Ewing's sarcoma is a rare, malignant tumour predominantly affecting young adolescent males. We describe a unique case of an isolated extra-skeletal metastasis from a skeletal Ewing's sarcoma primary, arising in the right sinonasal cavity of a young man who presented with severe epistaxis and periorbital cellulitis.

Histologically, the lesion comprised closely packed, slightly diffuse, atypical cells with round, hyperchromatic nuclei, scant cytoplasm and occasional mitotic figures, arranged in a sheet-like pattern. Immunohistochemical analysis showed positive staining only for cluster of differentiation 99 glycoprotein. Fluorescent in situ hybridisation identified the Ewing's sarcoma gene, confirming the diagnosis.

Complete surgical resection was achieved via a minimally invasive endoscopic transnasal approach; post-operative radiotherapy. Ten months post-operatively, there were no endoscopic or radiological signs of disease.

Metastatic Ewing's sarcoma within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important to enable early diagnosis. This case illustrates one potential management strategy, and reinforces the evolving role of endoscopic transnasal approaches in managing sinonasal cavity and anterior skull base tumours.

We report an extremely rare case of extramedullary plasmacytoma.

Case report and review of the English-literature concerning extramedullary plasmacytoma and multiple myeloma.

We present an unusual case of multiple extramedullary plasmacytomas, which, over a protracted course of 30 years, presented on different occasions at three separate sites in the head and neck. The patient was managed surgically on all occasions, and was disease-free at the time of writing.

Following review of the literature, we believe this to be the only case with this extremely unusual presentation. This case is noteworthy, not only because of the rarity of extramedullary plasmacytoma, but also because it highlights a number of important clinical issues. The diagnosis and management of extramedullary plasmacytoma require close cooperation between multiple disciplines.

The authors present an extremely rare case of carcinoma of the tympanic membrane.

A case report and review of the literature concerning carcinoma of the tympanic membrane and temporal bone are presented and discussed.

The authors present a patient with recurrent otorrhoea and an abnormal tympanic membrane. Biopsy was inconclusive, but resection demonstrated squamous cell carcinoma of the tympanic membrane. We also discuss the investigation, diagnosis, natural history and management of this rare condition, as well as the staging and management of tumours of the temporal bone and the differences between these closely related but prognostically different entities.

This rare entity can be managed by primary surgical resection if there is no evidence of metastasis.

We describe the first published case of papillary thyroid carcinoma metastatic to the temporal bone.

A 64-year-old woman presented with a large left temporal bone mass centred in the jugular foramen, initially thought to be a paraganglioma or schwannoma. She was simultaneously being investigated for a left-sided thyroid nodule, which was found to be unremarkable on repeated fine needle aspiration cytology. A biopsy of the temporal bone mass indicated that it was of thyroid origin. The patient underwent total thyroidectomy, which enabled a final diagnosis of follicular-variant papillary thyroid carcinoma with metastasis to the temporal bone.

Although biopsy is not the usual management for many types of temporal bone mass, pathological investigation is recommended if the tumour has an atypical growth rate, location, spread and/or radiological features. Metastasis of papillary thyroid carcinoma to the skull base is extremely rare, and correct diagnosis is essential in order to pursue an effective treatment plan.

To present a case of a child with a desmoplastic fibroma of the vidian canal, compressing the vidian nerve.

A 12-year-old girl with several years' history of right-sided facial pain was referred to our institution. Magnetic resonance imaging and computed tomography scans showed an expansile mass involving the right vidian canal. The patient underwent a complete endoscopic surgical resection aided by the FusionTM ENT navigation system. This was performed through a transnasal, trans-septal, trans-sphenoidal route via the right nostril, and achieved macroscopic clearance with minimal peri-operative morbidity. A biopsy of the lesion showed a fibro-osseous lesion consistent with desmoplastic fibroma.

Diagnosis and resection of this rare lesion at an earlier stage would have avoided delays in resolving the child's disabling pain. This emphasises the importance of early referral of unusual cases to tertiary centres.

We present an extremely rare case of isolated angiokeratoma of the tongue.

Case report and review of related literature.

An 18-year-old, male adolescent presented with a fleshy, intermittently bleeding mass in the posterior third and base of the tongue. The lesion was initially suspected to be a lingual thyroid or haemangioma, but histopathological features were consistent with angiokeratoma. Magnetic resonance imaging revealed that the lesion extended up to the vallecula and involved the lamina propria and superficial tongue musculature. No similar lesions were found elsewhere in the body. No metabolic derangements were identified in the patient or his family. The 2.6 × 1.5 × 0.5 cm mass was excised under general anaesthesia.

We present the 1st case of isolated lingual angiokeratoma in a male, the 4th such case overally, the largest ever documented. The lesion was situated in the posterior third and base of the tongue, a position not previously described.

The use of interosseous wire to fix bone grafts is well known. Herein, we describe a technique for fixation of an iliac crest bone graft for nasal augmentation, using a stainless steel wire.

A hole in the cancellous part of the graft guides the wire exactly into a groove in the cortical part, preventing slippage and ensuring rigid fixation. The wire is then threaded through a hollow spinal needle passed underneath the skin envelope; this avoids a dorsal incision and thus minimises scarring, reduces the risk of graft exposure and improves the aesthetic result.

This technique has two distinct advantages: prevention of wire slippage and avoidance of a dorsal nasal incision.

The described method uses an interosseous wire for rigid bone graft fixation, without a dorsal incision. This prevents wire slippage; it also achieves a good cosmetic result by improving the nasal contour via a cantilever effect which raises the nasal tip.

Complementary and alternative medicine is very much in the public arena as a treatment option. The pharmacodynamics of most complementary and alternative medicines are not well understood, and some can lead to significant adverse drug interactions. This report aims to present the case of a cancer patient who abandoned traditional medicine in favour of complementary and alternative medicine.

Case report.

The patient refused potentially curative adjuvant radiotherapy for his oral cavity carcinoma following surgery, and eventually succumbed to disease.

Recently, there appears to be much public awareness of and empathy for complementary and alternative medicine. Healthcare professionals should be aware of such therapies so that they can advise their patients in an informed manner. The role of such therapies in benign conditions may not be as critical as that in malignancy, where life-saving conventional treatment may be abandoned in favour of complementary and alternative medicine, with consequent loss of life.

To present a conservative surgical approach, via the transorbital route, for selected cases of infratemporal fossa involvement or inferolateral orbital tumours extending to the infratemporal fossa.

Case series report.

Department of ENT, CSM Medical University (King George Medical College), Lucknow, India.

One patient.

Operative feasibility, intra-operative access, post-operative morbidity and cosmesis.

This novel and relatively conservative technique provides excellent exposure in selected cases of infratemporal fossa involvement and has minimal post-operative morbidity. Cosmesis is excellent, as osteotomy is not required and scarring is minimal. There is no risk of trismus, Vth or VIIth cranial nerve injury, or cerebrospinal fluid leakage, and haemostasis is easily achieved.

Classical, open approaches to the infratemporal fossa involve considerable morbidity, while conservative approaches have their limitations. Diagnostic uncertainty over a small infratemporal fossa mass (perhaps an extension from an inferolateral orbital tumour) is an uncommon clinical challenge. The transorbital approach described is suited to benign and early malignant tumours, and has excellent results when combined with orbital exenteration (if needed). This paper discusses this approach's technical details and feasibility in different clinical situations, and compares it with other infratemporal fossa approaches.

We present a rare case of an unusual presentation of a pyriform sinus haemangioma in a normally healthy, Caucasian woman, including our management and a review of the literature.

A 44-year-old woman presented complaining of bringing up fresh blood whilst brushing her teeth, dysphonia, food sticking in her throat, and epigastric pain for the preceding 12 months, accompanied by a 17.5 kg weight loss. She underwent pre-operative computed tomography and subsequent complete excision of a pyriform sinus haemangioma using CO2 laser.

Haemangiomas are congenital vascular malformations and can affect any part of the body. They are not prevalent in adults and are rarely found in the pyriform sinus. Their common presenting symptoms and management have been previously documented; however, the presented case is unusual in both its presentation and management.

We report a rare case of successful surgical management of tubercular tracheal stenosis. There was no history of tracheostomy except for trauma management.

A 24-year-old man presented with breathing difficulty. He had previously sustained blunt chest injury, a fractured mandible and minor head injury in a traffic accident. Despite successful mandibular fracture fixation, he subsequently developed progressive breathing difficulty with stridor. The patient was treated successfully with surgical resection and bronchoplastic reconstruction. Post-operatively, endotracheal tuberculosis was diagnosed.

Endotracheal tuberculosis is rare despite the high incidence of pulmonary tuberculosis in India. Early diagnosis and prompt treatment are necessary to prevent tuberculous tracheobronchial stenosis, an extremely rare but serious clinical problem which can cause obstructive pneumonia and exertional dyspnoea. Surgical resection and bronchoplastic reconstruction is the established treatment for such stenosis. Patients with active tuberculosis usually respond to conventional antitubercular treatment.

We report a pregnant patient with a rapidly growing mass within the nasal cavity, which required pre-operative super-selective embolisation and subsequent removal under general anaesthesia after childbirth. We also discuss the clinical, radiological and histological characteristics of lobular capillary haemangioma, and its treatment.

Case report and literature review.

Lobular capillary haemangioma is a benign lesion originating in the vascular tissue of skin, mucosa, muscles, glands and bone. These lesions grow rapidly. Nasal localisation is rare. Microtrauma and pregnancy are the most commonly proposed aetiological factors. Reported incidence during pregnancy ranges from less than 2 per cent to approximately 5 per cent. The management of a pregnant woman with such a lesion may be complex, and depends on the severity of symptoms and the status of the pregnancy. Complete surgical excision, with or without pre-operative embolisation, is the treatment of choice.

This uncommon lesion should be considered in any pregnant patient with a mass in the mouth or nasal cavity.

To highlight the clinical importance of inflammatory myofibroblastic tumours of the respiratory tract in children, and to present a case series of three children which illustrates this tumour's variable clinical presentation.

The series includes: a nine-year-old girl with a diagnosis of juvenile idiopathic arthritis, who presented with finger clubbing and was found to have an inflammatory myofibroblastic tumour in her right upper lobe; a 15-year-old adolescent with a left main stem bronchial inflammatory myofibroblastic tumour, who presented with breathlessness and chest pain; and a 12-year-old girl with a tracheal inflammatory myofibroblastic tumour who presented with stridor. In each case, the tumour was resected surgically.

Inflammatory myofibroblastic tumour are a rare but clinically important and pathologically distinct lesion of the respiratory tract in children. The cases in this series highlight some of the varied clinical presentations of inflammatory myofibroblastic tumours, and illustrate some of this tumour's different anatomical locations within the paediatric respiratory tract.

We report an extremely rare case of giant fibrovascular polyp of the hypopharynx.

We present a 49-year-old man who had increasing difficulty swallowing, advanced respiratory distress and weight loss, as well as a hypopharyngeal mass protruding from his mouth.

Diagnosis was confirmed by endoscopic examination and computed tomography. A tracheostomy was required due to laryngeal obstruction by the regurgitated mass. The giant polyp was removed via per-oral endoscopic excision under general anaesthesia.

Fibrovascular polyps occur most commonly in the cervical oesophagus, and are extremely rare in the hypopharynx. They can grow to a very large size over several years. We discuss the symptoms, diagnosis and surgical treatment techniques for upper aerodigestive tract fibrovascular polyps, in the light of the literature.

To raise awareness of nasal malignant melanoma, a rare tumour, and to highlight the difficulty associated with its optimum management.

Case report and literature review.

A 71-year-old, Caucasian man was diagnosed with malignant melanoma in the right nasal cavity, after presenting with right-sided epistaxis. He underwent endoscopic medial maxillectomy; histological analysis confirmed that the resection margins were clear. However, within six months he re-presented with a metastatic deposit of malignant melanoma in his right external auditory canal, for which he underwent right temporal bone resection. There was no evidence of distant metastasis on radiological studies. Unfortunately, within a month the tumour recurred in the right nasopharynx. A multidisciplinary team decision was made to offer the patient palliative chemoradiotherapy.

Mucosal malignant melanoma of the nose is very rare, and aural metastasis from this primary site has not previously been reported. Optimum management must involve a multidisciplinary team.

We present the first report in the all English literature of a case of laryngeal squamous cell carcinoma metastasis to the orbit.

Case report of orbital metastasis from laryngeal carcinoma; clinical, radiological and pathological findings are discussed.

A 75-year-old man presented to the ENT department with a five-week history of left orbital pain, swelling and reduced vision. Past medical history included laryngectomy, bilateral neck dissection and post-operative radiotherapy for T4 N2c M0 squamous cell carcinoma of the supraglottis, 10 months earlier. Imaging showed an orbital mass extending along the roof and lateral aspect of the orbit into the optic canal and superior orbital fissure, and further posteriorly into the left cavernous sinus with meningeal enhancement in the left anterior cranial fossa. Histopathological analysis after biopsy showed the mass to be consistent with metastatic poorly differentiated squamous cell carcinoma.

After searching the all English literature, we report what is, to our best knowledge, the first case of laryngeal carcinoma metastasis to the orbit. Despite its rarity and poor prognosis, such a metastasis should be considered as part of the differential diagnosis of an orbital mass, as timely recognition can improve the patient's quality of life.

Nasal dermoid cysts are congenital lesions which are often diagnosed in infancy or childhood. However, a small number present in adulthood, and some extend intracranially. Traditional treatment for the intracranial portion of these cysts includes frontal craniotomy.

Two intracranial dermoid cysts were resected via a transnasal endoscopic approach, using 70° nasal endoscopy for complete visualisation and intracranial tumour removal. We describe our technique for the procedure itself and for reconstruction of the skull base defect.

The endoscopic transnasal skull base approach is an excellent alternative to a traditional frontal craniotomy, to achieve complete resection of intracranial dermoid cysts.

We present an unusual case of parapharyngeal cerebrospinal fluid collection causing upper airway obstruction following a temporal bone fracture.

Case report and literature review of temporal bone fracture associated with parapharyngeal cerebrospinal fluid collection.

A 19-year-old man presented with cerebrospinal fluid otorrhoea and temporal bone fracture following a head injury. He was discharged after 48 hours of observation. The patient returned within 6 hours with sudden unilateral neck swelling and stridor after blowing his nose. Flexible nasendoscopy and computed tomography showed extrinsic compression of the pharynx, with partial upper airway obstruction. A literature review using Pubmed™ and Medline™ identified no previously reported cases of parapharyngeal cerebrospinal fluid collection associated with temporal bone fracture.

This case illustrates a previously undescribed complication of temporal bone fracture. Raised intracranial pressure in the presence of a cerebrospinal fluid fistula may lead to airway obstruction, following temporal bone fracture.

We describe a rare case of an ectopic submandibular gland associated with atrophy of the ipsilateral floor of the mouth muscles.

Case report and review of the world literature regarding ectopic submandibular glands.

The reported patient had an ectopic submandibular gland associated with atrophy of the ipsilateral anterior digastric and mylohyoid muscles. This implies maldevelopment of these muscles in the floor of the mouth and arrest of the normal migration of the submandibular gland. The condition was diagnosed using magnetic resonance imaging and conventional submandibular gland sialography.

Submandibular gland ectopia in the floor of the mouth is a rare phenomenon. The described case represents the first report of an ectopic submandibular gland associated with atrophy of the ipsilateral floor of the mouth muscles. Radiologists and clinicians should familiarise themselves with this entity and its imaging findings, in order to prevent unnecessary biopsy of this benign condition.