Neck Sarcomas Research Articles

A Rare Case Report of Malignant Peripheral Nerve Sheath Tumor in the Infratemporal Fossa.

Introduction: Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors that develop from peripheral nerve sheath cells and they account for approximately 5% to 10% of all soft-tissue sarcomas. MPNSTs in the head and neck region represent approximately 2% to 6% of all head and neck sarcomas and account for 12% to 19% of all MPNSTs, and the infratemporal fossa is a rare site for MPNSTs. MPNSTs originating from the trigeminal nerve are extremely rare. Case presentation: A 19-year-old female presented with a 6-month history of left-sided facial pain and paresthesia on the same side. On examination, there was left-sided facial paresthesia at the third trigeminal nerve (V3) areas; computed tomography scanning and magnetic resonance imaging showed an infratemporal lesion and surgical resection was done. Histological examination and the immunostaining finding showed high-grade MPNST. Conclusion: MPNSTs in the head and neck region may manifest with nonspecific symptoms. The diagnosis often requires a combination of clinical, pathological, and immunohistochemistry studies. Treatment involves total surgical resection with adjuvant radiotherapy.

Institutional Review and Clinicopathologic Correlation of Head and Neck Sarcomas

Abstract Introduction/Objective Head and neck sarcomas are challenging to diagnose and treat. Sarcomas in the head and neck region cause significant morbidity and mortality due to the vital structures affected. The rare nature of head and neck sarcomas also creates challenges in studying these entities. Methods/Case Report We performed a retrospective review of 40 cases of head and neck sarcomas at our institution from January 2000 to December 2023. Cases of metastatic sarcoma to the head and neck region were excluded. Skin and thyroid primary cases were also excluded. Results (if a Case Study enter NA) The initial age of diagnosis ranged from 2 years to 86 years with an average age of 39.2 years old. Thirteen pediatric patients were identified with the most common diagnosis being embryonal rhabdomyosarcoma and the most common location being the pharynx. The most common diagnosis among adult patients was chondrosarcoma with the most common location being the sinuses. Most patients presented at stage T2N0M0 and had an average survival of 4.4 years. Pediatric patients had a slightly greater average survival of 8.4 years. Adult patients had an average survival of 2.1 years. Conclusion Overall, head and neck sarcomas show poor prognosis and arise more commonly in the pharynx in pediatric patients and the sinuses in adult patients.

Head and neck sarcomas: Thirty years of experience in a tertiary referral center in Brazil.

This study analyzed the demographics, clinicopathological, treatment, and survival characteristics of head and neck sarcomas (HNS) diagnosed in a tertiary reference center in Brazil. HNS cases were retrospectively retrieved from the Department of Pathological Anatomy of the School of Medical Sciences of the State University of Campinas. The medical records were examined to extract demographic, clinicopathological, and follow-up information. The Pearson chi-square test, Kaplan-Meier curve, and Cox proportional hazards regression model were employed to identify survival and potential prognostic factors. A total of 47 patients were included in the study. The majority were men (61.7%) with a mean age of 38.9 years. The nasal cavity (34.0%) was the most common anatomical site. The lesions are usually presented as volume increases (78.7%). The most common histological subtypes were chondrosarcoma, osteosarcoma, and alveolar rhabdomyosarcoma. Surgical excision alone was the most common treatment modality. Local recurrence was observed in 10 cases, and metastases in 3 cases. During a mean follow-up period of 71.9 months, from diagnosis to the last follow-up, 31 patients (65.9%) were alive without the disease. A total of 10 patients (21.3%) died of the HNS for a mean follow-up period of 14.3 months. The time to presentation of more than 6 months (p = 0.0309) and the presence of metastases (p = 0.0315) were identified as prognostic factors for survival, while male sex was found to be an independent prognostic factor for recurrence. In conclusion, the results of this study indicate that the occurrence of a shorter lesion time to presentation and the presence of metastases were associated with a reduction in survival rates in patients with HNS.

Improving head and neck sarcoma care: The impact of a specialized multidisciplinary team approach on diagnosis and patient outcomes

ObjectiveGlobally, head & neck sarcoma care pathways remain unclear. In 2018, the London Sarcoma Service (LSS) set up a dedicated head and neck sarcoma (HNS) multidisciplinary team (MDT) with a clear objective to provide formal access to super-specialist expertise in diagnosis, treatment planning and management of HNS. The aim of the study is to provide first results of a dedicated HNS MDT. MethodsAll patients discussed between 2018 and 2022, in HNS MDT, with a new histologically confirmed HNS diagnosis were included in the study. Demographics, anatomic site, morphology, MDT recommendation, treatment details and outcomes were obtained from electronic patient records. ResultsA total of 337 patients were discussed in the HNS MDT of which 178 patients were included in the study, with a median age of 53 years(range 2–94); 67 % were soft tissue sarcomas(STS) and 33 % were bone sarcomas(BS), of which 43 % and 71 % were high grade, respectively. 55 % BS and 39 % STS underwent surgery. 9 % of BS and 7 % of STS received adjuvant Proton Beam therapy. With a median follow-up of 2.16 years, recurrence was observed in 12 %, distant metastasis in 6 % of patients and overall survival was 72 %. ConclusionThe HNS MDT provides expertise on diagnosis and multi-modality management of HNS. STS are more likely to be misdiagnosed. Atypical imaging characteristics should trigger a specialist referral. Adequate surgery at first presentation remains the mainstay of treatment and the strongest prognosticator of overall survival. Formation of an expert working group specific to HNS must work towards streamlining sarcoma care.

Epidemiological Study of Head and Neck Sarcomas.

Head and neck sarcomas are uncommon tumors that account for less than 1% of head and neck malignancies and fewer than 5% of all sarcomas. This study aims to assess the head and neck sarcomas pattern in Isfahan, Iran, compare its results with previous findings, and investigate treatment methods and the recurrence and survival rate. This study was done on 160 patients diagnosed with head and neck sarcomas between 2001 and 2020 in four medical centers in Isfahan, Iran. The patients with head and neck sarcoma confirmed by biopsy were included. Patient information included sex, age at diagnosis, and full characteristics of the mass. Disease recurrence and 2-year and 5-year overall survival (OS) were investigated by phone. Among 160 patients, 94 (58.8%) were male. The mean age was 36.9 ± 23.2 years. The neck was the most reported location for sarcomas (N=34, 21.3%). Undifferentiated pleomorphic sarcoma was the most frequent histopathologic pattern (N=48, 30.0%). The brain and lungs were the most common destinations for metastasis. "Surgery and adjuvant therapy together" was the most common type of treatment (N=108, 68.8%). The 2-year and 5-year recurrence rates were N=100, 62.5%, and N=110, 68.8%, respectively. The 2-year and 5-year OS was N=103, 64.4%, and N=58, 36.6%, respectively. Eighty-nine patients have had metastasis (55.6%). Both 2-year and 5-year recurrence rates and OS were significantly correlated with metastasis. The OS within two years and five years were significantly higher in those treated only by "Surgery".

The role of pulmonary metastasectomy for non-primary lung cancer: Umbrella review of meta-analysis.

8639 Background: Lungs are the most common metastatic area for many cancers. Due to heterogeneous characteristics of primary cancers, the benefit of pulmonary metastasectomy (PM) has not been investigated other than colorectal cancers. Furthermore, well-designed trials in this field are difficult to undertake due to diverse clinical scenarios and therapy related to primary cancers. This study aims to investigate the clinical outcomes of PM for non-primary lung cancer by synthesizing existing literature. Methods: A systematic search for meta-analyses on PM for non-primary lung cancers was conducted, encompassing publications up to January 3, 2024. The analysis included eight primary cancer types: colorectal, renal cell, breast, adrenocortical, head and neck cancers, melanoma, and sarcoma. Primary outcomes, overall survival, and recurrence rates post-PM were assessed using random-effect models to account for study heterogeneity. Subgroup analysis categorized the survival rates based on primary cancer types, and a proportion meta-analysis was performed for comparison. Results: This study included 44 systematic-review articles and 157 individual studies, involving 23,694 patients who underwent PM for non-primary lung cancer. Patients had a mean age of 48.0 [95% CI 43.9-52.0] years, with 68.4% [95% CI 65.0-71.7] being male. About half of patients (47.1% [95% CI 40.8-53.5] had multiple metastatic lesions and 26.6% had bilateral involvement of lung. Sublobar resection was mostly performed (82%), and complete R0 resection was achieved in 87.2% [95% CI 83.0-90.8]. The pooled 5-year overall survival rate after PM was 41.2% [95% CI, 37.1-45.4%], with specific rates for each primary cancer type listed (Table). Patients with germ cell tumor demonstrated significantly higher survival rate than other cancers (p<0.05), while patients with melanoma had worst outcome (p<0.05). During follow-up, 57.6% [95% CI 46.4-68.1] had recurrence: 48% of them had intrathoracic only recurrence and 52% had extra-thoracic recurrence. Conclusions: The findings affirm that PM yields survival benefit. Overall survival rates following PM do not significantly differ based on primary cancer types, except for germ cell tumors and melanoma. Additionally, the recurrence rates are comparable to that of stage 4 advanced cancers other than germ cell tumor and is not significantly influenced by the primary cancer type. This highlights the importance of recognizing and incorporating PM in clinical practice when appropriate. [Table: see text]

Outcomes for Patients With Head and Neck Sarcoma Treated Curatively With Radiation Therapy and Surgery

Soft tissue sarcomas (STSs) of the head and neck (H&N) are rare malignancies that are challenging to manage. We sought to describe the outcomes of patients treated with curative intent using combined surgery and radiation therapy (RT) for H&N STS. We performed a single-institution retrospective review of patients with nonmetastatic STS of the H&N who were treated from 1968 to 2020. The Kaplan-Meier method was used to estimate disease-specific survival (DSS) and local control (LC). Multivariable analyses (MVAs) were conducted using Cox proportional hazards model. One hundred ninety-two patients had a median follow-up of 82 months. Tumors arose in the neck (n = 50, 26%), paranasal sinuses (n = 36, 19%), or face (n = 23, 12%). Most patients were treated with postoperative RT (n = 134, 70%). Postoperative RT doses were higher (median, 60 Gy; preoperative dose, 50 Gy; P < .001). Treatment sequence was not associated with LC (preoperative RT, 78% [63%-88%]; postoperative RT, 75% [66%-82%]; P = .48). On MVA, positive/uncertain margin was the only variable associated with LC (hazard ratio [HR], 2.54; 95% CI, 1.34-4.82; P = .004). LC was significant on MVA (HR, 4.48; 95% CI, 2.62-7.67; P < .001) for DSS. Patients who received postoperative RT were less likely to experience a major wound complication (7.5% vs 22.4%; HR, 0.28; 95% CI, 0.11-0.68; P = .005). There was no difference in the rate of late toxicities between patients who received preoperative or postoperative RT. H&N STS continues to have relatively poorer LC than STS of the trunk or extremities. We found LC to be associated with DSS. Timing of RT did not impact oncologic or long-term toxicity outcomes; however, preoperative RT did increase the chance of developing a major wound complication.

P16 and HPV status in head and neck sarcomas and sarcomatoid carcinomas

Human papillomavirus (HPV)-positive oropharyngeal carcinoma is a distinct type of head and neck carcinoma with improved prognosis. p16 immunostaining is often used as a surrogate marker for HPV infection in this particular setting. The aim of this study is to estimate the prevalence of p16 staining and HPV infection in head and neck sarcomatoid carcinomas as well as head and neck sarcomas. 21 sarcomatoid carcinomas and 28 head and neck sarcomas were tested for p16 positivity using immunohistochemical staining, and for high-risk HPV infection using In situ hybridization (ISH). 24 % of sarcomatoid carcinomas and 21 % of sarcomas were positive for p16 staining. All 49 cases were negative for HPV ISH. The results confirm that p16 staining is not specific and may not be associated with HPV infection in non-oropharyngeal head and neck sites. They also indicate that non-oropharyngeal head and neck sarcomatoid carcinomas are not likely to be HPV related.

A Retrospective Study of 291 Patients With Head and Neck Sarcomas: Treatment, Outcomes, and Prognostic Factors

AimsSarcomas constitute a group of rare malignant neoplasms, commonly subcategorized into soft tissue sarcomas (STS) and bone sarcomas. This study aims to describe the treatment modalities and outcome of head and neck sarcoma (HNS) patients in western Denmark and to identify prognostic factors for overall survival and recurrence in HNS patients. Materials and methodsThe Aarhus sarcoma registry, The National Danish Sarcoma Database, and the Danish National Pathology Registry were used to identify HNS adult patients diagnosed between 1979 and 2022. ResultsAltogether, 291 patients were included in this study. The prevalent histological subtypes were undifferentiated pleomorphic sarcoma (16%; 48/291) and leiomyosarcoma (15%; 44/291) for STS patients (n = 230) and chondrosarcoma (10%; 28/291) and osteosarcoma (7%; 19/291) for bone sarcoma patients (n = 61). Surgery with curative intent was performed in 71% (164/230) and 70% (43/61) of STS and bone sarcoma patients, respectively. Clear resection was achieved in 59% (97/164) of STS patients and 44% (19/43) of bone sarcoma patients. Eighty-nine patients relapsed (STS n = 66, bone sarcoma n = 23) after a median time of 2.7/5.5 years for STS/bone sarcoma patients. The five-year overall survival rates were 45% for STS patients and 66% for bone sarcoma patients.The following factors were significantly, negatively associated with overall survival in STS patients: Age (hazard ratio (HR)) = 1.02, p < 0.001), tumour size ≥5 cm (HR = 1.75, p = 0.003), metastatic disease (HR = 3.17, p < 0.001), high grade tumour (HR = 2.24, p = 0.004), previous cancer (HR = 2.84, p < 0.001), and high Aarhus composite biomarker score (ACBS) (HR = 4.56, p = 0.001). For relapse in STS patients, higher tumour grade (HR = 3.19, p = 0.014), intralesional margins (HR = 2.84, p < 0.001), ≥2 previous cancers (HR = 3.00, p = 0.004), and high ACBS (HR = 3.29, p = 0.047), were negatively associated. For bone sarcomas only higher age (HR = 1.02, p = 0.049) and intralesional margins (HR = 2.91, p = 0.042) were significant negative factors for overall survival. ConclusionMultiple prognostic factors for overall survival and relapse were identified, especially for STS patients.

Head and neck sarcomas: A series of 20 cases

Head and neck sarcomas are rare and histologically diverse malignancies, which represents a diagnostic challenge for radiologists because they have many overlapping imaging features. They account for less than 1% of all malignant tumors in the body. People of all ages can be affected, regardless of gender. Rhabdomyosarcoma is the most common histological form, especially in children. Due to the risk of metastasis, extension is absolutely necessary. Their treatment requires the intervention of a multidisciplinary team in specialized units to improve prognosis. The aim of our work is to review, through our series, the contribution of imaging resources in the diagnostic approach of tumor nature, to establish the "baseline" workup and post-therapeutic follow-up, as well as to analyze the epidemiology, clinical and histological characteristics of head and neck sarcomas.

Current status of head and neck sarcomas in Japan in 2016–2019: an analysis using the national cancer registry

BackgroundHead and neck sarcomas are especially rare in Asia, leading to limited clinical evidence. This study aimed to investigate the incidence, clinical features, treatment status, and outcome of these sarcomas using data from the National Cancer Registry in Japan.MethodsAll head and neck sarcomas diagnosed between 2016 and 2019 and recorded in the National Cancer Registry were analyzed. Data on sex, age, primary site, histological type, stage, treatment modality, and prognostic information were collected. Age-adjusted incidence and 3-year survival rates of patients with head and neck sarcomas were calculated.ResultsOverall, 635 head and neck sarcoma patients were identified. Head and neck sarcoma occurred more frequently in men and patients in their 70 s. The age-adjusted annual incidence rate was 0.125 per 100,000 patients in the 2015 Japanese model or 0.089 per 100,000 patients in the world population model. The nasal cavity and paranasal sinuses were the most frequent primary sites, with rhabdomyosarcoma as the most common histologic type. Treatment typically involved chemotherapy and/or radiation therapy for rhabdomyosarcoma and Ewing’s sarcoma, whereas surgical approaches for other types. Three-year survival rate of head and neck sarcoma patients was 64.8%.ConclusionsHead and neck sarcomas occurred rarely, but most frequently in the nasal cavity and paranasal sinuses in Japan. Poor outcomes were observed for sarcoma patients than for non-sarcoma head and neck cancer patients.

Dietetic service provision and nutritional interventions provided to patients with head and neck sarcoma: Findings from a national United Kingdom centre five-year audit

ObjectivesHead and neck sarcomas (HNS) represent a subset of malignancies which may be treated with multiple-modalities including chemotherapy followed by surgery and/or radiotherapy. Malnutrition is common in head and neck cancer (HNC) but limited literature exists determining nutritional interventions required in HNS. This audit aimed to investigate the dietetic input and nutritional interventions in HNS at a London sarcoma centre according to evidence-based HNC nutritional guidelines. MethodsRetrospective review between January 2018 to January 2023. Adult patients (≥18 years) newly-diagnosed with HNS treated at the tertiary centre were included. Demographic data and information on nutritional interventions was collected and statistically analysed. Results337 patients were referred to the HNS multidisciplinary meeting; 154 met the inclusion criteria; 66 received dietetic intervention (age = 45 [30.5] years; 47% (n = 31) male; baseline weight = 70.3 ± 18.13 kg; BMI = 24.2 [8.47] kg/m2). 19.7% (n = 13) were malnourished at presentation. Nutritional interventions were as follows: dietary counselling 100% (n = 66); oral nutritional support 90.9% (n = 60); oral nutritional supplements 75.8% (n = 50); enteral tube-feeding 54.5% (n = 36). 15.2% (n = 10) had a prophylactic gastrostomy; n = 4 pre-surgery and n = 6 pre-radiotherapy/proton-beam therapy±chemotherapy with 100% and 33.3% using the tube for ≥4 weeks respectively. Average duration of dietetic intervention was significantly longer for patients with high grade sarcoma compared to low grade (411.5 days and 136.8 days respectively, p = 0.000039) and for those undergoing multi-modality treatment compared to single-modality (402.9 days and 187.7 days respectively, p = 0.000932). ConclusionA fifth of patients with HNS were malnourished at presentation and the majority receiving dietetic intervention required nutrition support, indicating the need for nutritional management in HNS.

Management of head & neck sarcomas in adults: A retrospective study

The research purpose is to review the surgical approach and evaluate the results in adult patients with head and neck sarcomas.The histopathology varied, including two leiomyosarcomas, six malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, four dermatofibrosarcomas protuberans, three osteosarcomas, two angiosarcomas, one liposarcoma, one Ewing sarcoma, one synovial sarcoma, two unclassified/non-differentiated sarcomas and one solitary fibrous tumor. Surgical resection included maxillectomy, mandibulectomy, craniectomy, parotidectomy, scalp resection, face skin resection and laminectomy. The reconstruction was performed with one rectus abdominis flap, four radial forearm flaps, two latissimus dorsi flaps, two vascularized fibula flaps, two pectoralis major myocutaneous flaps, two trapezius flaps, two temporalis flaps, seven scalp flaps and two nasolabial flaps.The total patient number was 24. The hospitalization was uncomplicated, followed by postoperative radiotherapy in the majority of cases. In a mean 15-year follow-up period, 11 patients are still alive and disease-free. There were four recurrences treated with palliative radiotherapy.The surgical approach for head and neck sarcomas, including the achievement of a functionally acceptable result by organ sparing techniques, remains challenging. Wide resection combined with the appropriate reconstruction, particularly with microsurgical techniques, and followed by adjuvant radiotherapy or chemotherapy offer improved prognosis and quality of life.

Epidemiology and Outcomes of Soft Tissue Sarcomas: Insights from a Decade of Head and Neck Surgery at Sótero del Río Hospital, Santiago, Chile

Soft tissue sarcomas (STS) are rare malignant tumors of mesenchymal origin. They are associated with genetic and environmental risk factors. Their clinical manifestations are nonspecific, requiring a high level of suspicion. The first-line treatment is surgical. Positive margins are the only independent predictor of local recurrence and worse survival rates. Strict follow-up is recommended due to its high recurrence rate. Analyze the casuistry of STS treated with curative intent by the head and neck surgery team at the Sótero del Río Hospital (HSR) at Santiago, Chile between 2013 and 2023. A retrospective, descriptive study of patients with STS managed by the HSR head and neck surgery team. Clinical presentation, diagnostic and therapeutic tools, and oncological results are analyzed. 26 patients were included, 84.6% female, with an average age of 61.7 years. Only 30.8% presented identifiable risk factors. The most common histology was undifferentiated pleomorphic sarcoma (26.9%), and the predominant location was extremities (46.2%). 77.8% of head and neck sarcomas and 58.8% of trunk and extremity sarcomas occurred in advanced stages. Disease-free survival was 66.6%; 68.2% in patients with negative surgical margins, and 60.0% in positive margins at five years. STS is an infrequent pathology. This study corresponds to the first retrospective research on STS in Chile. We require establishing a specialized multidisciplinary team to optimize the management and follow-up of STS patients.

Pencil Beam Scanning Proton Therapy for Adolescents and Young Adults with Head and Neck Sarcomas

PurposeTo assess clinical outcomes of adolescents and young adults (AYAs) with head and neck sarcomas (HNSs) treated with pencil beam scanning proton therapy (PBSPT) and to report quality of life (QoL). Materials and MethodsTwenty-eight AYAs (aged 15 to 39 years) with HNS treated between January 2001 and July 2022 at our institution were included. The median age was 21.6 years. Rhabdomyosarcoma (39.3%), Ewing sarcoma (17.9%), chondrosarcoma (14.3%), and osteosarcoma (14.3%) were the most frequent diagnoses. Three (10.7%) patients were metastatic before PBSPT and 13 (46.4%) patients had a tumor with intracranial extension. The median total radiation dose was 63 GyRBE (range, 45 to 74 GyRBE). Thirteen (46.4%) patients received concomitant chemotherapy. Toxicity was reported according to the Common Terminology Criteria for Adverse Events (CTCAE), version 5.0 (US National Institutes of Health, Bethesda, Maryland). Survival was estimated using the Kaplan-Meier method. QoL was assessed using a PEDQOL (Pediatric Quality of Life Questionnaire) questionnaire. Self-reported outcomes were assessed using institutional questionnaires. ResultsWith a median follow-up of 57 months (range, 3.7 to 243 months), 5 patients (17.8%) had local failure (LF) only, 2 (7.1%) experienced distant failure (DF) only, and 2 (7.1%) had LF and DF. The estimated 5-year local control (LC) and distant control (DC) rates were 71.8% and 80.5%, respectively. The median times to LF and DF were 13.4 and 22.2 months, respectively. Four (14.3%) patients died, all but one from their HNS. Estimated 5-year overall survival was 90.7%. Six (21.4%) patients developed nonocular grade ≥3 toxicity, which consisted of otitis media (n = 2), hearing impairment (n = 2), osteoradionecrosis (n = 1), and sinusitis (n = 1). Four (14.3%) patients developed cataracts that required surgery. The 5-year freedom from nonocular grade 3 toxicity was 91.1%. No grade 4 or higher toxicity was observed. Adolescents rated their quality of life before treatment worse than their parents did. ConclusionExcellent outcomes with acceptable late-toxicity rates were observed for AYAs with HNS after PBSPT.

Outcomes for Patients with Head and Neck Sarcoma Treated Curatively with Radiation Therapy and Surgery

Soft tissue sarcomas (STS) of the head and neck are rare and diverse entities that are challenging to manage. Definitive treatment requires surgery, often with radiation therapy (RT). We sought to describe the outcomes of patients treated curatively with surgery and RT for head and neck STS. We performed a retrospective review of patients treated at a tertiary cancer center for non-metastatic STS of the head and neck between 1968 and 2020; over half were treated in the modern era defined as 2005 or after. Patients with rhabdomyosarcomas or cutaneous angiosarcoma were excluded. The Kaplan-Meier method was used to estimate actuarial overall survival (OS), disease-specific survival (DSS) and local control (LC). Multivariable analyses (MVA) were conducted using Cox proportional hazards model. Median follow-up was 82 months. Of the 192 patients, the majority were male (n = 111, 58%), White (n = 158, 82%), and median age was 49.5 [IQR 32.5-64]. The highest proportion of tumors were in the neck (n = 50, 26%), paranasal sinuses (n = 36, 19%), or face (n = 23, 12%). The most common histology was sarcoma, not otherwise specified (n = 44, 23%), followed by undifferentiated pleomorphic sarcoma (n = 32, 17%), and neurogenic sarcoma (n = 15, 8%). Most patients were treated with postoperative RT (n = 134, 70%). Patients treated with preoperative RT were older (median 56.5 yrs vs post-op 44 yrs, p = 0.009). Post-op RT doses were higher (median 60 Gy, pre-op 50 Gy, p<0.001), and margins were more likely to be negative in those treated with pre-op RT (n = 39, 67%, post-op 69, 51%, p = 0.02). 5-year LC, DSS, and OS for the entire cohort was 76% (95% CI 69-82), 74% (67-80), and 71% (64-77), respectively. LC was not affected by treatment sequence (pre-op RT 78% (63-88), post-op RT 75% (66-82), p = 0.48). Patients with negative margins had improved 5-yr LC (86% (77-92), positive/uncertain 65% (53-74), p = 0.003). On MVA, positive/uncertain margin was the only variable associated with LC (HR 2.54 (1.34-4.82), p = 0.004). Poorer 5-yr DSS was associated with higher grade (grade 3 75% (63-84), grade 1-2 89% (75-94), p = 0.02), and treatment era (pre-2005 68% (57-76), on/after 2005 80% (70-87), p = 0.04). These both remained significant on MVA (grade 3 HR 2.39 (1.07-5.36), p = 0.034; pre-2005 HR 2.31 (1.03-5.21), p = 0.043). Sixteen patients (8%) developed a late RT toxicity, including fibrosis (n = 4, 2%), necrosis (osteoradionecrosis n = 2, brain necrosis n = 1, soft tissue necrosis n = 1), and eye dryness (n = 2, 1%). Neither the timing of RT nor dose was found to be associated with developing a late RT toxicity. Head and neck STS continues to have relatively poorer local control than STS of the trunk or extremities. Timing of RT did not impact oncologic or long-term RT-related toxicity outcomes. However, more data are needed to ascertain whether preoperative RT may impact acute surgical toxicities. These data contribute to multidisciplinary care planning for patients with STS in these challenging anatomic locations.

Head and Neck Sarcomas Characteristics at Dr. Hasan Sadikin General Hospital for 5 Years (2017-2021)

Background: Sarcoma is a malignant neoplasm originating from mesodermal tissue with an incidence rate of 1% of neoplasms throughout the body. Head and neck sarcoma requires more complex management and until now, reviews of this disease are still very rare. This study aimed to report the incidence and characteristics of head and neck sarcoma during 2017-2021 at Dr. Hasan Sadikin General Hospital, Universitas Padjadjaran, Bandung.&#x0D; Methods: Research data was taken from patient medical records through SIRS at Dr. Hasan Sadikin General Hospital during the 2017-2021 period according to the ICD10 of head and neck malignancies and histopathological data.&#x0D; Results: During 2017-2021, 15 cases of head and neck sarcoma were found, consisting of 9 cases (60%) rhabdomyosarcoma, 2 cases (13.3%) fibrosarcoma, and 1 case each (6.7%) synovial sarcoma, undifferentiated pleomorphic sarcoma, angiosarcoma and dermatofibrosarcoma protuberans. The neck and shoulder area are the most frequently affected site (40%), followed by the scalp (26.7%), maxilla (20%), and oral cavity (13.3%). The highest incidence rates were in female (60%) and children aged &lt;17 years (46.7%).&#x0D; Conclusion: The incidence of head and neck sarcoma at Dr. Hasan Sadikin General Hospital Bandung is rare. The most frequent cases are rhabdomyosarcoma the most commonly affected areas are the neck and shoulders.

Quality and consistency of clinical practice guideline recommendations for PET/CT and PET: a systematic appraisal.

To systematically appraise the methodologies used for guidelines for positron emission tomography (PET) imaging and to compare the consistency of these recommendations. We searched PubMed, EMBASE, four guideline databases, and Google Scholar to identify evidence-based clinical practice guidelines pertaining to the use of PET, PET/computed tomography (CT), or PET/magnetic resonance in routine practice. We assessed the quality of each guideline using the Appraisal of Guidelines for Research and Evaluation II instrument and compared recommendations regarding indications for 18F-fluorodeoxyglucose (FDG) PET/CT. Thirty-five guidelines for PET imaging, published between 2008 and 2021, were included. These guidelines performed well in the domains of scope and purpose (median 80.6%, inter-quartile range [IQR] 77.8-83.3%) and clarity of presentation (median 75%, IQR 69.4-83.3%), but poorly in applicability (median 27.1%, IQR 22.9-37.5%). Recommendations for 48 indications in 13 cancers were compared. Considerable inconsistencies in the direction of whether to support the use of FDG PET/CT were observed in 10 (20.1%) indications pertaining to 8 cancer types: head and neck cancer (treatment response assessment), colorectal cancer (staging in patients with stages I-III disease), esophageal cancer (staging), breast cancer (restaging and treatment response assessment), cervical cancer (staging in patients with stage < IB2 disease and treatment response assessment), ovarian cancer (restaging), pancreatic cancer (diagnosis), and sarcoma (treatment response assessment). Current guidelines for PET imaging vary in methodological quality and provided considerably inconsistent recommendations. Efforts are needed to improve adherence to guideline development methodologies, to synthesis high-quality evidence, and to adopt standard terminologies. PROSPERO CRD42020184965. Guidelines for PET imaging provide considerably inconsistent recommendations and vary in methodological quality. It is suggested that clinicians be critical of these recommendations when applying them in practice, that guideline developers adopt more rigorous development methodologies, and that researchers prioritize research gaps identified by current guidelines. • PET guidelines vary in methodological quality and provided inconsistent recommendations. Efforts are needed to improve methodologies, synthesize high-quality evidence, and standardize terminologies. • Among six domains of methodological quality assessed by the AGREE II tool, guidelines for PET imaging performed well in scope and purpose (median 80.6%, inter-quartile range 77.8-83.3%) and clarity of presentation (75%, 69.4-83.3%), but poorly in applicability (27.1%, 22.9-37.5%). • Among the 48 recommendations (for 13 cancer types) compared, conflicts in the direction of whether to support FDG PET/CT use were observed in 10 (20.1%), for 8 cancer types (i.e., head and neck, colorectal, esophageal, breast, cervical, ovarian, pancreatic, and sarcoma).

Recurrent Giant Myofibroblastic Sarcoma of Neck in a Teenager: Overcoming Treatment Challenges

Sarcomas are rare tumors arising from a variety of mesenchymal tissues which are even rarer in head and neck region amounting 1% only of the 5% of sarcomas. About 80% of head and neck sarcomas originate in soft tissue while the remaining 20% arise from bone (Cormier and Pollock in J Clin 54:94−109, 2004). One among the commonly presenting variants of sarcomas in head and neck that our patient was diagnosed with is Low grade myofibroblastic sarcoma (LGMS). These even though uncommon have a predilection for head and neck region particularly the tongue (Mentzel et al. in Am J Surg Pathol 22:1228−38, 1998; Cai et al. in Virchows Arch 463:827−36, 2013; Meng et al. in Chin Med J 120:363–9, 2007; Demarosi et al. in Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 108:248–54, 2009). LGMS was reclassified as a distinct entity by the WHO classification of soft tissue tumors in 2002 (Qiu et al. in Oncol Lett 9:619−25, 2015). Oncological resection is the mainstay of treatment. In case of bulky tumor, resection and reconstruction would be challenging. We report a recurrent LGMS of 15-year-old girl who presented with a ginormous soft tissue swelling in her left neck region which made her day-to-day activities strenuous and made her non ambulatory and the line of treatment executed in order to get her back on feet, healthy & free of the debilitating tumor.

MO-0379 Clinical Outcome of AYAs with Head and Neck Sarcomas after Pencil Beam Scanning Proton Therapy

MO-0379 Clinical Outcome of AYAs with Head and Neck Sarcomas after Pencil Beam Scanning Proton Therapy