• Journal of Chest Surgery
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• v.47 no.1
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• pp.51-54
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• 2014

Primary tumors of the lung are uncommon in pediatric patients, particularly bronchioloalveolar carcinoma (BAC). An 11-year-old female suffering from back pain for 1 month was referred to Seoul St. Mary's Hospital for treatment of a pathologic fracture of the lumbar spine. Comprehensive evaluation disclosed numerous pulmonary metastases of rhabdomyosarcoma (stage IV). During chemotherapy, most of the lung lesions regressed, with the exception of two nodules. Wedge resections, intended for diagnosis and cure, yielded a histologic diagnosis of BAC.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.25-28
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• 2015

Mitochondrial disorders are rare metabolic diseases. They often present during neonatal period but with nonspecific clinical features such as feeding difficulties, failure to thrive, and seizures. Mitochondrial defects have also known to be associated with neurological disorders, as well as cancers. We report the first case of neonatal mitochondrial respiratory chain defect with sarcoma botryoides confirmed by pathologic diagnosis, suggesting another possible link between mitochondrial dysfunction and cancer.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.49 no.2
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• pp.96-99
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• 2023

Oral spindle cell/sclerosing rhabdomyosarcoma (SCRMS) with anaplastic lymphoma kinase (ALK) expression is extremely rare, and its diagnosis is very challenging in the absence of clinical or pathological indicators. This case presented with gingival swelling and alveolar bone resorption and was suspected clinically to be periodontitis. A biopsy was performed and, due to immunoreactivity with ALK, the patient was misdiagnosed with inflammatory myofibroblastic tumor. However, based on the combined histological and immunohistochemical features, a revised diagnosis of SCRMS with ALK expression was finally concluded. We believe that this report makes a significant contribution to the precise diagnosis of this rare disease for proper treatment.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.642-646
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• 2001

A 12-years-old female admitted to the hospital with the complaint of pain on the right upper chest area which persisted about 1 month prior to admission. $Caf{\acute{e}}$-au-lait spots of various size laying on a whole body and freckling on the axilla were found on physical examination. A huge mass was found on the plain chest X-ray and on chest MRI. The mass encroached thoracic spine, posterior rib, back muscles, and then into the neural canal and compressed thoracic spinal cord. On the 5th day of hospitalization, the patient complained tingling on the both legs and 2 days later, monoparesis on the right leg. Open thoracotomy and decompressive laminectomy was done to remove mass. Pathologic reports confirmed rhabdomyosarcoma, embryonal type.

• Journal of Genetic Medicine
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• v.6 no.1
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• pp.91-94
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• 2009

Werner syndrome (WRN), or adult progeria, is a very rare, autosomal recessive disorder characterized by the appearance of accelerated aging, including cataracts, gray hair, skin atrophy, and atherosclerosis. This syndrome is caused by mutations in the WRN gene and had a high risk of a spectrum of rare neoplasms including: i) non-epithelial malignant or pre-malignant tumors/conditions, osteosarcomas and soft tissue sarcomas, malignant melanomas, myeloid leukemia and myelodysplastic syndrome; ii) an epithelial neoplasm, thyroid carcinoma, and iii) meningiomas. Recently, authors experienced a case of Werner syndrome complicated by bone metastasis of rhabdomyosarcoma in a 20-year old Korean man. The patient revealed a painful mass on his right knee and progeroid features, short stature, scalp alopecia, abnormal dentition, craniofacial disproportion, hypothyroidsm, cataracts and osteoporosis. The onset of symptoms of Werner syndrome generally precedes any later symptoms of associated conditions, such as malignant tumor. Therefore, early recognition of Werner syndrome is important to assist identification of malignant tumors at an early stage in this patient group.

• Radiation Oncology Journal
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• v.4 no.1
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• pp.63-66
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• 1986

The malignant small round cell tumor of the thoracopulmonary region was described by Askin in 1979 and called as Askin Tumor. The Askin tumor is a rare, arises from the soft tissues of the chest wall or peripheral lung which is predominantly in younger generation. Clinical and pathologic appearance were very similar to Ewing's sarcoma and rhabdomyosarcoma but when examined it by electron microscopy, there was some different in morphology. The tumor tended to recur locally and did not seem to disseminated widely but the median survival was only 8 months.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.28 no.1
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• pp.285-297
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• 1998

This report details a case of 8-year-old girl showing failure of odontogenesis after chemo-radiation therapy for the rhabdomyosarcoma at the age of 4. The observed results were as follows : 1. Past history revealed that she had received for a total radiation dose of 4430cGy, 29 fractions in 6 weeks and chemotherapy with vincristine, actinomycin D and cytoxan, followed as maintenance phase for 2 years. 2. The patient was symptom -free and appointed for the treatment of multiple dental caries. 3. Oral examination showed hypoplastic enamel on whole erupted permanent teeth and showed retarded eruption. 4. Conventional radiograms showed failure of root development including abrupt cessation of root formation and root agenesis, and microdontia, missing teeth, irregular enamel, dislocation of the impacted teeth. Additional finding showed good healing bone pattern on the left mandibular ramus and angle area. 5. Cephalometric analysis revealed failure of bite raising due to incomplete eruption of all the first molars and made it possible to suspect entrapped mandibular growth and then Class II tendency growth. 6. There was correlation between the time of chemo-radiation therapy and the damage of the teeth.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.116-120
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• 2019

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3-7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function.

• Investigative Magnetic Resonance Imaging
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• v.15 no.2
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• pp.176-180
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• 2011

Metastases to the orbit usually affect the intraorbital fat and bone than the extraocular muscles. Metastasis to the extracoular muscles commonly occurs unilaterally, and diffuse enlargement of the bilateral extraocular muscles due to metastasis is extremely rare. In this report, we will describe a case of diffuse metastasis to the bilateral extraocular muscles from nasal rhabdomyosarcoma masquerading as thyroid associated orbitopathy. We will also discuss about the MR imaging findings helpful for differential diagnosis from thyroid associated orbitopathy.

• The Journal of the Korean bone and joint tumor society
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• v.1 no.1
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• pp.77-83
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• 1995

Malignant fibrous histiocytoma was classified as fibrosarcoma, liposarcoma, rhabdomyosarcoma in times past and it was first introduced in 1963 to refer to a group of soft tissue tumors and reported in bone in 1972. It was postulated that the origin of tumor cell was derived from histiocytes that could assume the appearance and function of fibroblasts("facultative fibroblasts") at first. But, recently the immunohistochemical studies suggested origin from a primitive mesenchymal cell. The malignant fibrous histiocytoma were commonly misdiagnosed as other tumors such as myxoid liposarcoma, pleomorphic rhabdomyosarcoma, osteogenic sarcoma, leiomyosarcoma, malignant schwannoma, giant cell tumor, hemangiopericytoma, dermatofibrosarcoma protuberans due to the broad morphologic spectrum. The seventeen cases of the malignant fibrous histiocytomas diagnosed at Hanyang University Hospital since 1979 were reviwed, and the suggestions are as follows : 1. No fundamental histologic differences between the MFHs of soft tissues and bones. 2. The other tumors must be excluded in order to diagnose MFH due to wide histologic variety of MFH. 3. The review of the initial tissue slide was necessary for adequate diagnosis because the recurrent MFHs showed different histologic findings from original tumors.