Abstract
Soft tissue chondromas are uncommon benign tumors found mostly in the hands and feet and rarely reported in the pediatric population. In this case presentation we describe a 10 year old boy who had an MRI for facial paralysis due to Ramsey Hunt Syndrome, which incidentally revealed a parapharyngeal mass. He underwent transoral resection of the mass without complication, and histopathology confirmed the diagnosis of soft tissue chondroma. This case is unique due to the unusual location of the tumor and its presentation in a child.
1
Case presentation
A 10 year old male presented with a two week history of an acute onset painful vesicular rash involving the left auricle and left side of his face over the third trigeminal nerve distribution. These symptoms progressed to severe left-sided otalgia, vertigo, and left-sided facial paralysis. He denied hearing loss, otorrhea, dysphagia, or odynophagia. He was diagnosed with Ramsay Hunt Syndrome and treated with acyclovir and steroids. An MRI brain was obtained, which revealed an incidental mass in his left parapharyngeal space ( Fig. 1 A ). Our department was consulted and a CT scan was ordered, revealing a 48 × 38 × 18 mm, well-circumscribed lesion of the parapharyngeal space between the constrictor muscles and pterygoids with multiple calcifications ( Fig. 1 B). It was decided that he would undergo excision of this tumor following the resolution of his herpes zoster infection to avoid exacerbation of his infection from surgical stress. In addition, the patient elected to undergo bilateral tonsillectomy for chronic tonsillitis. During the two-month interval before surgery, he developed mild dysphagia and globus sensation in the throat.
Resection of the left parapharyngeal mass was performed through a transoral approach. The patient was given preoperative clindamycin and dexamethasone. After removal of the left tonsil, the anterior tonsillar pillar was retracted laterally. An incision was made through the pharyngeal constrictor with electrocautery, revealing the well-encapsulated, somewhat lobulated mass extending inferolaterally just lateral to the pharyngeal constrictors ( Fig 2 A, B ). No significant nerves or blood vessels were encountered, and the mass was completely removed without difficulty. The wound was irrigated with antibiotics, and there was no evidence of residual tumor. The pharyngeal constrictors were re-approximated and the mucosa was closed primarily.
Pathology revealed a 54 × 45 × 17 mm nodular tan and pink encapsulated mass with multiple cartilaginous nodules, consistent with a soft tissue chondroma ( Fig. 3 A, B ). At his one-week post-operative visit, patient was doing well with no complaints of voice changes, fever, or swelling. Two years following surgery, there are no problems reported with swallowing.
2
Discussion
Soft tissue chondromas are benign, relatively rare cartilaginous lesions, with over ninety percent located close to tendon or joint capsules in the hands and feet . Since their first discovery by Morgan in 1842, only about 150 cases have been reported in the head and neck region, most commonly in the tongue and rarely in the cheek, parotid gland, masseter muscle, masticatory space, parapharyngeal space, and neck . There has only been one other case of a parapharyngeal chondroma reported in the literature, which occurred in a 20 year old man with a two-year history of gradual sensation of swelling of the pharynx with increased snoring . These tumors usually occur in adults in the fourth through sixth decades and are very rare in young children. In the literature there are less than ten pediatric cases of soft tissue chondromas, with most occurring in close proximity to joint spaces of the back, axilla, hand, hip, or knee . One previous case reported a soft tissue chondroma of the neck of a 5 year old extending from the thyroid cartilage to the sternal notch . To our knowledge this is the first report of a parapharyngeal soft tissue chondroma in a pediatric patient.
The etiology of extraskeletal chondromas is unknown. One theory is that these lesions arise from residual embryonal tissue in an area of preexisting fetal cartilage. The other belief is that they arise from metaplasia of pluripotential mesenchymal cells, which occurs due to some type of irritating stimulus . Since our patient’s lesion was discovered incidentally due to an unrelated illness, the pathology of his tumor is uncertain.
The signs and symptoms of head and neck chondromas depend on the tumor’s size, location, and growth rate. These tumors usually present as slow growing, firm masses that may be asymptomatic or cause pain and symptoms related to compression of nearby structures. When located in the neck, symptoms of upper airway obstruction can occur such as snoring or dyspnea . In children, this could be mistaken for asthma . Our patient’s tumor was discovered incidentally after he presented with Ramsey Hunt Syndrome. Although he was initially asymptomatic at presentation, he later developed mild dyspnea and a globus sensation in the throat.
Radiographic investigation of soft tissue chondromas shows well demarcated, extraosseous soft tissue masses. On CT they frequently appear as circumscribed, heterogeneously enhancing masses with chondroid calcifications, which are punctate, curvilinear, or ring-like. On MR imaging, these tumors are multilobulated with low to medium signal on T1-weighted images and high signal on T2-weight images . In our case, imaging revealed a well-circumscribed lesion of the parapharyngeal space between the constrictor muscles and pterygoids with multiple calcifications.
Soft tissue chondromas should be treated by wide local excision, owing to the 10–15% recurrence rate . A parapharyngeal space tumor can present a challenge to the surgeon regarding the appropriate surgical approach. There are four main ways to approach the parapharyngeal space including transoral, transcervical, transparotid, or transmandibular. The transoral approach has been favored in recent years because of its good exposure and little intra-operative hemorrhage . Kazeki and Nameki recently characterized a standardized method for selecting a surgical approach to benign pharyngeal space tumors based on preoperative imaging. They recommend the transoral approach for small tumors localized in the middle portion of the anterior parapharyngeal space and those not palpable in the neck or parotid region . In our patient, the mass was removed completely with the transoral approach with no harm to adjacent structures and minimal blood loss.
In conclusion, this case is remarkable not only for the patient’s young age but also for its unusual presentation and location in the parapharyngeal space. The mass was successfully removed by an uncomplicated perioral approach, and patient has remained asymptomatic without recurrence.
Acknowledgment
We would like to thank William P. Daley, MD for his assistance with the pathology imaging.
Author Attribution: Erin Smith wrote this manuscript. Luminita Rezeanu wrote pathologic descriptions. Jeffrey Carron reviewed and approved the content of the final draft. This case report has not been previously published in the literature. The authors have not received any financial support to complete this research.
Conflicts of Interest: None of the authors have conflicts of interest to disclose.
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