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Pseudomyogenic hemangioendothelioma (PMHE), a rare vascular neoplasm, was first described in 1992 asa fibroma-like variant of epithelioid sarcoma, and would be termed as epithelioid sarcoma-like hemangioendothelioma a decade later due to its significant histologic overlap with epithelioid sarcoma and diffuse cytokeratin expression. PHME is currently defined as a distinct, potentially intermediate malignant, rarely metastasizing neoplasm with vascular/endothelial differentiation. It is characterized by young age (typically less than 40 years old), extremity location (approximately ~80%), and t(7:19) SERPINE1::FOSB fusion as the most common molecular alteration. Herein, we report a case of a 59-year-old male presenting with multifocal lesions, including in the right temporalis muscle, right frontoparietal calvarium, right pterygoid muscles, and right mandibular condyle. Histologic examination of the right temporal lesion revealed a multinodular biphasic lesion composed of sheets and fascicles of elongated spindle and epithelioid cells infiltrating into the adjacent skeletal muscle. Admixed abundant neutrophilic infiltration is noted; however, areas of necrosis, increased mitosis, nuclear atypia, or rhabdomyoblast-like cells are absent. Immunohistochemical (IHC) staining showed that the tumor cells were diffusely and strongly positive for FOSB, pan-cytokeratin (AE1/AE3), CD31, and ERG. Molecular testing demonstrated a t(9:19) EGFL7::FOSB fusion mRNA. This constellation of morphological, IHC and molecular findings was consistent with a diagnosis of PMHE. This is the first reported case of multifocal PMHE with EGFL7::FOSB fusion in the head and neck area of a patient aged more than 50 years old. Since the differential diagnoses for PMHE includes high-grade malignancies with aggressive clinical behavior, coupled with the rare reports of PMHE in the head and neck region, awareness of this tumor in the head and neck region will avoid the misdiagnosis and overtreatment of this entity.

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Malu RafiObjectives This study was undertaken to evaluate the characteristics and treatment outcomes of patients with adenoid cystic carcinomas of the head and neck region treated at a tertiary cancer center in south India. Materials and Methods This was a retrospective study done on patients treated at the institute between 2004 and 2011. Clinicopathological details and treatment outcomes were captured from the treatment records to estimate the treatment outcomes and factors affecting them. Results There were a total of 140 patients with mean age of presentation of 46 years and a median follow-up of 65 months. The most common single site of presentation was the oral cavity. One-hundred eighteen patients (84.3%) underwent primary surgical resection in which 38% had negative surgical margins. Ninety-nine patients were given adjuvant radiotherapy and 18 patients received radical radiotherapy, mostly for surgically inaccessible and inoperable tumors. Median time to recurrence and distant metastasis was 37 and 34 months, respectively. The overall survival and disease-free survival estimate using the Kaplan-Meier method were 92.3 and 71.9%, respectively, at 5 years. Surgical removal of the primary tumor and the nodal stage had the most significant impact on the overall survival outcomes of these patients. Conclusion Surgery remains the most impactful treatment modality in the management of these rare epithelial tumors. The use of adjuvant radiotherapy may help to tackle the issues of perineural spread and inadequate surgical margins in technically difficult sites. Radical radiotherapy also has impressive response rates.

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Gouty tophi is a disease characterized by the long-term deposition of monosodium urate crystals in joints or cartilages. The most commonly affected site is the first metatarsophalangeal joint, and gouty tophi in the head and neck region are relatively rare. This article reports a case involving an elderly male with asymptomatic gouty tophi in the thyroid cartilage. The patient had a history of gout for over 10 years and presented with a painless thyroid mass that had been present for at least 3 years. He had not received systemic treatment. Preoperative contrast-enhanced cervical CT results indicated a high likelihood of gouty tophi. Postoperative pathology confirmed the mass to be consistent with gouty nodules. Following the procedure, the patient was treated symptomatically with non-busulfan tablets and colchicine. No recurrence was observed at the 1-year follow-up. This report highlights the need to consider gouty tophi in cases of prolonged gout history and abnormal imaging findings in the head and neck region. Appropriate management, including urate-lowering therapy and surgery, if necessary, can lead to optimal outcomes and prevent recurrences. Further research is warranted to enhance understanding and clinical management of this uncommon regional manifestation of gout.

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Introduction: The primary approach for managing skin cancer involves surgery, although radical radiotherapy (RT) may be considered as an alternative option in cases where patients decline the treatment themselves or are not eligible for surgical intervention. Herein we assess single-institution material in terms of the use of hypofractionated QUAD SHOT RT in patients disqualified from surgery. Material and methods: Between December 2019 and December 2022, nine patients with locally advanced non-melanoma skin cancer were disqualified from surgery and as a result were treated at the Radom Oncology Centre, Poland. Patients were treated with the Radiation Therapy Oncology Group 8502 QUAD SHOT regimen (14.8 Gy/4 fractions, twice-daily treatment with a 6 h interval, on 2 consecutive days). Courses were repeated every 4 weeks 3 times using volumetric modulated arc therapy (VMAT). Results: Grade 2 toxicities were observed in 4 of 9 (44.4%) patients, no grade ≥ 3 acute toxicity was observed. The median age was 79.1 (60-98) years. Irradiated areas were as follows: nose skin (2), cheek (2), eyebrow with eyelid (1), forehead (1), temple (1), sternum (1), and scapula (1). Performance status was as follows: WHO II - 5 patients (55.6%), WHO I - 3 patients, WHO III - one patient. One patient underwent 3 RT courses in 2 areas for a total of 6 treatment courses, 6 patients received 3 courses of treatment, and 2 patients received 2 courses. Additionally, as of 14 March 2023, four patients died of non-malignant causes. Conclusions: QUAD SHOT schedule with VMAT RT may be an effective palliative treatment method with a good response rate, which positively affects patients' quality of life in locally advanced non-melanoma skin cancer patients disqualified from surgery.

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Non-melanoma skin cancer of the head and neck (NMSCHN) is one of the most common malignancies worldwide, and its incidence is growing at a significant rate. It has been found to be aggressive in its spread and has the capacity to metastasize to regional lymph nodes. Cutaneous squamous cell carcinoma (cSCC) has a considerably high mortality rate. It has remarkable characteristics: diameter >2 cm, depth >5 mm, high recurrence, perineural invasion, and locoregional metastases. Aggressive cSCC lesions most commonly metastasize to the parotid gland. Also, immunocompromised patients have a higher risk of developing this aggressive cancer along with the worst prognostic outcomes. It is very important to discuss and assess the risk factors, prognostic factors, and outcomes of patients with cSCC, which will give clinicians future directives for making modifications to their treatment plans. The successful treatment of aggressive cSCC of the head and neck includes early detection and diagnosis, surgery alone or adjuvant chemotherapy, and radiotherapy as required. Multimodal therapy options should be considered by clinicians for better outcomes of aggressive cSCC of the head and neck.

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Synovial Sarcoma (SS) is a rare soft-tissue malignant tumour. Its presentation in the head and neck region is uncommon. Because of the complex anatomy of the head and neck region, surgery with clear margins is not achievable. In such cases, a multi-modality approach is required as there is no established standard of care. In this report, we share the case of a girl who presented with nasal obstruction. Imaging revealed a mass involving the left nasal cavity, paranasal sinuses without intracranial extension. It was diagnosed as synovial sarcoma. She underwent surgical excision and adjuvant radiation therapy (RT) to the tumour bed, followed by an incomplete course of chemotherapy. Later on, she developed systemic disease. Considering the rarity of this case and lack of guidelines for standard treatment, we report on this case to share our experience with management and treatment outcome.

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INTRODUCTION: In the present study, the advantages of superficial cervical plexus block (SCPB) were evaluated using a landmark-guided method. Our primary aim was to evaluate the analgesic efficacy of SCPB in various head and neck surgeries by observing intra- and postoperative requirements of the total dose of the systemic analgesic, visual analog scale (VAS) score, and the total duration of analgesia; vital parameters and associated perioperative complications were also observed. MATERIALS AND METHODS: Sample size was calculated using reference by taking the parameter pain score at 12 hours using MedCalc software v. 19.5.1 (MedCalc Software, Ostend, Belgium) with a mean difference of score 3±3.5 between two groups, 80% power, and 95% confidence interval (CI); the sample size for each group was 21. There were 30 patients in each group of ASA I, II, and III who were posted for mandibular, tympanomastoid and clavicular surgeries. Group A received general anaesthesia with systemic analgesia and Group B received general anaesthesia followed by SCPB with an injection of bupivacaine 0.25% 10ml on each side according to the site of surgery. VAS score, intra and postoperative analgesic requirement in 24 hours, time of first demand bolus, and peri-operative complications were noted. RESULTS: Intraoperative fentanyl requirement for group A was 97.5±13.75 µg as compared to group B (70.16±13.09 µg), postoperative injection paracetamol requirement was also significantly higher in group A (2566.66±504 mg) as compared to group B (833.33±874.28 mg). The total duration of analgesia was significantly higher in Group B (1191.33±375.36 min) as compared to Group A (122.0±50.88 min) with a p-value <0.0001. No significant complications were noted in any patient. CONCLUSION: SCPB provides better perioperative analgesia by decreasing intraoperative as well as postoperative systemic analgesic requirements and their associated side effects, with no significant perioperative complications in various head and neck region surgeries.

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Neuroendocrine carcinoma(NEC) is a malignant tumor derived from neuroendocrine cells, with distinct clinical, morphological and immunohistochemical characteristics. Neuroendocrine carcinoma of the head and neck is very rare in clinic. Larynx is the most common affected site, and the root of the tongue is extremely rare. The clinical manifestations are mainly eating pain, cauliflower like mass in the mouth, and ulcerative lesions that have not healed for a long time. Maxillofacial MRI and contrastenhanced CT are the most commonly used examination tools for such diseases, which can detect the spaceoccupying lesions of tumors. Neuroendocrine granules found in the cytoplasm under pathological light microscope can be diagnosed as neuroendocrine carcinoma. However, for most cases, it is difficult to make a diagnosis only under light microscope, and it is often necessary to make a diagnosis by means of immunohistochemistry and other technical means. This paper reports a case of neuroendocrine carcinoma of the root of the tongue, introduces its characteristics, diagnosis and treatment, and reviews the relevant literature of this case.

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Neuroendocrine carcinoma(NEC) is a malignant tumor derived from neuroendocrine cells, with distinct clinical, morphological and immunohistochemical characteristics. Neuroendocrine carcinoma of the head and neck is very rare in clinic. Larynx is the most common affected site, and the root of the tongue is extremely rare. The clinical manifestations are mainly eating pain, cauliflower like mass in the mouth, and ulcerative lesions that have not healed for a long time. Maxillofacial MRI and contrastenhanced CT are the most commonly used examination tools for such diseases, which can detect the spaceoccupying lesions of tumors. Neuroendocrine granules found in the cytoplasm under pathological light microscope can be diagnosed as neuroendocrine carcinoma. However, for most cases, it is difficult to make a diagnosis only under light microscope, and it is often necessary to make a diagnosis by means of immunohistochemistry and other technical means. This paper reports a case of neuroendocrine carcinoma of the root of the tongue, introduces its characteristics, diagnosis and treatment, and reviews the relevant literature of this case.

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BACKGROUND: Immune therapies are currently under intensive investigation providing in many cases excellent responses in different tumors. Other possible approach for immunotherapy is a targeted intratumoral delivery of interleukin 12 (IL-12), a cytokine with anti-tumor effectiveness. Due to its immunomodulatory action, it can be used as an imunostimulating component to in situ vaccinating effect of local ablative therapies. We have developed a phIL12 plasmid devoid of antibiotic resistance marker with a transgene for human IL-12 p70 protein. The plasmid can be delivered intratumorally by gene electrotransfer (GET). PATIENTS AND METHODS: Here we present a first-in-human clinical trial protocol for phIL12 GET (ISRCTN15479959, ClinicalTrials NCT05077033). The study is aimed at evaluating the safety and tolerability of phIL12 GET in treatment of basal cell carcinomas in patients with operable tumors in the head and neck region. The study is designed as an exploratory, dose escalating study with the aim to determine the safety and tolerability of the treatment and to identify the dose of plasmid phIL12 that is safe and elicits its biological activity. CONCLUSIONS: The results of this trail protocol will therefore provide the basis for the use of phIL12 GET as an adjuvant treatment to local ablative therapies, to potentially increase their local and elicit a systemic response.

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Desmoid fibromatosis (DF) is one of the rarest locally aggressive growing benign tumor entities. We present an overview of the literature and a rare clinical case of a 22-year-old female patient, who was diagnosed with aggressive DF in the left pharyngeal wall at the age of 4 years old.

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BACKGROUND: Dynamic contrast enhanced magnetic resonance imaging (DCE-MRI) can in vivo characterize tumor microvascular environment. The aim of the present study was to reveal the DCE-MRI findings and to determine the correlation between these findings and immunohistochemical data in head and neck squamous cell carcinoma (HNSCC). METHODS: Thirty-three patients diagnosed with primary HNSCC were evaluated retrospectively. DCE-MRI was conducted in all cases. CD34, CD105, and ki-67 expressions were analyzed with immunohistochemistry in tissue sections to determine micro-vessel density and proliferative activity. RESULTS: The DCE-MRI is a successful technique in distinguishing tumor tissue from normal tissue. It was determined that Ve, Ktrans, and ki-67 values were significantly higher in high-stage tumors and there were positive correlations between the Ktrans value (by standard ROI) and CD34 MVDmax and CD34 MVDmean values. No statistically significant correlation was determined between other parameters in DCE-MRI and immunohistochemical data, and T stage. DISCUSSION: DCE-MRI could successfully differentiate tumor tissue in HNSCC. Furthermore, it was observed that DCE-MRI had the potential to reveal certain immunohistochemical information in vivo.

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OBJECTIVES: To determine the clinicopathological features of epithelioid sarcoma presenting in head and neck region (HNES) and elucidate diagnostic key points and treatment options for HNES. MATERIALS AND METHODS: A total of 12 HNES cases were collected in our department from 2010 to 2020. Their clinical information and pathological features were documented, and relevant follow-up was performed. Immunohistochemistry was carried to analyze the protein markers of HNES. RESULTS: Of the 12 HNES cases, 10 were primary tumors and 2 were metastasized from foot and shoulder, respectively. The patients with primary tumors were significantly younger than those with metastasized ones (22.7 vs 41.5, p = .0157), and male patients outnumbered female patients (3:1). Of all HNES cases, 9 were classic subtype, and 3 were proximal subtype. HNES patients had a poor prognosis, with 5-year overall survival of 41.5% and 5-year relapse-free survival of 22.5%. A loss of INI1 was identified as the hallmark of HNES with 83.3% (10/12) of HNES cases presenting as EZH2 positive. CONCLUSIONS: HNES is more prevalent at younger ages and in males, has a poor prognosis, and exhibits a greater proportion of classic subtype than proximal subtype. EZH2 inhibitor has therapeutic potential in HNES.

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BACKGROUND: Despite the vivid progress in molecular and genetic profiling, extensive diagnosis and multiple therapeutic modalities, cancers of unknown primary in the head and neck region continue to be a formidable challenge. AIM: The purpose of the review is to present the most recent and well-established findings concerning cancers of unknown primary (CUPs) in the head and neck patients and consequently to provide medical specialists with essential information regarding the biology, pathology, histology, diagnosis and treatment of CUP in the head and neck region. MATERIAL AND METHODS: The Medline/PubMed database was searched by using the following keywords "CUP", "cancer of unknown primary", "CUP biology", "head and neck". The references of the publications of interest were also screened for relevant papers. There were no limitations in regard to the publication date. CONCLUSION: Unique biology and pathophysiology prompt the management of CUP to be demanding. The vivid rise in HPV-related CUPs during the last 20 years has put more focus on this phenomenon. New findings concerning the enigmatic biology of CUP provide fundaments for targeted therapy. Despite the availability of various diagnostic methods, the diagnosis of CUP continues to be a time-consuming, strenuous process that eventually provides answers. It remains controversial as to what combination of treatment methods is the most effective. There is no consensus on the value of combining chemotherapy with radiotherapy. Highly specific surgical treatments for particular histological types of CUP produce more satisfactory results. It is paramount to establish reliable guidelines concerning the diagnosis and treatment of CUP patients.

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BACKGROUND/AIM: Cutaneous squamous cell carcinoma (cSCC) is a common type of skin cancer. Options for palliative treatment include systemic agents and radiotherapy. Selection of a radiation regimen should consider the patient's survival prognosis. This study aimed to identify prognostic factors of survival after palliative radiotherapy for cSCC of the head-and-neck. PATIENTS AND METHODS: Ten factors were analyzed for survival in 12 patients including age, gender, tumor site, histological grade, primary tumor stage, lymph node involvement, distant metastases, upfront surgery, radiation dose and completion of radiotherapy. RESULTS: On univariate analysis, improved survival was significantly associated with lower histological grade (better differentiation) (p=0.022), no distant metastases (p=0.040) and completion of radiotherapy (p=0.014). In the multivariate analysis, lower histological grade (risk ratio=6.05, p=0.100) and completion of radiotherapy (risk ratio=4.87, p=0.115) showed trends. CONCLUSION: Predictors of survival were identified that can help design individual treatments. Patients require optimal supportive care as completion of radiotherapy was associated with better survival.

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Cutaneous squamous cell carcinoma (SCC) is the second most common non-melanoma skin cancer among Caucasians. We analyzed our data on tumors been treated between 2006 and 2016 at the Skin Cancer Center of an academic teaching hospital. Thousand hundred and ninety-four patients with 1296 SCC have been identified including 804 males (67.3%) and 390 females (32.7%). The mean age among females was significantly higher with (83.9 ± 7.9) years compared with males ([79.3 ± 8.1] years; P < .001). The most common tumor localizations were outer ears (n = 227, 17.5%), scalp (216, 16.7%), and forehead (215, 16.6%). The majority of tumors was stage I. 31.9% of patients had previous SCC or Bowen's cancer, 29.1% had actinic keratoses. Major non-dermatological comorbidities were cardiovascular diseases and diabetes. SCC were treated by delayed Mohs surgery. First Mohs procedure resulted in R0 status in 83.9%. Most cases with R1 resection were located on nose and eyelids. For a subpopulation of patients (n = 105), a structured questionnaire study evaluated changes in sun protection behavior after skin cancer. After the confirmed diagnosis of a cutaneous SCC concerns about harm to the skin by intense sunlight was reported by 78.4% (76) of participants, with 92.0% among females versus 73.6% among males. Eighty percent of females and 76.8% of males wanted to care more about sun-protection in the future, spend less time 84.0% (females) and 72.5% (males) in the sun, and adapt their leisure activities for a better sun protection in 92.0% (females) and 81.9% (males). Secondary prevention in males needs improvement.

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Spindle cell squamous cell carcinoma (SCSCC) represents a rare, dedifferentiated form of conventional squamous cell carcinoma (SCC) with propensity for head and neck. Morphological heterogeneity of this tumor poses significant diagnostic challenge for pathologists. This study aimed to evaluate detailed morphological and immunohistochemical (IHC) characteristics of a series of SCSCC cases. All head and neck SCSCCs diagnosed over seven years were retrospectively analyzed to assess morphological features in detail with evaluation of possible differential diagnoses. Elaborate IHC panel was performed in each case. Eleven cases (8 males, 3 females) were included. Most patients were in 5th to 7th decade. Oral cavity (n = 6; 54.5%); oropharynx (n = 3; 27.3%), and larynx (n = 2; 18.9%) were involved. Tumors were polypoidal (n = 6), ulceroproliferative (n = 3), or ulceroinfiltrative (n = 2). Microscopically, epithelial ulceration was common (90.9%). Squamous differentiation was evident on light microscopy in 6 (54.5%) cases as surface dysplasia (n = 1; 9.1%), infiltrative tumor (n = 4; 36.4%), or both (n = 1; 9.1%). The spindled tumor component showed markedly heterogeneous morphology mimicking mesenchymal malignancies or benign mesenchymal proliferations. IHC for epithelial markers (CK, EMA, p40, and p63) established epithelial differentiation in 40% (2 of 5) cases where it was not evident morphologically. CK showed highest positivity followed by p40 and p63. Aberrant mesenchymal marker expression was variably seen. Morphological diagnosis of SCSCC is challenging with several histological mimics. Surface dysplasia and component of invasive SCC in a predominantly spindled tumor are important subtle morphological clues. Systematic morphological approach aided by IHC is helpful in clinching the accurate diagnosis.

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The histology of basaloid squamous cell carcinoma (BSCC) can resemble that of adenoid cystic carcinoma (AdCC). Herein, we report two cases of BSCC with adenoid cystic-like features (BSCC-AdC). We collected cases of AdCC and BSCC of the head and neck region, extracted two cases with unusual histology, and reexamined them histologically and immunohistochemically. Case 1 involved an 81-year-old Japanese male, who had an elastic-hard mass on the left side of his tongue, and a biopsy examination suggested AdCC. Case 2 involved a 63-year-old Japanese male, who had a polypoid mass on his right hypopharynx. He was diagnosed with AdCC with high-grade transformation. Histologically, atypical cells in a myxoid stroma, which exhibited trabecular, nest-like, and/or cribriform growth patterns, and necrosis were observed in both cases. Case 2 displayed more marked cellular atypia than Case 1. Immunohistochemically, the tumor cells were diffusely positive for cytokeratin 5/6, p63/p40, SRY-related HMG-box 10 and Ki-67, but negative for other myoepithelial markers and p16. Finally, both cases were rediagnosed as BSCC-AdC. It is known that esophageal BSCC displays adenoid cystic-like features, and BSCC-AdC also sometimes occurs in the head and neck region. Clinicians should carefully differentiate BSCC-AdC from AdCC of the minor salivary glands and human papillomavirus-related carcinoma.

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We aimed to compare the geometric distortion (GD) correction performance and apparent diffusion coefficient (ADC) measurements of single-shot diffusion-weighted echo-planar imaging (SS-DWEPI), multiplexed sensitivity encoding (MUSE)-DWEPI, and MUSE-DWEPI with reverse-polarity gradient (RPG) in phantoms and patients. We performed phantom studies at 3T magnetic resonance imaging (MRI) using the American College of Radiology phantom and Quantitative Imaging Biomarker Alliance DW-MRI ice-water phantom to assess GD and effect of distortion in the measurement of ADC, respectively. Institutional review board approved the prospective clinical component of this study. DW-MRI data were obtained from 11 patients with head and neck cancer using these three DW-MRI methods. Wilcoxon signed-rank (WSR) and Kruskal-Wallis (KW) tests were used to compare ADC values, and qualitative rating by radiologist between three DW-MRI methods. In the ACR phantom, GD of 0.17% was observed for the b = 0 s/mm2 image of the MUSE-DWEPI with RPG method compared with that of 1.53% and 2.1% of MUSE-DWEPI and SS-DWEPI, respectively; The corresponding methods root-mean-square errors were 0.58, 3.37, and 5.07 mm. WSR and KW tests showed no significant difference in the ADC measurement between these three DW-MRI methods for both healthy masseter muscles and neoplasms (P > .05). We observed improvement in spatial accuracy for MUSE-DWEPI with RPG in the head and neck region with a higher correlation (R2 = 0.791) compared with that for SS-DWEPI (R2 = 0.707) and MUSE-DWEPI (R2 = 0.745). MUSE-DWEPI with RPG significantly reduces the distortion compared with MUSE-DWEPI or conventional SS-DWEPI techniques, and the ADC values were similar.

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PURPOSE: This study was aim to evaluate the patterns of failure according to radiotherapy (RT) target volume for cervical lymph nodes in metastases of unknown primary origin in head and neck region (HNMUO). MATERIALS AND METHODS: Sixty-two patients with HNMUO between 1998 and 2016 were retrospectively reviewed. We analyzed the clinical outcomes and primary site failure depending on the radiation target volume. The target volume was classified according to whether the potential head and neck mucosal sites were included and whether the neck node was treated involved side only or bilaterally. RESULTS: Potential mucosal site RT (mucosal RT) was done to 23 patients and 39 patients did not receive mucosal RT. Mucosal RT showed no significant effect on overall survival (OS) and locoregional recurrence (LRR). The location of primary site failure encountered during follow-up period was found to be unpredictable and 75% of patients with recurrence received successful salvage therapies. No significant differences in OS and LRR were found between patients treated to unilateral (n = 35) and bilateral neck irradiation (n = 21). Treatment of both necks resulted in significantly higher mucositis. CONCLUSIONS: We found no advantages in OS and LRR of patients with HNMUO when mucosal sites and bilateral neck node were included in the radiation target volume.