두개 기저부에 발생한 척삭종의 MR영상 소견

김대홍,이호규 凡石學術奬學財團 1999 凡石學術論文集 Vol.3 No.-

두개강내 척색종에 대한 임상적 고찰 및 치료성적 : Clinical Review and Therapeutic Outcome

김충현,하영일,송관영,한윤경 대한신경외과학회 1995 Journal of Korean neurosurgical society Vol.24 No.7

Chordomas are uncommon tumors arising from the embryonic remnants of the fetal noto-chord. The spheno-occipital region accounts for 36 percent of chordomas. The authors are presenting four cases of intracranial chordomas with clinical manifestation was analyzed. All patients were men and their mean age was 37 years old. The presenting complaints were diplopia in 2 patients and decreased visual acuity in the other two. Radiologic studies demonstrated extensive bony destruction of the skull base and soft mass with punctate calcification in all 4 cases. Treatment consisted of subtotal removal of tumor and subsequent radiotherapy). Histo-pathologically. three of the four patients had typical chordomas and the remaining case was confirmed as chondroid chordoma. The three typical chordomas recurred after initial treatment. 2 of these three were reoperated but in the 10 months and 33 months, respectively after initial diagnosis. The patient with chondroid chordoma remained well for 46 months after initial treatment without significant neurological deficits. In conclusion. therapeutic outcome of intracranial chordomas are grim because of their aggressive that frequent recurrence and inability to remove tumors completely. With the exception. chondroid chordoma has good prognosis and long term survival.

Two Cases of Dedifferentiated Chordoma in the Sacrum

Seon Chun Kim,Wonik Cho,Ung-Kyu Chang,Sang Min Youn 대한척추신경외과학회 2015 Neurospine Vol.12 No.3

Dedifferentiated chordoma (DC) is defined as a chordoma containing sarcoma components. DC is distinguished from conventional chordoma by the rapidity of tumor growth and the potential for distant metastasis. We report two cases of DC, which are developed in the sacrum. We reviewed the medical records and imaging studies of 2 patients diagnosed with DC and the literature published. In the first case, percutaneous biopsy revealed that it was conventional chordoma in the sacrum. Patient underwent radiation therapy (RT). Six years after the RT, the tumor recurred. Surgical removal was performed and the recurrent tumor was diagnosed as DC in histopathologic examination. In the second case, a patient underwent gross total resection of sacral tumor, which was diagnosed with conventional chordoma. Aggravated tumor was detected after 4 months, and patient underwent reoperation. The second operation revealed the transformation of the tumor into DC. The survival time of the patients after the diagnosis was 10 and 31 months. Dedifferentiated chordoma is a rare and highly aggressive tumor. De novo type exists, but it usually transformed from recurrent chordoma after surgical resection or radiation.

Chordomas: Histopathological Study in View of Anatomical Location

차윤진,서연림 대한의학회 2019 Journal of Korean medical science Vol.34 No.13

Background: Chordomas are aggressive bone tumors that have a predilection for the axial skeleton including the skull base and spinal/sacral bones. However, the histopathological and clinical differences between skull base chordoma (SBC) and sacral/spinal chordoma (SC) are unclear as previous studies have been focused on patient prognosis and treatment outcome. This study aimed to evaluate the clinicopathologic features and prognosis of chordoma according to its location. Methods: Patients with chordomas were enrolled, and the histopathologic features were compared according to the tumor location. Results: A total of 52 patients were enrolled. SBCs had more abundant chondroid matrix and diffuse growth pattern, while SCs had non-chondroid, myxoid matrix and a lobulating pattern, typical of chordoma. Old age and residual tumors were risk factors for shorter overall survival in SBCs. The chondroid matrix was an independent risk factor for shorter disease- free survival in the overall population. Conclusion: Chordomas have different histopathologic features depending on the anatomical location.

원발성 비인두 척삭종 1예

김용대,우현재,이형중,윤석근 대한이비인후과학회 2002 대한이비인후과학회지 두경부외과학 Vol.45 No.5

Chordomas arise mainly in the sacrococygeal region, and to a lesser extent, in the spheno-occipital region. When occurring in the later region, they may protrude into nasopharynx and rarely into the sinonasal tract, mainly sphenoid sinus. Histolo-often far advanced at the time of presentation. The current treatment is applying surgical excision with/without postoperative radiation. Since its first report in 1987, there have a several reports of primary nasopharyngeal chordoma throughout the world. In Korea, one case of sphenoidal chordoma was reported in 193, but there had been no report of primary nasopha-ryngeal chordoma. We report a recent case of primary nasopharyngeal chordoma we have treated by transnasal endoscopic surgical excision and present a review of the literature. (Korean J Otolaryngol 2002;45:515-8)

두개 기저부 척삭종의 세포유전학 및 분자생물학의 최신 지견

안정용(Jung Yong Ahn),심유식(Yu Shik Shim),조준형(Jun Hyung Cho),장종희(Jong Hee Chang),이규성(Kyu Sung Lee) 대한두개저학회 2008 대한두개저학회지 Vol.3 No.1

Chordomas are rare, low-to-intermediate grade malignant tumors arising from notochordal remnants in the midline skeletal axis. They account for <1% of central nervous system tumors and <5% of all primary malignant bone tumors. It is characterized by slow growth, local recurrence, and low metastasis rates. An increasing variety of techniques is now available to detect genetic alterations in chordomas, herein, we review the current knowledge of the genetic alterations in the skull base chordomas. The distribution of copy number changes is composed by two approaches; the low-resolution banding karyotyping and high-resolution whole genome CGH approach. The mapping of candidate genes in chordoma genesis awaits the application of high resolution targeted approaches. Chromosome 1p36.13 and 7q33 represent a candidate region for a chordoma gene. In gene expression study, many genes, such as HER2/neu, epidermal growth factor receptor, c-Met, platelet-derived growth factor receptor A and B, KIT receptors, E-cadherin, neural cell adhesion molecule, progesterone receptor B, estrogen receptor alpha, transforming growth factor alpha and basic fibroblast growth factor, fibronectin, and Cathepsin K, are differentially expressed and act a potential therapeutic target.

Spinal Cerebrospinal Fluid Seeding of a Clival Chordoma: A Case Report

백승환,유인규,김승민,박기석,손현진 대한영상의학회 2015 대한영상의학회지 Vol.73 No.1

Chordomas originate from remnants of the embryonic notochord and account for < 2% of all malignant bone tumors. Chordomas have a high rate of local recurrence. However, spinal cerebrospinal fluid (CSF) seeding of a chordoma is extremely rare. Here, we present a very rare case of clival chordoma with spinal seeding. Radiologists should consider spinal CSF seeding of a clival chordoma, particularly when accompanied by signs of dural perforation or caudal extension.

식도 선암종 감별을 요하는 비전형적인 위치에 발생된 흉부 척색종 - 증례 보고 -

Sam Supreeth MBBS,조창희,김상일,김영훈 대한척추외과학회 2020 대한척추외과학회지 Vol.27 No.3

연구 계획: 증례 보고목적: 52세 여성 환자에서 흉추에 발생한 식도 선암종 감별을 요하는 척색종을 보고하고자 한다. 선행 연구문헌의 요약: 원시 척삭 기원의 희귀 악성 종양인 척색종은 모든 골 악성 종양의 1-4%를 차지한다. 특히 후종격동에서 식도 선암종과 감별을요하는 척색종은 매우 특이한 사례이다. 대상 및 방법: 특이 병력 없는 52세 무증상 여성으로 후방 종격동 종괴가 우연히 발견되어 본원으로 내원하였다. 건강 검진 상 식도 위장관 내시경 검사에서 발견되어 매년 정기적으로 경과 관찰을 시행하였다. 경과 관찰 2년차에 크기 증가로 본원으로 의뢰되었다. 본 논문은 본원 IRB 승인(KC20ZASI0214) 을 받은 이후 진행되었다. 결과: 증가된 종괴에 대해 절제술이 시행되었고, 병리학 소견상 척색종으로 확인되었다. 수술 후 8개월차 CT 및 1년차 MRI 상 국소 재발은 없었다. 결론: 흉부 후종격동 척색종은 매우 드물다. 특히 면역 조직학적으로 식도 선암종을 흉내 내기 때문에 진단하기 까다롭고 진단을 놓치기 쉽다. 해부학적위치와 국소 침습적인 특성으로 인해 최적의 치료를 하기 위해서는 다학제적인 접근 방식이 필요하다. 약칭 제목: 비정형 위치 발생한 흉부 척색종 Study Design: Case report. Objectives: To report a case of chordoma mimicking adenocarcinoma in a 52-year-old woman. Summary of Literature Review: Chordoma, a rare malignant tumor of primitive notochordal origin, accounts for 1-4% of all bone malignancies. Chordoma mimicking esophageal adenocarcinoma in the posterior mediastinum is unique. Materials and Methods: A 52-year-old asymptomatic woman was referred to our center with an incidentally detected posterior mediastinal mass and no significant medical history. The mass was diagnosed on a routine medical screening by esophagogastroduodenoscopy and the patient was receiving yearly follow-up. An increase in size was noticed during the second year of follow-up on endoscopic ultrasonography and the patient was referred to the Department of Gastrointestinal Surgery of our hospital. This paper was written after receiving institutional review board (IRB) approval (KC20ZASI0214). Results: En bloc resection was performed for the growing thoracic mass and pathologic findings confirmed it to be chordoma. A followup computed tomography examination was conducted at 8 months, as well as magnetic resonance imaging at 1 year, and there was no local recurrence. Conclusions: Posterior mediastinal chordoma at the thoracic level is very rare. Furthermore, it is difficult to diagnose because its immunohistochemical characteristics mimic those of esophageal adenocarcinoma, meaning that it can be missed. Management is a challenge due to its anatomical location and additional local invasion. Therefore, a multidisciplinary approach is needed, including careful consultation with the patient to provide the optimal treatment for the best possible outcome.

천추부에 발생한 거대 척색종 치험 1례

서범신,나영천,박종태,Suh, Bum-Sin,Na, Young-Cheon,Park, Jong-Tae 대한성형외과학회 2010 Archives of Plastic Surgery Vol.37 No.5

Purpose: Chordoma is a rare primary osseous tumor arising from the remnants of the primitive notochord. It occurs once in 2,000,000. It is characterized by its slow growth, high frequency to invade destroy bone by direct extension. We experienced giant sacral chordoma and reconstructed with gluteal advancement flap. Methods: A 52-year-old woman presented with a 2-years history of gluteal pain. In the biopsy study revealed sacral chordoma. MRI study showed $13{\times}12{\times}10\;cm$ sized m0cs. We approached anterior and posterior resection and reconstructed with bilateral gluteus maximus advancement flap. Results: After the operation, blader and anal function were slightly decreased. But, 4 months later those were almost fully recovered. There was no significant complication and recurrence after 2-years follow-up. Conclusion: Chordoma is characterized by its slow growth, high frequency to invade and destroy bone by direct extension. Wide surgical resection is the only curative procedure. We report a ase of giant sacral chordoma which was successfully treated by anterior and posterior approach and reconstructed with bilateral gluteal advancement flap.

척삭종의 세침흡인 세포학적 소견

유한석,김민석,하화정,김정순,신명순,박선후,정진행,고재수,이승숙,Ryu, Han-Suk,Kim, Min-Suk,Ha, Hwa-Jung,Kim, Jung-Soon,Shin, Myung-Soon,Park, Sun-Hoo,Chung, Jin-Haeng,Koh, Jae-Soo,Lee, Seung-Sook 대한세포병리학회 2004 대한세포병리학회지 Vol.15 No.1

It is important to recognize the pathognomonic cytologic findings of chordoma, because of overlapping cytologic features between chordoma, chondrshdosarcoma, myxofilbrillary ependymoma, and metastatic adenocarcinoma. We reviewed the cytomorphologic features of 5 cases of chordoma diagnosed by fine needle aspiration cytology at Korean Cancer Center Hospital from 1987 to 2003. Clinical and radiographic findings of each case were reviewed. Four males and one female (29-54 years) had tumors involving the sacrum. Pain was the presenting symptom in 4 cases. The three cases showed moderate to high cellularity. In all cases, typical physaliferous cells with or without cytoplasmic processes were noted. In two cases, the background was myxoid with single scattered cells. Cell clusters showing cord-like arrangement were occasionally seen. The single or clustered cells showed mild cellular pleomorphism with slightly increased nuclear/cytoplasmic ratio. Mitotic figures were not seen. In our review, the recognition of physaliferous cells is the most important feature to diagnose chordoma and to differentiate it from other lesions mimicking chordoma.