Optimal preoperative therapy regimen in the treatment of resectable retroperitoneal sarcoma (RPS) remains unclear. This study compares the impact of preoperative radiation, chemoradiation and chemotherapy on overall survival (OS) in RPS patients.

The National Cancer Database (NCDB) was queried for patients with non-metastatic, resectable RPS (2006–15). The primary endpoint was OS, evaluated by Kaplan–Meier method, log-rank test, Cox multivariable analysis and propensity score matching.

A total of 1,253 patients met the inclusion criteria, with 210 patients (17%) receiving chemoradiation, 850 patients (68%) receiving radiation and 193 patients (15%) receiving chemotherapy. On Cox multivariable analysis, when compared to preoperative chemoradiation, preoperative radiation was not associated with improved OS (hazards ratio [HR] 0·98, 95% CI 0·76–1·25, p = 0·84), while preoperative chemotherapy was associated with worse OS (HR 1·64, 95% CI 1·24–2·18, p < 0·001). Similar findings were observed in 199 and 128 matched pairs for preoperative radiation and chemotherapy, respectively, when compared to preoperative chemoradiation.

Our study suggested an OS benefit in using preoperative chemoradiation compared to chemotherapy alone, but OS outcomes were comparable between preoperative chemoradiation and radiation alone.

Sarcoma of the head and neck is a rare condition that poses significant challenges in management and often requires radical multimodality treatment.

This study aimed to analyse current clinical presentation, evaluation, management dilemmas and oncological outcomes.

Computer records and case notes were analysed, and 39 patients were identified. Variables were compared using Pearson's chi-square test and the log-rank test, while survival outcomes were calculated using the Kaplan–Meier method.

The histopathological diagnosis was Kaposi sarcoma in 20.5 per cent of cases, chondrosarcoma in 15.3 per cent and osteosarcoma in 10.2 per cent. A range of other sarcomas were diagnosed in the remaining patients. The site of disease was most commonly sinonasal, followed by the oral cavity and larynx.

Wide local excision with clear resection margins is essential to achieve local control and long-term survival. There is a need for cross-specialty collaboration in order to accrue the evidence which will be necessary to improve long-term outcomes.

Radiation therapy is a commonly used treatment in head and neck oncology, whether used alone or as an adjunct to other treatments. Osteoradionecrosis and radiation-induced malignancy are both rare, late complications of radiation therapy.

This paper describes a case of radiation-induced sarcoma of the mandible initially presenting as osteoradionecrosis. In addition, the literature on the identification and treatment of these entities is reviewed.

The patient underwent excision and reconstruction of the presumed osteoradionecrosis. Final pathology demonstrated radiation-induced sarcoma. The patient suffered tumour recurrence at seven months post-operatively.

When evaluating a patient with osteoradionecrosis, clinicians should be cognisant of the risk of malignancy. This paper describes the first case of radiation-induced sarcoma presenting as osteoradionecrosis. Primary treatment of radiation-induced sarcoma is wide surgical extirpation, with a very limited role for chemotherapy. Overall survival for this entity is poor.

Ewing's sarcoma is a rare, malignant tumour predominantly affecting young adolescent males. We describe a unique case of an isolated extra-skeletal metastasis from a skeletal Ewing's sarcoma primary, arising in the right sinonasal cavity of a young man who presented with severe epistaxis and periorbital cellulitis.

Histologically, the lesion comprised closely packed, slightly diffuse, atypical cells with round, hyperchromatic nuclei, scant cytoplasm and occasional mitotic figures, arranged in a sheet-like pattern. Immunohistochemical analysis showed positive staining only for cluster of differentiation 99 glycoprotein. Fluorescent in situ hybridisation identified the Ewing's sarcoma gene, confirming the diagnosis.

Complete surgical resection was achieved via a minimally invasive endoscopic transnasal approach; post-operative radiotherapy. Ten months post-operatively, there were no endoscopic or radiological signs of disease.

Metastatic Ewing's sarcoma within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important to enable early diagnosis. This case illustrates one potential management strategy, and reinforces the evolving role of endoscopic transnasal approaches in managing sinonasal cavity and anterior skull base tumours.

To review the existing diagnostic modalities and treatment for primary tracheal synovial sarcoma, and to report a case of primary cervical synovial sarcoma arising in the trachea.

Retrospective.

Head and neck surgery unit at a tertiary university centre.

One case of primary cervical tracheal monophasic synovial sarcoma diagnosed by SYT–SSX gene rearrangement.

This patient underwent surgical resection of the synovial sarcoma, together with tracheal resection and primary anastomosis assisted by laryngeal-releasing manoeuvres, without complication.

Clinical, radiographical, pathological and surgical information were collected.

One year post-operatively, there was no evidence of recurrence.

Synovial sarcoma arising in the trachea is very rare. Diagnosis is confirmed by demonstrating the SYT–SSX gene rearrangement. The first-line treatment is surgery.

Given its rarity, varied histological presentation and often pseudosarcomatous appearance, nodular fasciitis is frequently misdiagnosed on pre-operative, intra-operative and final analyses.

Four cases of nodular fasciitis are reviewed.

Physical and radiological findings were consistent with a parapharyngeal tumour, probably neurogenic, a level four neck mass suspicious for lymphoma; a sternoclavicular mass in a patient with a history of breast cancer suspicious for metastasis; and a cheek mass consistent with an accessory parotid tumour. Fine needle aspiration results were consistent with a neurogenic tumour in two patients and an undifferentiated malignancy in two patients. Frozen section examination most commonly included masses with spindle-type cells. The final diagnosis of nodular fasciitis was made only after permanent section and immunohistological analysis.

In a patient with nonspecific results following investigation of a head or neck mass, nodular fasciitis should be considered. Use of appropriate immunohistochemical markers will aid in the final diagnosis.

We report a case of malignant peripheral nerve sheath tumour seen in our department.

We present case reports and a review of the world literature concerning malignant peripheral nerve sheath tumour.

A 38-year-old Caucasian woman was diagnosed with malignant peripheral nerve sheath tumour of the infraorbital nerve. She underwent a wide enbloc resection followed by a course of radiotherapy. A five-year post-operative magnetic resonance imaging scan revealed no evidence of recurrence.

Malignant peripheral nerve sheath tumours are rare in the head and neck. An awareness of their occurrence is important for early diagnosis. Management involves: accurate histological diagnosis; radiological imaging to define tumour extent and plan resection; wide surgical excision with histologically negative margins; reconstruction of the tissue defect; and post-operative radiotherapy.

Extraskeletal Ewing's sarcoma is a rare tumour of mesenchymal origin, which is histologically similar to primary osseous Ewing's sarcoma. The majority of cases have been reported in the lower limb and paravertebral region, with a few cases reported in the neck.

We report a patient with extraskeletal Ewing's sarcoma presenting with a right-sided neck mass, vocal fold palsy and T1 nerve root involvement. The detection of characteristic features on computed tomography and magnetic resonance imaging scans, in conjunction with histological analysis, was required to make the diagnosis.

Radiological imaging demonstrated that the tumour was infiltrating through the neural exit foramina of the lower cervical nerve roots, with encasement of the vertebral artery. The patient was managed with combination chemotherapy for systemic control and irradiation for local control.

Extraskeletal Ewing's sarcoma has a propensity to occur in a paravertebral location, being found rarely in the cervical region, and has a predilection to infiltrate through neural exit formina. Computed tomography and magnetic resonance imaging are helpful in the diagnosis of this rare tumour.

Malignant triton tumour is a relatively rare, aggressive sarcoma comprising both malignant schwannoma cells and malignant rhabdomyoblasts. Malignant triton tumour of the parapharyngeal space is exceptionally rare, with only one case being described in the literature. Malignant triton tumour of the cervical sympathetic nerve has not previously been reported.

We report a case involving the parapharyngeal space and arising from the cervical sympathetic nerve, and we review the management and outcome of the previous case reported in this rare location.

The parapharyngeal space is a unique location. Owing to this specific localisation, adjuvant therapy in addition to complete resection may be important in the treatment of malignant triton tumour in this rare location.