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Introduction Pediatric cystic sublingual masses often present a diagnostic dilemma for practitioners. Though uncommon, dermoid or epidermoid cysts can present in the sublingual space at any age and are often misdiagnosed as an inflammatory pseudocyst (ranula) or lymphatic malformation. Imaging may not always identify the underlying etiology, requiring physicians to maintain a high index of suspicion for these relatively rare oral cysts. Objectives To describe the presentation and treatment of sublingual dermoid and epidermoid cysts presenting to a tertiary children's hospital over 20 years. Methods A retrospective review of all pathology specimens identified as dermoid or epidermoid cysts within the sublingual space from 1999 to 2019. Patient charts were then reviewed for relevant clinical, imaging, and operative data. Results Twelve pediatric patients were identified (8 female, 4 male) with a mean age of 7.2 years (SD 5.6). Eighty six percent (6/7) of dermoid cysts were found in female patients, while 60% (3/5) of epidermoid cysts were in male patients. Multiple dermoid and epidermoid cysts were each found in one patient (8%). Two epidermoid cysts presented in the neonatal period. Preoperative diagnosis included nondiagnostic “cystic mass” (33%), ranula (25%), lymphatic malformation (LM) (17%), and dermoid/epidermoid cyst (17%). Two thirds of patients (8/12) underwent imaging, with all receiving either MRI or CT. Although MRI was the most likely to suggest the possibility of a dermoid/epidermoid cyst (2/4), ranula was the most common primary radiographic diagnosis (5/8). One patient underwent sclerotherapy for presumed LM one year prior to surgical excision of the cyst. Eleven patients (92%) underwent intraoral excision, one (8.3%) underwent a combined intraoral/extraoral approach. Conclusions To our knowledge, this review represents the largest case series of pediatric sublingual dermoid and epidermoid cysts to date. This series contained higher levels of epidermoid cysts and female patients than previously reported in the literature. Identifying more dermoid cysts in females and epidermoid cysts in males is also a new finding. MRI was superior to CT and US regarding the presence of a dermoid/epidermoid cyst. Frequently misdiagnosed, it is important to consider these relatively rare pathologies when treating children presenting with sublingual masses in order to avoid delayed and/or inappropriate treatment.

Introduction Pleomorphic myxoid liposarcoma is a rare and aggressive cancer seen in the pediatric population that has been previously associated with hereditable cancer disorders like Li Fraumeni syndrome. We present a case report and review of the relevant literature. Case presentation Pleomorphic myxoid liposarcoma presenting as a second primary tumor in a child with a strong family history for cancer led to diagnosis of Li-Fraumeni syndrome, which is associated with TP53 tumor suppressor gene inactivation. Management and outcome The tumor was fully excised, but postoperative radiation was deferred to limit future radiation-induced tumorgenesis. Discussion Pleomorphic myxoid liposarcoma is rare but aggressive, and should prompt caregivers to test for potential hereditable cancer disorders. Li-Fraumeni syndrome is associated with early onset neoplasia and development of recurrent primary tumors. Its presence affects treatment decisions and methods of surveillance. Chemoradiation should be used judiciously in this population.

Summary Objective: To understand the clinical presentation and management of salivary gland anlage tumor (SGAT). Design and methods: This case series includes a report of a newborn male who presented with acute airway obstruction secondary to a nasopharyngeal mass which was discovered in the course of the clinical evaluation. Six additional cases of SGAT from the pathology consultation files of one of the authors (LPD) presenting in similar fashion are also included. The relevant literature from 1966 to the present has been reviewed through a Medline keyword search utilizing terms “salivary gland anlage tumor”, “neonatal”, and “nasopharynx.” Results: Endoscopic evaluation identified a nasopharyngeal mass tethered to the posterior septum. Although CT and MRI were helpful in identifying the mass and excluding involvement of the surrounding structures, the imaging characteristics of the mass itself were nonspecific. The patient was taken to the operating room and the polypoid mass was removed transorally after lysis of its septal attachment. Pathologic examination revealed a SGAT, a recently described entity in neonates and young infants, who present with early onset respiratory distress. Since the initial report of nine cases by one of the co-authors (LPD), seven additional cases including the present one have been seen in consultation. Conclusions: Salivary gland anlage tumor of the nasopharynx is a rare cause of neonatal airway obstruction. Endoscopic evaluation and imaging studies are helpful in the exclusion of other etiologies, some of which may have intracranial extension. Simple excision has been curative to date. There have been no reported recurrences in any of the previously studied cases with clinical follow-up dating more than 5 years.

Lipofibromatosis is a recently described, benign neoplasm that presents in the pediatric population [J.F. Fetsch, M. Miettinen, W.B. Laskin, M. Michal, F.M. Enzinger, A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis, Am. J. Surg. Pathol. 24 (2000) 1491-1500]. It is a rare soft tissue tumor histologically distinct from other fibromatoses such as juvenile fibromatosis, fibrous hamartoma of infancy, calcifying aponeurotic fibroma, and lipoblastoma. Distinguishing histopathologic features of lipofibromatosis include abundant and disorganized adipose lobules traversed by bundles of spindled fibroblast-like cells. It most commonly presents in the extremities. We present a case involving a young girl, which we believe represents the first report of lipofibromatosis involving the neck.

Objective: To evaluate the frequency of intracranial and orbital complications occurring as simultaneous but separate complications of acute rhinosinusitis in the pediatric population. Methods: Records of children admitted to St. Louis Children’s Hospital between 1 January 1990 and 31 December 2002 were reviewed. Relevant literature was reviewed with the assistance of Medline. Results: We identified 74 patients with orbital complications related to acute rhinosinusitis. A bimodal age distribution was identified with one group above and one group below the age of 7 years. The frequency of intracranial complications in pediatric patients already admitted for orbital complications of acute rhinosinusitis was 0% for those less than 7 years of age and 9.3% (4/43) for patients 7 years and older. If surgery was required for orbital disease, these risks were 0 and 24% (4/17), respectively. The review found that patients with dual complications had a mean age of 15 years, 11 months, were male (100%), had subperiosteal abscesses located superiorly or superolaterally within the orbit, and presented with significant frontal sinus disease. All intraoperative cultures were polymicrobial. MRI was superior to CT for identification of intracranial extension. Conclusions: Simultaneous intracranial and orbital complications are rare but significant occurrences in acute pediatric rhinosinusitis. Because of the high incidence of intracranial findings, we recommend MRI in addition to CT scan along with aggressive management in children older than 7 years of age admitted with orbital complications of acute rhinosinusitis who also demonstrate risk factors for intracranial disease. These risk factors are discussed.

Summary Objective To identify the otolaryngological manifestations of posttransplant lymphoproliferative disorder (PTLD) in pediatric heart and lung transplant recipients. Methods A 14-year retrospective case series review (1990–2003) of children less than 18 years of age presenting with PTLD after receiving orthotopic heart and lung transplants at St. Louis Children's hospital (St. Louis, MO, USA). Results One hundred ninety seven cardiac and 246 pulmonary transplant patients were included in this review. Thirteen heart transplant patients developed PTLD with 39% (5/13) presenting in the head and neck. Thirty-two lung transplant patients developed PTLD with 25% (8/32) presenting in the head and neck. PTLD in cardiac transplants most often presented as adenotonsillar hypertrophy or cervical lymphadenopathy. The sinonasal cavity was the most common site of head and neck PTLD in pulmonary transplants patients. Conclusions This study suggests that head and neck involvement is more common than currently reported in the literature. As thoracic organ transplants become more frequent in children, otolaryngologists caring for this population should maintain a high index of suspicion for any suggestive findings. Evaluation must include tissue biopsy, as histopathologic examination is required for diagnosis.