Neurofibromatosis type 1 (NF 1) or Von Recklinghausen’s disease is an autosomal dominant condition characterized by multiple skin cafe´-au-lait lesions and multiple neurofibromas. In the head and neck neurofibromas have a predilection to arise in th deep planes of the neck. Surgical management is nearly always required for functional or cosmetic reasons or to exclude the possibility of malignant transformation. We present four cases of neurofibroma of the head and neck and illustrate the difficulties inherent in managing this condition, particularly the age range in which it may occur, the risk of malignant transformation, the possibility of abnormal bleeding and the morbidity that may be associated with the surgical resection. The role of magnetic resonance (MR) scan in the assessment and monitoring of these lesions is highlighted. We also report a unique association of a pharyngeal pouch with NF 1 in a young (25-year-old) patient.

A 41-year-old man presented to his general practitioner (GP) with a wheeze and dyspnoea on exercise. Asthma was diagnosed and treatment with inhaled corticosteroid and a beta2-agonist commenced. Despite this, his condition deteriorated over three weeks culminating in stridor, requiring emergency admission to hospital. Nasendoscopy revealed a polypoidal lesion in the upper trachea, acting like a ball valve. A local anaesthetic tracheostomy, secured his airway. Subsequent direct laryngoscopy allowed avulsion of the lesion. Alternative methods of airway management are discussed. Histology revealed a paraganglioma. The aetiology of paragangliomas is described and a literature review of tracheal paraganglioma is presented. Post-operative recovery was unremarkable. However, tumour recurrence occurred at nine months. A subsequent revision tracheostomy and laser resection has ensured disease-free survival, one year later. We recommend that acute onset wheeze, refractive to appropriate therapy, should be referred for urgent examination of the upper aerodigestive tract.

Solitary fibrous tumours are rare tumours originally described in the pleura. More recently there have been reports of these tumours arising at other sites including the parotid region. They are characterized histologically by a variety of growth patterns that can be confused by the unwary with other benign or malignant tumours particularly sarcomas. We present a case occurring in the pre-auricular region which presented diagnostic difficulties.

Inflammatory pseudotumour (IPT) is an idiopathic condition characterized by sclerosing inflammation, which mimics a neoplastic process. IPT involving the skull base and cervical spine is distinctly rare and usually indistinguishable from aggressive neoplasms or infection. We report a case of IPT involving the skull base and cervical spine. Initially the patient complained of headache and hearing loss without other neurological dysfunction. Two cycles of oral systemic steroid therapy resulted in only partial responses. Low dose radiotherapy was followed by quadriparesis as the lesion infiltrated into the cervical vertebral bodies. Subsequently a second course of radiation was administered to the whole cervical spine. Marked improvement was observed clinically and radiologically; however, the patient gradually deteriorated and died of sepsis. Given the aggressive nature of disease and the complications related to the long-term treatment, we suggest that a more aggressive therapeutic approach is suitable in extensive IPT of the skull base.

In this case report, we present a solitary metastasis of renal cell carcinoma inside the masseter muscle. To our knowledge, it is the very first case ever encountered.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a particularly rare variant of sweat gland carcinoma with characteristics of indolent and progressive course and high incidence of perineural invasion and local recurrence. However, regional lymph node metastasis in PCACC is exceedingly rare and its prognostic implication is unknown. Only two previous cases of recurrent scalp PCACC were reported to be associated with cervical lymph node metastases at 42 months and 20 years, respectively, after the initial treatment. We present a case of PCACC occurring in the left parotid region with regional neck lymph node metastasis in a 64-year-old man. Because the occurrence of lymph node metastasis seems to be associated with recurrent PCACC, we suggest that neck dissection may be included in the treatment for recurrent PCACC patients.

We present a rare case of inflammatory pseudotumour in the submandibular region. A review of the literature revealed that this is only the second case of inflammatory pseudotumour in the submandibular region to be reported. Clinical presentation and management of this condition are discussed.

Endoscopic laser dacryocystorhinostomy (DCR) is a recognized technique for the surgical treatment of epiphora. Nasolacrimal duct obstruction is surgically bypassed by creating a passage from the lacrimal sac to the nasal cavity (rhinostomy). Some patients have undergone endonasal laser-assisted DCR, and were found to have an obstructed rhinostomy at follow-up. However, they reported a subjective improvement in their symptoms. Five such patients, at six months follow-up, were found to have a non-functioning rhinostomy with fluorescein dye emerging from under the inferior turbinate. These five patients along with four controls had post-operative macrodacryocystograms (MDCG) to delineate the anatomical passage by which tears were entering the nasal cavity.

In the control group, clear passage of contrast into the middle meatus was demonstrated in three of the four subjects. In the study group, passage of dye to the inferior meatus, via the nasolacrimal duct was demonstrated in four of the five subjects.

It is well recognized that a proportion of patients suffering from epiphora will have a natural resolution of their symptoms. Our results demonstrate that the resolution of epiphora in some operated patients was due to a re-opening of the nasolacrimal duct, and not because of a patent rhinostomy.

Malignant tracheal stenosis presents a considerable challenge to the head and neck surgeon. The use of intraluminal stents has been previously described, but current literature notes a paucity of supportive physiological data. We describe the use of a self-expanding metallic Wallstent in the palliation of a case of papillary thyroid carcinoma. This is accompanied by significant improvement in quantitative pulmonary function.

A rhabdomyosarcoma of the head and neck region is a rare childhood neoplasm often presenting with vague symptoms that can easily mimic other diseases. We present an unusual case of an alveolar rhabdomyosarcoma of the soft palate in a three-year-old child, that presented as a peritonsillar abscess.

The parapharyngeal space is a complex anatomical area, which can give rise to a variety of both primary and metastatic neoplasms. We present an unusual case of metastatic adenocarcinoma of the breast, masquerading as a deep lobe parotid neoplasm, 15 years after the primary presentation. Metastatic breast carcinoma has been previously reported at other sites in the head and neck but this is the first report of metastases in the parapharyngeal space with an oropharyngeal presentation.

We report a case of glottic primary laryngeal lymphoma. Although the head and neck region is a frequent site of origin of extranodal non-Hodgkin’s lymphomas, laryngeal involvement is exceptional. Including this case, about 90 primary laryngeal lymphomas have been reported in the literature. Microscopic study showed a diffuse malignant lymphoma of high-grade malignancy (WF sub-division H). A diffuse, large, B-cell-type NHL was diagnosed histopathologically.

The patient was treated with combination chemotherapy, including cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP), which resulted in complete clinical remission after two courses. Four courses of combination chemotherapy were subsequently performed, making a total of six courses of combination chemotherapy. No recurrence has been observed during the 16-month follow-up period.

The masseteric space is an important tissue compartment of the neck, but disease in it is difficult to diagnose and treat. In this paper a case of a young adult male with an abscess of the submasseteric space is presented. Diagnosis was established by computed tomography (CT) of the neck, but the severity of the lesion was not accurately estimated. Surgical intervention was performed and a large quantity of pus was drained. A detailed medical history and clinical examination of the patient as well as CT are important tools in the accurate diagnosis and efficient treatment of the disease.

An unusual case of internal jugular thrombosis following routine tonsillectomy is presented. Guidelines for the clinical management of this case, with anticoagulants, do not exist.

Cervicofacial actinomycosis is known to affect many soft tissues and bony structures in the head and neck. However to the authors’ knowledge, actinomycosis of the post-cricoid region has not been previously reported. A case of a 74-year-old male who developed actinomycosis of the post-cricoid region after radiotherapy for a laryngeal carcinoma is presented. Actinomycosis should be considered in the differential diagnosis of dysphagia following radiotherapy for squamous cell carcinoma of the larynx, as early treatment is likely to result in a favourable outcome.

Granular cell tumour is a rare neoplasm that can occur in various sites. This report describes a 48-year-old female with a malignant granular cell tumour originating from the cervical sympathetic nerve trunk, who presented with hyperaesthesia of the left shoulder. The tumour had a maximum diameter of 86 mm and involved adjacent tissues directly.

Histopathologically, it showed considerable variation in the size and shape of nuclei, with occasional mitosis and an abundant granular cytoplasm. Immunohistochemically, the tumour reacted positively for S-100 protein and neuron-specific enolase, indicating its neural origin. Following total local excision of the lesion, the patient has been well without recurrence.

Calcium pyrophosphate dihydrate crystal deposition disease, exhibits several clinical manifestations, from absence of symptoms to severely destructive arthropathy or conditions simulating neoplasm, which is frequently related to the temporomandibular joint. Fifteen of the 31 reported cases of tophaceous pseudogout were found in the head and neck region. A patient presented with a parotid swelling, which initially was suspected to be malignant because of the following findings: radiodensity, progression into the joint, osseous destruction of the major ala of the sphenoid and a fine needle aspirate with crystals, osteoblasts, megakaryocytes and irregular cells of varying size. At surgery there was found a tumour consisting of a white, firm gritty material. It progressed to the skull base where material had to be left, because of the presence of the nerves and vessels. A frozen specimen was reported to be benign. Histological examination showed inflammatory cells, macrophages, a chondroid material with embedded metaplastic chondroid cells and giant cells of foreign body type. Crystal examination of X-ray diffraction revealed calcium pyrophosphate dihydrate.

We report a case of aspiration pneumonitis following the use of papain enzyme in a patient with chronic obstructive pulmonary disease with meat impacted in his oesophagus.

Maxillary sinus hypoplasia (MSH) is occasionally encountered in otorhinolaryngological practice. The hypoplastic sinus is liable to mucus retention, and cases of MSH usually present as a persistent maxillary sinusitis. Endoscopic surgery has been recommended as an effective treatment for the sinus infection. However, MSH is associated with anomalies of the lateral nasal wall which, if not recognized pre-operatively, can lead to inadvertent surgical damage to the orbit. Although some of these abnormalities can be evident endoscopically, the role of imaging in diagnosis and identification of important surgical landmarks is paramount. A posteriorly placed middle meatal antrostomy is recommended as the surgical treatment of choice.

Alveolar rhabdomyosarcoma of the parotid gland is a rare condition. The treatment should involve an aggressive multi-modality approach incorporating surgical removal of the tumour, radiotherapy, and multi-agent chemotherapy. The presentation of this condition along with the most appropriate line of treatment is discussed.