Analysis of Diagnosis and Treatment Strategies for Primary Juvenile Psammomatoid Ossifying Fibroma Complicated With Primary Aneurysmal Bone Cyst.

BACKGROUND: Juvenile Psammomatoid Ossifying Fibroma (JPOF) is a type of noncancerous bone tumor that usually affects adolescents in the craniomaxillofacial area. Clinical manifestations are usually symptoms caused by the tumor's invasive compression of surrounding tissues. Aneurysmal Bone Cyst (ABC) is also a benign bone tumor, and it typically occurs in long bones and the spine. Only 2% to 3% of cases occur in the head and neck. Due to the rarity of this combination of clinical cases, clinicians face difficulties in comprehensively understanding this complex lesion. Therefore, a comprehensive review of the clinical manifestations and characteristic imaging findings is necessary for surgeons. CASE PRESENTATIONS: On April 6, 2019, a 13-year-old boy presented with left maxillofacial bulge and pain for 1 month. Magnetic resonance imaging of the paranasal sinuses showed an irregular hive-like mass signal in the left maxillary sinus, and cystic changes with fluid levels were seen in the lesion. After the initial diagnosis of JPOF with primary ABC, we decided to perform a facial mid-facial resection of maxillary sinus tumor to remove the tumor tissue. Finally, after 3 recurrences and 4 operations, there was no tumor recurrence for 20 months after the last operation, and the patient was still under continuous follow-up. CONCLUSIONS: This case provided a reference for the diagnosis and treatment of JPOF combined with ABC. In particular, a new understanding of the association between the two diseases and the management of recurrence were proposed, which had the potential to improve clinical understanding of this complicated condition.

Abscopal effect in maxillary sinus cancer: Insights from two case reports and a literature review.

BACKGROUND: The abscopal effect is a rare phenomenon in which localized radiation therapy triggers tumor reduction in nontargeted areas. Although this phenomenon has been observed in various cancer types, it remains infrequent and not fully understood. CASE: Two patients with maxillary sinus cancer with distant metastases were treated with radiotherapy after immune checkpoint inhibitor (ICI) therapy. The patients demonstrated abscopal effects following ICI therapy and radiotherapy, showing shrinkage in metastatic areas not directly targeted by radiation. CONCLUSION: This report was reviewed to examine the synergistic effects of ICI and radiotherapy and to identify optimal strategies to enhance the abscopal effect in clinical practice. It has also touched on various ongoing debates and clinical trials aimed at understanding and exploiting this effect to improve cancer treatment. The exact mechanisms and optimal treatment protocols remain areas for future research.

Endoscopic Management of Maxillary Sinus Schwannoma.

ABSTRACT: Schwannomas are benign tumors originate from nerve sheath. In this article, the authors aimed to share our experience and review the literature on endonasal endoscopic intervention for a maxillary sinus schwannoma. A 30-year-old Caucasian female patient applying to ophthalmology clinic due to exophthalmos and proptosis in her left eye for the last 6 months. A well-defined mass was detected in left maxillary sinus that was filling and expanding the sinus. Endoscopic biopsy from the patient was reported as ancient schwannoma. The encapsulated mass was completely removed by performing a combined endoscopic medial maxillectomy and Caldwell-Luc procedure under general anesthesia. The authors have been following the patient for 2 years and there was no evidence of recurrence, however, a decrease in the volume of the maxillary sinus occurred, suggesting chronic maxillary atelectasis. In conclusion, schwannoma should be considered in the differential diagnosis of sinonasal masses.

Locally advanced undifferentiated sarcomatoid carcinoma of the right maxillary sinus with PDCD6-TERT fusion: A rare case report.

Sarcomatoid carcinoma of maxillary sinus tumor is extremely rare in head and neck tumors and has poor prognosis and frequently occurs to relapse locally after surgery. We first reported a case of locally advanced undifferentiated sarcomatoid carcinoma of right maxillary sinus with PDCD6-TERT fusion gene. The patient with a previous history of moderate alcohol drinking and smoking. The patient underwent surgical treatment. The tumor tissue using NGS analysis, no other driver gene mutations, and the PD-L1 IHC was negative. He received TPF regimen induction chemotherapy combined with anti-PD1 inhibitor and radiotherapy. The effect of treatment was good.

Radiologic criteria of retropharyngeal lymph node metastasis in maxillary sinus cancer.

OBJECTIVE: To determine the most appropriate radiologic criteria of metastatic retropharyngeal lymph nodes (RLNs) in patients with maxillary sinus cancer (MSC). MATERIALS AND METHODS: We retrospectively evaluated 16 consecutive patients who underwent magnetic resonance imaging (MRI) before and after the treatment of locally advanced squamous cell carcinoma of the maxillary sinus. The minimal and maximal diameters of all RLNS were recorded. RLNs were classified as metastatic on the basis of the MRI follow-up (f/u). RLNs were considered non-metastatic if stable disease continued until the final MRI f/u and metastatic in cases with different evaluations (complete response, partial response, progressive disease) determined using Response Evaluation Criteria in Solid Tumours (RECIST) ver. 1.1. The receiver operating characteristic curve (ROC) and area under the curve (AUC) were used to assess the accuracy of various criteria in the diagnosis of metastatic RLNs. RESULTS: Of the 34 RLNs in 16 cases observed on pretreatment MRI, 7 were classified as metastatic RLNs and 27 as non-metastatic RLNs. Using the radiologic criteria, metastatic RLNs tended to be diagnosed more accurately with the minimal axial diameter than with the maximal axial diameter (AUC; 0.97 vs. 0.73, p = 0.06). The most accurate size criterion of metastatic RLNs was a minimal axial diameter of 5 mm or larger, with an accuracy of 94.1% (32 of 34). CONCLUSIONS: The most appropriate radiologic criterion of metastatic RLNs in MSC is a minimal axial diameter of 5 mm or longer.

Imaging predictor of ophthalmic involvement in maxillary sinus cancer during super selective intra-arterial cisplatin infusion and concomitant radiotherapy (RADPLAT).

OBJECTIVE: To investigate the predictability of ophthalmic artery involvement in maxillary sinus cancer using preprocedural contrast enhanced CT and MRI. METHODS: We analyzed advanced (T3, T4a, and T4b) primary maxillary sinus squamous cell carcinoma treated with super-selective intra-arterial cisplatin infusion and concomitant radiotherapy (RADPLAT) from Oct 2016 to Mar 2020. Two diagnostic radiologists evaluated the tumor invasion site around the maxillary sinus using preprocedural imaging. These results were compared with the angiographic involvement of the ophthalmic artery using statistical analyses. We also evaluated our RADPLAT quality using complication rate, response to treatment, local progressive free survival (LPFS), and overall survival (OS). RESULTS: Twenty patients were included in this study. There were ten cases of ophthalmic artery tumor stain and there was a correlation between ophthalmic artery involvement and invasion for ethmoid sinus with statistically significant differences. Other imaging findings were not associated with ophthalmic artery involvement. CONCLUSIONS: Ethmoid sinus invasion on preprocedural imaging could suggest ophthalmic artery involvement in maxillary sinus cancer. It may be useful in predicting prognosis and treatment selection.

Ameloblastoma of the maxillary sinus: conservative surgical management considering high recurrence risk potential.

Ameloblastoma (AM) in the maxillary sinus is rare. This benign entity shows locally invasive, destructive and aggressive behaviour and a high rate of recurrence. Therefore, the course of treatment is radical resection. We report the case of a 38-year-old man presenting with signs of recurrent sinusitis in the Ear, Nose and Throat Department. Transnasal flexible endoscopy revealed a cystic mass in the right inferior and middle nasal passage. CT scan showed an obliterated right maxillary sinus with a ballooning effect and pressure atrophy of the lateral sinus wall, without possible differentiation of the middle and low nasal turbinate. The patient was treated with transnasal functional sinus surgery; pathology stated AM. AM in the maxillary sinus is rare, locally destructive and therefore as a gold standard is resected radically to prevent recurrence. We demonstrate a conservative approach; explicitly, we combined a transvestibular and functional endoscopic sinus surgery resection of the AM to maintain function and reduce the possibility of postoperative impairments. Whether the strategy of treatment for AM is conservative, it nonetheless can result in a recurrence-free status. Nevertheless, inclusion into an oncological follow-up-programme with regularly performed MRI and CT is recommended.

Localized Maxillary Sinus Papilloma: Management of Incidental Lesion.

Most maxillary sinus papillomas are confirmed when they have extended beyond the nasal cavity and are rarely found while localized in the maxillary sinus. We experienced two cases of localized papilloma in the maxillary sinus. Case 1 was a 69-year-old man with a localized left maxillary sinus lesion detected during a routine imaging examination. As the lesion was likely to be papilloma, we recommended that the patient undergo diagnostic surgery, which he refused. He experienced bloody rhinorrhea 1 year and 9 months after the first visit, and computed tomography (CT) showed increased lesions and bone destruction. Histological examinations revealed squamous cell carcinoma ex inverted papilloma. He died 5 years after the first visit. Case 2 was a 46-year-old woman in whom positron emission tomography/CT showed a localized right maxillary sinus lesion. Tissue biopsy results indicated oncocytic papilloma. Endoscopic resection was performed later. On an imaging examination, sinonasal papilloma was determined accidentally to be a localized lesion of the maxillary sinus. A detailed interpretation of the CT scan was useful in estimating sinonasal papilloma. Tissue biopsy or diagnostic surgery should be performed when sinonasal papilloma is suspected during appropriate image evaluation.

Targeting MET Amplification with Crizotinib in a Case of Sinonasal Undifferentiated Carcinoma.

Comprehensive molecular testing of individual tumors has led to the identification of novel molecularly defined cancer therapies and treatment indications. Given low frequencies of many molecular alterations, efficacy of therapies used to target them are often undefined, especially in the context of rare malignancies. Here we describe the first reported case of MET amplification in sinonasal undifferentiated carcinoma (SNUC), a rare cancer with a poor prognosis. The patient was treated with crizotinib, a tyrosine kinase inhibitor that targets c-MET, and experienced a complete response. Our report demonstrates the potential of employing precision oncology approaches in SNUC and other rare cancers.

Combined Simultaneous Multiportal Approach via Minimally Invasive Transciliary and Endoscopic Endonasal Approaches for Tumors Invading Both the Skull Base and the Sinonasal Area.

BACKGROUND: A combined transcranial and transfacial approach has long been the gold standard for surgical management of large tumors with sinonasal and skull base involvement. The extended endoscopic endonasal approach for such pathologies has its advantages, but it has flaws as well, such as anatomic limitations and more ponderous skull base reconstruction and thus higher risk of postoperative complications. Our primary technique for surgical treatment of these pathologies has been a combination of transfacial and minimally invasive transciliary supraorbital keyhole approaches. With the aim to further minimize invasiveness, potential complications, and unsatisfactory aesthetic outcomes during surgical treatment of large tumors invading both the sinonasal area and the skull base, we abandoned the transfacial approach and simultaneously combined the transciliary supraorbital keyhole approach with the endoscopic endonasal approach. METHODS: The well-known microscope-assisted minimally invasive approach via a transciliary supraorbital keyhole craniotomy was combined with the endoscopic endonasal approach. RESULTS: Six patients with different histologic types of tumors affecting the sinonasal area and the skull base were operated on. The mean operative time was 3 hours, there were no unexpected intraoperative or postoperative complications, and total tumor removal was achieved in each patient. None of the patients experienced complications associated with the surgery during follow-up. CONCLUSIONS: Our combined simultaneous multiportal approach enables total tumor eradication with reduced operative time and is associated with minimal intraoperative and postoperative complications, low mortality rate, and excellent cosmetic results.

Endoscopic maxillectomy: anatomo-radiological description of the "double" maxillary sinus window.

OBJECTIVES/HYPOTHESIS: To present a modified endoscopic medial maxillectomy (MEMM) approach to control maxillary sinus pathologies. METHODS: MEMM was completed in 13 fresh-frozen specimens. An MEMM includes cutting the nasolacrimal duct, inferior meatus flap, and repositioning the inferior turbinate (IT). The following measurements were obtained: length of IT, height from the nasal floor to valve of Hasner, height of the IT at the level of valve of Hasner, height of the IT at the insertion of the middle turbinate, and distance from the piriform aperture to the posterior wall of maxillary sinus and to the posterior border of palatine bone. Similar measurements were also performed on craniofacial computed tomography (CT) scans (n = 50). The surgical technique was performed in a case series (n = 8). RESULTS: The mean of the specimens was 82 (range 70-95) years old. The average area of the harvested inferior meatus flap area was 9.6 ± 1.0 cm2. In the radiologic study, the mean maximum antrostomy area was 8.8 ± 1.7 cm2 and the IT area overlapping the antral window was 5.8 ± 1.1 cm2, the area allowing a double window control was 3.1 ± 1.9 cm2, the posterior IT insertion length was 0.7 ± 0.4 cm, and the inferior meatus flap covering the inferior meatotomy had an area measuring 6.7 ± 1.7 cm2. Eight patients underwent MEMM for various benign conditions showing no recurrence after 26 month follow-up. CONCLUSION: The proposed modifications of MEMM provide a "double" window maxillary sinus control with access to all maxillary walls and preservation of the IT.

Management of FDG avid Benign Sinonasal Schneiderian Papilloma: A Case Report and Review of the Literature.

BACKGROUND: The inverted and oncocytic subtypes of sinonasal Schneiderian papillomas are benign tumors with possible rare malignant transformation and are typically managed with complete surgical resection and close follow-up. While computed tomography (CT) and magnetic resonance imaging (MRI) are mainstays in preoperative evaluation of bony invasion and soft tissue extension of the lesion, their imaging characteristics by 18F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) is less well characterized. OBJECTIVE: To describe the clinical presentation and management of a PET positive sinonasal lesion. To conduct a literature review of FDG uptake in benign sinonasal papillomas. METHODS: Case report (n = 1) and literature review of similar cases (n = 32). RESULTS: We report the case of a 69-year-old man presenting with an isolated left maxillary sinus mass with avid FDG uptake, discovered on PET/CT imaging. An endoscopic left maxillary mega-antrostomy provided successful definitive treatment for final pathologic diagnosis of oncocytic papilloma. Literature review of cases of sinonasal papillomas with avid FDG uptake found that oncocytic papillomas, on average, exhibit greater uptake than inverted papillomas and both may be mistaken as malignancies on PET. CONCLUSION: While PET imaging demonstrating avid FDG uptake is associated with an increased risk of malignancy, it does not rule out the possibility of a benign sinonasal papilloma nor other benign inflammatory lesions. Particularly, oncocytic papillomas may have very high FDG uptake and mimic malignant lesions.

Clinicopathological features of 214 maxillary sinus pathologies. A ten-year single-centre retrospective clinical study.

BACKGROUND: Diagnosis of maxillary sinus pathology must include the clinical radiological study (CRS) and histopathological analysis. The aim of this study is 1) to describe the clinicopathological features of maxillary sinus lesions, obtained successively in a single medical centre over the last 10 years and 2) to determine the sensitivity and specificity for the diagnosis of malignant lesions based exclusively on the CRS. METHODS: It is a single-centre observational retrospective clinical study on patients who attended the University Hospital Complex of Santiago de Compostela (CHUS) with sinus pathologies during the period of 2009-2019. RESULTS: The sample consisted of 133 men (62.1%) and 81 women (37.9%), with an average age of 46.9 years (SD = 18.8). In terms of frequency, the most frequent pathology was the unspecified sinusitis (44.4%), followed by polyps (18.2%), malignant tumours (9.8%), inverting papilloma (7.5%), fungal sinusitis (4.7%), cysts (3.7%), benign tumours (2.3%), mucocele (2.3%) and other lesions (1.9%). Cysts and benign tumours were diagnosed earliest Vs malignant tumours (65.2 years (SD = 16.1)) were diagnosed the latest (p < 0.001). Based only on the CRS for malignancies, diagnostic indexes were 71.4% sensitivity and 97.9% specificity, with a Kappa value of 0.68 with (p < 0.001). CONCLUSION: Maxillary sinus pathology is very varied with therapeutic and prognostic repercussions. CRS is sometimes insufficient and histopathological confirmation is essential.

A case report of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient.

INTRODUCTION: Primary immunodeficiency diseases (PIDs), a rare group of gene defects with different manifestations, are at great risk of malignancy. The incidence of diffuse large B-cell lymphoma in the sinusoidal tract is quite rare with nasal congestion, stuffiness, and pain in maxillary sinus manifestation. Human serine-threonine kinase 4 (STK4) deficiency affects the immune system with recurrent bacterial and viral infections, mucocutaneous candidiasis, cutaneous warts, skin abscesses, T- and B-cell lymphopenia, and neutropenia. PATIENT CONCERN: In this study we describe the infrequent incidence and successful treatment of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient with clinical manifestation of severe intractable headaches, unilateral swelling of her face, nasal congestion, stuffiness, and pain in maxillary. DIAGNOSIS: Clinical data including headaches, unilateral swelling of face, nasal congestion, stuffiness and pain in maxillary sinus with confirmed histopathology and magnetic resonance imaging finding confirmed sinusoidal diffuse large B cell lymphoma in a STK4 deficient patient. INTERVENTION: Six cycles of R-CHOP (rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisolone) were administered and after each cycle, G-CSF support was used. Chemotherapeutic drugs were administered with standard dose and no dose reduction was done during the treatment. IVIG treatment continued during the courses of chemotherapy. OUTCOME: The index patient achieved complete response at the end of chemotherapy courses and was in remission for about 8 months afterward, prior to the date of the present report. CONCLUSION: PID patient are often at increased risk of malignancies. Sinusoidal diffuse large B-cell lymphoma is quite rare and prognosis is variable. Early attention to patient's manifestation, suitable treatment, and monitoring manifestations caused by PID are critical.

Modified endoscopic Denker approach in management of inverted papilloma of the anterior maxillary sinus.

BACKGROUND: Inverted papilloma (IP) is a benign epithelial tumor of the sinonasal cavity that is locally aggressive and has a propensity for recurrence. Tumors involving the anterior maxillary sinus can be challenging to reach endoscopically, and have historically been approached through a Caldwell-Luc or canine fossa trephination. We present a case series of 22 patients with maxillary IPs originating from the anterior wall, which were successfully resected using a modified endoscopic Denker (MED) procedure. This technique enables access to the entire maxillary sinus without the need for a separate transseptal or sublabial incision. METHODS: A retrospective chart review was performed on patients who underwent the MED approach for management of maxillary IPs involving the anterior wall from 2012 to 2018. The demographic data, clinical presentation, radiographic findings, pathology, and surgical outcomes were reviewed. RESULTS: Twenty-two patients were identified, including 13 males and 9 females. Eighteen of the 22 patients had prior surgery and all had Krouse stage 3 disease. After a mean follow-up of 24 (range, 4-63) months, only 1 patient (4.5%) developed a recurrence, which was treated successfully with endoscopic resection. Complications included 1 patient with preoperative epiphora who required dacryocystorhinostomy, epistaxis in another, and 1 patient with transient upper lip numbness. No patients developed alar notching or pyriform aperture stenosis. CONCLUSION: The MED technique is highly effective for surgical resection of primary and recurrent maxillary IPs involving the anterior wall, providing complete access to the entire maxillary sinus. In many cases, the MED can obviate the need for an adjunctive sublabial or transseptal incision, while also providing excellent exposure for postoperative surveillance.

Surgical treatment of metastatic mesenchymal chondrosarcoma to the spine: A case report.

INTRODUCTION: Metastatic mesenchymal chondrosarcoma of the spine is a highly unusual disease without standard curative managements yet. The objective of this case report is to present a very rare case of metastatic chondrosarcoma to the spine successfully operated by surgical treatment. The management of these unique cases has yet to be well-documented. PATIENT CONCERNS: A 34-year-old woman presented with a 4-month history of continuous and progressive back pain and a 1-month history of radiating pain of bilateral lower extremities. The patient, who had been diagnosed of mesenchymal chondrosarcoma of maxillary sinus for 3 years, received surgical treatment of palliative endoscopic-assisted total left maxillary resection via mini Caldwell-Luc approach, and palliative enlarged resection due to the progress of residual lesions, followed by no adjuvant therapy. Multiple lytic, expanding lesions of the spine and paraspinal region with severe epidural spinal cord compression was identified. DIAGNOSIS: CT, MRI and bone scan of spine showed spinal cord compression secondary to the epidural component of the metastatic lesions. Post-operative pathology confirmed the diagnosis of metastatic spinal mesenchymal chondrosarcomas. INTERVENTIONS: The patient underwent posterior spinal canal decompression, resection of T12 and L3 lesions, internal fixation of T11-L5 pedicles, and cement augmentation of T12 and L3. OUTCOMES: The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 1-year follow-up visit. There were no complications associated with the spinal surgery during the follow-up period. CONCLUSION: Metastatic spinal mesenchymal chondrosarcoma, although rare, should be part of the differential diagnosis when the patient presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression and total resection of the metastatic chondrosarcoma when the tumor has caused neurological deficits or other severe symptoms. Osteoplasty by cement augmentation is also a good choice for surgical treatment in some patients.

Positron emission computed tomography and magnetic resonance imaging features of sinonasal small round blue cell tumors.

AIM: The sinonasal tract hosts numerous types of undifferentiated neoplasms, having small round cell morphology. The aim of this study was to determine whether sinonasal small round blue cell tumors (SRBCT) have distinct imaging features on computed tomography (CT), magnetic resonance imaging (MRI), and 18-fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT. METHODS: Seventy-three patients (43 male; Mage = 61.2 years) with histopathologically proven sinonasal SRBCT were retrospectively reviewed. Imaging features of SRBCTs including location, maximum dimension, margin characteristics, presence of calcification, sclerotic bone changes, intratumoral necrosis, tumor extension, bone destruction, bone remodeling, perineural spread, T1- and T2-weighted MRI signal intensity, qualitative features on diffusion-weighted imaging and 18F-FDG PET/CT, and pattern of contrast enhancement were analyzed using Fisher's exact test or the chi-square test. The maximum standardized uptake values (SUVmax) and apparent diffusion coefficient (ADCmean) values of SRBCT were compared by utilizing the Kruskal-Wallis test. RESULTS: There was a significant difference between SRBCT type regarding the tumor location (p = 0.006), 18F-FDG uptake pattern (p = 0.006), involvement of the orbit (p = 0.016) and pterygopalatine fossa (p = 0.043), the presence of perineural spread (p < 0.001), bone destruction (p = 0.034), and intratumoral necrosis (p = 0.022). Bone destruction and necrosis were more common in rhabdomyosarcoma. Perineural spread was common in sinonasal adenoid cystic carcinoma (ACC). Qualitative 18F-FDG uptake features as well as tumor location were significantly different between sinonasal ACC and sinonasal undifferentiated carcinoma. The ADCmean and SUVmax values were not statistically different between SRBCT types. CONCLUSIONS: Sinonasal SRBCTs have numerous distinct imaging features on CT, MRI, and 18F-FDG PET/CT that could be useful in the differentiation between lesions when the histopathologic diagnosis is inconclusive.