Surgical treatment for osteochondromas in pediatric digits.

PURPOSE: Osteochondromas in pediatric digits occasionally require surgical treatment due to restricted finger motion and/or angulatory deformity. However, the patients are growing children, and the indication for surgical treatment is controversial. We reviewed our cases in order to clarify characteristics of tumors and to report surgical outcomes. METHODS: We performed surgeries on 17 osteochondromas in the digits of 16 patients. The average age at surgery was 3.6 years. Ten of 16 patients had solitary osteochondroma, and 6 patients had been diagnosed with multiple osteochondromatosis. We classified osteochondroma into 3 types, according to their locations. Type A is located at the nonepiphyseal metaphysis of the bone (9 lesions), type B in the metaphysis on the epiphyseal plate side (5 lesions), and type C in the diaphysis (3 lesions). Surgery was indicated for either or both restricted motion and angulatory deformity. Simple excision of the tumor was performed in 14 lesions, wedge osteotomy in 2 fingers, and osteotomy with excision of tumor in 1 finger. The average follow-up period was 49 months (range, 14-155 mo). RESULTS: Surgical outcomes in types B and C were all good, the deformities were well corrected, and range of motion was improved. On the other hand, in type A, 2 cases still had more than 30° of restricted motion at the final follow-up, and another 2 cases showed more than 10° of angulatory deformity. CONCLUSIONS: For type A, early surgical treatment is recommended to prevent the progress of the finger deformity and to improve motion. When the tumors are oriented more laterally and include less than a third of the joint surface, we recommend sufficient tumor excision, which can include part of the articular surface.

Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome.

A 47-year-old man, who had been diagnosed as myelodysplastic syndrome (MDS), complained of a severe cough and a high-grade fever. Chest CT disclosed scattered small nodules and ground-glass opacities with interlobular septal thickening in both lung fields and a mass lesion in the right lower lobe. Pathological findings of the ground-glass opacities and the mass lesion obtained by video-assisted thoracoscopic surgery revealed the accumulation of eosinophilic amorphous material in the alveoli and confirmed the diagnosis of pulmonary alveolar proteinosis (PAP). Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in sera were below sensitivity, while the GM-CSF level was elevated in bronchoalveolar lavage fluid. He was diagnosed as secondary PAP associated with MDS.

Peripheral primitive neuroectodermal tumor of the chest wall of a 69-year-old man.

We report a case of peripheral primitive neuroectodermal tumor (pPNET), which belongs to the pPNET/Ewing's sarcoma family, arising in the chest wall of a 69-year-old man. He had high levels of serum neuron-specific enolase and pro-gastrin-releasing peptide, which are believed to be useful diagnostic blood markers for small cell lung carcinoma (SCLC). Microscopically, the tumor was composed of solid nests and sheets of monotous, primitive, small round cells with a few rosettes, making it difficult to distinguish from SCLC. Immunohistochemically, the tumor cells showed intense cell membranous immunoreactivity for MIC2 protein (CD99). EWS/FLI-1 chimeric mRNA that originated from the characteristic t(11;22)(q24;q12) chromosomal translocation was detected by RT-PCR and nucleotide sequence analysis. These results confirmed the diagnostic validity of the present tumor being a pPNET, thus raising the possibility that in the past, pPNETs which have arisen in the chest have been mistakenly diagnosed as SCLC.

[Two younger male patients with rapidly progressing pulmonary pleomorphic carcinoma].

We report 2 cases of pulmonary pleomorphic carcinoma. The patient in case 1 was a 44-year-old man who was admitted to our hospital complaining of dry cough and dyspnea on exertion. Chest radiography and CT showed a huge tumor with left pleural effusion. Percutaneous biopsy suggested malignancy. As distant metastasis was not found and as cytological examination of pleural effusion gave a result of class II, we attempted to perform left pneumonectomy. However, we performed partial resection of the tumor because it had invaded the aorta, pulmonary artery, pericardium and pleura. The pathological diagnosis was pleomorphic carcinoma, p-T4 N2 M0. After the operation, we performed systemic chemotherapy, including cisplatin and irinotecanm with little effect (PD). The patient died of progression of the tumor. Case 2 was a 34-year-old man who was admitted to our hospital complaining of fever and general malaise. Chest radiography and CT showed a tumor in the left upper lobe. As no distant metastasis was found, we tried left upper lobectomy. However, we performed left upper segmentectomy and lymph node dissection because of invasion of the aorta and the chest wall, and because of hypersegmentation of the left upper lobe. Pathological diagnosis was pleomorphic carcinoma, p-T4 N0 M0. After the operation, the mediastinum was subjected to radiation therapy. However, a metastatic tumor was found in the pelvis after this radiation was given. We performed systemic chemotherapy with substances including cisplatin, gemcitabine and vinorelbine, but with little effect (PD). The patient died of peritonitis and pleuritis due to the recurrence and progression of the tumor. Both cases had rapidly growing neoplasms showing little sensitivity to chemotherapy or radiotherapy. Pulmonary pleomorphic carcinoma is suggested to be type of lung cancer with a poor prognosis when the tumor is not resected in the early stages.

[A case of chronic hypersensitivity pneumonia with elevation of serum SP-D and KL-6].

A 73-year-old woman suffering from dyspnea on effort and chronic cough was admitted to our hospital. Chest computed tomography disclosed ground-glass opacities, irregular linear opacities and honeycombing distributed predominantly in the subpleural area. The serum levels of SP-D and KL-6 rose to 889 ng/ml, 1,755 U/ml, respectively. These findings indicated idiopathic pulmonary fibrosis. However, the number of lymphocytes and the CD4/CD8 ratio in the BAL fluid were elevated. Transbronchial lung biopsy specimens demonstrated alveolitis with granuloma formation. The evidence that she had lived in a house with a heavy fungal growth and that tests of precipitation in response to Penicillium corylophilum were positive confirmations of a diagnosis of chronic hypersensitivity pneumonia.

Glucose levels during oral glucose tolerance tests and gamma-glutamyl transpeptidase are predictors of change from normal to impaired glucose tolerance in healthy middle-aged Japanese men.

We evaluated the predictors of the development from normal to impaired glucose tolerance (IGT) in healthy middle-aged Japanese men. Forty male subjects who showed normal glucose tolerance (NGT) levels based on WHO criteria and who had undergone 75-g OGTT annually for 10 years were selected in the database of medical checkups retrospectively, and divided into two groups: those retaining NGT and those that developed IGT. Gamma-glutamyl transpeptidase (GGT) and the glucose levels at 30 and 60 min were significantly associated with the development of IGT in the Cox proportional hazard model. However, other clinical characteristics and the glucose levels at pre-load and at 120 min were not significantly associated with the development of IGT. GGT and the glucose levels at 30 and 60 min after the 75-g glucose load were predictors of development from NGT to IGT in healthy middle-aged Japanese men.