Neck Sarcomas Research Articles

Outcomes of the treatment of head and neck sarcomas in a tertiary referral center.

Head and neck sarcomas are a rare and heterogeneous group of tumors that pose management challenges. We report our experience with these tumors. Forty consecutive patients treated for 44 head and neck sarcomas between 1997 and 2014 were culled from our prospectively maintained head and neck database. Five patients were excluded. The adult cohort consisted 29 (83%) patients of a mean age of 57.7 years, with 33 sarcomas. The most common diagnoses were undifferentiated pleomorphic sarcoma (27%) and chondroblastic osteosarcoma (21%). Clear surgical margins were achieved in 24/33 (73%) lesions. Twenty-two patients received radiotherapy and/or chemotherapy. Fourteen patients developed local (n = 6), regional (n = 1) and distant (n = 7) recurrence. The overall 5-year survival was 66% with a mean survival interval of 66.5 months. Recurrent sarcoma, close (<1 mm) or involved surgical margins and advanced age were associated with statistically significantly reduced survival. The pediatric cohort consisted 6 (17%) patients, with a mean age of 9 years. Five patients had primary embryonal rhabdomyosarcomas and one had chondroblastic osteosarcoma. Clear surgical margins were achieved in five (83%) patients. All patients received adjuvant radiotherapy and/or chemotherapy. Mean survival interval was 102 months. Three patients developed local (n = 1) or distant (n = 2) recurrence. Twenty-three free and 8 pedicled flaps were performed in 25 patients. Eleven out of thirty-nine (28%) lesions in 11 patients developed a complication. In conclusion, head and neck sarcomas are best managed by a multidisciplinary team at a tertiary head and neck referral center and resection with clear margins is vital for disease control.

Analysis of Clinical Prognostic Factors for Adult Patients with Head and Neck Sarcomas

To evaluate the treatment, outcome, and prognostic factors in patients with head and neck sarcomas treated in an academic medical center. Case series. Academic medical center. We performed a retrospective analysis of adult patients (n = 97) with primary head and neck sarcomas treated between 2000 and 2012. We analyzed the treatment, outcome, and potential factors predictive of disease-free survival and disease-specific survival. We also evaluated the outcome and prognostic factors in patients with bone and soft tissue sarcomas. The median overall survival was 6.8 years, with 2-year and 5-year overall survival rates of 78% (95% confidence interval [CI], 66%-86%) and 59% (95% CI, 44%-72%), respectively. Univariable analysis revealed that age at diagnosis (>60 years: hazard ratio [HR], 2.7; 95% CI, 1.2-6.2; P = .01), surgical intervention (HR, 8.3; 95% CI, 3.5-19.5; P < .001), and metastatic disease (HR, 4.3; 95% CI, 1.3-13.6; P = .01) were significantly associated with disease-specific survival. In this study, patients over the age of 60 years at diagnosis and those with inoperable disease at initial presentation had significantly worse disease-specific survival. Surgical intervention remains the optimal treatment modality for those with resectable disease and was associated with significantly better survival in this heterogeneous series. Further multi-institutional studies are required to better define prognostic factors in individual histological subtypes.

Head and neck sarcomas: analysis of the SEER database.

To summarize the epidemiology of sarcomas occurring in the head and neck and identify prognostic factors for patient survival. Cross-sectional analysis of the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program. The SEER 18 registries, comprising sarcoma diagnoses made from 1973 to 2010, were queried for sarcomas arising in the head and neck. Pediatric and adult patients were analyzed separately, and multivariate and propensity-matched analyses were performed to identify predictors of disease-specific survival. In all, 11,481 adult cases and 1244 pediatric cases were identified. In adults, the most common histologic subtypes were malignant fibrous histiocytoma (MFH), Kaposi sarcoma, and hemangiosarcoma, while in the pediatric cohort, the most common histologic subtypes were rhabdomyosarcoma, MFH, and osteosarcoma. Cause-specific 2-, 5-, and 10-year survival rates were 76%, 66%, and 61% for adults and 84%, 73%, and 71% for pediatric patients. Multivariate analysis performed for adults revealed that male gender, absence of radiation therapy, and stage I disease were associated with improved cause-specific survival reaching statistical significance. However, a propensity-matched model demonstrated no significant difference in cause-specific survival between patients who received radiation and those who did not. Sarcomas, a heterogeneous group of malignant mesenchymal tumors, are uncommonly found in the head and neck. This study represents the largest analysis of patients with head and neck sarcomas in the literature and demonstrates the impact of age, gender, primary site, histology, and radiation status on overall prognosis.

Paediatric head and neck sarcoma: The London Sarcoma Service series

Paediatric head and neck sarcoma: The London Sarcoma Service series

Head and neck sarcoma: Analysis of 29 cases

ObjectivesThe purpose of this study was to analyze the outcome of patients suffering from head and neck sarcomas and to identify indicators of outcome. Material and methodsThe medical records of 43 patients treated between 2000 and 2010 were analyzed. All patients were suffering from primary head and neck sarcoma. The final study sample included 29 patients. ResultsMean survival was 56 months. Overall 2- and 5-year survivorship was 69% and 31% respectively. Parameters positively influencing survival were: male gender; non-smoker; alcohol consumption; age <18years; tumor size <5cm; location (nasal pyramid, jaw and maxillary sinus). Rhabdomyosarcoma and synovial sarcoma were the histological forms positively influencing prognosis. Age was the only parameter significantly influencing survival (P<0.05). ConclusionsThe present overall 5-year survivorship was similar to the lower limit of the available literature data. Age was the only proven indicator of outcome. In order to have more reliable data it is essential to set up broader databases.

Local control rates with five-fraction stereotactic body radiotherapy for oligometastatic cancer to the lung.

To report our institutional experience with five fractions of daily 8-12 Gy stereotactic body radiotherapy (SBRT) for the treatment of oligometastatic cancer to the lung. Thirty-four consecutive patients with oligometastatic cancers to the lung were treated with image-guided SBRT between 2008 and 2011. Patient age ranged from 38 to 81 years. There were 17 males and 17 females. Lung metastases were from the following primary cancer types: colon cancer (n=13 patients), head and neck cancer (n=6), breast cancer (n=4), melanoma (n=4), sarcoma (n=4) and renal cell carcinoma (n=3). The median prescription dose was 50 Gy in five fractions (range, 40-60 Gy) to the isocenter, with the 80% isodose line encompassing the planning target volume (PTV) [defined as gross tumor volume (GTV) + 7-11 mm volumetric expansion]. The follow-up interval ranged from 2.4-54 months, with a median of 16.7 months. The 1-, 2-, and 3-year patient local control (LC) rates for all patients were 93%, 88%, and 80% respectively. The 1-, 2-, and 3-year overall survival (OS) rates were 62%, 44%, and 23% respectively. The 1- and 2-year patient LC rates were 95% and 88% for tumor size 1-2 cm (n=25), and 86% for tumor size 2-3 cm (n=7). The majority (n=4) of local failures occurred within 12 months. No patient experienced local failure after 12 months except for one patient with colon cancer whose tumors progressed locally at 26 months. All five patients with local recurrences had colorectal cancer. Statistical analyses showed that age, gender, previous chemotherapy, previous surgery or radiation had no significant effect on LC rates. No patient was reported to have any symptomatic pneumonitis at any time point. SBRT for oligometastatic disease to the lung using 8-12 Gy daily fractions over five treatments resulted in excellent 1- and 2-year LC rates. Most local failures occurred within the first 12 months, with five local failures associated with colorectal cancer. The treatment is safe using this radiation fractionation schedule with no therapy-related pneumonitis.

Head and neck sarcomas: the UCLA experience

PurposeTo profile the clinical presentation, subtype distribution, and treatment results of sarcomas of the head and neck at a single tertiary academic center over an 11-year period. Materials and methodsA retrospective review was performed by examining the records and reviewing the pathology of 186 patients with head and neck sarcomas treated at UCLA Medical Center from 2000 to 2011. ResultsThe mean age of the study population was 49 ± 22years. 58% of the patients were male and 42% were female. Median duration of follow-up for the entire group was 18.5months. The most common presenting symptom was a mass lesion in 59.9% of patients. The nasal cavity/sinus was the most common presenting site seen in 22% of patients. Solitary fibrous tumor/hemangiopericytoma was the most common subtype. 15% of patients had evidence of prior radiation exposure. 26.3% of tumors were greater than 5cm and 35.5% were high-grade. Margins were positive in 31.2% of patients. Lymph node metastasis was rare at 6.5%. Perineural invasion was identified in 6.5%. Among all subtypes, 5-year recurrence-free survival and overall survival were 50% and 49%, respectively. Multivariate analysis demonstrated that grade and margin status were predictors of recurrence-free survival while grade and age affected overall survival. ConclusionsHead and neck sarcomas are a rare entity frequently presenting as a mass lesion. In our series, lesions tended to be high-grade with a significant portion of surgical specimens having positive margins. Grade and margin status were the most important predictors of survival.

A Brief Overview of Hyperthermia in Cancer Treatment

Despite the large economic and intellectual efforts, cancer is still not easily treatable disease by conventional therapies. This led ultimately to reconsider hyperthermia, like one of interesting treatment methods connected to immunity and tumor metabolism. Hyperthermia has also the ability to play an additional role when used together with the conventional methods of treatment: surgery, radiotherapy, chemotherapy and immunotherapy. Recent trials in Holland and Germany have demonstrated that hyperthermia can prolong life and decrease disease re-appraisal when used in combination with radiotherapy and chemotherapy. Some tumors seem more responsive than others. In this brief summary we will attempt to give a vision of hyperthermia from a physical stand point, but more importantly we will give clinical and biological aspects. The results obtained in these trials on certain types of cancers such as cervix cancer, recurrent breast cancer and head neck cancer, melanoma, sarcomas, liver, glioblastoma and pancreatic cancer support the use of this technique, although some clinical and technical problems persist and have not been completely resolved.

Prognostic Factors in Head and Neck Sarcomas: Analysis of the SEER Database

Objectives: 1) Summarize the epidemiology of sarcomas occurring in the head and neck. 2) Identify prognostic factors for patient survival. Methods: Cross-sectional analysis of the National Cancer Institute’s Surveillance, Epidemiology and End Results (SEER) Program. Results: The SEER 18 database, spanning the years 1973 to 2009, was analyzed. A total of 2134 cases of sarcoma arising in the head and neck were identified, accounting for 3.9% of all sarcomas. Of head and neck sites, the bones of the skull and face were most commonly affected (n = 856), followed by the oral cavity and pharynx (n = 532), nasal cavity and paranasal sinuses (n = 365), and major salivary glands (n = 192). Overall disease-specific 2-, 5-, and 10-year survival rates for head and neck sarcoma were 69.5%, 55.9%, and 49.9%, respectively. On Cox regression analysis, histologic subtype, tumor location, gender, and patient age were found to influence mortality rate significantly ( P &lt; 0.05), with male gender and older age portending worse outcomes. Conclusions: Sarcomas, a heterogeneous group of malignant mesenchymal tumors, are uncommonly found in the head and neck. This study represents the largest analysis of patients with head and neck sarcoma in the literature and demonstrates the impact of histology, tumor location, age, and gender on overall prognosis.

Head and neck sarcomas: a 12 year retrospective review of 40 cases

Head and neck sarcomas: a 12 year retrospective review of 40 cases

Head and Neck Sarcomas: A Comprehensive Cancer Center Experience

Head/neck sarcomas are rare, accounting for about 1% of head/neck malignancies and 5% of sarcomas. Outcomes have historically been worse in this group, due to anatomic constraints leading to difficulty in completely excising tumors, with high rates of local recurrence. We retrospectively analyzed cases of head/neck soft tissue sarcomas (STS) and osteogenic sarcomas managed in a multi-disciplinary setting at Fox Chase Cancer Center from 1999–2009 to describe clinicopathologic characteristics, treatment, outcomes, and prognostic factors for disease control and survival. Thirty patients with STS and seven patients with osteogenic sarcoma were identified. Most STS were high grade (23) and almost all were localized at presentation (28). Common histologies were synovial cell (6), rhabdomyosarcoma (5), angiosarcoma (4), liposarcoma (4) and leiomyosarcoma (3). The type of primary therapy and disease outcomes were analyzed. Cox proportional hazards regression analysis was performed to identify predictors of disease-free survival (DFS) and overall survival (OS). The HR and 95% CI for Cox model and median DFS/OS analyzed by Kaplan-Meier curves were calculated.

OP215: Radiation induced sarcomas of the head and neck

For over a century, ionising radiation has been known to induce malignancies in almost every organ, the most common being leukaemias and sarcomas. Radiation-induced sarcomas of the head and neck are rare tumours which present multiple resective and reconstructive challenges to the head and neck surgeon. We present our 12 year single-institution series of 17 radiation-induced sarcomas of the head and neck and compare their behaviour and outcomes to our series of 130 non-radiation induced head and neck sarcomas. Aim To compare the clinical, radiologic, pathologic characteristics and treatment outcomes of our series of 17 radiation-induced sarcomas of the head and neck with our cohort of 130 non-radiation induced head and neck sarcomas. Method We performed a retrospective study from the University College London Hospital Head and Neck Sarcoma Database of our 17 radiation-induced head and neck sarcomas over 2000–2012 including demographics, radiation characteristics, clinical and radiologic presentation, resective and reconstructive experience and treatment outcomes. Results and Conclusions Our series of radiation-induced head and neck sarcomas all had radiation doses of >40 Gy, a variable latency period and almost universally were high-grade sarcomas. Our treatment protocol consisted of neo-adjuvant chemotherapy and aggressive surgical resection with our series of radiation-induced sarcomas showing similar rates of clear surgical margins but reduced survival outcomes in comparison to non radiation-induced head and neck sarcomas.

OP216: Health related quality of life survey amoung head and neck sarcoma cases

Objectives Assessment of health-related quality of life (HR QOL) in head and neck sarcoma patients from diagnosis to 5 years after start of treatment. Study design A prospective, descriptive study. Methods 108 patients with head and neck sarcomas were looked at, and University of Washington QOL (UWQOL) questionnaires were used to assess the quality of life for all surviving patients. Patients were grouped into two groups: Maxillary sarcomas and Mandibular sarcomas. Results The mandibular group had more significant speech and swallowing difficulties affecting the quality of life. Pain was not significantly different between the groups. Problems with teeth, opening of the mouth, dryness in the mouth, and sticky saliva were persistent or worsening. Similar findings were found regardless of sex, age, or stage when clinical significant changes are considered. Conclusion There is very limited data available looking at the quality of life post resection of sarcoms in literature. The mandibular sarcomas affect function more than the maxillary and midface tumours.

Keynote Address V: Towards Personalized Treatment for Head and Neck Cancer

The yield of the cancer genome project is providing extraordinary potential for the identification of new drug targets, for selecting individuals for specific therapies, and for predicting the outcome of an individual with cancer. For a number of malignancies affecting the head and neck region, e.g. melanoma, lymphoma and sarcoma, we are already using genetic knowledge to select patients for specific targeted therapies. One example is the use of vemurafenib for patients with melanoma that harbors a BRAF mutation. For the majority of squamous cells carcinomas of the head and neck (HNSCC), genetic knowledge has not yet resulted in stratified approaches to therapy. Apart from site classification, SCCHN can also be grouped into those with p53 mutations (the vast majority of smoking and alcohol-induced tumors) and those harboring human papillomavirus (mainly HPV-16). HPV+ HNSCC is an emerging disease representing a distinct clinical and epidemiological entity. Understanding the genetic basis of both HPV+ and HPV- HNSCC could allow therapeutic targeting of affected pathways for a stratified medicine approach. Recent gene sequencing studies indicate that various mutations within the PI3 kinase (PI3K) signaling pathway are found in both subgroups. E.g. PIK3CA mutation or amplification, and PTEN inactivation by gene copy loss or mutation are present in over 50% of oropharyngeal SCC, suggesting that targeting the PI3K/AKT/mTOR pathway might be an attractive approach in these patients. Other receptor tyrosine kinase mutations occurring at low frequency e.gs FGFR1, FGFR3 and EGFR could be use to select patients for targeted therapies against these proteins, should they be established as driver mutations in these tumors. One significant challenge of targeted therapies based on gene sequencing information is the observation of intra-tumor genetic heterogeneity. Because HNSCC are often readily accessible to multiple biopsies, this is an area where knowledge gained from HNSCC could be exploited to other solid tumors. Another area of significant interest is the use of immune therapy, e.g. anti-PD1 or anti-CTLA4 antibodies to treat solid tumors. Biomarkers predicting which patients will respond to these approaches are in development. Although surgery, chemotherapy or chemo-radiotherapy will remain the mainstay of treatment for most HNSCC in the foreseeable future, the introduction of molecular targeted therapies and immune therapies will play an ever more important role in the future management of HNSCC.

Outcomes and Prognostic Factors of Post-irradiation and de novo Sarcomas of the Head and Neck: A Histologically Matched Case-Control Study

This study was designed to compare post-irradiation sarcomas (PIS) and de novo sarcomas (DN) of the head and neck in terms of tumor characteristics, prognostic factors, and survival outcomes. All (N=83) head and neck sarcoma patients treated at National Cancer Centre, Singapore (Feb 2002-May 2011) were included: DN (N=60; 72%); PIS (N=23; 28%). Clinicopathologic features and outcomes of all patients and histologically matched pairs were compared. Prognostic factors were identified using univariate and multivariate analyses. Median age, gender, smoking status, and tumor size were not significantly different. Significant differences were seen in histology (most prevalent: PIS-sarcoma-NOS; DN-angiosarcoma) and tumor subsite (most prevalent: PIS-nasal cavity and sinuses; DN-skin). Median latency of PIS development was 16.7 years. PIS patients had shorter overall survival (OS) and disease-specific survival (DSS) compared with DN patients, most clearly seen on histologically matched pair analysis: 2-year OS (PIS: 54%; DN: 83%; P=0.028). Multivariate analyses showed that age>50 years (hazard ratio (HR)=3.68; P=0.007), ever-smokers (HR=2.79; P=0.017), and larger tumor-size (cm) (HR=1.12; P=0.045) were associated with worse OS, and age at >50 years (HR=2.77; P=0.04) and ever-smokers (HR=2.94; P=0.021) were associated with worse DSS. When treated with curative intent, no significant survival difference was noted between DN and PIS patients. In our cohort, PIS constituted 28% of head and neck sarcomas. Poorer prognosis traditionally associated with PIS compared with DN was not seen amongst patients treated with curative intent.

Combined chemoradiation for head and neck region myxofibrosarcoma of the maxillary sinus

Adult sarcomas of the head and neck region (HNSs) are considered a rare clinicopathological entity. They account for only 2-15% of all adult sarcomas and for less than 1% of all head and neck malignancies. The preferred initial treatment option is wide surgical excision. Whenever surgery is considered infeasible, a frontline combined-modality approach including radiotherapy and chemotherapy might be proposed. We here report on a case of localized sarcoma of the maxillary sinus treated with induction chemotherapy and subsequent intensity-modulated radiation therapy (IMRT), achieving a persistent complete remission status. A 66-year-old man was referred to our institution hospital for left-sided facial pain with swollen left cheek and ipsilateral facial palsy. Magnetic resonance imaging showed a mass within the left maxillary sinus extending to the orbital floor and adjacent alveolar bones. Histological examination of the biopsy specimen demonstrated a myxofibrosarcoma. The patient underwent induction chemotherapy with gemcitabine 900 mg/m2 (days 1-8) and taxotere 80 mg/m2 every 3 weeks for 3 cycles and sequential simultaneous integrated boost (SIB) IMRT up to a total dose of 70 Gy/35 fractions to the macroscopic disease with 59.5 Gy/35 fractions to the level IB-II lymph nodes in the left neck. Treatment was well tolerated with mild acute toxicity. Complete remission was achieved at restaging MRI 6 months after the end of the combined modality approach. The patient remains in complete, unmaintained clinical and instrumental complete remission 18 months after treatment, with no late side effects. Combination therapy with induction chemotherapy and sequential SIB-IMRT could therefore be a promising modality for head and neck sarcomas, allowing for simultaneous tumor control and normal tissue sparing.

Diagnosis and Treatment of Head and Neck Sarcomas: Personal Experiences

Sarcomas are a rare group of tumors. They do not commonly manifest as malignancies in the head and neck region. The rare nature of these tumors has resulted in a limited number of publications. This is a descriptive study. We included patients with primary sarcomas of the head and neck, observed between 1991 and 2011 in the department of Otorhinolaryngology (University of Chieti, Italy). We studied the following variables: age, gender, race, origin, primary anatomical location of the neoplasm, pathology, diagnosis and type of cancer treatment employed. During the study period, we found 9 patients with head and neck sarcomas. Of this sample, 6 were males, and 3 were females. The man/woman ratio was 2:1. As far as age is concerned, the most affected age range was between 60 and 80 years. The mean age of the patients was 62. As far as pathology is concerned, we found 7 histopathological variations of sarcomas, and the most common type was the leiomyosarcoma. The most widely used treatment modality was surgical treatment. The data in this study show that head and neck sarcomas are rare tumors with high histological variability, which may involve different anatomical sites. Since these are rare and not well-known lesions, further epidemiological studies must be carried out, in order to give us more knowledge about the disease.

Adult Head and Neck Sarcomas: Rare Localization with Difficult Therapeutic Management

Background: Head and neck sarcomas are very rare tumors, accounting less than 1% from all malignancies of this region. They represent a heterogeneous group of tumors with distinct prognostic features. There have been significant improvements in characterizing these sarcomas using traditional morphologic assessments and immunohistochemical analysis, but treatment management is still a big challenge. Prognostic factors are essentially tumor grade, margin status and tumor size. Surgery remains the mainstay of treatment, despite the anatomic constraints, followed by radiation therapy. Aim: to show the rarity of the head and neck localization, and describe epidemiologic, clinico-radiological, histological features, treatment management and the evolution of these tumors. Methods: It is a retrospective descriptive study conducted in the department of medical oncology at Hassan II University Hospital between January 2007 and December 2011, including all patients with histologically proven melanoma of the anorectal area. Results: 10 cases were collected, that represented 7 % from all sarcomas reported at the study period. Five men and 5 women, the mean age was 34 years (range: 17-65years). Local imaging was performed by MRI in six cases and CT scan in 4 cases. It had showed locally advanced disease in 7 cases and among them 6 patients had a metastatic disease at the staging. Lung was the site of predilection of metastases. Histological analysis with the support of immunohisto-chemestry showed diverse histological subtypes. For the 3 cases with localized stages, the mainstay treatment was based on surgery followed by radiation therapy in one case with unclear margins. For metastatic stages, first line of chemotherapy was mainly based on: doxorubicin, ifosfamide, and cisplatine for osteosarcoma. Evolution was marked by disease controlee and remission in 2 patients who underwent surgery with clear margins. Unfortunately by recurrence in patient operated with unclear margins and by rapid progression for metastatic stages leading to death. Conclusion: Adult head and neck sarcomas are very rare tumors with poor prognosis, high aggressiveness and rate of recurrence. They are frequently diagnosed at advanced stages. Treatment management is still a big challenge given to the difficulty of surgery and modest response to chemotherapy.

Synovial sarcomas of the head and neck: Comparative analysis with synovial sarcoma of the extremities

This study analyzed synovial sarcoma (SS) of the head and neck in order to identify features associated with survival improvement and compared them with the survival of SS of limbs. Clinical charts and histopathologic material with analysis for SYT/SSX gene rearrangement of 16 patients were reviewed. The clinicopathologic features and their association with survival were analyzed and compared with 174 SS of limbs. The average age was 24.2 years (range 21-86). Eight cases occurred in each sex. The most frequent site was the parapharyngeal space (PPS). The mean tumor size was 5.38cm. Sixty-nine percent occurred in Stages II-III and 9% in Stage IV. Fifteen cases were excised: R0 resection in seven (46.7%) cases and R1 resection in eight (53.3%) cases. No patient with R0 resection has recurred, and three patients (37.5%) with R1 resection have recurred (p=0.035). Patients with R0 surgery had better survival rates compared to those who received other treatments (p=0.045). SS of head and neck showed a 5-year survival rate of 58% compared to 44.6% of the limbs (p=0.450). The most prevalent location was the PPS. Surgical resection with clear margins correlated with low recurrence. Head and neck sarcomas had similar survival rates compared to sarcomas of limbs.

A Clinicopathological Study of Head and Neck Sarcomas

A Clinicopathological Study of Head and Neck Sarcomas