Chordoma is a rare bone cancer for which there are no approved drugs. Surgery is the principle treatment but complete resection can be challenging due to the location of the tumours in the spine and therefore finding an effective drug treatment is a pressing unmet clinical need. A major recent study identified the transcription factor Brachyury as the primary vulnerability and drug target in chordoma. Previously, all-trans retinoic acid (ATRA) has been shown to negatively influence expression of the Brachyury gene, TBXT. Here we extend this finding and demonstrate that ATRA lowers Brachyury protein levels in chordoma cells and reduces proliferation of the chordoma cell line U-CH1 as well as causing loss of distinctive chordoma cell morphology. ATRA is available as a generic drug and is the first line treatment for acute promyelocytic leukaemia (APL). This study implies ATRA could have therapeutic value if repurposed for chordoma.

Chordomas are rare neoplasms originating along the neuraxis. Although they do not usually show cytological atypia, metastases have been reported in 30 per cent of cases. Survival rates in cases of skull base locations are low, and local recurrence is common after local excision. Radiation therapy is used in post-operative treatment and proton radiation therapy as the primary treatment. In the present paper we present the case of a 50-year-old Caucasian man affected by chordoma of the clivus, with liver and chest metastases, relapsed after several surgical local excisions, to discuss improvements in therapeutic and imaging techniques. Indium111 (In111) pentetreotide single photon emission computed tomography (SPECT) was employed to assess the presence of somatostatin receptors and to treat the tumour with radiolabelled Y90-DOTA-lanreotide. Imaging, performed 2 months afterwards, showedstable disease in the lungs but a local progression in the metastases, in comparison with pre-treatment uptake. These data suggest the usefulness of radiolabelled somatostatin analogues in the diagnosis and therapy of chordomas.

Primary chordoma of the paranasal sinuses are extremely rare tumours, with only a small number of cases verified and published in the literature. It appears that only five of these cases involved the ethmoid sinuses either as a primary or by local invasion, and of these documented cases only one other has been found to solely involve the ethmoid sinus. We present a case of primary ethmoid sinus chordoma treated by wide local surgical excision and present a review of the literature with regard to prevalence and treatment rationale.

The ISG viewing wand is an intra-operative guidance system with a proprioceptive robotic-like jointed arm. It provides surgeons with almost instantaneously reconstructed computer-generated CT or MRI images in two or three dimensions and can correlate any point within the operative field to its corresponding locus on the reformatted scan images. In addition to having been used in over 400 neurosurgical patients in Bristol, 14 patients with skull-base, cerebello-pontine angle or temporal bone lesions have also undergone wand-guided resections. The wand has proved to be particularly useful in pre-operative planning, allowing minimally-invasive incisions, providing per-operative navigation, identifying the relationship and proximity of important anatomical structures and in assessing the extent of lesion resection. We illustrate the advantages of intra-operative image-guidance by discussing four cases. The potential applications of this form of imaging technology to other otolaryngological procedures are discussed.

Immunocytochemistry (ICC) proved to be an essential adjunct in the fine-needle aspiration (FNA) cytological diagnosis of chordoma of the clivus in a 62-year-old woman. The cytological picture in routinely stained smears was not entirely diagnostic for chordoma due to the paucity of typical ‘physalipherous’ cells. To exclude other primary or metastatic neoplasms of the skull base possibly sharing the same cytological picture, additional direct smears were immunostained with antibodies specific for cytokeratin (CK), vimentin (VIM), SI00 protein (SIOOP), carcinoembrionic antigen (CEA), epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), CD68 antigen (KP1) and with the ‘panepithelial’ antibodies B72.3 and Ber-EP4. Chordoma cells showed the following immunoprofile: CK+/VIM−/S100P+/CEA−/EMA+/GFAP−/B72.3−/Ber-EP4−/CD68+. The pattern of immunoreactivity for CK, S100P and CEA confirms previously reported data, while the B72.3−/Ber-EP4−/CD68+ staining profile represents a novel observation. The etection of a CK+/S100+/CEA−/B72.3−/Ber-EP4− immunocytological profile of chordoma cells in aspirates is a basic requirement to exclude pertinent diagnostic differentials, such as metastatic carcinoma, ependymoma and sarcoma, and permits a reliable pre-operative diagnosis of the tumour by aspiration cytology.

An unusual case of a chordoma of the parapharyngeal space is presented with no direct involvement of the axial skeleton. Diagnosis was confirmed with the aid of immunocytochemical tests.