• Journal of Chest Surgery
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• 제32권2호
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• pp.189-193
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• 1999

폐에 발생하는 간엽 낭성 과오종(mesenchymal cystic hamartoma: MCH)은 매우 드문 종양으로 1986년에 처음으로 발표되었고 아직까지 유병율은 밝혀지지 않았다. 방사선 사진과 조직 검사에서 특징적으로 양측 폐에 다발성의 결절과 크기가 다양한 낭포들이 보인다. 결절들은 미성숙 간엽 세포들의 증식으로 이루어지고 결절의 크기가 점차 커지면서 낭포를 형성하게 되는데 낭포의 내경은 정상적 또는 화성 호흡 상피 세포로 이루어지고, 그 벽의 중간층은 방추형의 간엽세포충으로 되어 있다. 주증상은 객혈과 재발되는 기흉, 그리고 혈흉이다. 비교적 양성종으로 알려져 있지만 악성 변화의 가능성이 있다. 폐기포 절제술 시 폐 전체 표면에 다양한 크기의 낭포성 병소와 결절들이 육안적으로 관찰되었고, 조직 검사상 낭포의 내경은 호흡 상피로 둘러져 있었고, 그 벽의 중간층은 원시 간엽 세포층로 이뤄져있었다. 육안적 소견과 광학현미경하 소견이 간엽 낭성 과오종에 적합한 소견이었다.. 저자들은 자주 재발되는 기흉과 객혈이 있었던 27세의 여자에서 간엽 낭성 과오종을 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제49권4호
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• pp.302-305
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• 2016

A 16-year-old girl was transferred to the department of thoracic and cardiovascular surgery because of a spontaneous pneumothorax with prolonged air leakage. Chest computed tomography demonstrated a cystic lesion measuring $2{\times}3cm$ and involving the left upper lobe. Left upper lobectomy was performed via video-assisted thoracoscopic surgery. A pathologic examination of the specimen revealed a mesenchymal cystic hamartoma. Despite the rarity of pulmonary mesenchymal cystic hamartoma, it should be considered a potential cause of pneumothorax for patients with a large pulmonary cyst. Further, surgical resection must be considered because serious complications such as hemothorax, hemoptysis, and malignant transformation have been reported.

• 대한두개안면성형외과학회지
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• 제18권4호
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• pp.292-295
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• 2017

A 17-month-old boy was evaluated for a midline mass on his chin. The mass was anchored to the mentalis muscle with a stalk-like structure. The pathological diagnosis of the mass was rhabdomyomatous mesenchymal hamartoma. This is the first report of rhabdomyomatous mesenchymal hamartoma presenting as a midline chin mass in Korean pediatric patients.

• 대한세포병리학회지
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• 제3권1호
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• pp.30-36
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• 1992

Pulmonary hamartoma is the most common benign tumor of the lung but rarely encountered in routine cytologic work-up. We recentely experienced a case of fine needle aspiration cytology of pulmonary hamartoma in a 65-year old male patient. The characteristic cytopathologic features were 1) The mesenchymal components with cuboidal epithelial cells in a clean background, 2) The presence of fibromyxoid mesenchymal component composed of fibroblasts in a loose fibrillary stroma, and 3) Antler-liker fibromyxoid cluster having the rimming of epithelial cells which correspond to the epithelial glowing in cleft-like fashion in pulmonary hamartoma.

• Advances in pediatric surgery
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• 제7권1호
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• pp.31-36
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• 2001

Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood, Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4-32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from $10{\times}8.5cm$ to $34{\times}29cm$. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion. it may be confused. and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore. complete excision of the tumor with surrounding normal liver tissue is recommended.

• Journal of Chest Surgery
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• 제23권6호
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• pp.1244-1250
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• 1990

Hamartoma had been considered as a congenital malformation of a tumerous lesion since the original description by Albrecht in 1904. Pulmonary hamartoma is able to classified as endobronchial and intrapulmonary hamartoma clinically, and is considered to benign mesenchymal neoplasms which originated in peribronchial connective tissue, and so called mesenchymoma by someone. We have experienced 3 cases intrapulmonary hamartoma which were discovered during routine chest film check up at all cases and are able to detailed histologic examination by electron microscopy at one case.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권1호
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• pp.113-119
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• 2001

소아에서 간의 간엽 과오종은 주로 복부팽만을 주소로 내원하여 대부분 2세 이내에 발견되며, 비교적 크기가 큰 낭종의 소견을 보이는 것으로 알려져 있다. 저자들은 담도 폐쇄증으로 Kasai 수술받은 8개월 여아에서 복부 초음파 검사상 작은 기질성 종괴를 우연히 발견하여 병리학적으로 확진된 간엽과오종 1례를 보고하는 바이다.

• 한국간담췌외과학회지
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• 제26권4호
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• pp.407-411
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• 2022

Mesenchymal hamartoma of the liver (MHL) is a rare benign tumor that often presents in early childhood, and it rarely occurs in adulthood. Aberrant development of the portal tract is a known cause of MHL. Although limited information is available on the natural course of MHL, malignant transformation has been reported in a few cases. Here, we report a case of a 26-year-old female with intrahepatic cholangiocarcinoma secondary to unresected MHL. The patient underwent resection of the hepatic mass, which was diagnosed as MHL at 2 years of age, due to an increase in mass size and a suspicion of malignant transformation during work-up. Histopathology confirmed intrahepatic adenosquamous carcinoma in the background of MHL, with a T2N0M0 pathological stage (stage II). The surgical margin was free from tumor cells. The patient fully recovered postoperatively and started receiving adjuvant chemotherapy. Previous case reports have only reported about the development of undifferentiated embryonal sarcoma or angiosarcoma as malignant transformation of MHL. Cases of other malignancies have not been published; however, it is difficult to rule out the occurrence of various malignancies related to the portal tract when considering the pathogenesis of the disease. To the best of our knowledge, this is the first case report of adenocarcinoma of bile duct origin secondary to MHL. This case report suggests that aggressive surgical management should be considered after the initial diagnosis of MHL.

• 대한세포병리학회지
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• 제11권1호
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• pp.31-34
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• 2000

Pulmonary hamartoma is an uncommon benign tumor consisting of a mixture of loose fibromyxoid tissue, cartilage, fat, and cleft-like spaces lined by cuboidal or ciliated epithelium. Cytologically, the presence of a mesenchymal component is essential for the diagnosis of pulmonary hamartoma. We report the fine needle aspiration cytologic findings of two cases of pulmonary hamartoma. Case 1 was a 71-year-old woman with a mass, measuring $1.8{\times}1.5cm$ in the upper lobe of the right lung. Case 2 was a 51-year-old woman with a mass, measuring $2.3{\times}2.0cm$ in the lower lobe of the right lung. Fine needle aspiration cytology of both pulmonary masses revealed several sheets of loose fibromyxoid tissue fragments with focal cartilaginous differentiation and a few clusters of bland cuboidal epithelial cells on the bloody background. The diagnosis was histologically confirmed by needle biopsy.

• Advances in pediatric surgery
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• 제13권2호
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.