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.

Destructive Fibrosarcoma of the Maxillary Sinus.

Paranasal fibrosarcoma of nasal cavity and paranasal sinuses is a very rare malignant tumor. It is usually presented with nasal obstruction and epistaxis. In this clinical report, clinical symptoms, pathogenesis, and treatment principles of a paranasal fibrosarcoma originating from the right maxillary sinus and obstructing the right nasal passage are discussed.A 55-year-old male patient was admitted to the authors clinic with complaints of nasal obstruction and epistaxis lasting for 2 years. Anterior rhinoscopy revealed a mass lesion which obstructed the right nasal passage and caused frequent epistaxis. An opacity consistent with soft tissue lesion which was originated from the right maxillary sinus and filled the right nasal passage was observed in paranasal tomography. Magnetic resonance imaging revealed that the mass lesion was contrasted. Tumor was seen to erode orbital floor, and lateral and anterior walls of the maxillary sinus. Biopsy result was reported as papilloma. The patient was treated with Denker approach as anterior wall of the maxillary sinus was eroded by the tumor lesion and the mass lesion was excised. The patient received postoperative radiotherapy as pathological diagnosis was reported as paranasal fibrosarcoma.

Adenoid Cystic Carcinoma of the Maxillary Sinus with Isolated Trigeminal Anesthesia.

Adenoid cystic carcinoma (ACC) is a rare malignant secretory gland tumor. It is characterized by slow growth, long clinical course, local recurrences, and distant metastases. In the sinonasal tract, it most commonly arises in the maxillary sinus. It often presents at an advanced stage with perineural spread (PNS). Our patient presented with left-sided facial numbness without other symptoms. The numbness was localized to the left cheek, left side of nose, and left upper lip. Magnetic resonance imaging (MRI) of the brain revealed an enhancing lesion involving the left maxillary sinus with orbital invasion and posterior extension into the cavernous sinus. Transnasal endoscopic exploration with tissue removal revealed ACC. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed no evidence of distant metastases. Presentation of sinonasal ACC (SNACC) is variable depending on the involved structures. Characteristic PNS with ACC may cause neuropathic symptoms. This case displays a unique presentation of an advanced ACC of the maxillary sinus manifesting as isolated unilateral trigeminal anesthesia without sinonasal symptoms. The patient also failed to demonstrate any ocular or oculomotor symptoms despite extensive involvement of the orbit and surrounding structures. This case highlights the importance of recognizing ACC due to its association with late symptomatic manifestations. It also reinforces the need for clinical diligence with the workup of new onset neuropathic symptoms in the maxillary distribution of the trigeminal nerve.

Acquired von Willebrand syndrome with a type 2B phenotype: diagnostic and therapeutic dilemmas.

In this report, we provide evidence of an acquired von Willebrand syndrome (AVWS) with a type 2B phenotype rather than the expected type 1 or 2A. The patient was referred prior to surgical removal of a fibrous mass within the maxillary sinus. His first bleeding 7 years earlier following a retinal tear had been complicated by monocular blindness. Several mucocutanous bleedings followed. Hematological investigations revealed von Willebrand factor (VWF):Ag 91 IU/ml, factor VIII 86 IU/ml, VWF:RCo 34 IU/ml and profound thrombocytopenia with platelet clumping. VWF multimer analysis showed a loss of high-molecular-weight multimers and his plasma aggregated normal platelets under low ristocetin concentration, consistent with type 2B von Willebrand disease (VWD). Sequencing of VWF exon 28 and of the platelet GP1BA gene to investigate the possibility of platelet-type VWD failed to reveal mutations. Serum protein electrophoresis showed a monoclonal IgG protein and led to the diagnosis of monoclonal gammopathy of unknown significance (MGUS), raising suspicion of an AVWS. Over 2 years, he experienced severe gingival bleedings and traumatic intracerebral hemorrhage. Following debridement of the sinus mass, the patient required 20 units of packed red blood cells, despite high-dose Humate-P, continuous Amicar and twice-daily platelet transfusions. Bleeding finally ceased following infusion of activated factor VIIa. A history of prior uncomplicated vasectomy and tendon laceration, no family history of bleeding, the inability to identify a causative mutation in either exon 28 VWF or platelet GP1BA and the MGUS led to diagnosis of AVWS with a type 2B phenotype. This case highlights the difficulties in assigning a diagnosis and the management of bleeding in a patient with an atypical presentation of AVWS.

Concomitant inverted papilloma and fungus ball in unilateral maxillary sinus.

PROBLEM: The concomitant appearance of an inverted papilloma and a fungus ball in unilateral maxillary sinus is rare. These disease entities may be difficult to distinguish before surgery. METHODOLOGY: A male patient presented with the characteristic symptoms of chronic rhinosinusitis. A preoperative sinus computed tomography scan revealed unilateral sinus opacification, hyperdense calcified spots, and bony erosion of the medial maxillary sinus wall. RESULTS: During the operation, a cheesy, clay-like fungus ball was removed. In addition, a papillary appearance of the sinus mucosa led to the suspicion of inverted papilloma behind the fungus ball and nasal polyps. The histopathology showed an inverted papilloma concomitant with a fungal ball and some inflammatory polyps. CONCLUSION: In addition to preoperative imaging, one should be aware of the potential etiology; careful intraoperative reevaluation is imperative for clinicians to avoid misdiagnosis and to provide adequate management of the underlying disease.

Rehabilitation of an extraoral and intraoral defect complicated with microstomia. A study case.

A 72-year-old man was referred from the surgery department for rehabilitation following surgical resection of Basaloid carcinoma. The first surgical intervention involved the anterior palatal region and was restored with a simple obturator. Two years later further surgery was undertaken to excise a recurrent tumor in the nose and part of the cheek. This resulted in an exposed nasal cavity and maxillary sinus. In addition, there was a small oral aperture composed of thin tissue that stretched to its maximum due to scar formation. The defect was restored with a full thickness skin flap but it subsequently broke down leaving the midface exposed with limited mouth opening due to tissue contraction and scar formation after the flap operation. The defect was rehabilitated with Co-Cr obturator intraorally and a silicone nose retained to the naso-palatal extension of the obturator by a magnet extraorally. This resulted in practically good retention, placement, and adaptation of the two parts of the prosthesis.

Cavernous hemagioma of the maxillary sinus: a rare cause of epistaxis.

BACKGROUND: A case of cavernous hemangioma of the maxillary. METHOD: A 25 year old lady presented with history of nasal obstruction, recurrent epistaxis and headache. On exam she had a mass in the left osteomeatal complex that was friable and bled easily on palpation. Computerized tomography of the paranasal sinuses 2mm coronal cuts showed complete opacification of the left maxillary sinus and magnetic resonance imaging revealed a 1.5 x 3 x 2.5 cm lobulated soft tissue mass lesion that enhanced with intravenous gadolinium administration, but no flow-void signals were present. Histologically, the mass was made up of dilated and anastomosing blood vessels. RESULT: The radiologic findings may be attributed to a malignant lesion especially when extensive bone erosion is present. CONCLUSION: Cavernous hemangioma of the maxillary sinus is a very rare benign entity that presents with recurrent epistaxis and nasal obstruction.

Nighttime facial pain induced by myxoma of the maxillary sinus: a case report.

Myxomas of the maxillary sinus are uncommon benign tumors arising from the primitive mesenchyme. They may be related to dental malformations or missing teeth but may also occur without any abnormalities. They usually result in facial deformity manifested by slow, painless bony expansion. Although these tumors are well-circumscribed, they are more extensive than they appear due to their local aggressiveness and bone erosion. Therefore, it should be widely resected with maximal preservation of surrounding structures to prevent recurrence. This article reports a case of myxoma of the maxillary sinus and nighttime facial pain in a 33-year-old male who underwent curettage following debridement with a microdebrider via an intranasal endoscopic approach and surgery involving a modified Caldwell-Luc approach.

[Unilateral maxillary sinusitis: a cavernous haemangioma with bone destruction]. / Einseitige Sinusitis maxillaris : Kavernöses Hämangiom mit knöchernen Arrosionen.

Haemangioma originating in the paranasal sinuses are a rare entity. In the case of unilateral sinusitis the differential diagnosis should include tumors. The following case of a 30-year-old female patient with a therapy-resistant sinusitis showed bone destruction and a maxillary shadow on computed tomography. The histological exam resulted in a cavernous haemangioma.

[Cystic lesion with a displaced tooth in the maxillary sinus]. / Zystischer Kieferhöhlenprozess mit verlagertem Zahn.

Ameloblastomas are epithelial odontogenic tumors in the mandibula or maxilla with potential local infiltrating growth; therefore, relapses can occur after incomplete resection. Among the different histological subtypes, the following are of clinical importance: The so-called unicystic ameloblastoma, radiologically presenting as a common dentigerous cyst, and the so-called extraosseous ameloblastoma. This case report describes the rare combination of a unicystic ameloblastoma with extraosseous localization in the maxillary sinus and association with a displaced tooth. This unusual constellation can cause major diagnostic problems.

[Maxillary sinus osteoma associated with a mucocele]. / Ostéome du sinus maxillaire associé à une mucocèle.

INTRODUCTION: Maxillary sinus osteomas are rare benign tumours with a poorly documented clinical evolution. Their craniofacial localization may be part of a syndrome. We report a case of maxillary sinus osteoma associated to a mucocele and a cyst probably of dental origin, with no sign of associated Gardner syndrome. CASE REPORT: A 52-year-old woman consulted for chronic maxillary sinusitis and an oral vestibular fistula. The CT-scan suggested a mucocele with reactive bone formation. Macroscopically, a pediculated bone tumour was found next to a mucocele, and to a cyst probably of dental origin. The anatomopathological examination led to a diagnosis of cancellous osteoma. DISCUSSION: Several hypotheses have been made on the etiology of sinus osteomas. When identified, screening for Gardner's syndrome should be implemented because of the associated risk for colic malignancy.

[Decompensated sinusitis complicated by cancer of the nasal cavity and the maxillary sinus].

This paper was designed to illustrate the probability of the development of an oncological disease of the nasal cavity and the paranasal sinuses in young subjects presenting with long-standing decompensated chronic sinusitis; also, it demonstrates the possibility of long-term remission after surgical elimination of the inflammatory process. A 28 year-old patient is described having decompensated chronic pansinusitis complicated by sepsis and secondary suppurative dacryocystitis. Multiple surgical interventions on several paranasal sinuses for the treatment of this pathology proved inefficient and were followed by sanation rhinosinusotomy with the simultaneous removal of the malignant tumour. The long-term monitoring of paranasal sinus condition after radical pansinusotomy and ablation of the tumour followed by a course of radiotherapy gave evidence of the malignant process with simultaneous elimination of inflammatory changes in paranasal sinuses.

Malignant tumors of the maxillary sinus: Prognostic impact of neurovascular invasion in a series of 138 patients.

BACKGROUND: Maxillary sinus cancer is a rare disease with heterogeneous biologic behavior. The pattern of neurovascular invasion is known to be an important prognosticator in head and neck cancers, but has not been studied in maxillary malignancies. MATERIALS AND METHODS: Patients undergoing surgery-based treatment with curative intent for a malignancy of the maxillary sinus at the Unit of Otorhinolaryngology - Head and Neck Surgery of the University of Brescia between November 2000 and October 2018 were included. A description of the characteristics of the patients, tumors, and treatments has been performed along with uni- and multi-variate analysis of prognostic factors. Tumors were classified based on the presence of perineural (P0/P1) and lymphovascular invasion (V0/V1) in 4 categories: P0V0, P1V0, P0V1, and P1V1. RESULTS: One hundred-thirty-eight patients were included. Mean age at surgery was 61.0 years. Most patients (60.1%) were affected by non-salivary carcinomas, and most tumors (73.9%) were high-grade cancers. One hundred-seven (77.5%) tumors were classified as pT4. The large majority of patients received bi- or tri-modality treatment. Sixty-three (45.7%) cases were classified as P0V0, 32 (23.2%) as P1V0, 7 (5.1%) as P0V1, and 36 (26.1%) as P1V1. T category, nodal status, and neurovascular invasion were significantly associated with prognosis. Perineural and lymphovascular invasion were associated with the topographical growth of the tumor. CONCLUSIONS: Maxillary cancer is often diagnosed at an advanced stage and in most cases requires a multimodal approach. Perineural and lymphovascular invasion are frequent and have a different impact on prognosis and topographical extension of the tumor.

Anaplastic lymphoma kinase expression and prognosis in inflammatory myofibroblastic tumours of the maxillary sinus.

Inflammatory myofibroblastic tumours (IMT) of the nasal cavity and nasal sinus are rare and, although over 50 cases have been reported in the English-language literature, their precise aetiology and biological behaviour have not been elucidated. Recent studies suggest that anaplastic lymphoma kinase (ALK)-positive tumours have a very low risk of metastasis, but ALK reactivity does not appear to correlate with recurrence. Between March 2002 and December 2008, we encountered three cases of maxillary sinus IMT and investigated them to determine the clinicopathological course, prognosis and immunohistochemical expression of ALK. Two of the patients died < 13 months after the initial diagnosis and the third had multiple recurrences. All three cases were immunohisto chemically negative for ALK expression. IMT of the sino-nasal tract is rare and may undergo malignant transformation in a minority of cases. The three cases manifested progressive extension with bone destruction and multiple recurrences, and two cases had a fatal outcome and one case had high recurrence.

Nasal obstruction as the first symptom in a patient with a calcifying epithelial odontogenic tumour (CEOT).

UNLABELLED: Calcifying epithelial odontogenic tumour (CEOT), also known as Pindborg tumour, is a rare, benign odontogenic neoplasm. A case of an intra-osseous CEOT in the maxilla is presented in which unilateral nasal obstruction and progressive difficulty in breathing were the first clinical symptoms. Dental practitioners might be the first clinicians to come across such tumours, during investigation of missing or non-erupted maxillary teeth, ie canines, and they should be alerted by any unilateral nasal obstruction symptoms. Diagnostic features and treatment options of the tumour are discussed in relation to its histological typing. CLINICAL RELEVANCE: This manuscript highlights the importance of accurate clinical and radiographic investigation for the dental practitioner when assessing missing maxillary teeth.

Extranodal diffuse large B cell lymphoma of maxillary sinus presenting as a palatal ulcer.

A multitude of disease processes ranging from periodontitis to malignancies can lead to formation of solitary ulcer on the palate. Hence solitary ulcers of palate can often be a challenging one to diagnose. We report an interesting case of a diffuse large B cell lymphoma of the maxillary sinus which perforated the palatal bone and presented clinically as a palatal ulcer. Initially the lesion manifested as a small ill-defined swelling in the posterior palatal slope in relation to 24and25 which were mobile and hence was erroneously diagnosed as chronic periodontal abscess. This paper is intended to stress the relevance of including non-Hodgkin's lymphoma in the differential diagnosis of solitary palatal ulcers as it may be often misdiagnosed as more common reactive or inflammatory lesions.

Sinonasal inverted papilloma associated with small cell neuroendocrine carcinoma: A case report and literature review of rare malignancies associated with inverted papilloma.

We report a rare case of sinonasal inverted papilloma (IP) associated with small cell neuroendocrine carcinoma (SNEC). To our knowledge, this is the first report to describe SNEC found during the treatment of sinonasal IP. Surgery and five cycles of cisplatin plus etoposide with concurrent intensity modulated radiation therapy were performed. Neither local recurrence nor distant metastasis was noted during 6 years of post-diagnostic follow-up. The prognosis of SNEC is very poor. Treatment planning for sinonasal IP should consider a possible association with this rare but aggressive malignancy, whose treatment is completely different from that of squamous cell carcinoma, a malignancy which is commonly associated with IP. We also performed a PubMed review of the literature to identify the incidence and pathological diagnosis of associated malignancy. Among a total of 5286 cases of sinonasal IP (61 studies), the incidence of associated malignancy was 8.02% in squamous cell carcinoma, 0.19% in transitional cell carcinoma, 0.04% in mucoepidermoid carcinoma, 0.02% in verrucous cell carcinoma and 0.02% in adenocarcinoma. The incidence of associated malignancy was significantly higher in East and Southeast Asia (11.0%) and North America (10.4%) than in Europe (3.9%) (p=0.04 and p=0.03, respectively; T-test).

Keratocystic odontogenic tumor invading the right maxillary sinus: a case report.

Keratocystic odontogenic tumor (KCOT) is a benign intraosseous neoplasm of the jaw. Involvement of the maxillary sinus is an unusual presentation. We present the case of a 23-year-old man with extensive KCOT and impacted third molar in the right maxillary sinus. The clinical, radiological, and histological features of this tumor and its surgical management are discussed.

[Compound odontoma as a cause of chronic maxillary sinusitis]. / Odontoma compuesto como causa de sinusitis maxilar crónica.

Sinusitis of dental origin is a relatively frequent entity, but the presence of an odontoma in the sinus as a source of this pathology is exceptional. Here we present a case of a young patient who presented chronic maxillary sinusitis over 2 years, originating in an odontoma located in the sinus drainage area.