the patients was 26.5 years old and the ratio of males to females was 13:3. In most cases, corticosteroid therapy was effective and nine spontaneously improved cases were reported. To determine the indications for steroid therapy, further examination is needed.

Gorham's disease complicated by chyloma of the chest wall.

Gorham's disease is a rare disorder characterized by a non-malignant proliferation of thin-walled lymphatic vessels that result in progressive bony destruction and often extend into the surrounding soft tissues. Chylothorax is a common complication in cases of mediastinal involvement. Here we report a case of a 22-year-old male with Gorham's syndrome complicated by chyloma of the chest wall, but without chylothorax. To our knowledge, this is the first report demonstrating an abnormality of the parietal pleura prior to the clinical development of the chylothorax.

Pulmonary adenocarcinoma complicated with pulmonary infarction presented as intrapulmonary metastases: a report of a case.

Pulmonary adenocarcinoma complicated with a pulmonary infarction presenting as an intrapulmonary metastasis is relatively rare. We present a case of pulmonary infarction manifesting as intrapulmonary metastases of lung cancer. A previously healthy 59-year-old woman was admitted to our hospital for evaluation of abnormal shadows in the right lower lung field. Laboratory tests showed no abnormalities except for a slight elevation of carcinoembryonic antigens (CEAs). Computed tomography (CT) of the chest revealed a hilar mass lesion with parenchymal lesions in the periphery of the right lower lobe, highly suspected to be a pulmonary adenocarcinoma with intrapulmonary metastases. A diagnosis of pulmonary adenocarcinoma was confirmed by a transbronchial brushing examination. A right middle and lower bilobectomy with mediastinal lymph node dissection was due to hilar lymphadenopathy and a lower lobe invasion of the main tumor. Histopathological findings of the resected specimens revealed poorly differentiated adenocarcinoma of the lung with N1 (number 11i and 12 l) disease and multiple pulmonary infarctions with coagulation necrosis and recanalization. Our case suggests that pulmonary infarction associated with lung cancer should be considered as one important cause of peripheral pulmonary nodules.

Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography.

Tracheoesophageal fistula (TEF) or bronchoesophageal fistula may be congenital, inflammatory, neoplastic, or secondary to trauma. Congenital TEF or bronchoesophageal fistula is usually associated with esophageal atresia and is readily diagnosed in infancy. But if it is not associated with esophageal atresia, it may persist until adulthood. Some theories have been proposed to explain this delay in diagnosis. We present a case of a 70-year-old man with congenital TEF. The TEF was successfully diagnosed by multidetector-row CT esophagography.

[Serum amyloid protein A was a useful marker for steroid tapering in a case of MPO-ANCA-associated vasculitis].

A 55-year-old man was admitted complaining of hemosputa, fever and dyspnea. The chest radiographs and computed tomography showed a diffuse alveolar filling pattern; suggesting alveolar hemorrhage. Laboratory data demonstrated renal dysfunction with hematuria and proteinuria and serum MPO-ANCA was also elevated. Respiratory failure progressed rapidly within two days. Steroid pulse therapy and plasmapheresis was performed. Thereafter, symptoms and chest radiograph findings improved dramatically. However proteinuria persisted as steroid administration was tapered. Renal biopsy demonstrated gromeluronephritis and interstitial lymphocyte infiltration. After administering a second course of steroid semi-pulse therapy, her proteinuria improved. C-reactive protein and MPO-ANCA decreased to normal levels after the initial steroid therapy, but serum amyloid A protein (SAA) gradually elevated. The second course of steroid pulse therapy normalized SAA, and proteinuria improved. Based on these findings, SAA seems to be a more sensitive marker for steroid tapering than either CRP or MPO-ANCA.

[A case of a slow-growing, non-mucin-producing bronchioloalveolar carcinoma with a 9-year clinical history before thoracoscopic surgery].

A 73-year-old woman was followed up with high-resolution computed tomography (HRCT) from 2000 through 2002 because an area of ground-glass opacity in area S1 + 2 of the left upper lobe was found on HRCT in August 2000. The opacity was present on ordinary CT scans obtained in 1993, but had not been recognized. Thoracoscopic partial resection of the left lung was performed on October 8, 2002. Pathologic examination of the resected specimen showed localized bronchioloalveolar carcinoma (Noguchi's type B). Tumor doubling time was 1718 days from 1993 to August 2000 and 273 days from August 2000 through 2002. This case of adenocarcinoma with bronchioloalveolar growth was characterized by initial slow growth followed by accelerated growth, no mucin production, and a long interval from the detection of opacity to surgery.