Sarcoidosis of the larynx in a child

Sarcoidosis is a multisystem disease of unknown etiology characterized by non-caseating granulomatous inflammation of various organs, but most frequently involving the lungs of young adults. Sarcoidosis is rare in the pediatric age group, however numerous extensive reviews have been published. The most commonly seen initial manifestations in childhood are non-specific constitutional symptoms such as lethargy, fatigue and malaise, followed by cough, dyspnea, fever, weight loss, and lymphadenopathy in order of decreasing frequency. The diagnosis is one of exclusion and is established when clinical and radiological findings are supported by histological evidence of widespread non-caseating epithelial cell granulomas in more than one organ, or a positive Kveim test. Laryngeal involvement is usually part of the systemic disease, but isolated laryngeal sarcoidosis has been reported in adults. We report here a case of isolated laryngeal sarcoidosis in a 13 year old girl. The differential diagnosis and management are discussed.     

Malignancy of the larynx in a child

Squamous cell carcinoma of the larynx in children is rare. The management of laryngeal malignancy is more difficult in children than adults for several reasons: the aggressive nature of a tumor that is often diagnosed late in children; the delicacy of pediatric anatomic structures; intraoperative blood loss; long-term post-treatment complications; and psychological factors particular to children. A tracheostomized 13-year-old boy came to us with a 4-month history of hoarseness, breathing difficulty, and swelling in the neck. A detailed examination revealed that a transglottic tumor had infiltrated the thyroid and cricoid cartilage, the upper two tracheal rings, and the thyroid gland. Such an infiltration has not been previously reported. The lesion proved to be a well-differentiated squamous cell carcinoma. We performed a wide-field total laryngectomy, which was followed by radiotherapy. Unfortunately, the child survived only 3 years postoperatively.     

Mucoepidermoid carcinoma of the larynx in a child

Laryngeal carcinoma in childhood is rare and most commonly classified as squamous cell carcinoma. Although mucoepidermoid carcinoma of the larynx has been noted in adults, it has not previously been reported in a child. A 13-year-old boy presented to our department with an epiglottic mass which was determined histologically to be mucoepidermoid carcinoma. A total epiglottectomy was performed and one year postoperatively the child is without disease.     

Plexiform neurofibroma of the larynx in a child

A case of a female child of six years of age with a plexiform neurofibroma of the larynx caused by von Recklinghausen's disease is presented. Laryngeal involvement in neurofibromatosis type 1 (NF1) is rare and only 19 paediatric cases have been reported. The tumour was biopsied and lateral pharyngotomy with supraglottic hemilaryngectomy was performed in order to relieve obstructive symptoms. Problems related to this unusual tumour localization are discussed and a review of the literature is presented.     

Carcinoma in situ of the larynx

A retrospective study of carcinoma in situ of the larynx at the British Columbia Cancer Institute indicates that radiotherapy, using a tumoricidal dose of Co 60, is the treatment of choice for this condition. Between 1940 and 1972, 43 patients with carcinoma in situ of the vocal cords were seen. A follow-up of five years or more was possible in 28 of these cases. Twenty-two were treated primarily with a tumoricidal dose of radiotherapy. Twenty-one of the 22 were free of disease for at least five years. This study, therefore, shows a five-year cure rate of almost 100 percent for patients treated with radiotherapy. It also brings out two further points regarding carcinoma in situ of the larynx; namely, an apparent increase in its incidence, and the presence of co-existing invasive carcinoma in some cases. We feel that since the incidence of laryngeal carcinoma has not increased, this apparent increase probably represents a greater awareness by both the pathologist and the clinician. We have also achieved more accurate diagnosis since the introduction of routine microlaryngoscopy. The single radiotherapy failure in our series was due to failure to diag: Nose co-existing invasive carcinoma. This would seem to be the most likely cause of similar failures reported in the literature. Carcinoma in situ should be managed as follows:

• 1 Any patient found to have carcinoma in situ on laryngeal biopsy must have careful microlaryngoscopy with examination of the hypopharynx, larynx, subglottic larynx, and upper trachea.
• 2 If the lesion is small and confined to one cord, complete stripping of that cord is indicated.
• 3 If both cords are abnormal, stripping and/or careful multiple biopsies are essential.
• 4 If a small localized lesion is found, repeat cord stripping is performed in one month.
• 5 If any biopsy reveals invasive carcinoma, the patient must not be classified as a case of carcinoma in situ.

     

Carcinoma in situ of the glottic larynx

Carcinoma in situ (CIS) is part of the histopathologic spectrum of laryngeal disorders where invasive squamous cell carcinoma is the endpoint of cellular disarray. Few reports consider prognostic indicators that predict which lesions become invasive. Forty-one patients with CIS of the glottic larynx were analyzed for risk factors that would predict invasive cancer. Anterior commissure involvement by CIS resulted in 92% conversion to invasive squamous cell cancer compared to 17% of lesions limited to the mobile fold. Epidermal growth factor receptors were also analyzed and were found not to be helpful in predicting invasion. Lesions of the mobile fold should be removed endoscopically and the patient should be observed closely for recurrence. Anterior commissure involvement that is inaccessible to complete laser ablation should be radiated, and the patient should be observed carefully.