Ewing's Sarcoma of Mandible: A Case Report with Review of Literature.

Background: Ewing sarcoma (ES), a rare malignancy, comprises whatever the age, 4-15% of all primary bone tumors. It represents 1% of all malignant tumors in children and is the fourth most common bone malignancy after myeloma, osteosarcoma, and chondrosarcoma. Case description: A 12-year-old boy came to the Oral Surgery Department of Bretonneau Hospital referred by his dentist with a rapidly evolving swelling in the left mandibula for 6 weeks, which was initially diagnosed as a facial cellulitis. Cone beam computed tomography (CBCT) showed a poorly defined, expansile, and osteolytic tumor on the left side of the mandible. Clinical and radiographic findings were in favor of an aggressive primitive bone tumor. A mandibular biopsy under general anesthesia was performed in the Department of Surgical Oncology at Institut Curie in Paris, revealing an ES. Conclusion: Mandibular ES can mimic dental infections when swelling is the main clinical manifestation, which can lead to a delayed diagnosis. A correlation between clinical, radiological, histopathological, and immunohistochemical with cytogenetics is needed to confirm the diagnosis. Moreover, smaller tumors have better survival.Dentists must therefore be aware of the clinical signs of ES in order to quickly refer patients to a specialized department. How to cite this article: Bellut N, Lutz CM, Lesnik M, et al. Ewing's Sarcoma of Mandible: A Case Report with Review of Literature. Int J Clin Pediatr Dent 2024;17(2):187-190.

A rare intraosseous synovial sarcoma of the mandible: A case report.

INTRODUCTION: Synovial sarcoma is a relatively common high-grade soft-tissue sarcoma. This lesion accounts for 5-10 % of soft-tissue sarcomas, which tend to appear in the limbs, especially the lower limbs. Synovial sarcoma in the neck is rare and causes involvement of the head and neck in 6-7 % of cases. Intraosseous involvement of the mandible is rare. In this report, a rare intraosseous synovial sarcoma of the mandible is reported. PRESENTATION OF CASE: A 29-year-old man with a complaint of painless outgrowth of the gingiva in the posterior region of the left mandible. In the intraoral examination, an exophytic, firm, smooth and well-defined lobulated mass on the alveolar ridge, extending from distal of the second premolar to mesial of the second molar, was observed. On a radiographic examination, a radiolucent lesion with an ill-defined border was seen in the left body of the mandibular with perforation of the buccal cortex. In a histological examination, immunohistochemistry confirmed synovial sarcoma. The patient underwent surgery with wide margins, and radiotherapy after surgery. DISCUSSION: The main treatment method is surgery with a wide margin. Radiotherapy as an adjuvant treatment along with surgery is the second most common treatment method. Radiotherapy is recommended in cases of involvement of the margin of the lesion, size greater than 5 cm, and recurrence of the lesion. Chemotherapy after surgery has been used less frequently. CONCLUSION: Surgical resection with a wide margin is the main treatment. Adjuvant radiation therapy and chemotherapy can be helpful in tumor control, especially in monophasic cases.

Malignancy of Nose and Paranasal Sinuses: An Institutional Study in Central India.

INTRODUCTION: Malignancy of the nose and paranasal sinuses is a highly heterogeneous tumor group that arises from various cell types commonly seen in the fifth to sixth decades of life, with twice as much commonness in males. Patients present with varied clinical presentations like nasal obstruction, facial swelling, orbital complications, etc. Squamous cell carcinoma and adenocarcinoma are the most common variant. Surgery followed by adjuvant chemo or radiotherapy is the treatment of choice. METHODS: The study was undertaken in the Department of Otorhinolaryngology All India Institute of Medical Sciences, Bhopal, India, from 2021 to 2023. It was a retrospective study in which patients diagnosed and underwent treatment in the last 2 years were enrolled. Data were retrieved from the medical record department and surgical registry. Twenty-eight patients were recruited for the study. Detailed history, clinical examination, imaging findings, surgical plans, postoperative adjuvant therapy details, and histopathological findings were recorded. RESULTS: There were 18 (64.2%) males and 10 (35.8%) females, with a male-to-female ratio of 1.8: 1. The mean age of patients was 50.5 years. Facial swelling was the most frequent symptom (n=15, 54%). Twenty-one (75%) patients use chewable tobacco, while sixteen (57%) are smokers. All our patients belong to the lower socioeconomic group. Endoscopic resection was done in 15 (62.5%) patients, and combined open and endoscopic approaches were used in 9 (37.5%) patients. The most common histological variant was squamous cell carcinoma (n=8, 28%). CONCLUSION: Malignancy of the nose and paranasal sinus is very rare. They presented with varied masked clinical presentations of benign diseases. Early identification and high clinical suspicion, along with imaging studies, are pivotal in managing malignancy of the nose and paranasal sinuses.

A malignant peripheral nerve-sheath tumor: A CARE case report.

INTRODUCTION: Malignant peripheral nerve-sheath tumor (MPNST) is an extremely rare sarcoma of the nasal cavity and paranasal sinuses. Non-specific clinical and radiological presentation and challenging histological diagnosis make it little known by physicians. We describe a case of maxillary sinus MPNST, following CARE guidelines. CASE REPORT: A 62-year-old woman consulted for swelling of the right cheek and hard palate with several months' progression. CT and MRI revealed a tissue mass in the right maxillary sinus with osteolysis of the orbital and maxillary floors, hard palate and lateral and medial walls of the maxillary sinus. Biopsy confirmed diagnosis of low-grade MPNST. After total resection and 60Gy adjuvant radiotherapy, 2-year follow-up showed no signs of recurrence. CONCLUSION: MPNST in the nasal cavity and paranasal sinuses is very rare. Because of a high risk of recurrence, wide resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.

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.

BCOR-Rearranged Sarcoma of the Tonsil.

A tonsillar mass in a young patient with no medical issues routinely presents as an infectious process. Practitioners must maintain a broad differential if diagnostic testing does not support an infection. Neoplasm must be excluded. Otolaryngologists must consider malignancies other than squamous cell carcinoma, the most common oropharyngeal malignancy, and lymphoma. Rare tumors, such as sarcomas, must also be considered. Otolaryngologists must be familiar with the proper management of rare oropharyngeal malignancies.

Anterior Skull Base Sarcomas: Report of Characteristics and Outcomes at a Tertiary Care Cancer Center.

Objective This study was aimed to describe our institutional experience on characteristics and treatment outcomes of sinonasal sarcomas invading the anterior skull base. Design Present study is a retrospective review. Setting The study was conducted at an academic cancer care center. Participants Thirty-one patients with skull base sarcomas treated with primary surgery from 1979 to 2015 were identified for this study from a preexisting database. Main Outcome Measures Survival and recurrence outcomes using the Kaplan-Meier method were the focus areas of the study. Results The median age was 44 years (range: 13-69 years). Twenty patients were male (64.5%). Twenty-nine patients underwent open craniofacial resection (93.5%) and two patients underwent endoscopic resection (6.5%). The majority of tumors were staged pT4 (77.4%). The most common pathologies were leiomyosarcoma (19.4%), osteosarcoma (16.1%), and chondrosarcoma (12.9%). Of those with known margin status, 10 patients had positive/close margins (32.2%) and 16 patients had negative margins (51.6%). Most tumors were high grade (74.2%). Twenty-three patients (74.2%) received adjuvant radiation and four patients (12.9%) received adjuvant chemotherapy. There were nine postoperative complications (29%) including one mortality and three cerebrospinal fluid leaks. There were 10 local, 2 regional, and 5 distant recurrences over a median follow-up of 74 months (range: 1-300 months). The 5-year disease-specific survival (DSS) was 69.8%. The 5-year locoregional recurrence-free probability (RFP) was 63.2% and the 10-year distant RFP was 71.7%. The 5-year DSS for high grade tumors was 64.2 and 85.7% for low grade tumors ( p = 0.117). Conclusion This study contributes an updated analysis of anterior skull base sarcomas. Five-year DSS is approximately 70%. Analysis of survival outcomes based on grade, tumor size, and other factors is limited by small sample size and the rarity of these tumors.

Tracheal chondrosarcoma: A case report, systematic review, and pooled analysis.

BACKGROUND: Tracheal chondrosarcoma is a rare malignancy, and formal treatment guidelines have not been established due to the lack of high quality studies. Best evidence at this time is limited to case reports. AIM: Explore the role of surgical intervention, radiation therapy, and chemotherapy, and the long-term outcomes for these interventions for tracheal chondrosarcoma. METHODS AND RESULTS: A literature search was performed using PubMed (1959-2020) and ResearchGate (1959-2020) using medical subject heading terms "tracheal chondrosarcoma" OR "trachea chondrosarcoma." Additional reports were identified within reviewed articles and included for review. Articles pertaining to chondrosarcomas of the lung, bronchus, larynx, or other head and neck subsites were excluded. Cases of chondromas were excluded. Thirty-five patients with tracheal chondrosarcoma were identified in the literature since 1959. Advanced age was significantly associated with recurrent or persistent disease (p = .003). The majority (77%) of cases were treated with open surgical resection, with an open approach and negative surgical margins being significantly associated with being disease-free after treatment (p = .001 and p < .001, respectively). Adjuvant radiotherapy was reserved for those unfit for surgery or for recurrent disease. Tumor size, extra-tracheal extension, tumor calcification, location, and initial diagnosis were not associated with tumor recurrence. CONCLUSION: Non-metastatic tracheal chondrosarcoma can be treated by adequate surgical resection, with little to no role for adjuvant radiotherapy or chemotherapy. Open surgery and negative margins were associated with oncologic control, while advanced age was associated with recurrent or persistent disease.

Pretreatment tumor sampling and prognostic factors in patients with soft-tissue sarcoma of the head and neck.

BACKGROUND: Insufficient preoperative work-up and consequent intralesional or marginal resection of soft-tissue sarcomas of the head and neck (STSHNs) is common. METHODS: This retrospective cohort study comprised 63 patients with STSHN treated at the Helsinki University Hospital between 2005 and 2017. We assessed the effect of pretreatment tumor sampling on surgical margin status and need for supplemental surgery, as well as prognostic factors and survival. RESULTS: The lack of representative pretreatment biopsy specimen was associated with unfavorable margin status. Primary surgery at a non-academic center was associated with need for supplemental surgery. The 3-year overall survival (OS) was 68%, disease-specific survival (DSS) 71%, and recurrence-free survival (RFS) 61%. Higher tumor grade and primary tumor size over 5 cm were associated with reduced DSS. CONCLUSIONS: Diagnosis and management of STSHNs should be centralized to experienced academic centers. Decision-making between needle biopsy, open biopsy, or upfront radical surgery depends on tumor location and size.

Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature.

Biphenotypic sinonasal sarcoma (BSNS) is a rare recently described distinct spindle cell sarcoma which arises exclusively in the sinonasal region and is characterized by concomitant neural and myogenic differentiation. Before this neoplasm was characterized, most were classified as other entities including adult fibrosarcoma, monophasic synovial sarcoma and malignant peripheral nerve sheath tumor. By immunohistochemistry, these tumors characteristically express S100 and smooth muscle actin (SMA) and/or muscle specific actin (MSA). Most cases harbor rearrangements of PAX3 (paired box gene 3), and the most frequent translocation partner is MAML3 (mastermind like transcriptional coactivator 3). Herein, we described three cases of BSNS involving the nasal cavity with or without paranasal sinus involvement. We also did a literature review of the clinical features, histologic and immunophenotypic findings, cytogenetics, pathogenesis and behavior of this rare entity.

Understanding giant cell sarcoma of the head and neck: A population-based study.

BACKGROUND: Giant cell sarcomas (GCS) are rare head and neck neoplasms. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was analyzed for all patients who have been diagnosed with GCS from 1973 to 2014. RESULTS: Four hundred and forty cases of GCS of the head and neck were identified. The average age at diagnosis was 74.4 years, 86.8% were white, 82.5% were male, 70.7% were insured, and 88.2% lived in an urban metropolitan region. Connective tissue was the most frequent primary site (42.5%). The 5Y-DSS rate was 91.1%, while the 5Y-OS was 54.6% for all cases. Patients treated with surgery alone had the highest 5Y-DSS rate of 94.5. T-classification odds ratio was a significant predictor of survival accounting for confounding variables on multivariate analysis. CONCLUSIONS: GCS presents most frequently in connective tissue of the head and neck with overall high probability of survival. The treatment of choice is surgery alone.

Assessment of Prognostic Factors and Adjuvant Treatment Modalities in Adult Head and Neck Soft Tissue Sarcoma Patients Treated With Upfront Surgery.

Objectives Head and neck soft tissue sarcomas (HNSTSs) are a heterogeneous group of rare tumors. Surgical resection with negative margins remains the standard primary treatment for patients with HNSTS. The role of chemotherapy (CT) and radiotherapy (RT) remains controversial. In this multicenter study, we aimed to demonstrate the real-world assessing prognostic factors and the effect of adjuvant treatment modalities in adult patients with HNSTS treated with upfront surgery. Methods We included a total of 47 patients who underwent curative-intent resection of a primary HNSTS between 2000 and 2019. Results The median follow-up was 29 months. The median age of patients was 51 years, and 66% of patients were male. The median relapse-free survival (RFS) of the study population was 31 months (range: 1.0-61.1 months), and the median overall survival (OS) was 115 months (range: 60.8-169.2 months). The univariable analysis revealed that treatment modalities showed a significant impact on RFS (p = 0.021); however, no difference was found in its impact on OS (p = 0.137). R0 resection did not showed impact on RFS (p = 0.130), but a significant association was found with OS (p = 0.004). In multivariable analysis, T stage of the tumor (hazard ratio [HR]: 3.834; 95% CI: 1.631-9.008; p = 0.002) and treatment with surgery and sequential RT and CT (HR: 0.115; 95% CI: 0.035-0.371; p < 0.001) were independent factors associated with RFS. R0 resection was independently associated with OS (HR: 4.902; 95% CI: 1.301-18.465; p = 0.019). Conclusion Our study revealed that R0 resection improved OS, and T3-4 stage of tumor was a negative independent factor for RFS in surgically resected HNSTS patients. The use of sequential CT and RT after resection was associated with a better RFS, which emphasizes the importance of multidisciplinary evaluation of the treatment of HNSTS. Randomized prospective studies are needed.

Diagnostic use of fine-needle aspiration cytology and core-needle biopsy in head and neck sarcomas.

The diagnostic role of fine-needle aspiration cytology (FNAC) and core-needle biopsy (CNB) has not been comprehensively assessed in head and neck sarcomas. A systematic review of published cases (1990-2020) was conducted. Diagnostic performance of both FNAC/CNB to determine tumor dignity and histopathological diagnosis was calculated. One hundred and sixty-eight cases were included for which FNAC (n = 156), CNB (n = 8), or both (n = 4) were used. Predominant histologies were skeletal muscle, chondrogenic and vascular sarcomas. FNAC correctly assessed dignity in 76.3% and histology in 45% of cases. Dignity was significantly better for vascular tumors, metastatic and recurrent specimens, and worse for chondrogenic sarcomas. CNB showed a 92% accuracy to identify dignity and 83% for histopathology. FNAC and CNB are useful methods for the diagnosis of head and neck sarcomas, particularly well-suited in the context of recurrent or metastatic disease. The role of CNB remains largely unexplored for this indication.

Epithelioid Sarcoma of the Tongue: An Unusual Tumor Mimic for Squamous Cell Carcinoma.

INTRODUCTION: Epithelioid sarcoma is a rare soft tissue malignancy that usually presents in the distal extremities along fascial planes, aponeuroses or tendon sheaths. Very rarely, it presents as a primary or metastatic lesion of the head neck. METHODS: Chart review and comprehensive literature review using PubMed and Google Scholar. RESULTS: A 17-year-old non-smoker was referred for evaluation of an ulcerative lesion of the right anterior-lateral tongue, progressing over several months. Incisional biopsy was concerning for squamous cell carcinoma. He underwent partial glossectomy and bilateral selective neck dissections, with reconstruction using a radial forearm free flap. Final pathology was consistent with epithelioid sarcoma, proximal type, demonstrating perineural invasion and close margins. Post-operative PET scan showed no persistent nor metastatic disease. He underwent post-operative radiation therapy to a total dose of 56 Gy to the primary site. CONCLUSION: Epithelioid sarcoma is a rare malignancy usually presenting in the extremities of young adults, which uncommonly presents in the head and neck as a primary or metastatic lesion. The infrequency of these lesions has prevented development of evidence-based treatment recommendations. As with most sarcomas, surgery is the mainstay of therapy for epithelioid sarcoma, while radiation has been used in an adjunctive role. Although a rare lesion, epithelioid sarcoma should be considered in the differential diagnosis for atypical epithelioid lesions of the tongue and oral cavity when patient demographics, gross lesion characteristics, or histopathology are not entirely consistent with more common lesions, such as squamous cell carcinoma.

Laryngeal Synovial Sarcoma: A Systematic Review of the Last 40 Years of Reported Cases.

Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct investigation and management, and treatment is largely based on what is known for synovial sarcoma of the upper and lower limbs. This PROSPERO-registered study aims to review the diagnostic methods, treatment regimens, and survival outcomes for patients with synovial sarcoma of the larynx. A systematic search of databases Medline, Embase, SCOPUS, and Web of Science was undertaken in December 2017. The literature search identified 1031 potentially relevant studies, and after the deletion of duplicates and excluded papers, 98 full-text articles were screened. A total of 39 cases were reviewed from 32 studies in the data extraction. The average age at the time of laryngeal synovial sarcoma diagnosis was 32 years (range, 11-79 years). In all cases (n = 39), patients underwent wide surgical excision, with 20 patients requiring a partial or total laryngectomy. A total of 18 patients received adjuvant and 3 received neoadjuvant radiotherapy. Chemotherapy was used in 10 cases, with ifosfamide the most frequently used agent. There was considerable variability in the order and combinations of the abovementioned treatments. No clinicopathologic factors or treatment regimens were associated with improved overall survival or lower rate of recurrence. There is a paucity of literature and heterogeneity in clinical approaches to this highly aggressive sarcoma. Reporting of cases must be standardized and formal guidelines must be established to guide clinical management.

Open resection and reconstruction of a Nasoseptal Chondrosarcoma: case report and literature review.

BACKGROUND: Primary chondrosarcomas of the nasal septum are rare, with a variety of clinical features that evade detection and treatment. While endoscopic surgery has become increasingly accessible, open approaches may be needed to provide adequate visualization for tumour ablation and reconstruction. We report the resection and reconstructive considerations of a septal chondrosarcoma. CASE PRESENTATION: A 75-year-old woman presented with a 3-year history of a slow growing, firm mass in the nasal tip causing protrusion and septal fullness. Computed Tomography scan of the paranasal sinuses revealed a well-circumscribed, 2.2 cm mass at the anterior nasal septum extending into the right vestibule. Biopsy of the cartilaginous lesion confirmed the diagnosis of a low-grade chondrosarcoma by histopathology. The tumour was removed using a transcolumellar open rhinoplasty approach with a large septal resection. Primary reconstruction of the surgical defect was performed using an L-shaped strut from the nasal keystone area to the columella. Follow-up examinations demonstrated no evidence of recurrent disease with satisfactory functional and cosmetic outcomes at 1-year. CONCLUSION: This report describes a case of nasal septal chondrosarcoma successfully treated with surgical excision using an open rhinoplasty approach. Only 5-10% of chondrosarcomas are located in the head and neck region and arise rarely in the nasal septum in approximately 2-4%. With this mass, an open rhinoplasty approach was required to allow optimal exposure of the margins and to facilitate reconstruction without disruption of normal sinonasal anatomy and function. Although rare, chondrosarcoma of the nasal septum should be considered in the differential diagnosis of nasal masses.

Malignant peripheral nerve sheath tumor of nasal cavity and paranasal sinus with 13 years of follow-up-A case report and review of literature.

Although extremely rare, sarcomas including malignant peripheral nerve sheath tumors should be considered in the differential diagnosis of sino-nasal tract lesions. Long-term cure is possible through definitive operative management followed by adjuvant therapy.

Using Image-guided Intensity-modulated Radiotherapy on Patients With Head and Neck Soft-tissue Sarcoma.

BACKGROUND: Image-guided intensity-modulated radiotherapy (IG-IMRT) is increasingly being used to treat patients with soft-tissue sarcoma (STS) of the head and neck. Although there is no comparison between IMRT and conventional radiation therapy (CRT) concerning their efficacy. In this analysis, we compared CRT and IMRT outcomes for head and neck STS. PATIENTS AND METHODS: Sixty-seven patients who underwent radiotherapy between 1994 and 2017 were identified. RESULTS: The median follow-up was 31 months. Of the 67 patients, 34% were treated with CRT technique and 66% with IG-IMRT. The locoregional relapse rate following IMRT was 21% versus 70% with CRT (p<0.001) and the 5-year locoregional control was 69% versus 28%, respectively (p=0.01). IG-IMRT was associated with non-significant, less acute, and chronic adverse events. In the multivariate analysis, a significant influence of radiation technique on locoregional control was confirmed (p=0.04). CONCLUSION: IG-IMRT seems to be associated both with higher locoregional control as well as lower acute and chronic toxicities.

Nasal Chondrosarcoma of the Lower Lateral Cartilage.

Head and neck chondrosarcomas are incredibly rare with documented cases arising from skull base, maxilla, larynx, and nasal septum. We present the first reported case of chondrosarcoma arising from the lower lateral cartilage of the nose treated with surgical resection and primary reconstruction.

Multimodality treatment of head-and-neck soft-tissue sarcomas and short-term outcomes: Analysis from sarcoma medical oncology clinic.

BACKGROUND: Head-and-neck soft-tissue sarcomas (HNSTS) are extremely rare and lack definite guidelines. METHODS: We retrospectively analyzed consecutive adult patients with HNSTS who presented to our sarcoma medical oncology clinic from January 2016 to October 2017. RESULTS: There were a total of 30 patients. Unresectable localized disease was seen in 13 (43%) patients, metastatic disease 10 (34%) patients, while resectable disease in 7 (23%) patients only. Among unresectable localized disease, 3 (25%) patients could be converted to resectable disease after neoadjuvant therapy. Median follow period was 11 months. Progression-free survival was 19 months in patients with resectable disease and 6 months in patients with the unresectable/metastatic disease. Median OS was not reached. CONCLUSION: Unresectable HNSTS has a poor outcome. Neoadjuvant therapy can be tried in selected cases for achieving respectability or for vital organ preservation until robust data are available. A multidisciplinary approach for local control is crucial in managing unresectable HNSTS.