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Nasopharyngeal chordoma

Sakkarapani V, Panicker C

Med J Malaysia, 1977 Mar;31(3):208-12.
PMID: 904513
Matched MeSH terms: Chordoma/diagnosis*; Chordoma/pathology
Reconstruction of large sacral defects following tumour resection: a report of two cases

Cheong YW, Sulaiman WA, Halim AS

J Orthop Surg (Hong Kong), 2008 Dec;16(3):351-4.
PMID: 19126905

Sacral tumours often present surgical resection and reconstruction challenges. Wide resections result in large sacral defects and neoadjuvant radiotherapy impairs wound healing. The wounds need to be covered with bulky, well-vascularised, healthy tissues. We present 2 cases where large sacral defects were reconstructed following tumour resection. Both defects were reconstructed with inferiorly based, transpelvic, pedicled vertical rectus abdominis myocutaneous flaps. This is a robust flap and carries a well-vascularised muscle bulk and skin paddle. The donor site is distant from the lesion site and is thus unaffected by both the resection and radiotherapy. This is a useful flap for reconstructing large sacral defects.

Matched MeSH terms: Chordoma/pathology; Chordoma/surgery*
Fulltext Rare case of neglected large sacral Chordoma in a young female treated by wide En bloc resection and Sacrectomy

Phang ZH, Saw XY, Nor NFBM, Ahmad ZB, Ibrahim SB

BMC Cancer, 2018 Nov 14;18(1):1112.
PMID: 30428857 DOI: 10.1186/s12885-018-5012-3

BACKGROUND: Sacral chordoma is a locally aggressive malignant tumour originating from ectopic notochordal cells. The natural history of sacral chordoma is a slow growing tumour arising at the midline of the lower sacrum that can invade the sacrum and progressively increase in size expanding cranially and anteriorly. Metastasis is very rare even when the tumour is large. Sacral chordoma affects males more than females and is more commonly found in middle age and elderly patients.
CASE PRESENTATION: A 25 years old female had neglected an extremely large midline sacral mass for 2 years. On presentation to hospital, she had been bed bound for the past 2 years. The sacral mass was so large that it prevented her from lying down supine and sitting on the wheelchair comfortably. Clinical examination showed a 40 cm × 30 cm × 20 cm hard mass over the sacrum that involved both buttocks and the gluteal fold. Neurological exam of bilateral lower limb was normal. Computed Tomography Scan of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus; and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles. Biopsy taken confirmed Chordoma. This patient was managed by a multidisciplinary team in an Oncology referral centre. The patient had undergone Wide En Bloc Resection and Sacrectomy, a complex surgery that was associated with complications namely bleeding, surgical site infection and neurogenic bowel and bladder. Six months post operatively the patient was able to lie supine and sit on wheelchair comfortably. She required extensive rehabilitation to help her ambulate in future.
CONCLUSION: This is a rare case of neglected sacral chordoma in a young female treated with Wide En Bloc Resection and Sacrectomy associated with complications of this complex surgery. Nevertheless, surgery is still worthwhile to improve the quality of life and to prevent complications secondary to prolonged immobilization. A multidisciplinary approach is ideal and team members need to be prepared to address the complications once they arise.

Matched MeSH terms: Chordoma/pathology; Chordoma/surgery*
Fulltext Endoscopic Transoral Resection of an Axial Chordoma: A Case Report

Taran S, Yusof AH, Yusof MI

Malays Orthop J, 2015 Nov;9(3):75-77.
PMID: 28611918 MyJurnal DOI: 10.5704/MOJ.1511.015

Upper cervical chordoma (UCC) is rare condition and poses unique challenges to surgeons. Even though transoral approach is commonly employed, a minimally invasive technique has not been established. We report a 44-year old Malay lady who presented with a 1 month history of insidious onset of progressive neck pain without neurological symptoms. She was diagnosed to have an axial (C2) chordoma. Intralesional resection of the tumour was performed transorally using the Destandau endoscopic system (Storz, Germany). Satisfactory intralesional excision of the tumour was achieved. She had a posterior fixation of C1-C4 prior to that. Her symptoms improved postoperatively and there were no complications noted. She underwent adjuvant radiotherapy to minimize local recurrence. Endoscopic excision of UCC via the transoral approach is a safe option as it provides an excellent magnified view and ease of resection while minimizing the operative morbidity.

Matched MeSH terms: Chordoma
Fulltext Atypical Clival Chordoma in an Adolescent without Imaging Evidence of Bone Involvement

Hashim H, Rosman AK, Abdul Aziz A, Roqiah AK, Bakar NS

Malays J Med Sci, 2014 Sep-Oct;21(5):78-82.
PMID: 25977639

Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. This usually provides insight for a diagnosis. Here we present a case of a non-enhancing, pre-pontine mass that was hypointense on T1W and hyperintense on T2W in an adolescent. No clival bone erosion was observed. Based on the age group, imaging findings, and lack of clival erosion, a provisional diagnosis of epidermoid cyst was made and the tumour was resected. This patient was eventually diagnosed with a clival chordoma based on histopathological examination.

Matched MeSH terms: Chordoma