Description

A 53-year-old man presented to our service with a 1-month history of subacute bowel obstruction. Clinical examination was unremarkable with normal power and sensation in the lower limbs. This patient had no medical or surgical history. MRI of the pelvis identified a well-defined presacral midline lesion of 9.9 cm diameter, which appeared to arise from the S2 nerve root and below (figure 1A,B). These nerve roots control anal sphincter tone and prohibit faecal incontinence. Whole spine MRI and CT-thorax abdomen pelvis CT-TAP) out ruled metastasis. A biopsy confirmed diagnosis of chordoma. This patient was discussed by a multidisciplinary team consisting of orthopaedics, colorectal, vascular and plastic surgeons. Anterior mobilisation of the lesion, with ileostomy due to expected loss of anal tone and supporting vessel ligation, was undertaken. A wide sacral en-masse resection at the level of S1 disc and inferior nerve roots, incorporating partial gluteus maximus and piriformis bilaterally, was achieved by a posterior approach with a latissimus dorsi flap for reconstruction (figure 2A,B,C). Histopathology confirmed clear margins.

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Figure 1

A sagittal (A) and axial (B) MRI of a well-defined midline 9.9 x 9 cm arising from the sacral spine.

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Figure 2

Topographical view of patient's lumbar spine; preoperative (A) with suture identifying the biopsy site (blue arrow) and 6 weeks postoperative (B) with sacral (green arrow) and latissimus dorsi (purple arrow) wounds. Chordoma lesion (C) and sacrum excised, en-masse.

Chordomas are rare slow-growing malignant tumours of embryonic notochord remnants. They comprise 1%–4% of all primary bone tumours.1 Most common locations include the base of skull and sacrococcygeal regions. Sacrococcygeal chordomas can affect bowel, urinary and vascular structures resulting in obstruction. Diagnosis includes plain radiography, MRI and CT-TAP to identify metastasis. Definitive diagnosis is achieved with a tissue biopsy. Differential diagnosis includes chondrosacroma and tuberculosis. Treatment by surgery is a wide-margin en-masse resection. Poor prognosis has been identified with a large tumour size and incomplete marginal clearance.2 Clinical trials with immunotherapy, supported by the chordoma foundation, are currently underway for advanced metastatic disease.3