• Journal of Chest Surgery
• /
• 제47권1호
• /
• pp.51-54
• /
• 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.

• 대한유전성대사질환학회지
• /
• 제15권1호
• /
• pp.25-28
• /
• 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
• /
• 제49권2호
• /
• pp.96-99
• /
• 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
• /
• 제30권5호
• /
• pp.642-646
• /
• 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
• /
• 제6권1호
• /
• pp.91-94
• /
• 2009

저자들은 출생당시 특이소견 없었고, 유전질환의 가족력이 없는 20세 남자 환자에서 우측하지에 골전이가 동반된 횡문근 육종을 발견하고, 저신장, 탈모, 백내장, 치열이상, 골다공증, 갑상선기능저하증, 작은 손과 발, 사지는 가늘고 체간이 두터우며, 신체에 비해 큰 머리등 특이한 외모를 가진 워너증후군으로 진단한 1례를 경험하였기에 문헌 고찰과 함께 보고하고자 한다.

• Radiation Oncology Journal
• /
• 제4권1호
• /
• pp.63-66
• /
• 1986

흉폐부위에서 발생하는 악성소세포종양은 1979년 Askin이 보고했고 이를 Askin종양이라고 부른다. Askin종양은 상당히 희귀하며 흉벽에 연조직이나 폐가장자리에서 잘 생기며 특히 젊은 층에 호발한다. 임상적, 병리학적으로 Ewing's sarcoma와 Rhabdomyosarcoma와 상당히 흡사하나 정밀한 전자현미경 관찰시 병리학적으로 별개에 종양으로 간주되었다. Askin종양은 국소재발이 많고 전신으로 잘 터지지 않는 경향이 있으나 생존율은 상당히 나쁜 것으로 되어 있다. 임상적으로나 병리학칙으로 Askin종양에 해당되는 24계 환자를 치료결과와 더불어 보고한다.

• 치과방사선
• /
• 제28권1호
• /
• pp.285-297
• /
• 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
• /
• 제23권2호
• /
• pp.116-120
• /
• 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
• /
• 제15권2호
• /
• pp.176-180
• /
• 2011

안와에 발생하는 전이암은 주로 안와 지방이나 뼈에 발생하며 한쪽에 국한되어 발생하고, 양쪽 외안근의 미만성비대 형태로 발생하는 경우는 매우 드물다. 저자들은 코에 생긴 횡문근육종 환자에서 치료 후 추적 관찰 중 발생한 안와의 전이암이 양쪽 외안근의 미만성 비대 형태로 발생하여 갑상선 안병증으로 오인되었던 1예를 보고하고 갑상선 안병증과 감별하는데 도움을 받을 수 있는 소견이 무엇인지에 대해 분석해 보고자 한다.

• 대한골관절종양학회지
• /
• 제1권1호
• /
• pp.77-83
• /
• 1995

1. 연부 조직과 골 조직사이에서의 악성 섬유성 조직구종의 근본적인 조직학적 차이는 없었다. 2. 악성 섬유성 조직구종은 조직학적 소견이 다양하므로, 확진을 위해서는 다른 종양을 반드시 배제하여야한다. 3. 재발시 다른 형태의 조직 소견을 보여줄 수 있기 때문에 정확한 진단을 위해 환자의 과거력 및 과 거의 조직학적 검토가 필수적이라 사료된다.