A healthy 58-year-old woman presented with recurrent swelling and pain of the nose and both auricules. Bruits were heard over both carotid arteries. Magnetic resonance angiography revealed stenosis of both internal carotid arteries. Relapsing polychondritis was diagnosed. These symptoms improved after treatment with prednisolone and azathioprine. Although relapsing polychondritis is sometimes associated with systemic vasculitis, large vessel arteritis is rare and can negatively affect prognosis. We conclude that the detection of systemic vascular lesions, including those involving the central nervous system, can play an important role in the diagnosis of relapsing polychondritis and that early treatment is essential for a good outcome.

In Langerhans’ cell histiocytosis, radiological findings on temporal bone involvement show destructive bone lesions involving the mastoid, with the squamous part and middle ear less affected. Computed tomography (CT) is the preferred imaging modality for describing the extent of temporal bone involvement. CT also has an important role in monitoring disease activity and response to treatment. Bone scintigraphy seems to be less sensitive than radiography in detection of these lesions.

A patient presented with sudden hearing loss on her first visit to our department. Gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) of the posterior cranial fossa portrayed an intracanalicular tumour image (2-3 mm), and the pure tone average (PTA) and speech discrimination score (SDS) values were 65 dB and 60 per cent, respectively. Surgical intervention to remove the suspected tumour was scheduled by the translabyrinthine approach. Intracanalicular observations by the retrolabyrinthine approach revealed limited oedema on the inferior vestibular nerve with vascular dilation. The tumour image disappeared two years after the operation. Surgical findings and the post-operative course advocate that gadolinium-DTPA-enriched MRI image of an intracanalicular lesion such as arachnoiditis might produce a false-positive result.

Maffucci’s syndrome is a rare congenital non-inherited condition, characterized by multiple enchondromata, cutaneous haemangiomata and more recently spindle-cell haemangioma-endotheliomata. It is associated with an increased risk of malignancy including intracranial chondrosarcomas. Early diagnosis is crucial; screening patients with Ollier’s disease is recommended.

The treatment of choice for these intracranial cartilaginous tumours is complete surgical excision but this is often difficult to achieve due to difficult access and relationships with neurovascular structures. An alternative treatment is proton-beam therapy.

We report a case of Maffucci’s syndrome; illustrating the typical clinical and radiological features as well as the known complications of the condition.

We report a case of a peripheral neuroectodermal tumour (pPNET) of the cerebellopontine angle of a 67-year-old woman. The patient’s age at presentation was highly unusual. This case highlights the difficulties encountered, both clinically and pathologically, in securing the correct diagnosis of such a rare condition presenting in this relatively inaccessible area. The development of the nomenclature and classification of neuroectodermal tumours is traced. Recent advances in immunohistochemistry and genetic typing have shown the close relationship between pPNET and the previously difficult to classify Ewing’s sarcoma and Askin’s tumour.

Necrotizing granulomatous nodules are a common feature in patients with rheumatoid disease, affecting 20 per cent of seropositive patients. Although most commonly found subcutaneously at points of pressure, these nodules may occur in the connective tissues elsewhere in the body. In the upper aerodigestive tract, nodules have been described in diverse locations, but commonly in the trachea, larynx and the ear. We present the first reported case of a rheumatoid nodule affecting the nasal septum.

A case of inflammatory myofibroblastic tumour of the larynx in a 74-year old man is reported. The lesion presented as a polypoid tumour arising in the left true vocal fold. The patient died of non-tumour-related causes and the autopsy showed persistence of the laryngeal tumour. Multicentricity and distant metastases were not detected. The literature about this entity and its differential diagnosis is reviewed and briefly commented upon.

We report an atypical lipoma arising in the tongue of a 43-year-old man who presented with an indolent dorsal lingual swelling. Atypical lipomas contain multivacoulated lipoblasts, which distinguishes them from benign lipomas. The superficial location in this case distinguishes this tumour from well-differentiated liposarcoma, which is biologically similar in lacking the propensity for metastasis. The superficial location of atypical lipoma allows a complete resection, which is often not possible for the deep-seated counterpart. Atypical lipoma and well-differentiated liposarcoma, if left in situ, may undergo transition to de-differentiated liposarcoma. Atypical lipoma should be completely excised with a cuff of normal tissue in order to prevent repeated local recurrence and the possibility of de-differentiation.