• Journal of Chest Surgery
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• v.25 no.6
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• pp.598-604
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• 1992

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and acounts for 6% to 15% of all cases of childhood cancer, Rhabdomyosarcoma in seventh most common form of childhood neoplasms, following acute leukemia, tumors of the central nervous system, lymphoma neuroblastoma, Wilm`s tumor, bone tumor. Rhabdomyosarcoma can arise anywhere in the body, but primary site in the thorax is relatively rare. We experienced a case of aveolar rhabdomyosarcoma arising from intercostal muscle, A 12 year-old woman was suffered from the intermittent left chest pain radiating to the scapular area and dyspnea, On physical examination, pulmonary friction rub was heard on the left upper lobe area. Qn adimission, the chest simple radiography revealed a 7 x 6, 5cm sized radio-opaque mass with pleural effusion in the superior mediastinum and the CT showed a well difined radio-opaque mass including the destructed 2nd rib and pleural effusion. The percutaneous tra-nsthoracic needle aspiration biopsy was likely to show blastoma. After the chemotherapy[vincristine, actinomycin-D, cyclophosphamde] was done to treat blastoma, the pleural effussion was subsided and the mass was slightly decreased by 4.5x 4. 5cm. For treatment and diagnosis, we performed en-bloc resection and the defected chest was reconstucted with Gortex patch. Grossly, the specimen was colored graysh-white and arised in between two ribs The microscopic findings showed that the tumor cells were small round with scant pinkish cytoplasm on the H-E stain and the tumor cell nests were grouped by reticulum fibers and showed alveolar pattern on the silver stain The electromicroscopic finding presented that the cytoplasm contained tangled fibrillar and flocculent materials. The histopathologic findings were compatable with laveolar rhabdomyosarcoma. She was discharged without any complication. After discharge, she has been treated with radiation theraphy and chemotheraphy, and not recurred untill last follow-up We report a case of alveolar rhabdomyosarcoma arising to intercostal muscle, developed in 12 year-old waman, with brief review of literatures.

• Archives of Plastic Surgery
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• v.36 no.4
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• pp.507-511
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• 2009

Purpose: Adenoid cystic carcinoma (ACC) is a rare malignant epithelial neoplasm derived from the salivary glands. In some cases, ACC may arise in other primary sites, such as skin. We report a case of adenoid cystic carcinoma arising the scalp skin of 69 - year - old woman. Methods: A 69 - year - old woman presented with a tender scalp nodule. A local wide excision was performed. Histopathologic examination was revealed the adenoid cystic carcinoma with basaloid cells in a cribriform pattern. The resection margins were free of tumor. Two years later a tumor recurred in the scarred area. The lesion was removed surgically and the histopathological diagnosis of adenoid cystic carcinoma was again established. After two years, tumor recurred again and diatant metastasis of the lung was diagnosed. A surgical wide excision was done and the close regular follow - up for recurrence was done. Two years later, third recurrence of the scalp was observed. We also performed the wide local excision with tumor free margin. Results: We experience the recurrent adenoid cystic carcinoma of the scalp with pulmonary metastasis. We have performed the wide local excision for three times. The patient has been followed up for 10 years with regular work - up for recurrence and metastasis Conclusion: primary cutaneous adenoid cystic carcinoma is a rare skin neoplasm with a high potential for recurrence after local excision. The standard treatment of ACC is wide local excision with tumor - free margins established by permanent section.

• Journal of Chest Surgery
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• v.24 no.1
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• pp.72-82
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• 1991

From May 1978 to Sep. 1990, 106 patients who had been diagnosed as primary lung cancer and operated on at the Department of Thoracic & Cardiovascular Surgery, Han Yang University, were clinically evaluated. 1. The peak incidence of age was 5th decade of life[37.7%] and 6th decade[29.2%]. Male to female ratio was 3.8: l. 2. Most of symptoms were respiratory, which were cough, chest pain, hemoptysis, and asymptomatic cases were 2.9%. 3. Histopathologic classifications were squamous cell carcinoma[53.7%], adenocarcinoma [23.8%], bronchioloalveolar cell carcinoma[6.6%], undifferentiated large cell carcinoma[6.6%], small cell carcinoma[3.8%], adenosquamous carcinoma[3.8%] and others[1.8%]. 4. Methods of operation were pneumonectomy 49.1%[52cases], lobectomy 21%[22cases] bilobectomy[6cases], lobectomy with wedge resection[3cases], exploration 21.9%[23cases], and resectability was 78.3%. 5. Staging classifications were Stage I [22.6%], Stage II [11.3%], Stage IIIa[42.6%], Stage IIIb[21.7%] and Stage lV[1.6%]. Resectability by Stage; Stage I was 100%, II 100%, IIIa 84.4% and IIIb 30.4%. 6. Causes of most of inoperable cases were invasion of mediastinal structures and diffuse chest wall, and others were contralateral lymph node invasion and malignant pleural effusion. 7. Operative mortality was 6.7% which caused by arrhythmia, sepsis, pulmonary edema, and radiation pneumonitis. 8. On the long term follow up of the resectable cases, overall 1 year survival rate was 58.5 %, 2 year 39%, and 5 year 19.5%. Five year survival rate was 40% in Stage I, 25% in Stage II and 11.7% in Stage Illa. As for the method of operation, the higher 5 year survival rate was observed in lobectomies[33.3%] than in pneumonectomies[10.3%].

• Journal of Genetic Medicine
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• v.13 no.1
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• pp.41-45
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• 2016

Marfan syndrome (MFS) is an inherited connective tissue disorder with a mutation in the fibrillin-1 (FBN1) gene. Fibrillin is a major building block of microfibrils, which constitute the structural component of the connective tissues. A 10-year-old girl visited our hospital with the chief complaint of precocious puberty. According to her medical history, she had a pulmonary wedge resection for a pneumothorax at 9 years of age. There was no family history of MFS. Mid parental height was 161.5 cm. The patient's height was 162 cm (>97th percentile), and her weight was 40 kg (75th-90th percentile). At the time of initial presentation, her bone age was approximately 11 years. From the ophthalmologic examination, there were no abnormal findings except myopia. There was no wrist sign. At the age of 14 years, she revisited the hospital with the chief complaint of scoliosis. Her height and weight were 170 cm and 50 kg, respectively, and she had arachnodactyly and wrist sign. We performed an echocardiograph and a test for the FBN1 gene mutation with direct sequencing of 65 coding exons, suspecting MFS. There were no cardiac abnormalities including mitral valve prolapse. A cytosine residue deletion in exon 7 (c.660delC) was detected. This is a novel mutation causing a frameshift in protein synthesis and predicted to create a premature stop codon. We report the case of a patient with MFS with a novel FBN1 gene missense mutation and a history of pneumothorax at a young age without cardiac abnormalities during her teenage years.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.325-331
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• 2002

Postpneumonectomy syndrome is a rare complication that usually occurs in younger patients within the first year after a right total lung resection. Its clinical presentations are stridor, dyspnea, and recurrent pulmonary infections. An airway obstruction secondary to the extreme mediastinal shift and ratation after a pneumonectomy is the main mechanism. It is commonly complicated with tracheobronchomalacia due to longstanding airway compression. The management modalities involve a repositioning of the mediastinum with volume expansion of the pneumonectomy site by a expandable prosthesis. however, other methods including an endobronchial stent insertion should be considered in the presence of a tracheobronchomalacia or in poor surgical candidates. Here we describe a case of postpneumonectomy syndrome complicated by a bronchomalacia, which was successfully treated with a self-expandable endobronchal stent.

• Tuberculosis and Respiratory Diseases
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• v.63 no.6
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• pp.521-525
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• 2007

Positron emission tomography/computed tomography (PET/CT) is valuable for the diagnosis of malignancies. However, PET/CT is unable to discriminate exactly between inflammation and a neoplasm. We report a case of a 50-year-old man with pulmonary paragonimiasis that was suspicious for lung cancer, as detected by PET/CT. The use of PET/CT revealed multilobulated consolidation on the right lung and patchy consolidation on the left lung, with increased fluorodeoxyglucose (FDG) uptake. In addition, the left paraaortic lymph node (LN) and peripancreatic LN showed enlargement with increased FDG uptake. Lung cancer with multiple lymph node metastases was suspected from the increased standardized uptake values (SUV>4.5) determined by PET/CT. We performed wedge resection via video-assisted thoracic surgery (VATS) and found Paragonimus westermani eggs in the involved tissues.

• Korean Journal of Bronchoesophagology
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• v.5 no.1
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• pp.78-84
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• 1999

Broncholithiasis is defined as a condition in which calcified material is present within the bronchial lumen. It is a rare but troublesome disease that can cause life-threatening complications such as massive fatal hemoptysis. Therefore, pulmonary resection is frequently required to remove the broncholiths and irreversibly damaged parenchyma. We experienced 3 cases of broncholithiasis. In one case, a 36 year old female patient suffered from coughing, massive hemoptysis with lithoptysis caused by intrinsic obstructive broncholiths in the right middle and lower lobe. In the 2nd case, a 41 year old male patient complained of long-standing blood tinged sputum and frequent pneumonic symptoms for 10 months because of extrinsic broncholithiasis where the calcified peribronchial lymph node eroded into the bronchial lumen of the right lower lobe. The remaining case involved a 30 year old female patient who complained of intermittent blood-tinged sputum induced by intrabronchial broncholith in the orifice of the right middle lobe bronchus. Two patients underwent bilobectomy(right middle and lower lobe) for removal of the broncholiths, damaged bronchi and parenchyma. The other patient was treated with right middle lobectomy and stone removal by bronchotomy of bronchus intermedius. In all patients, the post-operative course was uneventful.

• Tuberculosis and Respiratory Diseases
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• v.41 no.6
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• pp.658-662
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• 1994

The leiomyosarcoma is uncommon tumor that consists of 10% of all sarcoma incidence and commonly arise from retroperitoneum or mesentery. But leiomyosarcoma arose from mediastinum is very rare and only incidental case report is present. Mediastinal leiomyosarcoma may originate from superior vena cava, pulmonary artery, small vessels of alveoli, esophagus and cardiac muscle. Common symptoms that are related with leiomyosarcoma of mediastinum are cough and dyspnea but dysphagia, chest pain and hemoptysis can be produced. Although long term survival after complete resection of tumor was reported in localized disease, there was no effective therapy that prolong the survival in patients who had disseminated disease or huge tumor mass. We report the case of posterior mediastinal leiomyosarcoma confirmed by aspiration cytology and immunohistochemical staining, along with a review of literature.

• Tuberculosis and Respiratory Diseases
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• v.48 no.1
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• pp.91-95
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• 2000

Mucous gland adenoma of the bronchus is a rare benign tumor arising from the bronchial mucous gland. It accounts for less than 0.5% of all lung tumors. In adults, tracheal tumors are most often malignant. Among benign tumors arising in the trachea, mucous gland adenoma of the trachea is extremely rare. First case was reported by Ferguson and Cleeland in 1988, as "Mucous gland adenoma of the trachea". Microscopic study shows it to arise from normal submucosal mucous glands forming glandular or tubular structures composed of mucous secreting cells. Common symptoms were cough, hemoptysis, recurrent and protracted pneumonia, shortness of breath, and wheeze. Duration of symptoms before diagnosis varied from a few weeks to 10 years with prolonged symptoms being usual. Management of these tumors should be complete excision, including pulmonary resection because two instances of recurrence after local excision have been reported.

• Journal of Chest Surgery
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• v.39 no.3 s.260
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• pp.244-247
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• 2006

Granular cell tumors (GCT) are uncommon benign neoplasms. Their location is mostly in the the skin, tongue, and breast; appearance in other parts of the body is rare, but it has been reported. They have also been reported to occur synchronously in multiple organs and metachronously in a single organ. The incidence of GCTs in the tracheobronchial tree is unknown and pulmonary GCTs are uncommon, with approximately 100 reported cases in the literature. We present the case of a 33-year-old man with a granular cell tumor of the left main bronchus. The tumor was found at bronchoscopy performed to exclude suspected endobronchial mass with symptoms of pneumonia. Biopsies revealed the histological pattern of a benign granular cell tumor. He underwent resection of the left main bronchus followed by end to end anastomosis of left main bronchus. He has not had any recurrence of the tumor during the 1 year follow-up.