Extraosseous Chordoma of the Nasopharynx in Paediatric Age

Guilarte, Jhonder Xavier Salazar; Méstre, Manuela Sancho; Albert, Juan Ramón Gras

doi:10.1016/j.otoeng.2012.07.011

Cited by 2 publications

(3 citation statements)

References 8 publications

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“…Extraosseous chordoma of the nasopharynx is a rare malig nancy that originates from the remnants of the notochord, a primitive tissue of embryonic origin around which the skull base and axial skeleton develop (12,(20)(21)(22). Extraosseous chordomas commonly affect individuals aged 50-60 years (23,24).…”

Section: Discussionmentioning

confidence: 99%

“…In the present case report, the patient presented with cranial neuropathies, pain and difficulty swallowing. Local recurrence with an incidence of 50-68% is common due to the aggressive nature of this tumor (12,20,22,27), and systemic metastasis to the lungs, bones, liver and lymph nodes are found in 17.8-43% of patients with chordoma (4,27).…”

Section: Discussionmentioning

confidence: 99%

“…Salazar Guilarte et al (22) reported the case of a 7-year-old girl with a 4x3x5-cm nasopharyngeal chordoma, who first underwent transoral endoscopic resection. Subsequently, stereotactic ablative radiotherapy was used to treat the 2-cm residual tumor with good results; dynamic IMRT was used to deliver 42 Gy in 4 fractions.…”

Section: Discussionmentioning

confidence: 99%

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Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Yeh

2021

Mol Clin Oncol

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Chordomas are slow-growing aggressive tumors that account for 1-4% of all bone tumors. The anatomical distribution of chordomas includes 50-60% in the sacrococcygeal region, 25-30% in the skull base and 15% in the mobile spine. Virchow was the first to describe and term these tumors as ‘ecchordosis physaliphora’ in 1857, and Muller established their notochordal origin in 1895. Extraosseous chordomas of the nasopharynx are very rare, and they exhibit similarities with other lesions of the nasopharynx, presenting as a soft tissue mass. Gross total resection combined with postoperative radiotherapy offers the best chance of long-term control. We herein present the case of a 63-year-old female patient with complaints of left temporal headaches, dizziness, left nasal obstruction, left maxillary area numbness, left ear hearing loss and swallowing difficulty. Computed tomography imaging examination revealed an 8.2x3.2x5.7-cm space-occupying lesion with central necrosis in the nasopharynx and oropharynx, partially occluding the pharyngeal lumen; the mass had infiltrated the left parapharyngeal space, the left medial and lateral pterygoid muscle and the left parotid gland, with bone erosion of the left mandible. The patient was diagnosed with extraosseous chordoma of the nasopharynx, conventional type, stage IIB. The patient underwent surgery and high-dose radiotherapy and local control of the chordoma was achieved. However, the patient succumbed to a lung metastasis. The details of the case are discussed, and a review of the current medical literature is presented to provide an updated discussion on the current status of nasopharyngeal chordoma research.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Yeh

2021

Mol Clin Oncol

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Endoscopic Resection of Pediatric Skull Base Tumors: An Evidence-Based Review

et al. 2018

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Objectives To perform a systematic review examining experiences with endoscopic resection of skull base lesions in the pediatric population, with a focus on outcomes, recurrence, and surgical morbidities. Methods PubMed/MEDLINE, Cochrane Library, Embase, and Web of Science databases were evaluated. Studies were assessed for level of evidence. Bias risk was evaluated using the Cochrane Bias tool, Grades of Recommendation, Assessment, Development and Evaluation (GRADE), and Methodological Index for Non-Randomized Studies (MINORS) criteria. Patient characteristics, pathology, site of primary disease, presenting symptoms, stage, procedure specific details, and complications were evaluated. Results were reported using the Preferred Reporting Systems for Systematic Reviews and Meta-Analysis guidelines. Results Ninety-three studies met criteria for inclusion, encompassing 574 patients with skull base tumors. The GRADE and MINORS criteria determined the overall evidence to be moderate quality. The most common benign and malignant pathologies included juvenile nasopharyngeal angiofibromas (n = 239) and chondrosarcomas (n = 11) at 41.6 and 1.9%, respectively. Of all juvenile nasopharyngeal angiofibroma tumors, most presented at stage IIIa and IIIb (25.8 and 27.3%, respectively). Nasal obstruction (16.5%) and headache (16.0%) were common symptoms at initial presentation. Surgical approaches included endoscopic endonasal (n = 193, 41.2%) and endoscopic extended transsphenoidal (n = 155, 33.1%). Early (< 6 weeks) and late (>6 weeks) complications included cerebrospinal fluid leak (n = 36, 17.3%) and endocrinopathy (n = 43, 20.7%). Mean follow-up time was 37 months (0.5–180 months), with 86.5% showing no evidence of disease and 2.1% having died from disease at last follow-up. Conclusion Endoscopic skull base surgery has been shown to be a safe and effective method of treating a variety of pediatric skull base tumors. If appropriately employed, the minimally invasive approach can provide optimal results in the pediatric population.

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Extraosseous Chordoma of the Nasopharynx in Paediatric Age

Cited by 2 publications

References 8 publications

Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Endoscopic Resection of Pediatric Skull Base Tumors: An Evidence-Based Review

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