Chordomas In Children Research Articles

Outcomes of Endoscopic Resection in Pediatric Skull Base Chordoma: A Systematic Review.

The endoscopic approach has been recommended as a primary option for treating chordomas, and it is associated with better resection rates and fewer surgical complications than transcranial surgery. This review aimed to assess the long-term consequences andevidence in the current literature regarding the endoscopic approach's efficacy in treating skull-base chordoma in children. A systematic review was conducted based on the PubMed, Web of Science, and EMBASE databases to examine the clinical outcomes of endoscopic endonasal surgery for pediatric skull base chordoma tumors. The review included studies published in English that employed specific research designs and reported on pediatric patients with skull base chordoma.Of the 268 studies initially considered, 25 met our eligibility criteria and were included in the final analysis. The average age of the patients was 11.5 years, with approximately equal numberof males and females. The endoscopic endonasal approach (EEA) was the most commonly used modality. Gross total resection (GTR) was achieved in 62.7% of patients, while 18.09% had a subtotal resection (STR), and 13.83% had near-total resection only. Most patients showed significant to moderate improvement from their baseline condition and had no recurrence during their follow-up. Our findings further endorse that the endoscopic approach is a viable primary treatment option for pediatric skull base 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.

Surgical management of clival chordomas in children

Objective: Describe the surgical management of clival chordomas in the pediatric population, with an emphasis on techniques of endoscopic endonasal surgery. Finding: Endoscopic endonasal approaches to the clivus provide access from posterior clinoids to foramen magnum. The clivus is divided into thirds (superior, middle, and inferior), each with corresponding vascular and neural structures. The abducens nerve is at greatest risk for injury due to spread of tumor within the interdural space to Dorello's canal. Despite decreased pneumatization of the sinuses in pediatric patients, the same techniques can be applied to this population. The contralateral transmaxillary approach enhances the ability to achieve a gross total resection when tumor extends laterally into the petrous apex. A multi-layer reconstruction with vascularized tissue minimizes the risk of cerebrospinal fluid leak. Conclusion: Endoscopic endonasal surgery can be safely applied to the pediatric population and provides the best opportunity for complete surgical excision for most tumors.

Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law.

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors. Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. Long-term overall and progression-free survival in children harboring skull basechordomascan be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Clinical Features and Prognostic Factors of Children and Adolescents with Clival Chordomas

Chordomas in children and adolescents are rare, and minimal published information is available. Our aim was to research clinical features and prognostic factors associated with clival chordomas in younger patients. The study included 25 patients (10 males and 15 females). Average follow-up time was 42.95 months (range, 2-108 months). Kaplan-Meier analysis was used to determine overall survival (OS) and progression-free survival. Pearson correlation was used to perform correlation analysis. Immunohistochemistry was used to detect Ki-67, cytokeratin 8 (CK8), platelet-derived growth factor receptor-β, and brachyury expression levels. Average age (± SD) of patients was 14.44 (3.44) years. Total resection was achieved in 5 cases, and nontotal resection was achieved in 20 cases. Average OS of patients with Karnofsky performance scale score ≥90 and patients in the total resection group was significantly longer compared with OS of patients with Karnofsky performance scale score <90 (P= 0.041) and patients in the nontotal resection group (P= 0.0497). Patients with lower Ki-67 expression levels had longer OS (P= 0.0207). Progression-free survival of patients with lower CK8 expression levels was significantly longer compared with patients with higher CK8 levels (P= 0.008). Expression levels of platelet-derived growth factor receptor-β were significantly correlated with expression levels of brachyury (P= 0.016). In children and adolescents with clival chordomas, higher preoperative Karnofsky performance scale score, total resection, and lower levels of Ki-67 and CK8 expression are favorable prognostic factors. Platelet-derived growth factor receptor-β may play a role in tumorigenesis and epithelial-mesenchymal transition of clival chordomas.

Clinical and Histopathologic Features of Chordomas in Children and Young Adults

Chordoma is a tumor of the axial skeleton that is distinctly uncommon in children and adolescents. Previous observations have suggested that chordomas in pediatric patients differ from adult chordomas in presentation, morphology, and behavior. This study examines the clinical and histologic features of chordomas in young patients (< or =25 years old). All cases from the Mayo Clinic files were graphed according to age and a bimodal distribution was observed. The 35 cases representing the youngest population were selected for review. Histopathology ranged from low cellularity tumors with lobulated architecture and abundant myxoid matrix (conventional chordoma), to those with varying amounts of chondroid matrix (chondroid chordoma), to more cellular tumors (atypical chordoma), and finally to neoplasms with high-grade spindle-cell differentiation (dedifferentiated chordoma). Over an average follow-up period of 129 months (range 1 to 501 months), there were 13 deaths (37%) and 3 patients with metastasis. This survival rate was slightly better than the reported mortality rate for adults with chordoma, but there was a subset of young patients (those with atypical chordoma) that had a significantly worse survival rate, suggesting that histologic subtyping may be predictive of prognosis.

Modified transoral approach for resection of skull base chordomas in children

Chordomas are rare slow growing, locally destructive tumours originating from remnants of the primitive notocord and are found most commonly in the clivus and saccrococcygeal region. These tumours usually present in early adult life but on occasion can present in childhood. The combination of the skull base location and paediatric patient size makes access to these tumours particularly challenging. We report a multidisciplinary technique used in two cases in children where a modified extended palatal split was undertaken to allow greater access for tumour excision. This approach allows for good access to the skull base region to allow for maximal tumour resection. This technique also appears to have minimal impact on palatal function and no adverse effects on the upper airway management.

Modified Transoral Approach for Resection of Skull Base Chordoma in Children

Introduction: Chordomas are rare, slow-growing tumors arising from the remnants of the notochord and are found in close association with the axial skeleton. These tumors usually present in early adult life but on occasion present in childhood. Pediatric skull base tumors are particularly challenging to access to allow for maximal surgical resection. We report a multidisciplinary technique used in 2 cases in children, where an extended palatal split was undertaken to allow neurosurgical access for excision to be attempted.

Base of Skull Chordomas in Children and Adolescents

Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23% of cases. Fourteen tumors (19%) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81%. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.

Base of skull and cervical spine chordomas in children treated by high-dose irradiation

To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity.

Clival Chordoma in Childhood: A Report of Two Cases

Clival Chordoma in Childhood: A Report of Two Cases

Cranial chordomas in children and young adults.

A series of 12 surgically treated and pathologically proven cranial chordomas presenting in children and young adults is reviewed. These rare tumors occur in relation to the path of the primitive notochord; four were parasellar, six were mid-clival, and two were inferior clival in location. Pathological evaluation revealed five of the tumors to have a significant chondroid component. Follow-up studies ranging from 1 month to 21 years revealed that eight patients were alive, all of whom had received radiation therapy following surgery.

Sacrococcygeal chordomas in children

Two children with sacrococcygeal chordoma are described. In childhood the chordoma is a highly malignant, locally infiltrative, and rapidly fatal tumor. Distant metastases are uncommon. Radiotherapy is the only definitive treatment, although our cases were totally resistant to all forms of therapy.

Intracranial Chordoma in Children

Chordoma has been generally regarded as a neoplasm of adult life, derived from remnants of notochord. It can occur anywhere along the axial skeleton but is most frequently found at the two poles; in the sacrococcygeal region and intracranially in the clivus. Sacrococcygeal chordomas are the most frequent of these tumors in children and were reviewed in 1933 by Montgomery and Wo lman. 1 Clival chordomas in children occur infrequently and have received even less attention. This report deals with the presence of a chordoma of the clivus, metastatic to the lungs, in a 21/2-yearold boy who, after review of the literature, would appear to be the youngest recorded case with this disease. In addition, the clinical, radiographic, and pathologic features of intracranial chordoma in children are reviewed and discussed. Report of a Case The patient's illness began when he was 21/2 years old with rapid onset of bilateral