Incidence Of Chordoma Research Articles

Dedifferentiated Sacral Chordoma: a case report

Sacral chordoma is the most common primary bone tumour bone in the sacrum. Though the incidence of chordoma is rare. Chordoma is divided into subcategories such as conventional chordoma, chondroid and dedifferentiated. Within these three categories the rare. Among the rarity, women and young adult were the least population to be diagnosed. We presented a case of a young female with dedifferentiated sacral chordoma.

ВОЗМОЖНОСТИ ПРОТОННОЙ ТЕРАПИИ В ЛЕЧЕНИИ ХОРДОМ ОСНОВАНИЯ ЧЕРЕПА У ДЕТЕЙ: СЕРИЯ КЛИНИЧЕСКИХ НАБЛЮДЕНИЙ

Chordomas are rare malignant tumors that account for no more than 4% of primary bone tumors. The incidence of chordomas in children does not exceed 5%. Tumor removal is the primary method of treatment, however, due to the high frequency of non-radical surgery, most patients require an adjuvant course of radiotherapy. Photon radiation therapy in doses of 50–60 Gy does not provide the required antitumor effect. At the same time, the use of a proton beam allows delivering a dose of 72 Gy or more with low toxicity. The article presents 4 clinical case reports of base-skull chordoma in children describing their own experience with proton therapy. An analysis of radiation exposure plans was carried out, with an evaluation of the main qualitative indicators characterizing the high-dose coverage of tumours and the radiation load on healthy tissues. The case reports described confirm that the technique of proton irradiation with a pencil beam makes it possible to safely administer ultra-high doses of radiation in close proximity to healthy tissues, including in children. Further improvement of the irradiation planning technique is required, which will improve the target coverage with a high dose of ionizing radiation.

Lumbar Chordoma Presenting as an Epidural Collection with Vertebral Destruction- An Unusual Cause of Lumbar Canal Stenosis

Chordomas are locally aggressive neoplastic lesions that arise from physalipherous cell nests that originate from notochordal remnants left behind during early foetal development. Chordomas of the mobile spine (C3-L5) constitute less than 5% of the overall incidence of chordomas in the spine. They generally are osteodestructive leading to vertebral collapse and severe deficits including paraplegia and quadriplegia. Here authors presented a case of 26-year-old female presented with severe bilateral L5 radicular pain, no deficits and intact bladder and bowel control. The pain was progressive and resistant to analgesic medication. On imaging a collection in the lumbar spine emanating from the L5 vertebral body extending into the canal leading to secondary spinal canal stenosis was observed. On surgical exploration a soft friable vascular mass compressing the dural tube and the exiting nerve roots was observed and the mass was analysed and found to be a chordoma. Although, these lesions are seen to compress the vital neural elements of the spine, the presence of a collection in the spinal canal was unusual and resembled tuberculosis which is a much common lesion, or pyogenic osteomyelitis of the vertebral body. Both differentials were proven wrong. Such an approach not only detected and treated the lesion early, but good rehabilitation and adjuvant therapy was initiated enabling excellent overall quality of life to the patient.

Chordoma: update on disease, epidemiology, biology and medical therapies.

Chordoma is an exceedingly rare subtype of bone sarcoma. This review aims to provide a comprehensive insight into chordoma epidemiology, and an update on the recent advances in disease, biology and medical therapies. The incidence of chordoma is approximately 0.08/100 000 and the 5-year overall age-adjusted relative survival is 72% in the United States and 61% in Europe. Over the last years, significant steps forwards have been done in the comprehension of chordoma complexity, with insights gained into the biology and morphology of this disease. New entities have been described and potentially druggable molecular targets identified. This is becoming all the more relevant today, as new potentially active agents are under development. Chordoma is a complex disease because of its rarity, biological heterogeneity and peculiar clinical behaviour. Despite the progress done, the outcome in this disease remains unsatisfactory and the identification of active systemic treatments remains an urgent, unmet medical need.

Aberrant Hyperactivation of Akt and Mammalian Target of Rapamycin Complex 1 Signaling in Sporadic Chordomas

Chordomas are rare, malignant bone neoplasms in which the pathogenic mechanisms remain unknown. Interestingly, tuberous sclerosis complex (TSC) is the only syndrome in which the incidence of chordomas has been described. We previously reported the pathogenic role of the TSC genes in TSC-associated chordomas. In this study, we investigated whether aberrant TSC/mammalian target of rapamycin complex 1 (mTORC1) signaling pathway is associated with sporadic chordomas. We assessed the status of mTORC1 signaling in primary tumors/cell lines of sacral chordomas and further examined upstream of mTORC1 signaling, including the PTEN (phosphatase and tensin homologue deleted on chromosome ten) tumor suppressor. We also tested the efficacy of the mTOR inhibitor rapamycin on signaling and growth of chordoma cell lines. Sporadic sacral chordoma tumors and cell lines examined commonly displayed hyperactivated Akt and mTORC1 signaling. Strikingly, expression of PTEN, a negative regulator of mTORC1 signaling, was not detected or significantly reduced in chordoma-derived cell lines and primary tumors. Furthermore, rapamycin inhibited mTORC1 activation and suppressed proliferation of chordoma-derived cell line. Our results suggest that loss of PTEN as well as other genetic alterations that result in constitutive activation of Akt/mTORC1 signaling may contribute to the development of sporadic chordomas. More importantly, a combination of Akt and mTORC1 inhibition may provide clinical benefits to chordoma patients.

Chordoma in Finland.

During the period 1953-1971, 20 cases of chordoma were reported to the Finnish Cancer Registry. Twelve of the patients were males. The mean annual (crude) incidence of chordoma in Finland was 0.30/10(6) in males, and 0.18/10(6) in females. Fifteen of the tumours were sacral, three vertebral, and two cranial. Local recurrences were common, and distant metastases were observed in 60 per cent of the cases; this exceeds the proportion usually mentioned in the literature. The commonest treatment was surgery combined with postoperative high-dose irradiation. The relative 5-year survival rate was 35 per cent, and the 10-year rate 18 per cent.