Alessandra Rinaldo

A review of neuroendocrine neoplasms of the larynx: update on diagnosis and treatment.

Neuroendocrine neoplasms of the larynx have been divided into those of epithelial or neural origin. The latter consist of paragangliomas while the epithelial origin group can be divided into the typical and atypical carcinoids and small cell neuroendocrine carcinomata, the latter consisting of the oat cell type, the intermediate cell type and the combined cell type. There are now over 500 cases of neuroendocrine neoplasms of the larynx in the literature. The diagnosis is primarily based on light microscopy, and, in some instances, it may be supported by special histochemical studies. It should be confirmed by immunocytochemical and/or ultrastructural investigation. The different biological behaviour of neuroendocrine neoplasms of the larynx makes a specific diagnosis of paramount importance, since treatment depends on diagnostic accuracy. Typical carcinoid is an extremely rare lesion. It is treated preferably by conservative surgery; elective neck dissection is not necessary because of the lack of lymph node metastases at diagnosis. Chemotherapy and/or radiotherapy have not been effective in the limited number of patients treated thus far. Prognosis is excellent with cure following surgery. Atypical carcinoid is the most frequent non-squamous carcinoma of the larynx. The mainstay of treatment is surgery. Elective neck dissection should be performed because of the high likelihood of cervical lymph node metastases. Primary radiation therapy with adjuvant chemotherapy is not indicated. The survival rate is 48 per cent at five years and 30 per cent at 10 years. Although the larynx is one of its most common extrapulmonary sites, small cell neuroendocrine carcinoma is still a rare tumour. Surgical results for this tumour have been disappointing and is reserved for cases of local relapse with no evidence of metastasis. Chemotherapy and radiotherapy currently appear to offer the least disabling and most effective forms of therapy. The two- and five-year survival rates are 16 per cent and five per cent, respectively. Paraneoplastic syndromes have occasionally been reported in association with carcinoid tumours (typical and atypical) and small cell neuroendocrine carcinoma. There have been also rare reports of an elevated neuropeptide serum level. Paraganglioma is the only laryngeal neuroendocrine neoplasm with a female preponderance (3:1). Confusion with atypical carcinoid has led to incorrect diagnosis and inappropriate classification schemes, erroneously suggesting that laryngeal paraganglioma has the potential for aggressive behaviour. Conservative surgery represents the treatment of choice; elective neck dissection is not necessary, and the prognosis is excellent.

Synovial sarcoma of the larynx and hypopharynx

Synovial sarcoma represents a mesenchymal malignancy of unknown histogenesis that most often occurs in the lower limbs of young adults. The head and neck region is a relatively rare location, in which the hypopharynx and larynx are, respectively, the most and least often affected anatomic sites. Histologically, synovial sarcomas are classified into monophasic and biphasic variants. Immunohistochemistry plays a major part in the differential diagnosis, enabling the demonstration of epithelial differentiation. Both monophasic and biphasic synovial sarcomas are characterized cytogenetically by the reciprocal translocation t(X;18)(p11.2;q11.2) between chromosomes X and 18. Two cases of synovial sarcoma arising in the larynx and in the hypopharynx and in which cytogenetic analysis detected a diagnostic t(X;18) chromosome aberration are reported here.

Is primary radiotherapy an appropriate option for the treatment of verrucous carcinoma of the head and neck

The literature on verrucous carcinoma of the head and neck was reviewed to analyse the use of primary radiation therapy in the treatment of this lesion. The results emphasize an overall local control rate of 43.2 per cent, and 6.7 per cent of true anaplastic transformation following irradiation. Diagnosis remains the fundamental problem: without a correct diagnosis of verrucous carcinoma, no correct treatment can be applied.

Level I dissection for laryngeal and hypopharyngeal cancer: is it indicated?

Squamous cell carcinoma of the larynx and hypopharynx tends to metastasize frequently to cervical lymph nodes, the location of which depends mainly on the site of the primary lesion. Five anatomical levels of cervical nodes have consequently been defined to standardize the terminology used to describe which lymph node groups are at risk for metastatic spread. Level I includes the submental and submandibular triangles. This review considers the role of these triangles in neck dissection and concludes that, unless there is clear evidence of spread, the inclusion of the level I triangles in the neck dissection is unwarranted since these nodes are not really at risk. There is therefore an important role for selective neck dissection in suitable cases of squamous cell carcinoma of the larynx and hypopharynx.

Selective lateral neck dissection for laryngeal cancer with limited metastatic disease: is it indicated?

The most important prognostic factor in cancer of the larynx is the presence of cervical metastatic disease, which is the most common type of recurrence in such patients. Because micrometastases cannot be detected pre-operatively at present, selective lateral neck dissection is increasingly recommended as the standard treatment for patients with a clinically negative neck in order to reduce the recurrence rate. In cases of N+ disease, selective lateral neck dissection can be as valid as modified radical neck dissection, providing patients have only limited, occult metastatic disease.

Selective lateral neck dissection for laryngeal cancer in the clinically negative neck: is it justified?

Selective lateral neck dissection is a recently-introduced surgical procedure for the treatment of cervical lymph nodes believed to be at risk of metastasis from primary malignant neoplasms of the upper respiratory and digestive tracts. Its value in the management of the clinically negative neck in cancer of the larynx is discussed.

Impact of phenotype on treatment and prognosis of laryngeal malignancies.

An overview of the impact of the phenotype on treatment and prognosis of different laryngeal malignancies is presented.

Book Reviews / Erratum

The updated fourth edition of the Color Atlas of ENT Diagnosis is an illustrated first introduction examination, diagnosis and therapy of the most frequent ear, nose and throat diseases. The more than 500 good-to-excellent color pictures are of great help to the beginner to become familiar with the conditions typically seen in the ENT field. The text is divided into five main sections: examination; ear; nose and face; pharynx and larynx; head and neck. As the stated purpose of this small pocket book is not to be a textbook but a pictorial survey, the majority of the book is reserved for the excellent pictures. Although most of the color photographs are self-explanatory every illustration is accompanied by a short descriptive text. Moreover, small paragraphs introduce special issues like laryngectomy or audiologic examination. I suggest a reference list for additional reading. All in all this small pocket atlas will be of great help to medical students, ENT trainees and general practitioners seeking a quick reference during daily practice. Elmar Oestreicher, Munich Maria Sanna, Hiroshi Sunose, Fernando Mancini, Alessandra Russo, Abdelkader Taibeh

Book Review and Announcement

A group of 17 well-known specialists from the UK (11), USA (3), Canada, Ireland and the Netherlands contributed to this very useful book. In 11 chapters, all problems of education and managemant of deaf children are discussed in detail. The content of the book is divided into three sections, which are relatively well balanced. A wide spectrum of audiometric methods, correct diagnostics of hearing loss and rehabilitation difficulties, especially of multiply handicapped deaf children, is described in the first chapter. Multiply handicapped children are characterized by their high heterogeneity with many sensory, physical, emotional, intellectual, social and educational needs. The main classes with typical problems in hearing assessment and aural rehabilitation are deaf-blind children, deaf children with severe learning disability, Down’s syndrome children with a high incidence of hearing impairment, deaf children with cerebral palsy and autistic children. The second section of the book entitled ‘Listening/Learning Devices’ refers precisely to everything concerning hearing aids, earmoulds, cochlear implants and tactile aids. Teachers must have the skills and knowledge so that they can provide the best possible support for both the hearing-aided and cochlear-implanted children in their care. The efficient use of all kinds of aids for deaf children will be heavily dependent on the audiologist and teacher of the deaf child in ensuring that an appropriate programme of training is implemented. The chapters of the third section entitled ‘Knowledge and Practice’ describe how the deaf child can be provided with good management which will involve the co-operation of parents and families, educational audiologists as well as teachers, support assistants and of course the children themselves. The teacher of the deaf has the responsibility to set child-centred goals that will lead to the optimum development of listening, language and learning. Effective use of residual hearing is not only a question of finding the right hearing aid, the amplification system for the right context is very important too. An overview of all modern technological aids for school-rooms helps to inform about the possibility how to prepare a friendly soundscape and how to better the listening environment. The acoustic conditions of the rooms can basically influence the listening to and understanding of speech. Therefore the auditory environment is highlighted along with the positive influence of the use of music and structured listening programmes. The residual hearing can be maximized by providing high-quality amplification, a favourable acoustic environment and planned opportunity to actively develop raised auditory awareness. A long list of references is to be found at the end of each chapter of the book. The book Audiology in Education provides an essential resource for all professionals involved with diagnostics, rehabilitation and educational management of deaf children. From me the best recommendation only. Anton Kollár, Brno