[Analysis of imaging features of rare tumors in nasal cavity and paranasal sinus].

Objective:To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods:The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results:Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion:Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry.

Ethmoid Osteoblastoma: A Case Report and Pooled Analysis of Cases.

Osteoblastoma is a typically noncancerous bone tumor commonly found in the spine and long bones of the arms and legs. It is exceedingly rare for this tumor to occur in the paranasal sinuses. We present a case of osteoblastoma in a 13-year-old boy affecting the ethmoid sinus, which manifested as exophthalmos. A computed tomography scan revealed an expansive lesion in the right ethmoid sinus, causing compression and displacement of the orbital contents to the right. The lesion exhibited a mix of ground glass opacity and dense bone. On magnetic resonance imaging, the less dense areas of the tumor showed strong enhancement, while the densely sclerotic regions appeared as signal voids on all imaging sequences. A combined transorbital and transnasal approach was performed to remove the tumor. Histologically, the tumor consisted expansile growth surrounded by a sclerotic rim of inter-anastomosing trabeculae of woven bone set within loose edematous fibrovascular stroma. This case highlights the unusual occurrence of osteoblastoma in the ethmoid sinus, a location seldom associated with this type of tumor, and adds to the existing literature on this topic and offers a new surgical approach to managing this entity.

Diffuse Large B-Cell Lymphoma of the Frontal Sinus: A Case Report.

Diffuse large B-cell lymphoma (DLBCL) is the most common type of Non-Hodgkin Lymphoma (NHL). It often involves the gastrointestinal tract, head and neck, and skin, but virtually any tissue or organ can be affected. The primary NHL of the nasal cavity and paranasal sinuses are extremely rare, causing diagnostic and therapeutic difficulties. We present the case of a 49-year-old woman with a 4-week history of diplopia and right superior eyelid swelling. Clinical, radiological, and histological examination led to the diagnosis of DLBCL of the right frontal sinus with anterior invasion of subcutaneous soft tissues and posterior intracranial involvement of the frontal region. She underwent three cycles of MATRIX chemotherapy, three cycles of R-DA-EPOCH, and CAR-T therapy. Unfortunately, treatments were unsuccessful and the patient died 11 months after diagnosis. In conclusion, an early diagnosis of DLBCL of the frontal sinus is difficult as it is often confused with other nasal pathologies. This causes a delay in treatment.

Comparison of three fixed-field IMRT plans for nasal cavity and paranasal sinus tumors / 中华放射医学与防护杂志

Objective:To compare three fixed-field intensity-modulated radiotherapy (IMRT) plans for nasal cavity and paranasal sinus tumors, including the coplanar IMRT (C-IMRT) plan and the non-coplanar IMRT(NC-IMRT) plan which were based on a conventional C-arm LINAC (Trilogy), and the coplanar IMRT (H-IMRT) plan based on an O-ring LINAC (Halcyon).Methods:Based on the data of 10 patients in the Ningbo First Hospital from December 2018 to December 2021 with nasal cavity and paranasal sinus tumors who underwent postoperative radiotherapy, this study redesigned three IMRT plans with the same prescribed doses and optimization objectives. Then, this study compared the doses of target volumes and organ at risks(OARs), the validation pass rates, and the execution time of these plans. Friedman test was employed in this study, and multiple comparisons were further made in cases of different results.Results:The differences in the conformal index (CI) of PTV and PTV boost of the three plans were statistically significant ( χ2 = 7.51, 9.69, P < 0.05). The multiple comparisons showed that the median CI of the H-IMRT plan was higher than that of the NC-IMRT plan ( Z = 2.53, 2.68, P < 0.05). The differences in other parameters of target volumes were not statistically significant. Compared with the C-IMRT plan, the H-IMRT plan reduced the Dmax of bilateral lenses, bilateral corneas, ipsilateral optic nerve, and ipsilateral eyeball ( Z = 2.80, 2.80, 2.80, 2.80, 2.81, 2.09, P < 0.05). Compared with the C-IMRT plan, the NC-IMRT reduced the Dmax of bilateral lenses, corneas, and eyeballs and contralateral optic nerve ( Z = 2.80, 2.66, 2.80, 2.70, 2.29, 2.29, 2.65, P < 0.05) and reduced the Dmean of bilateral eyeballs ( Z = 2.80, 2.80, P < 0.05). Compared with the NC-IMRT plan, the H-IMRT plan reduced the Dmax of the ipsilateral lens and cornea ( Z = 2.50, 2.08, P < 0.05), but increased the Dmax of the contralateral optic nerve and the Dmean of bilateral eyeballs ( Z = 2.80, 2.80, 2.80, P < 0.05). The validation pass rate of the three plans met the institutional standards, and the differences were not statistically significant. Moreover, the H-IMRT plan had the shortest median execution time (172.00 s), followed by the C-IMRT plan (337.50 s), and the NC-IMRT plan (388.00 s). Conclusions:The verification pass rate of the three plans can achieve the requirements of treatment implementation. The three plans had similar dosimetric differences in target volumes. However, the H-IMRT and NC-IMRT plans can protect the normal tissues (especially optical organs) more effectively than the C-IMRT plan, which is conducive to reducing the toxicity after radiotherapy and provides space for local dose increase or the radiotherapy for the treatment of tumor recurrence. The execution efficiency of the three plans is in the order of H-IMRT > C-IMRT > NC-IMRT. It is necessary to select appropriate radiotherapy equipment and technology according to actual situations.

Ethmoid glomangioma and oncogenic osteomalacia: a case report.

BACKGROUND: Glomangioma is a benign tumor of mesenchymal origin, derived from the glomus body. It is responsible for the thermal regulation of the dermis. The occurrence of oncogenic osteomalacia related to glomangioma is rare. Only two cases have been reported thus far. CASE PRESENTATION: A 32-year-old female, Brazilian, presented diffuse pain, during pregnancy, that developed progressively, limiting her mobility. Imaging showed a femoral neck fracture, and rheumatological laboratory examination showed hypophosphatemia. Also, the patient reported episodes of epistaxis during childhood and recurrence along with progressively right nasal obstruction. Endoscopic resection of the tumor was performed, and immunohistochemistry was conclusive for glomangioma. This case report describes the third case in which endonasal endoscopic surgery resulted in a favorable outcome. CONCLUSION: This case of glomangioma-induced oncogenic osteomalacia suggests that surgeons and clinicians should consider sinonasal tumors as a differential diagnosis of osteomalacia, and endonasal endoscopic surgery should be a possible curative resection.

Clinical Spectrum and Outcomes of Ocular and Periocular Complications following External-Beam Radiotherapy for Inoperable Malignant Maxillary Sinus Tumors.

PURPOSE: To highlight the clinical spectrum, management, and outcomes of ocular/periocular complications following high-dose external-beam radiotherapy (EBRT) for inoperable malignant maxillary sinus-involving tumors (MMST). METHODS: A retrospective, interventional case series. All patients who were diagnosed with inoperable MMST (with orbital involvement) and treated with high-dose fractionated EBRT (65 Gy in 30 fractions) at James Cook University Hospital, UK, were included. RESULTS: Seven patients with advanced MMST (T4aN0M0-T4bN2cM0) were included and were followed up for 23.8 ± 10.2 months. Severe lid margin disease, dry eye, and neurotrophic keratopathy were universally observed. Other complications included cicatricial conjunctivitis (71%), corneal perforation (57%), limbal stem cell deficiency (LSCD; 43%), glaucoma (29%), and superimposed candida keratitis (14%). Amniotic membrane transplant (AMT; 71%), tarsorrhaphy (43%), tectonic keratoplasty (29%), and evisceration (14%) were warranted. Intact corneal epithelium was observed in all patients and good corrected-distance visual acuity (≥20/60) was observed in 3 (43%) patients at final follow-up. CONCLUSION: High-dose EBRT for inoperable MMST can lead to a wide array of severe ocular/periocular complications. AMT serves as a potentially useful treatment modality to restore the ocular surface integrity after severe radiation keratopathy. We advocate active monitoring for any evolving ophthalmic complications during and after EBRT to enable timely intervention.

Sinonasal Undifferentiated Carcinoma in a Patient Previously Treated for an Intestinal-Type Adenocarcinoma: Metachronous Neoplasms or Recurrence of a Different Tumor Type?

A case of a sinonasal undifferentiated carcinoma occurring 2 years after a successfully surgically treated intestinal-type adenocarcinoma has never been reported. Treatment modality of paranasal sinus cancer strictly depends on histology so it is important to recognize a different tumor type in the follow-up of these patients.

Oncogenic Osteomalacia with Elevated Fibroblast Growth Factor 23: A Rare Case of Paranasal Sinus Tumor Onset.

Tumor-induced osteomalacia, also known as oncogenic osteomalacia, is a rare, acquired paraneoplastic disease characterized by hypophosphatemia and renal phosphate wasting. We report on the case of a 52-year-old-man admitted to our hospital for bone and muscular pains and difficulty in walking. He underwent a computed tomography (CT) scan of the legs that documented fractures in the right tibia, femur, and fifth metatarsus. Laboratory findings showed hypophosphatemia and elevated levels of parathyroid hormone (PTH). The first diagnosis was osteomalacia, treated with calcium and vitamin D, without any benefit. So he underwent a whole body CT scan, showing a small expansive lesion occupying the left frontal sinus. Furthermore, we found high serum levels of fibroblast growth factor 23 (FGF23) using the enzyme-linked immune sorbent assay (ELISA) assay. The patient underwent endoscopic surgical resection of the frontal tumor with complete clinical remission and the histopathological diagnosis of an ossifying fibromyxoid tumor. This is a rare case of oncogenic osteomalacia due to a paranasal sinus tumor. The main symptoms are not associated with nasal sinus involvement but with over-expressed FGF23. To conclude, physicians should never underestimate the chance of paraneoplastic syndrome in the head and neck district, even if such an occurrence is uncommon in this location. The clinical symptoms may be aspecific and not related to nose problems, making the differential diagnosis very difficult.

Giant Frontal Sinus Osteomas: Demographic, Clinical Presentation, and Management of 10 Cases.

BACKGROUND: Osteomas are rare benign and slow-growing osteogenic tumors mainly involving frontal and ethmoid sinuses. OBJECTIVES: The primary objective of our study is to present the management of cases of giant frontal sinus osteomas. Secondarily, we describe our modified unilateral osteoplastic flap approach without obliteration to remove these osteomas. METHODS: Retrospective chart review at a tertiary academic center ("Hôpital de l'Enfant-Jésus") from July 2006 to October 2016. Demographics characteristics, tumor characteristics, presenting symptoms, frontal sinus surgery technique (osteoplastic flap, endoscopic surgery, or a combination of both), and outcomes of giant frontal sinus osteomas (≥30 mm) were recorded. For laterally placed osteomas, tumors with posterior wall involvement, orbital roof involvement, or intracranial extension, the modified unilateral osteoplastic flap approach was used. A decision-making algorithm is proposed for the choice of surgical approach. RESULTS: Ten giant frontal osteomas were analyzed (7 men and 3 women). The mean age at diagnosis was 38 years old (range, 24-55 years; median, 39 years; standard deviation, 11 years). The most common presenting symptom was headache (43% of symptomatic patients). Five patients had complications preoperatively due to tumoral extension (sinusitis, cellulitis, mucocele, optic nerve compression, and convulsions). One patient was treated endoscopically, 3 patients had an open approach and 6 patients had a combined technique. One patient experienced a postoperative complication (local infection treated with oral antibiotics). Six patients had minimal residual tumor with one patient needing reoperation. CONCLUSION: Osteomas are rare paranasal sinus tumors. Due to the proximity to noble structures, a giant frontal osteoma should be managed surgically. The modified unilateral osteoplastic flap without obliteration offers good long-term surgical and aesthetic results. Osteomas are not known for malignant transformation and recurrences are rare; thus, subtotal resection is warranted and safe when a cleavage plan is not found.

Intensity-modulated radiotherapy for paranasal sinuses and base of skull tumors.

Paranasal sinus and skull base tumors are rare aggressive head and neck cancers, and typically present in the locally advanced stages. As a result, achieving wide surgical resection with clear margins is a challenge for these tumors, and radiotherapy is thus usually indicated as an adjuvant modality following surgery to optimize local control. Given the integral role of radiotherapy in the management of this subgroup of head and neck tumors, the advent of intensity-modulated radiotherapy (IMRT) has led to substantial improvement of clinical outcomes for these patients. This is primarily driven by the improvement in radiation dosimetry with IMRT compared to conventional two dimensional (2D)- and 3D-techniques, in terms of ensuring dose intensity to the tumor target coupled with minimizing dose exposure to critical organs. Consequently, the evident clinical benefits of IMRT have been in reduction of normal tissue toxicities, ranging from critical neurological symptoms to less debilitating but bothersome symptoms of eye infections and radiation-induced skin changes. Another domain where IMRT has potential clinical utility is in the management of a subset of non-resectable T4 paranasal sinus and skull base tumors. For these inoperable lesions, the steep dose-gradient between tumor and normal tissue is even more advantageous, given the crucial need to maintain dose intensity to the tumor. Innovative strategies in this space also include the use of induction chemotherapy for patient selection. In this review, we summarized the data for the aforementioned topics, including specific discussions on the different histologic subtypes of paranasal sinus and skull base tumors.

Pediatric Benign Paranasal Sinus Osteoneogenic Tumors: A Case Series and Systematic Review of Outcomes, Techniques, and a Multiportal Approach.

BACKGROUND: Pediatric benign paranasal sinus osteoneogenic tumors have previously been difficult to treat due to the surgical constraints of the developing skull. Progression may lead to intracranial complications or orbital and optic nerve compression. OBJECTIVE: To assess the presentation and management of benign paranasal sinus osteoneogenic tumors. METHODS: Systematic review of the symptoms and surgical treatment of these lesions, and a case series of 2 adolescents with osteomas impinging on the orbit treated with a multiportal approach. RESULTS: Forty-eight cases of osteomas, osteoblastoma, or osteoid osteomas were reviewed from 43 articles. The average age was 14 years, 67% of tumors were osteomas, and 48% were greater than 3 cm in longest dimension. Compared to osteoblastomas, osteomas had a greater male to female ratio (4.3:1; P = .02), presented at older age ( P = .03) with a smaller tumor burden ( P = .003), and had prior trauma or surgery ( P < .001). The ethmoid, frontal, maxillary, and sphenoid sinuses were affected in decreasing order of frequency. A purely endoscopic sinus surgery approach was taken in 31% of cases, open approach in 62% of cases, and combined approach in 7% of cases. Osteoblastoma patients had a 32% rate of recurrence, compared to only 3% among osteoma patients. On univariate logistics regression of patient demographics, tumor characteristics, and surgical approaches predictive of surgical outcomes, only tumor type was predictive of postoperative recurrence (odds ratio: 0.07; 95% confidence interval, 0.007-0.77; P = .029). CONCLUSION: There was no association between surgical approach and postoperative complications or rate of recurrence. However, a transnasal and transorbital multiportal approach avoids facial incisions.

Anterior Skull Base Glomangioma-Induced Osteomalacia.

Oncogenic osteomalacia (OO) is an uncommon but treatable cause of osteomalacia related to tumor production of FGF23, usually caused by benign mesenchymal neoplasms. Paranasal sinus glomangiomas are a rare cause of OO, with only one previously reported case. Here we describe a second case (first reported in English) of paranasal sinus glomangioma-induced osteomalacia in a 42-year-old man. He presented with weakness and multiple spontaneous fractures, and was found to have an ethmoid sinus glomangioma with intracranial extension. The tumor was removed via endoscopic endonasal approach to the anterior skull base, which resulted in complete resolution of symptoms and no further evidence of disease 1 year postoperatively.

Proton therapy for tumors of the base of the skull.

Relative to conventional photon irradiation, proton therapy has distinct advantages in its ability to more precisely target tumor while shielding adjacent normal tissues. In the setting of skull base tumors, proton therapy plays a critical role in the dose-escalation required for optimal tumor control of chordomas, chondrosarcomas, and malignancies of the paranasal sinuses and nasal cavity. For benign tumors such as craniopharyngiomas, pituitary adenomas and meningiomas, proton therapy can limit long-term adverse effects, such as secondary malignancies. This review summarizes published literature to date regarding the role of proton therapy in skull base tumors and introduces emerging proton therapy approaches such as pencil-beam scanning (PBS).

[Nasal cavity and paranasal sinus cancer]. / Radiothérapie des cancers des cavités nasosinusiennes.

The nasal cavity and parasinusal cancer are rare (10% of tumors of the head and neck) and are mainly represented by squamous cell carcinoma of the nasal cavity or the maxillary sinus and adenocarcinoma of the ethmoid sinus (occupational disease, wood dust). The most common clinical sign is nasal obstruction, but tumors can also manifest as rhinorrhea and/or epistaxis (usually unilateral signs). A magnetic resonance imaging of the facial structure is systematic for staging before treatment. The treatment consists of a first surgery if the patient is operable with a resectable tumor. If it is not the case, the treatment consists of radiotherapy (RT) associated with chemotherapy (CT) according to the initial data (T3/T4 or N+). After first surgery, RT is indicated (except T1N0 with complete resection) associated with a CT based on postoperative data (capsular effraction or incomplete resection). Lymph node irradiation is considered case by case, but is indicated in any nodal involvement. RT must be an intensity modulated RT (IMRT), static or dynamic, and must be imagery guided (IGRT). According to ICRU 83, doses to organs at risk and target volumes must be carried. Finally, after a post-treatment baseline imaging between 2 and 4 months, monitoring will be alternated with the ENT surgeon every 2 or 3 months for 2 years, then every 4 to 6 months for 5 years.

Surgical risk factors for recurrence of inverted papilloma.

OBJECTIVES/HYPOTHESIS: To identify variations in surgical technique that impact the recurrence of inverted papilloma following endoscopic excision. STUDY DESIGN: Retrospective cohort. METHODS: Data from 127 consecutive patients who underwent endoscopic excision of inverted papilloma and oncocytic papilloma at a tertiary care medical center from 1998 to 2011 were reviewed. Patient demographics, comorbidities, tumor stage, and intraoperative details, including tumor location and management of the base, were evaluated to identify factors associated with tumor recurrence. RESULTS: Recurrence of papilloma occurred in 16 patients (12.6%). Mean time to recurrence was 31.0 months (range, 5.2-110.0 months). Mucosal stripping alone was associated with a recurrence rate of 52.2% (12/23 patients), compared to 4.9% (3/61 patients) when the tumor base was drilled, 4.7% (1/21 patients) when it was cauterized, and 0.0% (0/22 patients) when it was completely excised (P = .001). Increased recurrence rate was associated with tumors located in the maxillary sinus (P = .03), as well as the performance of endoscopic medial maxillectomy (P = .001) and external frontal approaches (P = .02). CONCLUSIONS: Drilling, cauterizing, or completely excising the bone underlying the tumor base during endoscopic resection reduces the recurrence rate of inverted and oncocytic papilloma, when compared to mucosal stripping alone. Surgeons who perform endoscopic resection of these tumors should consider utilization of these techniques when possible. LEVEL OF EVIDENCE: 4.

Intracranial extension of Schneiderian inverted papilloma: a case report and literature review.

Inverted papilloma is an uncommon primary nasal tumor. Despite its benign nature, this tumor represents three typical characteristics: a high propensity of recurrence, local aggressiveness and association with malignancy. Inverted papilloma can reduce the patient's quality of life due to compromised nasal function, extension to the orbit and brain. The authors reported the unusual case of a 72-year-old male patient with inverted papilloma, which fatally extended to the intracranial temporal fossa after multiple recurrences. To the authors' knowledge, this is the twelfth case in the literature of inverted papilla extending into the temporal fossa. The current and pertinent literature in English, French and German was reviewed, and an algorithm for managing inverted papilloma was also proposed.

Radiation Therapy of Maxillary Sinus Cancer / 대한치료방사선과학회지

PURPOSE: Maxillary sinus cancers usually are locally advanced and involve the structures around sinus. It is uncommon for this cancer to spread to the regional lymphnodes. For this reason, local control is of paramount important for cure. A policy of combined treatment is generally accepted as the most effective means of enhancing cure rates. This paper reports our experience of a retrospective study of 31 patients treated with radiation therapy alone and combination therapy of surgery and radiation. MATERIALS AND METHODS: Between July 1974 and January 1992, 47 patients with maxillary sinus cancers underwent either radiation therapy alone or combination therapy of surgery and radiation. Of these, only 31 patients were eligible for analysis. The distribution of clinical stage by the AJCC system was 26%(8/31) for T2 and 74%(23/31) for T3 and T4. Eight patients had palpable lymphadenopathy at diagnosis. Primary site was treated by Cobalt-60 radiation therapy using through a 45degree wedge-pair technique. Elective neck irradiation was not routinely given. Of these 8 patients, the six who had clinically involved nodes were treated with definite radiation therapy. The other two patients had received radical neck dissection. The twenty-two patients were treated with radiation alone and 9 patients were treated with combination radiation therapy. The RT alone patients with RT dose less than 60 Gy were 9 and those above 60 Gy were 13. RESULTS: The overall 5 year survival rate was 23.8%. The 5 year survival rate by T-stage was 60.5% and 7.9% for T2 and T3, 4 respectively. Statistical significance was found by T-stage (p<0.005). The 5 year survival rate by N-stage was 30% for N(-) and 8.3% for N(+), but statistically no significant difference was seen(p30.1). The 5 year survival rate for RT alone and combination RT was 22.5% and 27.4%, respectively. The primary local control rate was 65%(20/31). CONCLUSION: This study did not show significant difference in survival between RT alone and combination RT. There is still much controversy with regard to which treatment is optimum. Improved RT technique and development of multimodality treatment are essential to improve the local control and the survival rate in patients with advanced maxillary sinus cancer.