A case of chordoma invading multiple neuroaxial bones:report of ten years follow up

Abstract

AIm: Chordoma is a rare, slow-growing primary malignant tumor of the axial skeleton, arising from the embryonic cells of primitive notochord. Chordomas may arise at different sites of the vertebral column simultaneously or more probably they may metastasise along the neural axis insidiously. Recurrence despite radical surgery and following adjuvant therapy is possible. mAterIAl and methOds:A 46-year-old female patient presented weakness and numbness of the lower extremities. She was operated for clivus chordoma five years ago at another institute.results: First the patient underwent surgery for resection of the tumor at the cervical region. a second surgery was performed to resect tumor on the foramen magnum and at the C1 level. Histologic examination of the removed vertebra confirmed the diagnosis of chordoma involving the vertebral body. Radiotherapy was administered after the second surgery. Follow-up neurological and radiological examinations revealed no abnormal neurological symptoms 2,5 years after second surgery. There were no distant organ metastases.COnClusIOn: A patient with diagnosed chordoma of the spine must be investigated with MRI of other regions of the neuraxis to exclude second or even third source of chordoma metastases. In metastatic chordoma cases, radical or gross total resection should be performed for each lesion but if complete surgical resections are impossible, preoperative or postoperative radiation therapy should be planned to improve life expectancy.KeywOrds: Chordoma, Cervical tumor, Thoracal tumor, Spinal tumor, Metastasis ÖZ AmAÇ: Kordoma, omurganın ender rastlanan, yavaş ilerleyen malign karakterli bir tümörüdür. Primitif notokordun embryo hücrelerinden köken alır. Omurganın farklı bölgelerinde, birbirinden bağımsız kordoma odakları eş zamanlı olarak gelişmeye başlayabilir. Fakat daha sıklıkla, birinci tümör odağından kaynaklanan metastatik kordoma lezyonları tüm nöral aks boyunca yayılabilir. Radikal cerrahiye ve ek onkolojik tedaviye rağmen rekürens sık görülür. yÖntem ve GereÇler: Bu sunumda tartışılan 46 yaşındaki kadın hasta, bacaklarında kuvvet ve his kaybı şikayetiyle kliniğimize başvurmuştur. Hasta, beş yıl önce, ayrı bir merkezde klivus kordoması tanısıyla opere edilmiştir.BulGulAr: Hasta öncelikle servikal bölgedeki tümör için opere edilmiştir. İkinci seansta foramen magnum ve C1 seviyesindeki tümör çıkarılmıştır. Patolojik inceleme sonucunda, omurga cismini tutan kordoma lezyonu tanısı konmuştur. İkinci cerrahi seansından sonra hasta radyoterapiye yönlendirilmiştir. Bu tedaviden ikibuçuk yıl sonra yapılan değerlendirmede, hastanın nörolojik muayenesinde progresyon gözlenmemiştir.sOnuÇ: Omurganın herhangi bir lokalizasyonunda kordoma tanısı konulan bir hastada, tüm spinal bölge manyetik rezonans görüntüleme (MRG) tetkiki ile incelenmelidir. Bu hastalarda birden fazla kordoma odağına rastlanabilir. Birden fazla odak tespit edilmesi durumunda, her ayrı tümör odağı için rezeksiyon planlanmalıdır. Total rezeksiyonun mümkün olmaması durumunda, yaşam süresini uzatmak...

Multicentric chordoma in a child

“…To our literature search, we found only three cases of multicentric chordoma in adults. [ 6 7 8 ] We report the first case of multicentric chordoma in pediatric age group.…”

Combined Therapy for Distant Metastasis of Sacral Chordoma

“…Peritoneal metastasis (also known as peritoneal carcinomatosis, when extensive) is relatively common, particularly in tumors of the abdomen and pelvis; it generally implies a poor prognosis, often with a significant impact on palliation [ 8 , 9 ]. The most common cancers to invade the peritoneal region are ovarian cancer, gastric cancer, colorectal cancer, appendiceal malignancies, gallbladder carcinoma, pancreatic carcinoma, primary peritoneal malignancy, and hematogenous spread (breast cancer, lung cancer, and malignant melanoma) [ 9 ].…”

“…MRI is much more sensitive than CT in diagnosing peritoneal metastasis (i.e., by 85–90%). Peritoneal metastases are not locally treated, although systemic treatment may have some effect [ 8 , 9 ]. There has been no report in the literature of peritoneal metastasis of chordomas.…”

“…There has been no report in the literature of peritoneal metastasis of chordomas. Aydın et al [ 8 ] report chordoma cells being identified in the peritoneal fluid of patients who had chordomas [ 8 – 10 ]. In the present case, we detected peritoneal metastasis in control MRI and CT scans.…”

Proton beam therapy in the management of skull base chordomas: systematic review of indications, outcomes, and implications for neurosurgeons

“…[3] In human embryos, the notochord forms the axial skeleton which later forms the skull base and vertebrae. [4] Normally, by 1-3 years of age the notochord is replaced by fibrocartilage tissue i.e. the notochord disintegrates.…”