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At least 19 recordsLinked to original sources
PubMedDate not supplied

Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research.

A Head and Neck Sarcoma Registry was established by the Society of Head and Neck Surgeons to review treatment results of a rare tumor by surgeons with special interest in this anatomic site. Two hundred fourteen patients were analyzed. There were 194 adult tumors and 20 pediatric tumors. The major sites included parotid and neck, 20%; face and forehead, 18%; maxilla and palate, 13%; scalp, 12%; mandible, 11%; paranasal sinuses, 7%; larynx, 2%; and oral cavity, 5%. Eighty-four percent were resectable. The disease-free survival was 56%; overall survival was 70% at 5 years. Major determinants of survival were adequacy of resection (margins free of tumor) and tumor type. Survival differed according to tumor cell type (tumor grade was not available). Patients with chondrosarcoma and dermatofibrosarcoma had survival approaching 100%. Patients with malignant fibrous histiocytoma (MFH) and fibrosarcoma (FSA) had intermediate survival of 60% to 70%. The worst survival, less than 50% at 5 years, occurred in patients with osteosarcoma, angiosarcoma, and rhabdomyosarcoma in decreasing order. This suggests a rationale for identifying high-risk patients for prospective adjuvant protocols. This study emphasizes the value of recording uncommon tumors to provide relevant information for future study and possibly therapy.

Adolescent↗
PubMed2005

Multidisciplinary management of head and neck sarcomas.

BACKGROUND: Head and neck sarcomas are extremely rare. This article reviews the management and outcomes in a multidisciplinary clinic. METHODS: The records of 41 male and 19 female patients (mean age, 50 years) were reviewed. Forty percent underwent surgical resection only, 35% underwent surgery and adjuvant therapy, and 25% underwent radiotherapy and/or chemotherapy without surgery. Seventy-one percent had complete histologic clearance. RESULTS: The mean follow-up was 3 years and 10 months, with an overall 5-year survival rate of 60%. Completeness of surgical excision was highly significant in determining 5-year local control (p < .025), and the addition of adjuvant radiotherapy had a major effect on local control, but only if complete surgical clearance had been achieved (p < .025). As expected, patients with more aggressive tumors had a significantly poorer overall prognosis, and achieving local control led to an enhanced 5-year survival (p < .025). CONCLUSION: These tumors are best managed in multidisciplinary clinics, and the mainstay of treatment is wide local excision and planned postoperative adjuvant radiotherapy.

Adolescent↗
PubMed2002

Sarcomas of the head and neck.

Sarcomas of the head and neck are extremely rare, accounting for less than 1% of all neoplasms of the head and neck. These sarcomas arise in both soft tissues and bone and thus cannot be treated by a single approach. The clinical behavior of these tumors varies considerably. Patients with low-grade lesions are prone to local recurrence, whereas those with high-grade lesions develop both local recurrence and disseminated disease. An additional complicating factor is the multiple sites in which sarcomas may arise in the head and neck. All of these factors make it difficult to ascertain the optimal treatment approach for sarcomas. This article reviews the current literature (as well as the author's own experience) and provides a general treatment guideline for sarcomas of the head and neck.

Clinical Trials as Topic↗
PubMed2004

Update in management of head and neck sarcoma.

PURPOSE OF REVIEW: This update addressing head and neck sarcoma presents new data published after January 2003. This new information is presented following review of contemporary management principles established before 2003. RECENT FINDINGS: The application of advances in molecular and genetic techniques to characterize individual sarcomas has improved classification schemes and hopefully will lead to individually tailored therapy. Maturation and refinements of large tumor registries are permitting more detailed analysis of larger contemporary series of the rare head and neck sarcomas. Improved long-term survival of patients treated for sarcoma in their childhood has allowed study of the morbidity induced by treatment. Novel treatment strategies are under study to decrease morbidity without diminishing the chance for cure. SUMMARY: Progress in improving the treatment of sarcoma of the head and neck has been slow due to the lack of a large clinical experience with this rare neoplasm. Advances continue and are anticipated to be most striking in the study of genetic mechanisms.

Disease Management↗
PubMed1996

Head and neck sarcomas: a review.

Sarcomas of the head and neck are rare. They present a challenge to the head and neck oncologist that is frequently quite different from that found in epithelial, salivary, and endocrine malignancies of the head and neck. The focus of this article is on diagnostic criteria, prognostic indicators, and therapy, and due to the large number of lesions, an in-depth review of the commonest: osteosarcoma (OS), chondrosarcoma (CS), rhabdomyosarcoma (RMS), fibrosarcoma (FS), malignant fibrous histiocytoma (MFH), and synovial sarcoma (SS). Less common variants will also be mentioned. Twelve cases are presented, consisting of OS(2), RMS(1), CS(3), MFH(2), SS(2), FS(1), and miscellaneous(1). Conclusions consist of an enunciation of the general principles of management of sarcoma of the head and neck.

Adult↗
PubMed2026

Next-generation sequencing in head and neck sarcoma: a single-centre institutional experience and review of the literature.

Head and neck sarcomas (HNS) are rare, heterogeneous malignancies representing less than 1% of head and neck cancers. Their complex anatomy and overlapping morphologies pose significant challenges for traditional diagnosis. We aimed to evaluate the clinical utility of next-generation sequencing (NGS) within a tertiary referral centre and synthesise these findings with current global molecular standards. We conducted a retrospective review of an original, previously unpublished, cohort of 12 patients with histologically verified HNS treated at University College London Hospital (UCLH) between 2023 and 2024. Molecular profiling included targeted DNA (RMH200) and RNA-fusion panels. This was supplemented by a qualitative synthesis of 16 key studies (2010-2026) identified through a systematic search strategy. In the institutional cohort, NGS provided definitive diagnostic or therapeutic clarification in 66% of cases (8/12). Key findings included the identification of pathognomonic fusions (such as EWSR1::FLI1, PAX3::MAML3), a novel MAMLD1::VGLL3 fusion, and actionable variants such as BRAF V600E and MYOD1. Furthermore, the formal exclusion of Neurotrophic tropomyosin receptor kinase (NTRK) fusions in some cases allowed for therapeutic streamlining. Literature synthesis aligned these results and emphasised the need for NGS for more accurate diagnosis and adequate treatment. NGS is a clinical necessity in the management of ultra-rare HNS. By transitioning from traditional morphology to high-resolution molecular interrogation, clinicians can resolve diagnostic ambiguity and identify targeted therapeutic pathways. Integration with emerging 2026 standards, including epigenetic classification and liquid biopsy monitoring, represents the future of precision surgery in head and neck sarcoma.

Humans↗
PubMed1999

Sarcomas of the head and neck.

Sarcomas are rare malignancies of mesenchymal origin. Computed tomographic and magnetic resonance imaging characteristics, as well as histologic findings and epidemiology, of sarcomas of the head and neck are reviewed. The sarcomas discussed include rhabdomyosarcoma, fibrosarcoma, osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, alveolar soft part sarcoma, Ewing's sarcoma, and synovial sarcoma. The imaging findings of these entities frequently are nonspecific. Imaging, particularly magnetic resonance imaging, has a major role in defining the extent of these tumors. This is important because complete surgical excision is the preferred method of treatment. Imaging also is useful in planning radiation therapy and determining prognosis.

Head and Neck Neoplasms↗
PubMed1989

Management and prognosis of head and neck sarcomas.

Between 1962 and 1988, a total of 104 patients with head and neck rhabdomyosarcoma (RMS) and 17 patients with nonrhabdomyosarcoma (NRMS) were evaluated and treated at St. Jude Children's Research Hospital. All parameningeal sites (middle ear, orbit, or nasopharynx) were excluded from further analysis; thus, 50 patients represent the cohort of head and neck sarcomas for this review. Survival was good in this group of patients, 28 of 50 being alive and disease-free at last follow-up. Twenty of the 38 patients with RMS were alive and disease-free. Similarly, 8 of the 12 patients with NRMS were disease-free at a median follow-up of 5 years. However, the site and size of the primary tumor impacted on the extent of the initial resection and further treatment in addition to surgery. Although the treatment policy evolved over time to a stage-specific strategy for treatment modalities, the data suggest that surgery alone may be sufficient initial therapy for a subset of patients. For patients in whom complete resection is not achieved, the addition of radiotherapy and chemotherapy may result in improved survival.

Adolescent↗
PubMed1993

Head and neck sarcomas: prognostic factors and implications for treatment.

One hundred and thirty patients with soft tissue sarcoma of the head and neck were treated at the Royal Marsden Hospital between 1944 and 1988. Pathological review was possible in 103 of these cases; only pathologically reviewed cases have been analysed. The median age at presentation was 36 years, and 53% were male. Four had neurofibromatosis type I, and one previous bilateral retinoblastoma. Six had undergone previous radiotherapy, 12 to 45 years prior to developing sarcoma. The tumours were < or = 5 cm in 78% of cases and high grade in 48%. Only one patient presented with lymph node metastases and only one with distant metastases (to lung). Malignant fibrous histiocytoma was the commonest histological type, occurring in 30 cases. The overall 5 year survival was 50% (95% CI 39-60). Local tumour was the cause of death in 63% of cases and 5 year local control was only 47% (95% CI 36-58) with local recurrence occurring as late as 15 years after treatment. The only favourable independent prognostic factor for survival was the ability to perform surgery (other than biopsy), with or without radiotherapy, as opposed to radiotherapy alone (hazard ratio 0.39; P = 0.003). Only one patient had a biopsy with no further treatment. Favourable independent prognostic factors for local control at 5 years were site (tumours of the head as opposed to the neck, hazard ratio 0.42; P = 0.02) and modality of treatment (combined surgery and radiotherapy compared to either alone, hazard ratio 0.31; P = 0.002). Patients in the combined modality and single treatment modality groups were well balanced for T stage, grade and tumour site. The patients in the combined treatment group had less extensive surgery, yet their local recurrence-free survival was longer. Unlike soft tissue sarcomas at other sites, those in the head and neck region more often cause death by local recurrence. The addition of radiotherapy to surgery may result in longer local recurrence-free survival.

Adult↗
PubMed1996

[Head and neck sarcoma].

We reviewed patients with primary soft tissue and bone sarcomas treated in our department. Malignant tumors arising in somatic, connective, soft tissues, especially those of the head and neck, are uncommon and few reports have been published. Between 1980 and 1995 15 patients with head and neck sarcoma were seen. The major sites involved included: paranasal sinuses and orbits (2 patients), neck (4) and 1 each in the forehead, temporal region, mandible, parapharyngeal space, tongue, hypopharynx, larynx, cervico-mediastinal region and nasal cavity. Follow-up ranged from 1-16 years. The youngest patient was a 12-year-old male. Rhabdomyosarcoma, one of the most common mesenchymal tumors found in the head and neck, especially in children, was excluded from our study because of its different clinical behavior and its chemosensitivity, unlike other sarcomas. Most of our patients were treated with wide-field resection; 12 received postoperative radiation, and 3 combined surgery and chemotherapy. Wide excision with negative margins is considered an important, positive, prognostic factor, as the extent and adequacy of excision largely determines survival and the incidence of local recurrence. All our patients underwent initial, wide, local excision of the lesion. Our observations strongly suggest that wide resection is the most effective means of therapy for sarcoma of the head and neck. Radical neck dissection was not only performed for manifest regional neck metastases, but was also used for completing the enlarged, wide-excisions of liposarcomas. The worst survival was in patients with osteosarcoma, angiosarcoma and fibrosarcoma, with an absolute 5-year survival of 60% (9/15).

Adult↗
PubMed2005

Sarcomas of the head and neck. Results from the treatment of 25 patients.

AIMS: Head and neck sarcomas comprise a heterogenous and biologically diverse group of rare neoplasms. In an effort to clarify some of the obscure clinical behavior of head and neck sarcomas, we present our experience and review the relevant literature. METHODS: Retrospective analysis of patients with histologically proven head and neck sarcomas treated in a tertiary Hospital Department between 1992 and 2002. RESULTS: During this period, 25 patients with head and neck sarcomas were registered. Follow-up ranged from 8 to 144 months. Twenty-three patients were treated with surgery as the primary modality; 14 were treated by surgery alone. Clear margins were obtained in all of them and local control was achieved in 12/13. The 2- and 5-year survival rates for the entire group were 80 and 40%, respectively. Mean overall survival time of our patients was 62 months (median 52 months). CONCLUSIONS: Surgical treatment remains the cornerstone of therapeutic management of head and neck sarcomas.

Adolescent↗
PubMed1994

[Treatment of primary soft tissue sarcoma of the head and neck].

Soft tissue sarcomas of the head and neck account for less than 1% of all malignant neoplasms in this region. A significantly increased risk of treatment failure is associated with high histologic grade, leading to an aggressive treatment. The medical records of 17 patients with soft tissue sarcomas of the head and neck were reviewed. They were divided into two groups according to age: 13 adults and 4 children. The former underwent wide surgical excision with postoperative radiotherapy in some cases and possibly chemotherapy. The 2 and 5 years survivals were 54% and 46% respectively. The latter, with rhabdomyosarcoma, underwent multimodality treatment (chemotherapy, surgery, radiotherapy). The 2 years survival was 50%. The mainstay of treatment of soft tissue sarcomas of the head and neck in adults remains surgery. Patients with incomplete resection or high grade tumour should receive more aggressive treatment (surgery and postoperative radiotherapy). The use of systemic chemotherapy has showed no statistically significant improvement in local control or survival. However, for rhabdomyosarcoma in children, chemotherapy is an essential part of treatment.

Adolescent↗
PubMed1999

Radiation induced sarcoma of the head and neck.

BACKGROUND: Radiation-induced sarcoma of the head and neck (RISHN) is a long-term complication of treatment. The rarity of this tumour is reflected in the very few series reported in the English language medical literature. The incidence of RISHN is, however, likely to increase due to progressive aging of the population combined with improved survival in head and neck cancer patients resulting from better treatment regimes. Diagnosis and management of this problem can be extremely challenging and the overall outlook has been reported to be very bleak. As survival data from isolated case reports cannot be expected to provide reliable information on outcome, we have reviewed 69 cases reported in the English medical literature since 1966 and pooled this information with our experience in treatment of RISHN. PATIENTS AND METHODS: Ten patients with features of a RISHN were treated at the Royal Marsden Hospital between 1944 and 1997. The features of RISHN, treatment, and outcome were analysed in these patients. Additionally, 61 eligible patients with RISHN reported in the literature between 1966 and 1997 were pooled with nine of our patients to form the RISHN group (n = 70). This group was then compared for survival with 124 patients with a diagnosis of head and neck sarcoma registered on the Head and Neck Sarcoma database at the Royal Marsden Hospital (SHN group). Lifetables were constructed using the Kaplan-Meier method and compared using the log-rank test. RESULTS: There was no site of predilection for RISHN, but malignant fibrous histiocytoma (MFH) was the commonest pathological diagnosis. The period of latency between initial radiation therapy and diagnosis of RISHN ranged from 9 to 45 years with a median of 17 years. Surgery was the mainstay of treatment and follow-up ranged from 6 months to 15 years with a median of 48 months. The actuarial five-year disease free survival in these patients was 60%. CONCLUSION: There is at present little or no prospect for effective prevention of RISHN and therefore, a high index of suspicion based on the patient's symptoms assumes great importance in the outcome of these patients. Although surgical management of RISHN is challenging because of the close proximity of the tumour to important regional structures and the technical difficulties of operating in an irradiated area, complete surgical excision appears to offer the best means for palliation and the only realistic chance for long-term survival.

Adolescent↗
PubMed1987

Synovial sarcoma of the head and neck.

Synovial sarcoma, a malignant soft-tissue tumor that occurs primarily in the extremities, is seen rarely in the head and neck. Although known to behave aggressively in the extremities, recent reports suggest that the course of synovial sarcoma may be more indolent when it occurs in the head and neck. Five new cases of synovial sarcoma of the head and neck are described herein. Of four patients with adequate follow-up, three died of pulmonary metastases at 1, 2 1/2, and 8 years following initial treatment. The fourth patient is alive with disease at the primary site six years after diagnosis. The poor prognosis for patients with this disease suggests that aggressive treatment is necessary.

Adult↗
PubMed1988

Granulocytic sarcomas of the head and neck.

Granulocytic sarcomas (chloromas) are rare tumors with a predilection for head and neck sites. In a review of six cases from two teaching institutions, chloromas were associated with the development of acute myelogenous leukemia or a relapse in five cases. Treatment modalities included chemotherapy and radiation therapy for problematic lesions. Our only survivor received combination therapy despite a negative bone marrow examination. Work-up should include a computed tomographic scan or magnetic resonance imaging to evaluate the extent of the tumor. Bone marrow examination and biopsy should be performed and specimens prepared for immunohistochemical studies or electron microscopy. Recommended treatment includes both aggressive chemotherapy and 2000 to 3000 cGy of external-beam radiotherapy to the tumor when possible.

Adolescent↗
PubMed2004

Management of sarcomas of the head and neck.

Soft tissue and bone sarcomas in the head and neck are rare tumors. The 1000 to 1500 yearly cases in the United States are distributed among at least 10 main histologies and multiple head and neck subsites. Although this makes structured studies difficult to perform and high-level evidence-based treatment algorithms difficult to find, basic treatment recommendations can be made from the existing literature for most histologies and subsites. This paper discusses the epidemiology, natural history, and treatment approaches for several of the most common head and neck sarcomas, including the "adult soft tissue sarcomas," osteosarcoma, chondrosarcoma of the larynx, angiosarcoma, rhabdomyosarcoma, desmoid tumors, and dermatofibrosarcoma protuberans.

Chondrosarcoma↗