Malignancy of the larynx in a child.

Abstract

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 widefield total laryngectomy, which was followed by radiotherapy. Unfortunately, the child survived only 3 years postoperatively.

Introduction

Head and neck malignancy is more common in adults than in children. Nevertheless, physicians must be aware that a small percentage of cases does occur in the pediatric age group. The first reported case of squamous cell carcinoma in a child was published in 1868 by Rehn (the patient was a 3-year-old boy). [1] Since then, 57 other cases have been reported. [2] In this article, we report a new case of pediatric squamous cell carcinoma, which was treated with surgery and radiotherapy.

Case report

A tracheostomized 13-year-old boy came to us with a 4-month history of hoarseness, breathing difficulty, and swelling in the neck. The patient had earlier undergone tracheostomy for stridor. Thereafter, he had undergone direct laryngoscopy and biopsy at another center; histopathology identified only an angiomatous polyp.

Our examination revealed that the lumen of the patient's metal tracheostomy tube was compromised by a tumor mass. Indirect laryngoscopy identified the growth as a smooth, pink, exophytic mass, which was located below the level of the epiglottis and which covered the entire glottis and supraglottis. On inspection and palpation of the neck, a diffuse, hard, tender, 5 x 6-cm area of swelling was noted in the front (figure 1). The lesion involved the thyroid and cricoid cartilage, the upper two tracheal rings, and both lobes of the thyroid gland. No neck nodes were palpable.

We made a clinical diagnosis of a transglottic malignancy. The tumor was staged as a T4 NO MX stage IV malignancy. A lateral neck x-ray detected a soft-tissue mass that involved the larynx and trachea and extended from the C3-C7 vertebral bodies. On direct laryngo-pharyngoscopy under general anesthesia, a large smooth mass in the supraglottic region could be seen obscuring the inlet of the larynx. Both the pyriform fossa and the postcricoid region were normal. The mass bled on touch. Histopathologic examination of a biopsy specimen identified a well-differentiated squamous cell carcinoma.

After receiving motivational counseling, the patient was taken for surgery under general anesthesia. A widefield total laryngectomy with a total thyroidectomy and removal of the upper three tracheal rings was performed (figure 2). A drain was inserted and the wound was closed (figure 3). Examination of the excised specimen showed that a transglottic tumor had involved the upper two tracheal cartilages, the laryngeal cartilage, and the thyroid gland (figure 4). Histopathologic examination again identified a squamous cell carcinoma (figure 5). The patient was put on thyroxine and calcium, and he was discharged on postoperative day 15 (figure 6). Two months later, he received 6,000 cGy of radiotherapy. Although the boy stopped attending school, he did learn esophageal speech. Unfortunately, he died 3 years postoperatively as the result of a local recurrence.

Discussion

Carcinoma of the larynx is most common in adults aged 55 to 75 years; it is rare in children. When it does occur in children, it is most common in those aged 11 to 15 years, followed by those aged 6 to 10 years; it is least common in children aged 1 to 5 years. [1,3] In adults, 90% of cases affect men; in children, the male-to-female ratio is 3:2. [1,3]

It has been noted that squamous cell carcinoma is the most common type of malignancy seen in children. [1,2] Mucoepidermoid carcinoma, rhabdomyosarcoma, and non-Hodgkin's lymphoma have also been reported in children. [2,4]

Predisposing factors. An extensive review of the literature indicated that the principal predisposing factor for laryngeal carcinoma is irradiation of benign head and neck lesions, particularly juvenile laryngeal papillomas. [2] Other known risk factors include active and passive smoking, exposure to certain chemical agents (e.g., asbestos), and a family history of malignant tumors. Immunodeficiency can impair the control of human papillomavirus (HPV) infection, and coinfection with HPV 18 and HPV 33 is not uncommon in cases of laryngeal cancer. [5]

Diagnosis. When they seek treatment, most affected children have an upper airway obstruction, hoarseness, cough, and dyspnea. The correct diagnosis of a laryngeal carcinoma is largely dependent on a high index of suspicion when interpreting these symptoms. The close clinical resemblance of this disease to more common and benign childhood conditions often leads to diagnostic and therapeutic delays. For example, diagnosis can be delayed when symptoms are attributed to normal pubertal vocal changes or upper respiratory tract infections. [2]

Most affected children do not have neck nodes; those who do can be misdiagnosed as having inflammatory cervical lymphadenopathy. McGuirt and Little reported one case in which a child developed a cervical metastasis. [2] Our patient did not have a neck metastasis. Rather, the 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 tumor's invasion of the tracheostoma was blamed on the considerable delay in the diagnosis of the malignancy. In earlier reported cases, the principal site of origin was the vocal folds; other sites were the supraglottic and subglottic regions. Our patient had a transglottic malignancy.

To avoid any delay in diagnosis, it is advisable to examine any child with a suspected malignancy by direct laryngoscopy (rigid or flexible) under general anesthesia and to obtain a biopsy analysis of any suspicious lesion. Radiographic studies of the bone, liver, and spleen and abdominal ultrasonography can also be performed to search for metastasis. Computed tomography and magnetic resonance imaging can assist in staging, and they are also helpful in following the response to therapy. [1,2]

Treatment. The extent of treatment depends on the tumor's stage, the presence or absence of laryngeal cartilage involvement, and the presence or absence of metastasis. Preservation of laryngeal function is a primary consideration. The use of surgery, radiotherapy, and chemotherapy--alone and in various combinations--has been described by various authors. However, successful management and the attainment of good long-term outcomes are hindered by several problems relating to the operative procedure, blood loss, complications, and certain psychological factors:

Operative procedure. Laryngectomy in children is more difficult than in adults because a child's structures are more delicate and the working space is limited.

Blood loss. The amount of blood loss in a child relative to body weight is an important consideration, and rigorous attention should be paid to minimizing it.

Complications. Children who undergo radiotherapy encounter more complications than do adults, primarily because they live so much longer following treatment. [3] The surgeon must anticipate that the growth of irradiated structures might be arrested, that abnormalities might occur in the patient's offspring, and that the patient might develop a secondary neoplasm.

Psychological aspects. It is most difficult to explain to young patients the nature of their disease, the type of operation that is going to be performed, and its aftereffects.

Once a tumor involves the cartilages of the larynx and thyroid, surgery and postoperative irradiation is the treatment of choice. Malignancies of the larynx are more aggressive in children than in adults, probably because they are recognized at a later stage. As a result, many affected children have a poor prognosis.

References

(1.) Zalzal GH, Cotton RT, Bove K. Carcinoma of the larynx in a child. Int J Pediatr Otorhinolaryngol 1987;13:219-25.

(2.) McGuirt WF Jr., Little JP. Laryngeal cancer in children and adolescents. Otolaryngol Clin North Am 1997;30:207-14.

(3.) Gindhart TD, Johnston WH, Chism SE, Dedo HH. Carcinoma of the larynx in childhood. Cancer 1980;46:1683-7.

(4.) Mitchell DB, Humphreys S, Kearns DB. Mucoepidermoid carcinoma of the larynx in a child. Int J Pediatr Otorhinolaryngol 1988;15:211-5.

(5.) Simon M, Kahn T, Schneider A, Pirsig W. Laryngeal carcinoma in a 12-year-old child. Association with human papillomavirus 18 and 33. Arch Otolaryngol Head Neck Surg 1994;120:277-82.