PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s annual visual diagnosis competition. This year, in addition to the 10 finalists I will be presenting at the National Conference and Exhibition I will be sharing four cases online in advance of the conference. This is the second of the four cases.

This case was submitted by…

Dr. Walker was assisted on presenting this case by Morgan Bowling, MD, a Pediatric Emergency Medicine Fellow at Newark Beth Israel Medical Center and Children’s Hospital of New Jersey and Ravi Thamburaj, MD, a Pediatric Emergency Medicine Attending Physician at Newark Beth Israel Medical Center and Children’s Hospital of New Jersey.

A 14-year-old male presented with sore throat and difficulty swallowing. His symptoms started approximately one month prior to presentation but had progressively worsened, leading to voice hoarseness and weight loss due to dysphagia. He had been referred to the otolaryngology (ENT) about one month prior from his pediatrician who prescribed a course of oral steroids and cephalexin, which he took as prescribed, with no improvement in symptoms. He had been scheduled for a future tonsillectomy. 

On initial exam, he was tachycardic to the 120s, in mild distress but afebrile, maintaining normal oxygen saturation on room air. He was in distress secondary to pooling of secretions and in moderate pain.  His voice was hoarse. His throat exam was significant for bilaterally enlarged tonsils without exudate or deviation of the uvula. There was no appreciable swelling of the jaw or face but there was some right sided, mildly tender lymphadenopathy. He had no stridor or trismus but did have a “hot potato” voice. The patient had significant pooling of phlegm and saliva. With suctioning of the copious secretions, a tan, irregularly bordered mass was visualized in the posterior pharynx. His lung exam showed clear breath sounds bilaterally. His cardiovascular exam was significant for only tachycardia with a regular rhythm. His abdomen exam was negative for hepatosplenomegaly. Remainder of exam including neurological exam was normal. 

Initial labs were significant for:

  • White blood cell count 18.6 x 103/mcL
  • High hemoglobin and hematocrit, 15.3 g/dL and 46.3% respectively
  • Platelet count was 435 x103/mcL
  • Low MCV of 73.3 fL
  • Renal panel – Sodium was 137 mmol/L, potassium 4.6 mmol/L, chloride 100 mmol/L, Co2 31.0 mm/L, calcium 10.6 mg/dL, glucose 103 mg/mL, BUN 13.0 mg/dL, creatinine 1.040 mg/dL
  • Liver profile – Bilirubin 0.4 mg/dL, albumin 4.0 g/dL, ALT 18Unit/L, AST 11 Unit/L, alkaline phosphatase 142 Unit/L
  • Mononucleosis screen, COVID-19, flu, and rapid strep negative
  • Throat and blood cultures were obtained

Coronal CT of the neck with contrast

Saggital CT of the neck with contrast

Computerized tomography (CT) of the neck with contrast was performed which showed 4.6 x 4.2 x 7.6 cm (AP x TRV x CC) heterogenous lesion anterior to and separate from the prevertebral soft tissues. Multiple vascular structures were seen within this lesion as well as a 3 cm cystic and /or necrotic component. There was significant narrowing of the oropharynx/ airway to approximately 4 mm over approximately 4 cm in length extending inferiorly from the level of the epiglottis. There was no nasopharyngeal, oropharyngeal, or base of the tongue mass. There were no masses seen in the hypopharynx or larynx. The trachea was patent and midline. There were no masses within the parapharyngeal or masticator spaces. There were small bilateral submental (level IA), submandibular (level IB), jugular chain (level II through IV), and the posterior triangle (level V) lymph nodes, which do not meet CT criteria for enlargement. There was a 1 cm spiculated noncalcified solid nodules in the left upper lung lobe. 

Due to findings on the CT scan, ENT was consulted and evaluated the patient at beside in the pediatric emergency department and performed a flexible laryngoscopy which did visualize the mass but could not fully visualize the remainder of the oropharynx but could not clearly delineate the diagnoses but was concerning for possible malignancy.

The patient was initially admitted to the local PICU, and later transferred to a quaternary facility with specialized ENT services where the ultimate diagnosis was made.

A. Peritonsillar Abscess

B. Palatal Torus

C. Synovial Sarcoma

D. Arteriovenous Malformation

E. Lymphangioma

C. Synovial Sarcoma

On the first day of his PICU stay at the outside hospital, the patient was evaluated by the intensivists, otolaryngology and oncologic teams. The decision was made to undergo resection of the mass which they agreed was likely malignant.  Due to the location of the mass, it was decided by ENT that a tracheostomy should be placed to ensure a definitive airway during the procedure. Prior to operation, patient was treated with dexamethasone, allopurinol and rasburicase as recommended by the pediatric oncologic teams. Post procedure, the patient had additional imaging done to determine if any metastases of the primary mass was found. CT of the chest showed a few scattered pulmonary nodules, but no other signs of metastases were noted. Pathology of the mass was found to be a pharyngeal synovial sarcoma. Patient has been started on induction neoadjuvant chemotherapy including doxorubicin and ifosfamide.  

Synovial sarcoma is a malignant mesenchymal neoplasm that represents approximately 5-10% of all soft tissue tumors and the age-adjusted incidence is about 0.81/1,000,000 in children and most commonly occurs in adolescents and young adults, between ages 15-40. These lesions originate from primitive mesenchymal cells and can occur anywhere and is the most common soft tissue sarcoma of the foot. It was originally named for the frequent presentation in the soft tissue spaces around large joints. Head and neck synovial sarcomas are rare, and account for 3-10% of all total cases.

Clinical manifestations vary, but 50% of patients present with pain. Other common findings include dysphagia, dyspnea, hoarseness, and headache. These tumors are slow growing, often increasing in size over 1-2 years, and due to the young age at presentation, they are often misdiagnosed as other pathologies.

The gross pathology is usually tan or grey and either multinodular or multi-cystic. On histology, the masses display monomorphic spindle cell sarcoma with epithelial differentiation.

There is no standard treatment but often includes wide-local excision followed radiation with or without chemotherapy. Survival rate is difficult to determine in the literature, but the 5-year survival rate is through to be estimated around 59-75%, depending on patient age, size, grade, anatomic location, surgical success, and response to adjunct therapies. It commonly metastasizes to the lungs and pleura.

References

Gazendam AM, Popovic S, Munir S, Parasu N, Wilson D, Ghert M. Synovial Sarcoma: A Clinical Review. Curr Oncol.  2021;28(3):1990-1920. Published 2021 May 19. DOI: 10.3390/curroncol28030177.

Thway K, Fisher C. Synovial sarcoma: defining features and diagnostic evolution. Ann Diagn Pathol. 2014;18(6):369-380. doi:10.1016/j.anndiagpath.2014.09.002

Wang Y, Zhu F, Wang K. Synovial sarcoma of the floor of the mouth: a rare case report. BMC Oral Health. 2020;20(1):5. Published 2020 Jan 6. DOI:10. 1186/s12903-019-0961-8.

Bouchet J, Hervé G, Lescaille G, Descroix V, Guyon A. Palatal torus: Etiology, clinical aspect, and therapeutic strategy. Journal of Oral Medicine and Oral Surgery. 2019;25(2):18. doi:10.1051/mbcb/2018040

Manjunath SM, Shetty S, Moon NJ, et al. Arteriovenous malformation of the oral cavity. Case Rep Dent. 2014;2014:353580. doi:10.1155/2014/353580

Kolay SK, Parwani R, Wanjari S, Singhal P. Oral lymphangiomas – clinical and histopathological relations: An immunohistochemically analyzed case series of varied clinical presentations. J Oral Maxillofac Pathol. 2018;22(Suppl 1):S108-S111. doi:10.4103/jomfp.JOMFP_157_17