• Asian Pacific Journal of Cancer Prevention
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• 제17권3호
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• pp.1449-1452
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• 2016

Background: Adult rhabdomyosarcoma (RMS) is a rare and aggressive disease with limited data compared with pediatric RMS. The goal of this study was to determine the treatment outcome and identify factors related to survival outcome in Thailand. Materials and Methods: Adult patients (${\geq}15$ years old) with the pathological diagnosis of RMS between 1985 and 2010 were reviewed. The data were retrospectively reviewed from the pathological results and medical records from Srinagarind Hospital, Khon Kaen University. Results: A total of 34 patients were identified in the study. The median age at diagnosis was 35.5 years and the most common primary sites were extremity and the head and neck region. The incidence of pleomorphic RMS increased with age and none was found in those aged younger than 20 years old. The median survival time was 9.33 months (95%CI: 5.6-13.1). The 1- and 5- year survival rates were 38.2% (22.3-54.0) and 20.6% (9.1-35.3). On multivariate analysis, age and size of tumor did not predict better outcome while chemotherapy and surgery were significantly associated with longer survival. Conclusions: Outcome of adult RMS was poor. Surgery and chemotherapy are strongly associated with better prognosis and multimodality treatment should be incorporated in the clinic.

• Asian Pacific Journal of Cancer Prevention
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• 제15권4호
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• pp.1771-1774
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• 2014

The rhabdomyosarcoma (RMS) is the most common type of soft tissue tumor in children and adolescents; yet only a few screens for oncogenic mutations have been conducted for RMS. To identify novel mutations and potential therapeutic targets, we conducted a high-throughput Sequenom mass spectrometry-based analysis of 238 known mutations in 19 oncogenes in 17 primary formalin-fixed paraffin-embedded RMS tissue samples and two RMS cell lines. Mutations were detected in 31.6% (6 of 19) of the RMS specimens. Specifically, mutations in the NRAS gene were found in 27.3% (3 of 11) of embryonal RMS cases, while mutations in NRAS, HRAS, and PIK3CA genes were identified in 37.5% (3 of 8) of alveolar RMS (ARMS) cases; moreover, PIK3CA mutations were found in 25% (2 of 8) of ARMS specimens. The results demonstrate that tumor profiling in archival tissue samples is a useful tool for identifying diagnostic markers and potential therapeutic targets and suggests that these HRAS/ PIK3CA mutations play a critical role in the genesis of RMS.

• Journal of Yeungnam Medical Science
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• 제28권1호
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• pp.70-76
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• 2011

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal (ERMS), alveolar (ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC (vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

• 대한수의학회지
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• 제49권4호
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• pp.361-363
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• 2009

Rhabdoid tumor is an aggressive neoplasm of animals and human. It is similar with rhabdomyosarcoma histopathologically. But cellular origin of this neoplasm showed no striated muscle origin by immunohistological and ultrastructural studies. Castrated male Shit-tzu dog, 6 years old, had a mass in the left gluteal region near to the tail. The mass was examined histopathologically and immunohistologically. Histopathologically, the tumor was consisted of large polygonal cell with abundant eosinophilic cytoplasm. The nuclei in some cells were marked eccentrically located. Immunohistochemically, many neoplastic cells were positive for vimentin. These findings were similar to histopathological and immunohistological features of human rhabdoid tumor and few rhabdoid tumors in animals.

• Journal of Chest Surgery
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• 제22권1호
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• pp.116-122
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• 1989

During 22-year period ending in June 1988, operation was performed on 22 patients with primary heart tumor at Yonsei University College of Medicine. Mean age was 38.8*3.03[mean \ulcornerEM] ranging from 14 to 63 years old. Twenty cases were myxomas. Others were fibromyxoma and rhabdomyosarcoma. All patients complained of dyspnea on exertion. Nine cases had palpitation. Other constitutional symptoms were weight loss [7 cases], headache [4 cases], generalized edema [3 cases] and cough [3 cases]. Five cases had neurological symptoms and signs preoperatively. Preoperative NYHA Class was poor [Class II; 8 cases, III; 9 cases and IV; 5 cases]. The most common site of tumor origin was fossa ovalis limbus [16 cases; 72.8%]. Because of severe mitral regurgitation and of recurrent myxoma, 2 cases were reoperated for mitral valve replacement. Postoperative complications were postoperative mitral regurgitation [5 cases; 22.8%] and arrhythmia [4 cases; 18.2%], one of which was supra-His bundle block. All patient survived operation. Follow-up of 15 patients [mean 28.95*8.3 months] was good as functional class 1[8 cases] or II [7 cases]. More effective adjuvant therapy will be necessary to improve long-term prognosis for malignant primary heart tumor.

• Journal of Chest Surgery
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• 제25권12호
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• pp.1422-1427
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• 1992

Askin tumor is rare malignant small round cell tumor that orgins from interconstal nerve of chest in children It was not until 1979 that Askin first reported that tumor. Although few sporadic reports had been reported, its incidence were too low to analize its clinical featurs. That tumors prognosis is so grave that no therapy would success to cure, but early diagnosis and enbloc excision with following combind chemotherapy and radiotherapy will prolong their survival. Other small round cell tumors of chest wall that must differentiate are Ewing`s sarcoma, rhabdomyosarcoma, lymphoma, neuroblastoma and pulmonary bla-stoma. The most prominant histologic charactersistics of this tumor is neuron specific eno-lase which is detected with immunohistochemistry technique, and neurosecretary electron dense granules within cytoplasm. We expirienced a case of Askin tumor occuring 12-year-old female who has huge right lower chest mass with dull chest pain. She have been underwent excision and postoperative radiotherapy. We are following her up for months and there is no evidence of local recurrence.

• 한국임상수의학회:학술대회논문집
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• 한국임상수의학회 2007년도 추계국제학술대회 및 컨퍼런스
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• pp.568-568
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• 2007

• Journal of Chest Surgery
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• 제8권1호
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• pp.29-36
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• 1975

We have experienced 49 cases of tumors of chest wall at St. Mary`s Hospital from Jan. 1963 to Dec.1974. In four cases of them, the reconstruction of chest wall defects performed. 1] Out of 49 cases of tumors of the chest wall, 27 cases were benign tumors, 14 cases metastatic malignant tumors, and 8 cases primary malignant tumors. 2] Twenty-six cases [50%] of tumors of the chest wall were on the bony cage. Among them benign tumors were 9 cases [35%], metastatic malignant tumors 14 cases [53%], and primary malignant tumors 3 cases [12%]. Of these, 24 cases were located on the ribs and 2 cases on the sternum.3] The malignant tumors of bony chest wall were excised in en bloc resection including involved ribs. The wide defects of bony chest wall were reconstructed by means of displacement of neighboring ribs and mobilized diaphragm, in the two osteogenic sarcomas of rib, and of prosthesis with silastic sheets in one rhabdomyosarcoma and one metastatic adenocarcinoma of lung.

• 대한세포병리학회지
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• 제7권2호
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• pp.218-224
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• 1996

We describe two cases of metastatic Wilms' tumor in the lung with emphasis on the cytologic features of specimens obtained by needle aspiration. One of them was extrarenal Wilms' tumor. The findings were correlated with the histopathologic features of the primary lesion. Cellular components in the fine needle aspiration cytology (FNAC) slides included blastemal, epithelial, stromal and inflammatory cells with immature tubular differentiation and rosette formation. Recognition of these cellular components in FNAC smears help in establishing FNAC diagnosis of Wilms' tumor. The blastemal cells were represented by small to medium sized cells with scanty cytoplasm having ill-defined borders and round to slightly oval nuclei with evenly dispersed chromatin and small marginated nucleoli. They were seen in our two cases. The differential diagnosis includes neuroblastoma, malignant lymphoma, malignant rhabdoid tumor, clear cell sarcoma, Ewing's sarcoma and embryonal rhabdomyosarcoma. In conclusion, making a definite cytologic diagnosis of metastatic Wilms' tumor may be possible by light and electron microscopy and immunohistochemical staining. The above findings may contribute to the diagnosis of FNAC of metastatic Wilms' tumor.

• Clinical and Experimental Pediatrics
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• 제61권1호
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• pp.30-34
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• 2018

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 ($GGT{\rightarrow}GAT$), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.