Pediatric parapharyngeal lesions: Criteria for malignancy Lobna El Fiky a, *, Tamer Shoukry b, **, Ossama Hamid c,1 a Ain Shams University, 48 Ibn El Nafees Street, 6th District, Madinet Nasr, 11371 Cairo, Egypt b Ain Shams University, 15 El Khalifa El Maamoun Street, Heliopolis, Cairo, Egypt c Ain Shams University, 50 El Hegaz Street, Heliopolis, Cairo, Egypt 1. Introduction Parapharyngeal tumors are rare tumors of the head and neck located in a complex anatomical region [1–4]. Nonetheless, the parapharyngeal space (PPS) is of particular importance, for the diversity of structures it houses and thus the varied nature of the tumors it can contain [1,2,5,6]. The clinical presentation of these tumors is always subtle, which hinders early diagnosis with a standard physical examination [1,3,7]. A lesion in the PPS can arise primarily within the space or more commonly extend secondarily from the surrounding fascial compartments or can represent metastatic disease [2,5,8,9]. Most available studies in the literature discuss PPS tumors in the adult population. In children, there are a wide variety of tumors that can be found as miscellaneous case reports [10–14]. Unlike adults, there are differences in pathomorphologic spectrum of pediatric PPS tumors [15]. The salivary gland tumors and paragangliomas that dominate in adult population are extremely rare in pediatric population where the most common tumor to be encountered is that of neurogenic origin [14]. Both benign and malignant tumors may arise from any structure contained within the PPS in adults as well as pediatric population [6,12,16,17]. Nevertheless, the diagnostic and surgical challenges of PPS lesions in children are much more delicate than in adult population [10,18] (Table 1). With the advent of CT scan and MRI, the radiological evaluation of the PPS lesions has advanced markedly providing an improved understanding of the anatomy and allowing improved differential diagnosis based on characteristic displacement of fascial planes [7,9,19,20]. Imaging studies of this region must be obtained before attempting biopsy or excision, as vascular lesions may present in the PPS [21]. International Journal of Pediatric Otorhinolaryngology 77 (2013) 1955–1959 ARTICLE INFO Article history: Received 5 April 2013 Received in revised form 10 September 2013 Accepted 13 September 2013 Available online 1 October 2013 Keywords: Parapharyngeal space tumors Pediatric head neck malignancy Neurogenic tumors Metastatic head neck tumors ABSTRACT Introduction: Parapharyngeal space (PPS) pediatric lesions represent a heterogeneous group of uncommon neoplasms of both benign and malignant nature. These tumors tend to be locally advanced by the time they are diagnosed; consequently, an early presumptive diagnosis of malignancy is of utmost importance. Objective: To highlight the occurrence of malignant PPS tumors in pediatric population, to determine their incidence and to describe probable warning clues for diagnosis. Patients and methods: The charts of 23 pediatric patients with PPS swellings were retrospectively reviewed to analyze clinical and pathologic data. We describe possible criteria to suspect malignancy by clinical and radiological examination. Results: Twenty-three patients presented with neoplastic lesions in the PPS, benign tumors in 43.5% and malignant in 56.5%. Malignancy was suspected in 13 cases according to combined clinical and radiological criteria: a painless intraoral and neck swelling in children below one year old, presentation with cranial nerve palsy, nasopharyngeal mucosal or sub-mucosal irregular lesion, the presence of multiple lymph nodes with no evidence of infection, and adjacent bony skull base destruction with intracranial extension. Conclusion: Occurrence of malignancy in PPS tumors in the pediatric population is not uncommon. Clinicians should deliberately look for associated secondary signs that predict malignancy. The surgeon must understand the pathological spectrum of tumors of this space and the proper use of imaging studies to make a preoperative diagnosis. This allows for planning of a sound surgical approach and a proper preoperative counseling. ß 2013 Elsevier Ireland Ltd. All rights reserved. * Corresponding author. Tel.: +20 2 2741840; fax: +20 2 6708390; mobile: +20 127461137. ** Corresponding author. Tel.: +20 1007233186. E-mail addresses: elfikylobna@hotmail.com, elfikylobna@gmail.com (L. El Fiky), tamshok2008@hotmail.com, tamshok@yahoo.com (T. Shoukry), ossamahamid@yahoo.com (O. Hamid). 1 Tel.: +20 1222145199/128487849; fax: +20 2 26394964. Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.09.010