Introduction Rhabdomyosarcoma (RMS) is the most frequently encountered soft tissue sarcoma in pediatric patients, with 35% arising in the head and neck region. Primary intraosseous rhabdomyosarcomas are however exceptionally rare with fewer than 10 cases identified in the literature. Recent studies describe a distinct group of primary bone RMS with gene alterations involving EWSR1 or FUS. We report an interesting case of a primary spindle cell RMS affecting the mandible of a 6-year-old girl. Case Details The child presented with left facial swelling and pain. Clinically the left mandible was enlarged. An infective cause was suspected and oral antibiotics prescribed, but with no improvement. Computed tomographic mandible imaging showed an aggressive lesion arising from the bone, occupying the masticator space and extending into the lateral skull base. Incisional biopsies demonstrated a high-grade spindle cell neoplasm, associated in part with irregular bony islands. The differential diagnoses primarily included osteosarcoma and rhabdomyosarcoma. The case was sent to a tertiary referral center and a spindle cell rhabdomyosarcoma was confirmed. The possibility of the recently described intraosseous rhabdomyosarcoma with either EWSR1 or FUS gene rearrangements was excluded by FISH. Following MDM discussion, the patient was commenced on neoadjuvant chemotherapy. The patient is awaiting her second cycle of chemotherapy. Conclusions RMS in children has a favorable prognosis with 5-year survival rates of > 70% reported. Prognosis is determined by clinical and biological factors with spindle cell RMS considered to be intermediate risk. Better prognosis is also described in fusion-negative cases, with a multimodality treatment approach typically adopted

Introduction Olfactory neuroblastoma (ONB) is an uncommon malignant neuroectodermal sinonasal tumor, which comprises approximately 2% to 3% of all sinonasal tumors. These tumors originate from specialized sensory neuroepithelial/neuroectodermal olfactory cells and occur most frequently in the upper nasal cavity, in the region of the cribriform plate. All age groups are affected, and patients often present with locally advanced disease resulting from delay in presentation. This is attributed to the slow growth by tumors and nonspecific symptomatology. Objective To evaluate the histologic features and management outcomes for patients with ONB at our institution. Results Over a 10-year period (2009-2019) 6 patients were identified with ONB. The majority of tumors (n = 4) were locally advanced at the time of presentation with extension beyond the nasal cavity and paranasal sinuses (Kadish stage C). One tumor was stage B, and one was stage A. Three tumors were Hyams grade I, 2 tumors were grade II, and 1 tumor was grade III. One grade I tumor demonstrated ganglioneuromatous differentiation. Glandular differentiation was seen in 1 grade II ONB. All patients had surgical resection, with 3 patients receiving postoperative radiotherapy. One patient was found to have multiple bony and left cervical metastases by cross-sectional staging imaging and therefore received postoperative chemotherapy. All patients are alive with 4 patients free from disease (follow-up period ranging from 1-108 months). Conclusions At our center patients typically presented with advanced disease with most patients receiving multimodality treatment comprising surgery and radiotherapy. All patients are alive with only 1 patient with disseminated disease.

Human papillomavirus (HPV)-positive oropharyngeal cancer (OPSCC) is associated with improved survival compared with HPV-negative disease. However, a minority of HPV-positive patients have poor prognosis. Currently, there is no generally accepted strategy for identifying these patients. We retrospectively analysed 270 consecutively treated OPSCC patients from three centres for effects of clinical, pathological, immunological, and molecular features on disease mortality. We used Cox regression to examine associations between factors and OPSCC death, and developed a prognostic model for 3-year mortality using logistic regression analysis. Patients with HPV-positive tumours showed improved survival (hazard ratio (HR), 0.33 (0.21–0.53)). High levels of tumour-infiltrating lymphocytes (TILs) stratified HPV-positive patients into high-risk and low-risk groups (3-year survival; HPV-positive/TILhigh=96%, HPV-positive/TILlow=59%). Survival of HPV-positive/TILlow patients did not differ from HPV-negative patients (HR, 1.01; P=0.98). We developed a prognostic model for HPV-positive tumours using a ‘training’ cohort from one centre; the combination of TIL levels, heavy smoking, and T-stage were significant (AUROC=0·87). This model was validated on patients from the other centres (detection rate 67%; false-positive rate 5.6%; AUROC=0·82). Our data suggest that an immune response, reflected by TIL levels in the primary tumour, has an important role in the improved survival seen in most HPV-positive patients, and is relevant for the clinical evaluation of HPV-positive OPSCC.

Introduction: 1–2% of head and neck malignancies are Adenoid Cystic Carcinoma (ACC). Disease relapse is common but occurs late. Treatment is surgical with post-operative radiotherapy when incomplete resection has occurred. Radiotherapy does not benefit overall survival and the literature is contradictory regarding its control of local disease.

Background: The UK incidence of oral cancer (OSCC) has risen significantly, and is estimated to rise further over the next decade. OSCC is an heterogenous disease, staged currently using the TNM classification, supplemented with pathological information from tumour and loco-regional lymph nodes. Although patients with advanced disease show reduced survival, there is no single pathological or molecular feature that identifies aggressive tumours at an early stage.