An adolescent girl with syndrome of inappropriate antidiuretic hormone secretion preceding the diagnosis of olfactory neuroblastoma - a case report.

Objectives: We present an adolescent in whom olfactory neuroblastoma (ONB) was detected on follow-up magnetic resonance imaging (MRI) 2.5 years after SIADH diagnosis. Our case contrasts prior pediatric reports in which ONB and SIADH were diagnosed concurrently. Case presentation: A previously healthy 13-year-old girl was found to have SIADH during evaluation for restrictive eating. Work-up ruled out adrenal, thyroid and paraneoplastic causes, diuretic use, and vasopressin receptor and aquaporin channel mutations. Brain MRI was normal except for paranasal sinus (PNS) inflammatory changes to the left fronto-maxillary sinuses and frontoethmoidal recess. The sodium levels normalized with fluid restriction (800-900 ml/m2/day). Multiple repeated attempts to liberalize fluid intake resulted in recurrent hyponatremia. Follow-up brain MRIs 4 and 11 months after the initial presentation showed persistent PNS inflammatory changes. A subsequent brain MRI 31 months after initial presentation demonstrated a lesion in the left frontoethmoidal recess extending into the left nasal cavity and biopsy showed low grade ONB. The patient underwent surgery with normalization of serum sodium on liberalized fluid intake. Seven days after surgery, she had recurrence of SIADH, and brain MRI showed remnant of the ONB at the fovea ethmoidalis. She completed adjuvant radiotherapy though her SIADH persisted. Conclusions: Our case highlights the importance of considering ONB in the evaluation of children with SIADH. Idiopathic SIADH is rare in children and if no cause is identified, computed tomography of sinuses and nasal endoscopy should be considered earlier in the work-up of these patients, particularly in the absence of sinus symptoms.

The syndrome of inappropriate antidiuretic hormone associated with nasal and paranasal malignant tumors.

PURPOSE: To investigate the clinical characteristics of the syndrome of inappropriate antidiuretic hormone (SIADH) associated with nasal and paranasal malignant tumors. METHODS: Patients with locally advanced or recurrence/metastatic malignant tumors of the nasal and paranasal sinuses were included. The SIADH was diagnosed according to the diagnostic criteria. The clinical characteristics of SIADH patients were retrospectively analyzed. RESULTS: Six patients (6/188, 3.2%) met the diagnostic criteria of SIADH, including four olfactory neuroblastoma (4/26, 15.4%), one neuroendocrine carcinoma (1/9, 11.1%), and one squamous cell carcinoma (1/63, 1.6%). Five patients (83.3%) had severe hyponatremia; however, the hyponatremia could be improved by fluid restriction or tolvaptan. Three patients' SIADH were recovered during the chemotherapy and the other three were recovered after the surgery. CONCLUSION: The incidence of SIADH associated with nasal and paranasal malignant tumors is relatively more common in olfactory neuroblastoma and neuroendocrine carcinoma. The hyponatremia caused by SIADH may be corrected by fluid restriction or tolvaptan, and the SIADH may be recovered through anti-tumor therapy.

Patient With Unilateral Nasal Obstruction and a Nasal Mass.

A 65-year-old man presented with a 2-year history of left nasal obstruction and large tumor in the left nasal cavity. What is your diagnosis?

Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature.

Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.

An exceptionally rare case of Cushing's syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood.

OBJECTIVES: Ectopic adrenocorticotropic hormone secretion/syndrome (EAS) is caused by excess secretion of ACTH leading to hypercortisolism by non-pituitary, commonly malignant origins. We present a rare case of esthesioneuroblastoma (ENB) complicated by EAS in the follow-up period. CASE PRESENTATION: A child presented with nasal obstruction at the age of 10 months. Polypoid mass obstructing the right nasal passage was detected. Magnetic resonance imaging (MRI) showed a lesion limited within the nasal cavity. The lesion was completely removed by nasal endoscopic surgery. The pathologic examination revealed a diagnosis of esthesioneuroblastoma. It was confined to the nasal cavity so chemotherapy/radiotherapy was not administered and began to follow up. At 28 months of age, he presented with rapid weight gain. Laboratory data were consistent with Cushing's syndrome (CS). High-dose dexamethasone suppression test and imaging studies led us to think of ectopic ACTH syndrome originated from ENB relapse. After partial resection of the tumor, ketoconazole treatment was started along with chemotherapy. Hypercortisolemia was kept under control with ketoconazole treatment as long as the treatment was maintained. CONCLUSIONS: Cushing syndrome is a rare endocrine disorder. Adrenal sources of hypercortisolism and ectopic sources of ACTH overproduction should be investigated especially in young children.

Functional Olfactory Neuroblastoma Inducing Symptomatic SIADH.

Olfactory neuroblastoma (ONB) is a rare malignancy of the sinonasal cavity, originating from neuroepithelial olfactory cells. Olfactory neuroblastoma can be difficult to diagnose due to its anatomic position and variable symptomatic presentation, leading to diagnosis at a more advanced stage. Here, we present the case of a 35-year-old man with no previous medical history who had a bicycle accident secondary to syncope. He was found to be hyponatremic and suspected to have syndrome of inappropriate antidiuretic hormone secretion (SIADH). In the workup of SIADH, a brain magnetic resonance imaging revealed a mass in the left middle meatus. The lesion secreted inappropriate amounts of ADH, resulting in symptomatic paraneoplastic SIADH. This ultimately led to the early recognition and successful resection of this rare ONB. The patient has remained disease-free for over 5 years. This case is a fortunate example of a functional malignancy of the sinonasal tract that was discovered early and successfully treated as a result of symptomatic SIADH.

Magnetic resonance imaging findings in patients with idiopathic olfactory dysfunction and normal findings on nasoendoscopy.

OBJECTIVE: In presentations of anosmia or dysosmia, magnetic resonance imaging may be required to screen for intracranial pathology such as olfactory neuroblastomas and other intracranial masses impacting on the olfactory pathway. This study aimed to establish positive magnetic resonance imaging findings of anosmia or dysosmia for scans performed before the coronavirus disease 2019 pandemic. METHODS: The study examined the outcome of patients who presented with isolated olfactory dysfunction and who underwent magnetic resonance imaging between 2015 and 2019. RESULTS: Of the 131 patients, 41 (31.3 per cent) had normal scan findings, 50 (38.2 per cent) had insignificant paranasal mucosal disease and 6 (4.6 per cent) had mucosal thickening significant enough to require additional intervention. These interventions included repeat nasoendoscopy or commencement of intranasal or oral steroids. No patients had olfactory neuroblastoma. CONCLUSION: Only 4.6 per cent of the magnetic resonance imaging scans revealed abnormal findings related to anosmia or dysosmia, and none required ENT surgical intervention. None of the magnetic resonance imaging scans identified an olfactory neuroblastoma or intracranial masses impacting on the olfactory pathway.

Esthesioneuroblastoma presenting as syndrome of inappropriate antidiuretic hormone secretion.

Hyponatremia is the most common fluid electrolyte disorder in hospitalized patients. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the main cause of normovolemic hyponatremia, it can be caused by diverse etiologies: malignant tumors are the most feared cause that clinician persists in finding. Exceptionally, SIADH can complicate Esthesioneuroblastoma (ENB) or olfactory neuroblastoma, a rare tumor of the nasal sinus cavities. We report the case of a 26-year-old female patient admitted for recurrent headaches and vomiting, with a profound normovolemic hyponatremia at the initial assessment. Biological explorations have concluded in a SIADH. Imaging showed a mass of the left nasal cavity with extensions to the ipsilateral paranasal sinuses. The biopsy of the lesion, under endoscopic control, was inconclusive. The anatomopathological study, after surgical removal, concluded in ENB. The postoperative evolution was marked by the normalization of the natremia.

Extramedullary Plasmacytoma of Nasal Cavity: A Case Report and Literature Review.

Extramedullary plasmacytoma (EMP) is a tumor characterized by plasma cell proliferation in organs or tissues outside the bone marrow. It originates from B lymphocytes and can occur in all extramedullary tissues and organs of the body. Primary EMPs of the nasal cavity are relatively rare and mostly presented as case reports. Nasal EMP usually manifests as nasal obstruction, epistaxis, and progressive dyspnea. A 64-year-old man was admitted to our hospital because of rhinorrhagia with nasal obstruction. Computed tomography (CT) revealed a mass in the right nasal passage, which we resected under nasal endoscopy. At the 2-year follow-up, positron emission tomography/CT showed osteolytic bone destruction in the third cervical vertebra and its accessories, accompanied by increased metabolism. We considered the possibility of progression to myeloma, but the patient refused another puncture biopsy. We reviewed the literature for the clinical characteristics and CT findings of 14 patients pathologically confirmed with EMPs of the nasal cavity. All patients had nonspecific clinical manifestations, such as nasal obstruction and epistaxis. Computed tomography mostly showed a uniform-density polypoid mass in the nasal cavity. Therefore, nasal EMP should be included in the differential diagnosis of nasal polyps and other nasal tumors.

Esthesioneuroblastoma Presenting With Orbital Hypertelorism and Foster-Kennedy Syndrome.

ABSTRACT: Esthesioneuroblastoma is a rare neoplasm originating from the olfactory neuroepithelium at the cribriform plate. The superior nasal cavity is primarily affected. Morbidity and mortality are related to locally destructive growth as well as metastatic potential. Orbital involvement is associated with decreased survival. The authors describe a case of advanced esthesioneuroblastoma with bilateral orbital involvement, presenting with a rare constellation of orbital hypertelorism and Foster-Kennedy Syndrome.

Nasal septal angiofibroma: less vascular behaviour than observed in radiological imaging, a rare case in a 51-year-old woman.

Angiofibroma of the head and neck is classically found in the nasopharynx of an adolescent male. We present a rare case where an angiofibroma was diagnosed in the anterior nasal septum of a 51-year-old woman, the eighth nasal septal angiofibroma reported in a female. This case highlights how an angiofibroma in an older woman has a less vascular behaviour than one in an adolescent male, even though radiological enhancement may suggest that the lesion is highly vascularised. Nasal septal angiofibromas can be managed by biopsy and removed by simple endoscopic resection with minimal haemorrhage. Although very rare, extranasopharyngeal angiofibroma/nasal septal angiofibroma should be included in the differential diagnosis when patients of all ages present with nasal obstruction secondary to a nasal mass, with or without epistaxis.

The Efficiency of Routine Histopathological Examination for Bilateral Nasal Polyposis.

The necessity to routinely carry out histopathologic examinations of surgically removed polyps in patients with chronic rhinosinusitis with bilateral nasal polyps (CRSwNP) is uncertain. The aim of this study was to describe the histopathology of polyps from a large series of patients who underwent endoscopic sinus surgery for CRSwNP and the rate of neoplasms. This is a retrospective study of the histopathological result of all patients with CRSwNP who underwent functional endoscopic nasal surgery and in whom histologic examination was performed, from 2006 to 2015, in a tertiary medical center. Data on demographics and whether a nasal lesion was suspected for neoplasm prior to surgery were analyzed. Two hundred thirty-five patients underwent bilateral nasal polypectomy and histopathologic examinations of the surgical specimens. The rate of neoplasms in nonsuspicious cases was 2.3% (5 cases) and all were inverted papilloma. To conclude, inverted papilloma and other neoplasms are considered rare incidental findings in patients presenting with bilateral nasal polyps. This study demonstrated a higher rate of the latter than that reported in the literature. Our study further emphasizes that despite the low incidence of these pathologies, a histopathologic examination of every nasal polypectomy is warranted so as not to miss potential serious pathologies. We stress that a routine histopathological examination should be practiced at least in the elderly population.

Inverted Papilloma Completely Obstructing Anterior Nasal Orifice.

Sinonasal inverted papilloma is a benign lesion that occurs in the nasal cavity and paranasal sinuses. Fungiform papillomas have been described as arising from the septum or the nasal vestibule, while inverted and cylindrical papillomas have been characterized as developing from an attachment site on the lateral nasal wall or a mucosal surface in the paranasal sinuses. Here, we present a rare case of an inverted papilloma that completely obstructed the anterior nasal orifice.