Donor site sequelae and patient satisfaction after head and neck reconstruction with a radial forearm free flap.

PURPOSE: Radial forearm free flap (RFFF) being a workhorse flap in head and neck reconstruction, we investigated its donor site delayed consequences. METHODS: Multicentric case series evaluating 189 patients who underwent RFFF for carcinologic reasons at least 6 months before. Patients and surgeon's appreciation regarding the aesthetic and functional consequences of the flap harvest on their daily life were evaluated by questionnaires using likert scales. Medical data were collected from patients' charts retrospectively. RESULTS: Thirty percent of patients had at least one sequelae. Experiencing a graft necrosis did not worsen long-term results. Cosmetic satisfaction was significantly worst for women, according to patients' and surgeons' opinion. For 81% and 92% patients, respectively, the flap harvest had no impact on daily life nor sport practice. Ratio between the benefits of reconstruction and the sequelae at the donor site was judged "excellent" for 77% patients. CONCLUSIONS: Although objectively important, RFFF morbidity has little impact in head and neck cancer patient's life.

NUT carcinoma in children and adults: A multicenter retrospective study.

BACKGROUND: Nuclear protein of the testis (NUT) carcinoma (formerly NUT midline carcinoma) is an aggressive tumor defined by the presence of NUT rearrangement with a poor prognosis. This rare cancer is underdiagnosed and poorly treated. OBJECTIVE: The primary objective of this study was to describe the clinical, radiologic, and biological features of NUT carcinoma. The secondary objective was to describe the various treatments and assess their efficacy. METHODS: This retrospective multicenter study was based on review of the medical records of children and adults with NUT carcinoma with specific rearrangement or positive anti-NUT nuclear staining (>50%). RESULTS: This series of 12 patients had a median age of 18.1 years (ranges: 12.3-49.7 years). The primary tumor was located in the chest in eight patients, the head and neck in three patients, and one patient had a multifocal tumor. Nine patients presented regional lymph node involvement and eight distant metastases. One-half of patients were initially misdiagnosed. Specific NUT antibody was positive in all cases tested. A transient response to chemotherapy was observed in four of 11 patients. Only two patients were treated by surgery and five received radiotherapy with curative intent. At the end of follow-up, only one patient was still in remission more than 12 years after the diagnosis. Median overall survival was 4.7 months (95% confidence interval [CI]: 2.1-17.7). CONCLUSION: NUT carcinoma is an aggressive disease refractory to conventional therapy. Early diagnosis by NUT-specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma to identify the specific rearrangement of NUT gene is useful to propose the optimal therapeutic strategy.

Multicenter prospective micro-costing study evaluating mandibular free-flap reconstruction.

Free-flap mandibular reconstruction is a highly specialized procedure associated with severe complications necessitating re-interventions and re-hospitalizations. This surgery is expensive in terms of health workers' time, equipment, medical devices and drugs. Our main objective was to assess the direct hospital cost generated by osseocutaneous free-flap surgery in a multicentric prospective micro-costing study. Direct medical costs evaluated from a hospital perspective were assessed using a micro-costing method from the first consultation with the surgeon until the patient returns home, thus confirming the success or failure of the free-flap procedure. The mean total cost for free-flap intervention was 34,009€ (5151-119,604€), the most expensive item being the duration of hospital bed occupation, representing 30-90% of the total cost. In the event of complications, the mean cost increased by 77.3%, due primarily to hospitalization in ICU and the conventional unit. This surgery is effective and provides good results but remains highly complex and costly.

Can Early Dissection of Cervical Lymph Nodes Improve Prognosis in Squamous Cell Carcinomas of the Hard Palate.

Squamous cell carcinomas of the hard palate and maxillary alveolar ridge are rare tumours with a poor prognosis. Lymph node dissection is recommended for tumours with nodal involvement, and is rarely performed in the absence of adenopathy. We report a series of patients with squamous cell carcinomas and evaluate the rate of lymph node invasion and its impact on survival. This is a retrospective study of 72 patients treated for squamous cell carcinoma of the hard palate and maxillary alveolar ridge between January 1, 1998 and December 31, 2008 in two cancer centres. Using clinical and radiological assessment, tumours were classified as T1-T2 in 25 patients (34.7%) and T3-T4 in 47 patients (65.3%). At diagnosis, 16 (22.2%) patients had clinical and/or radiological nodal involvement and 7 patients (9.7%) distal metastasis. Among N0 patients, 13 (18%) experienced isolated lymph node recurrence. Two-year global survival was 60%; 5-year survival was 34%. The rate of lymph node invasion observed in squamous cell carcinoma of the hard palate and maxillary alveolar ridge does not differ from other oral cavity sites. Because nodal recurrence worsens the prognosis of such a patient, lymph node dissection should be considered at an early stage, even among N0 patients.

Impact of extensive surgery in multidisciplinary approach of pterygopalatine/infratemporal fossa soft tissue sarcoma.

BACKGROUND: To evaluate a strategy whereby extensive surgery ± external radiotherapy (RT) could improve local control in pterygopalatine/infratemporal fossa (PIF) sarcoma. PROCEDURE: Forty-one patients with a diagnosis of sarcoma involving the PIF and referred to our Institute from 1984 to 2009 were included in the analysis. Patients received multidrug chemotherapy and radiotherapy ± surgery, depending on the period of treatment. RESULTS: The median age at diagnosis was 7.6 years (range: 0.1-22 years). There were 36 RMS, 3 undifferentiated sarcoma and 2 other soft-tissue sarcomas. Sixty-eight percent of patients had meningeal risk factors at diagnosis. Local treatment consisted of RT alone in 19 patients, surgery in combination to RT in 19 patients and surgery alone in 3 patients. The local progression rate (LPR) at 5 years was 45% for the entire population, 59% for the 19 patients treated with RT alone and 34% for the 22 patients who had surgery as part of their treatment. All locoregional failures after extensive surgery occurred at the skull base and/or in leptomeningeal spaces. CONCLUSIONS: Multidisciplinary approach including extensive surgery for PIF sarcoma is feasible and yields good local control with 15/22 patients in local complete remission. Future studies are warranted to confirm these promising results, to evaluate the possibility of avoiding RT or limiting the RT field, and to extend the indication for extensive surgery to other "worse" sites of PM sarcoma such as the paranasal sinuses.

CT-scan prediction of thyroid cartilage invasion for early laryngeal squamous cell carcinoma.

Treatment choice for laryngeal cancer may be influenced by the diagnosis of thyroid cartilage invasion on preoperative computed tomography (CT). Our objective was to determine the predictive value of CT for thyroid cartilage invasion in early- to mid-stage laryngeal cancer. Retrospective study (1992-2008) of laryngeal squamous cell carcinoma treated with open partial laryngectomy and resection of at least part of the thyroid cartilage. Previous laser surgery, radiation therapy, chemotherapy and second primaries were excluded. CT prediction of thyroid cartilage invasion was determined by specialized radiologists. Tumor characteristics and pathologic thyroid cartilage invasion were compared to the radiologic assessment. 236 patients were treated by vertical (20 %), supracricoid (67 %) or supraglottic partial laryngectomy (13 %) for tumors staged cT1 (26 %), cT2 (55 %), and cT3 (19 %). The thyroid cartilage was invaded on pathology in 19 cases (8 %). CT's sensitivity was 10.5 %, specificity 94 %, positive predictive value 13 %, and negative predictive value 92 %. CT correctly predicted thyroid cartilage invasion in only two cases for an overall accuracy of 87 %. Among the false-positive CT's, tumors involving the anterior commissure were significantly over-represented (61.5 % vs. 27 %, p = .004). Tumors with decreased vocal fold (VF) mobility were significantly over-represented in the group of false-negatives (41 vs. 13 %, p = .0035). Preoperative CT was not effective in predicting thyroid cartilage invasion in these early- to mid-stage lesions, overestimating cartilage invasion for AC lesions and underestimating invasion for lesions with decreased VF mobility.

Reconstruction of subtotal pharyngolaryngectomy using a fasciocutaneous free flap with cartilage graft: A case series of 17 patients.

INTRODUCTION: Subcricoid-hemilaryngopharyngectomy (SCHLP) with a reconstruction using a fasciocutaneous free flap armed with cartilage graft (FFACG) aims to avoid permanent tracheostomy while still maintaining the laryngopharyngeal functions. The purpose of this study is to report the outcome of this surgical approach. MATERIALS AND METHODS: Retrospective study including 17 men operated between 2001 and 2019. Specific survival rate included death caused by cancer or SCHLP complications. Complications, functional and oncological outcomes were evaluated retrospectively. RESULTS: There were no locoregional recurrences. One patient died due to inhalation pneumonia 3 years after surgery. Tracheostomy was closed in 13 patients (76.5%). Mean decannulation time was at six [1-14] months after surgery. CONCLUSION: SCHPL with FFACG could avoid total pharyngolaryngectomy with good oncologic results. However, tracheotomy is extended and deglutition recovery is long with high risk of aspirations. These complications justify that such surgery should be realized only on selected patients by experienced surgical teams. Expertise of the surgical team is critical.

Continuous Ropivacaine Peroneal Nerve Infiltration for Fibula Free Flap in Cervicofacial Cancer Surgery: A Randomized Controlled Study.

Introduction: Pain after cervicofacial cancer surgery with free flap reconstruction is both underestimated and undertreated. There is a rational for regional anesthesia at the flap harvest site, but few studies describe it. We assessed the influence of common peroneal nerve infiltration on pain and opioid consumption in patients having oropharyngeal cancer surgery with fibular free flap mandibular reconstruction. Methods: After institutional review board (IRB) approval and written informed consent, fifty-six patients were randomly allocated to perineural catheter with ropivacaine infiltration (ROPI) or systemic analgesia (CONTROL). In the ROPI group, an epidural catheter was placed by the surgeon before closure, and ropivacaine 0.2% 15 mL, followed by 4 mL/h during 48 h, was administered. The primary outcomes were pain scores and morphine consumption during the 48 h postoperative period. We also measured ropivacaine concentration at the end of infusion. Finally, we retrospectively assessed long-term pain up to 10 years using electronic medical charts. Results: Perineural infiltration of ropivacaine significantly reduced pain scores at the harvest site only at day 1, and did not influence overall postoperative opioid consumption. Ropivacaine assay showed a potentially toxic concentration in 50% of patients. Chronic pain was detected at the harvest site in only one patient (ROPI group), and was located in the cervical area in the case of disease progression. Discussion: Although the catheter was visually positioned by the surgeon, continuous ropivacaine infiltration of the common peroneal nerve did not significantly reduce postoperative pain, but induced a blood concentration close to the toxic threshold at day 2. Further studies considering other infiltration locations or other dosing schemes should be tested in this context, both to improve efficacy and reduce potential toxicity.

Influence of postoperative radiotherapy target volumes in unilateral head and neck carcinoma of unknown primary: A multicentric study using propensity score.

OBJECTIVE: To compare the impact of two radiation modalities on loco-regional control, survival and tumour emergence, after node dissection for an unilateral head and neck carcinoma of unknown primary (HNCUP). MATERIALS AND METHODS: This is a multicentric retrospective study of 138 patients with unilateral HNCUP treated between 2002 and 2017. The absence of primary tumour was assessed by a systematic panendoscopy and positron emission tomography. Neck dissection was initially performed for all patients. Radiation Therapy was delivered on ipsilateral lymph node areas in 62 cases (44%: UL-RT group) and on bilateral lymph node areas and the entire pharyngeal mucosa in 77 cases (56%: COMP-RT group). Impact of radiation modalities on locoregional control and overall survival was assessed using propensity score matching method in order to balance baseline characteristics between the two groups. RESULTS: The population included 80.4% men, 80.4% smokers, 32.6% P16 positive tumours and 71.0% extracapsular extension. After a median follow-up of 5 years, the locoregional control rate was 80.3% in the UL-RT group and 75.3% in the COMP-RT group (p = 0.688). The corresponding rate of contralateral lymph node recurrence was 0% versus 2.6% (p = 0.503) and the rate of tumour emergence was 11.5% versus 9.1% (p = 0.778). No significant difference was observed between the UL-RT and the COMP-RT groups for overall survival (p = 0.9516), specific survival (p = 0.4837) or tumour emergence (p = 0.9034). CONCLUSION: UL-RT seems to provide similar outcomes as COMP-RT in unilateral HNCUP post-operative management.

Management of unilateral head and neck carcinoma of unknown primary: Retrospective analysis of the impact of postoperative radiotherapy target volumes.

BACKGROUND: We compared the outcome of postoperative unilateral cervical nodes radiotherapy (UL-RT) vs bilateral cervical nodes plus total mucosal irradiation (COMP-RT) in the management of head and neck carcinoma of unknown primary (HNCUP). METHODS: HNCUP, defined by the absence of primary despite a PET-CT combined with a panendoscopy, were treated with curative intent by initial ipsilateral neck dissection. Sixty-nine patients with unilateral HNCUP were included: 23 received UL-RT while 46 received COMP-RT. Carcinologic outcomes and long-term quality of life (QOL) according to the QOL Questionnaire for Head and Neck 35 were assessed. RESULTS: Within 6.3 years of median follow-up, there was no significant difference in primary tumor emergence rate (P = .68), cervical node recurrence rate (P = .34), or overall survival (P = .33) between UL-RT and COMP-RT groups. A trend toward QOL improvement was observed in the UL-RT group. CONCLUSION: UL-RT seems to provide similar outcomes as COMP-RT in unilateral HNCUP management.

Impact of HPV Infection on the Immune System in Oropharyngeal and Non-Oropharyngeal Squamous Cell Carcinoma: A Systematic Review.

OBJECTIVES: To review the current knowledge regarding the involvement of human papilloma virus (HPV) infection and the immune system in the development of head and neck squamous cell carcinoma (HNSCC). METHODS: An electronic literature search was conducted to identify articles published between 1990 and 2019 pertaining to tumor-infiltrating immune cells (TICs) in HNSCC using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Issues of clinical relevance, including tumor location, the number of tumor samples, the inclusion of additional specimens (dysplastic or normal mucosa), tumor size, methods used for HPV detection, relationship between antigen expression and patient characteristics (age, gender, smoking, alcohol consumption, etc.), and prognostic data (overall survival (OS) and recurrence-free survival (RFS)) were assessed by four blinded investigators. RESULTS: The search identified 335 relevant studies, of which 41 met the inclusion criteria. Of these, 7 studies focused on the peripheral blood immune cell concentration in patients with HNSCC according to HPV status, and 36 studies investigated TICs in the intraepithelial and/or stromal compartment(s) according to HPV status. The immune cells studied were CD8+ T cells (N = 19), CD4+ T cells (N = 7), regulatory T cells (Tregs, N = 15), macrophages (N = 13), myeloid-derived suppressor cells (MDSCs, N = 4), and Langerhans cells (LCs, N = 2). CONCLUSIONS: Irrespective of tumor location, CD8+ and CD4+ T cells appear to play a key role in the development of HPV-related HNSCC, and their infiltration is likely associated with a significant impact on OS and RFS. To date, the roles and prognostic value of Tregs, macrophages, DCs and MDSCs remain unclear.

Botulinum toxin for radiation-induced facial pain and trismus.

OBJECTIVE: To report the efficacy of botulinum toxin A for radiation-induced pain, trismus, and masticator spasm in head and neck cancer. PATIENTS AND METHODS: This prospective nonrandomized study included patients in complete remission with radiation-induced pain and trismus with or without masticator spasms. Fifty units of Botox (Allergan) or 250 units of Dysport (Ipsen) were injected transcutaneously into the masseter muscles. Jaw opening was measured and patients answered 20 questions about jaw opening, pain, and cramps, before injection at 1 month. RESULTS: Nineteen patients (7 women, 12 men) were included. Median time after radiation therapy was 5 years (range, 11 months to 22 years). At 1 month, no significant increase in jaw opening was recorded. Improvement was noted in the functional domain (P = 0.004), for pain (P = 0.002) and cramps (P = 0.004), but not in the social (P = 0.83) or emotional (P = 0.43) domains. No side effects occurred. CONCLUSIONS: Botulinum toxin did not improve trismus but significantly improved pain scores and masticator spasms (oromandibular dystonia).

Early assessment of feasibility and technical specificities of transoral robotic surgery using the da Vinci Xi.

The latest generation Da Vinci® Xi™ Surgical System Robot released has not been evaluated to date in transoral surgery for head and neck cancers. We report here the 1-year results of a non-randomized phase II multicentric prospective trial aimed at assessing its feasibility and technical specificities. Our primary objective was to evaluate the feasibility of transoral robotic surgery using the da Vinci® Xi™ Surgical System Robot. The secondary objective was to assess peroperative outcomes. Twenty-seven patients, mean age 62.7 years, were included between May 2015 and June 2016 with tumors affecting the following sites: oropharynx (n = 21), larynx (n = 4), hypopharynx (n = 1), parapharyngeal space (n = 1). Eighteen patients were included for primary treatment, three for a local recurrence, and six for cancer in a previously irradiated field. Three were reconstructed with a FAMM flap and 6 with a free ALT flap. The mean docking time was 12 min. "Chopsticking" of surgical instruments was very rare. During hospitalization following surgery, 3 patients experienced significant bleeding between day 8 and 9 that required surgical transoral hemostasis (n = 1) or endovascular embolization (n = 2). Transoral robotic surgery using the da Vinci® Xi™ Surgical System Robot proved feasible with technological improvements compared to previous generation surgical system robots and with a similar postoperative course. Further technological progress is expected to be of significant benefit to the patients.

Salvage surgery after failure of very accelerated radiotherapy in advanced head-and-neck squamous cell carcinoma.

PURPOSE: To assess the efficacy and toxicity of salvage surgery for local or cervical nodal recurrence after accelerated radiotherapy for locally advanced head-and-neck squamous cell carcinoma (HNSCC). METHODS AND MATERIALS: We reviewed the medical records of the 136 patients with HNSCC who had been treated in three consecutive clinical trials at the Institut Gustave-Roussy using a very accelerated radiotherapy regimen (62 to 64 Gy with 2 daily fractions of 1.8 to 2 Gy over 3.5 weeks). Sixty-nine patients of the 136 initial patients (51%) had local or neck lymph nodes relapse, or both. RESULTS: Sixteen of these 69 patients (23%) had undergone salvage surgery for recurrence locally (n = 8) or in the cervical nodes (n = 8). All 16 had initially been diagnosed with locally advanced oropharyngeal carcinoma (T4, 11 patients; T3, 5 patients), and 13 had initially had cervical node involvement. After salvage surgery, 6 patients had had a local recurrence; 7, cervical node recurrence; and 3, distant metastasis. Thus, salvage surgery had been successful only in 3 patients. The 3- and 5-year overall actuarial survival rates were 20% and 11%, respectively. Eight patients had major postoperative wound complications, including carotid rupture in three cases. CONCLUSION: Salvage surgery for relapse after very accelerated radiotherapy for advanced HNSCC is infrequently feasible and is of limited survival benefit. It should be used only in carefully selected cases.

Tetranucleotide microsatellite instability in surgical margins for prediction of local recurrence of head and neck squamous cell carcinoma.

PURPOSE: Postoperative radiotherapy is used to prevent local recurrence of head and neck squamous cell carcinoma in patients with positive surgical margins. We sought to determine whether tetranucleotide microsatellite instability could be detected in surgical margins and used to predict local recurrence. EXPERIMENTAL DESIGN: We prospectively collected tumor and surgical margin specimens from patients with head and neck squamous cell carcinoma who had undergone surgical resection at Institut Gustave-Roussy during a 1-year period. Margins were considered positive if extensive pathological examination revealed either carcinoma within 5 mm or dysplasia. We tested five tetranucleotide microsatellite markers (UT5085, L17686, D9S753, ACTBP2, and CSF1R) in the tumor specimens and paired surgical margins of the patients whose margins were negative on pathological examination. RESULTS: Pathological examination revealed that among the 76 patients, 22 had positive margins; therefore, these patients were excluded. Of the 54 remaining patients, 26 (48%) had tumors informative for markers UT5085, L17686, or both; the other 3 markers were not informative. Seven (27%) of the 26 informative tumors had the same instability pattern in the surgical margins. At a median follow-up of 26 months, 5 of the 7 local recurrences occurred in patients with molecularly positive surgical margins. A strong, independent association was found between positive surgical margins and local recurrence (P = 0.01; hazard ratio, 6.49). CONCLUSIONS: Tetranucleotide microsatellite instability in surgical margins may be a useful biomarker to predict local recurrence of head and neck squamous cell carcinoma in patients with apparently disease-free margins.

Locoregional effects of pegylated liposomal doxorubicin (Caelyx) in irradiated area: a phase I-II study in patients with recurrent squamous cell carcinoma of the head and neck.

Aim of this study was to determine the antitumour activity and toxicity of pegylated liposomal doxorubicin (Caelyx) in pretreated patients with locally recurrent and/or metastatic squamous cell carcinoma of the head and neck (SCCHN). Caelyx was administrated as 1 h infusion every 3 weeks at doses of 35 mg/m2 (group A) and then subsequently given at 45 mg/m2 (group B). 26 patients received a total of 87 cycles. The median number of cycles was 3 (range 1-7). Four out of 24 evaluable patients (17%, 95% confidence interval (CI) 0.5-32%) showed significant evidence of antitumour activity, with tumour necrosis being observed in 2 patients. Grade 3-4 neutropenia was observed in only 2 patients. There were no grade 3-4 mucosal, skin, digestive, cardiac or hepatic toxicities. Caelyx has activity against locally recurrent SCCHN and is well tolerated up to 45 mg/m2, but a careful utilisation of this drug is required for tumours relapsing in irradiated areas.

[Functional surgery for head and neck squamous cell carcinoma]. / Chirurgie conservatrice des cancers des voies aérodigestives supérieures.

Surgery for head and neck squamous cell carcinoma can alter speech, swallowing, and cosmoses. Recent tendency is to avoid mutilating surgery unless the tumour is aggressive or resistant to chemotherapy and or radiotherapy. Functional surgery is being widely employed, and for example it may vary between conventional partial surgery and endoscopic laser surgery for small sized vocal cord cancers. Various new reconstructive procedures have been developed to help early functional restoration. Loco-regional flaps can be used to replace gums and avoid dental extractions. Free flaps with micro-vascular anastomosis can be employed for immediate reconstruction of extensive surgical defects involving pharyngeal wall, tongue, mandible and mid-face to restore better function and cosmoses. Few recently developed techniques can be also employed in selected cases of laryngo-pharyngeal cancers to avoid permanent laryngeal mutilation. Another goal of functional surgery is to decrease the postoperative radiotherapy or chemo-radiotherapy sequelae, and obtain successful postoperative functional rehabilitation.

Determinants of patient delay in doctor consultation in head and neck cancers (Protocol DEREDIA).

INTRODUCTION: Reducing the time between the onset of the first symptoms of cancer and the first consultation with a doctor (patient delay) is essential to improve the vital prognosis and quality of life of patients. Longer patient delay is linked to the already known sociodemographic, socioeconomic, socioeducational, sociocultural and socioprofessional factors. However, recent data suggest that some sociocognitive and emotional determinants may explain patient delay from a complementary point of view. The main objective of this study is to assess whether, in head and neck cancer, patient delay is linked to these sociocognitive and emotional factors, in addition to previously known factors. METHODS AND ANALYSIS: We intend to include in this study 400 patients with a not yet treated head and neck cancer diagnosed in one of six health centres in the North of France region. The main evaluation criterion is 'patient delay'. Sociocognitive, emotional, medical, sociodemographic, socioeconomic, educational, professional and geographic factors will be assessed by means of (1) a case report form, (2) a questionnaire completed by the clinical research associate together with the patient, (3) a questionnaire completed by the patient and (4) a recorded semidirective interview of the patient by a psychologist (for 80 patients only). The collected data will be analysed to underline the differences between patients who consulted a doctor earlier versus those who consulted later. ETHICS: The study has obtained all the relevant authorisations for the protection of patients enrolled in clinical trials (CCTIRS, CCP, CNIL), does not involve products mentioned in article L.5311-1 of the French Code of Public Health, and does not imply any changes in the medical care received by the patients. The study began in October 2012 and will end in June 2015. TRIAL REGISTRATION: ID-RCB 2012-A00005-38.

Osteosarcomas of the mandible: are they different from other tumor sites?

BACKGROUND: Osteosarcomas of the mandible (MOS) affect 1/10 million persons/year, mostly the young adult. Due to lack of specific data, the treatment of MOS is extrapolated from that of extragnathic OS but varies widely between institutions. MATERIALS AND METHODS: We aimed at providing a focused description of MOS histologies and grades through the English literature, at determining the evidence-based role of chemotherapy, of adjuvant radiation therapy and the potential of reconstructive surgery tailored through modern pre-operative multi-modal imaging. RESULTS: The estimated proportion of high grade MOS was 58%. However, low-grade MOS may be underestimated as they are mostly reported as case reports. The intermediate grade was hardly found in the literature. Estimated weighted-mean proportions of chondroblastic and osteoblastic MOS were 37% and 46%, respectively. Multimodal imaging modalities including MRI has a great potential for accurate pre-operative assessment of tumor extensions into bone and soft tissues. Surgery is the mainstay of treatment and margins the most important factor. The role of neoadjuvant chemotherapy in treating occult systemic metastases and in increasing the probability of clear margins is controversial, as well as the histology-dependent response to chemotherapy. The role of adjuvant radiotherapy (mostly proposed for positive margins) and/or adjuvant chemotherapy is still controversial. Crude survival is around 77% and local control around 67%. Local failure is the main cause of death in MOS compared to extragnathic sites.