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.

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.

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.

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.

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.

A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome with Recurrent Olfactory Neuroblastoma.

A 40-year-old woman who had a history of recurrent olfactory neuroblastoma presented with full moon face, central obesity, buffalo hump, impaired glucose tolerance and bilateral cervical lymph node swelling. Laboratory tests showed morbidly elevated levels of adrenocorticotropic hormone (ACTH) and cortisol, which were not suppressed by high-dose (8 mg) dexamethasone. Biopsies of the enlarged cervical lymph nodes revealed ACTH-positive metastatic olfactory neuroblastoma, and ectopic ACTH syndrome was diagnosed. Metyrapone was used to suppress cortisol production and resulted in decreased levels of ACTH and cortisol. Bilateral cervical tumor resection further reduced the ACTH and cortisol levels, accompanied by a reduction in the metyrapone dosage. Cushing's syndrome was alleviated through ACTH-producing tumor removal.

Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy.

BACKGROUND: Olfactory neuroblastoma (ONB) is a sinonasal malignancy seldom seen in clinical practice. It is also known by various other names like esthesioneuroblastoma, esthesioneuroepithelioma, esthesioneurocytoma, and esthesioneuroma. Surgery and radiation therapy are considered as standard treatment modalities for ONB; however, the role of chemotherapy is not well established. AIMS: We aim to define the role of chemotherapy in the neoadjuvant setting in a case of ONB. METHODS AND RESULTS: We report a young female patient presenting with a naso-facial swelling causing facial disfigurement, proptosis, decreased visual acuity, and poor performance status. She was diagnosed with advanced-stage ONB. Prompt administration of chemotherapy led to the improvement in the symptoms and rapid regression of the tumor mass. Later on, the tumor mass was excised completely without any neurological deficit. CONCLUSION: This report justifies the role of neoadjuvant chemotherapy in the management of ONB.

Occult olfactory neuroblastoma presenting with multiple bone metastases: a case report.

RATIONABLE: Olfactory neuroblastoma (ONB) is a rare malignant tumor of the nasal cavity, the primary local symptoms are usually inconspicuous. Patients are often admitted to various specialties based on different primary symptoms, which may result in delayed diagnosis and even a misdiagnosis. PATIENT CONCERNS: Here we report a case of ONB that presented initially as multiple ostealgia without any local symptoms of the tumor and primarily misdiagnosed as multiple myeloma. The patient was a 47-year-old female with bone pain at multiple sites. The initial diagnosis was considered as multiple myeloma. However, the morphologic examination of bone marrow suggested that the tumor cells originated from the nervous tissues. After the positron emission computed tomography scan, the primary lesion in the nasal cavity was located, and a biopsy was performed. DIAGNOSIS: The final diagnosis of ONB was confirmed by histopathological tests. INTERVENTIONS: The patient was treated with metronomic chemotherapy. OUTCOMES: The symptoms of bone pain were significantly relieved 3 months later. The emission computed tomography scan of the whole body bones and the magnetic resonance imaging of the head showed that the tumor size did not change significantly and proved a progression-free of the disease. LESSONS: It is a reasonable strategy to identify the original latent tumor by a prompt positron emission computed tomography scan when the primary diagnosis indicates a metastatic disease, especially for the occult malignancies like ONB.

Adrenocorticotropic Hormone-Secreting Esthesioneuroblastoma with Ectopic Cushing's Syndrome.

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. 68Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.

Isolated primary maxillary sinus esthesioneuroblastoma presenting as idiopathic syndrome of inappropriate antidiuretic hormone.

Esthesioneuroblastoma is an uncommon tumour, and isolated primary involvement of the maxillary sinus is exceedingly rare. Esthesioneuroblastoma has infrequently been reported as a source of paraneoplastic ectopic hormone production. We report a case of isolated primary maxillary esthesioneuroblastoma, presenting as idiopathic syndrome of inappropriate antidiuretic hormone (SIADH). A 17-year-old girl presented with symptoms consistent with SIADH and no sino-nasal symptoms. MRI to exclude pituitary tumour revealed an isolated lesion of the right maxillary sinus. Biopsy demonstrated esthesioneuroblastoma. The lesion was removed endoscopically as a single en bloc specimen. Following resection, the sodium level returned to normal. This is only the third report in the literature of a primary maxillary esthesioneuroblastoma presenting as SIADH.

Pulmonary nocardiosis mimicking small cell lung cancer in ectopic ACTH syndrome associated with transformation of olfactory neuroblastoma: a case report.

BACKGROUND: Pulmonary nocardiosis frequently develops as an opportunistic infection in cell-mediated immunosuppressive patients, and sometimes requires differentiation from pulmonary malignancy. Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a neoplastic disorder which leads to impaired cell-mediated immunity, and is commonly associated with small cell lung cancer (SCLC). Because pulmonary infection and causative malignancy can appear as pulmonary lesions with EAS, differentiation of these diseases remains a critical issue for physicians. CASE PRESENTATION: A 52-year-old woman with progressive lower limb paralysis and general fatigue was referred to us. She had been diagnosed with olfactory neuroblastoma (ONB) and treated with surgery and radiation therapy 10 years before the referral and had required stereotactic radiosurgery and chemotherapy 4 years later for a relapse of the ONB. On referral, she presented with Cushing's syndrome with elevated cortisol and ACTH levels. Potassium supplement improved her symptoms; however, a month later, she was urgently hospitalized due to acute pleuritic chest pain on inspiration. Chest computed tomography revealed left lower lobular consolidations and a contralateral nodule in the right middle lobe. The clinical history and laboratory work-up suggested that her Cushing's syndrome had most likely arisen from EAS. Additionally, the lungs were suspected as the ACTH source due to high levels of progastrin-releasing peptide and progressive pulmonary consolidation with a contralateral nodule, suggesting SCLC. However, histological examination from bronchoscopy revealed no evidence of malignancy, and Nocardia cyriacigeorgica was isolated from bronchoalveolar lavage fluid. Sulfamethoxazole/trimethoprim improved her pulmonary lesions. Somatostatin receptor scintigraphy revealed strong tracer uptake in the ONB lesions, indicating that the origin of the EAS was the olfactory tumor. However, histological examination of ONB specimens resected 10 years earlier showed no intracytoplasmic immunopositivity for ACTH. CONCLUSIONS: We highlight a rare case of pulmonary nocardiosis, which was associated with EAS mimicking SCLC, and was related to ONB transformation. Nocardiosis has to be considered even though anamnestic, clinical, and radiological aspects suggest the presence of metastasis. Additionally, physicians should carefully monitor patients with ONB for the development of Cushing's symptoms because the tumor can transform into an ACTH-producing form, even after long-term follow-up.

Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review.

RATIONALE: Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It has a subacute onset with the following symptoms: cognitive dysfunction, seizures, irritability, hallucinations, and short-term memory loss. Herein, we report the case of a 35-year-old man with PLE, an olfactory neuroblastoma (ONB) admixed with craniopharyngioma, and serum anti-Hu antibodies. PATIENT CONCERNS: The patient presented with generalized seizures, short-term memory loss, and a polypoid mass located high in the nasal cavity. INTERVENTIONS: He underwent surgical resection of the tumor and postoperative chemoradiotherapy with concurrent intra-arterial cisplatin administration. DIAGNOSIS: Pathological examination indicated an ONB admixed with craniopharyngioma. OUTCOMES: The patient's neurological symptoms gradually diminished after surgery. No evidence of recurrence was observed during a 4-year follow-up. LESSONS: We reported a histologically unusual heterogeneous tumor that comprised ONB and craniopharyngioma. This is the first reported case of PLE with anti-Hu antibodies possibly associated with ONB admixed with craniopharyngioma.

Ectopic Cushing's syndrome secondary to olfactory neuroblastoma.

We present the case of a patient with Cushing's syndrome secondary to ectopic ACTH secretion. A MR of the head showed a left-sided nasal mass extending down from the cribriform plate. The patient underwent endoscopic resection with nearly complete removal of the mass. Histological examination showed an ACTH-secreting olfactory neuroblastoma (ONB). The patient's cortisol levels returned to normal range after surgery and have remained normal for over a year. ONB is a rare cause for ectopic ACTH secretion. This case highlights the diagnostic and management difficulties in patients with ectopic ACTH secretion, and provides a brief review of ONB.

Idiopathic SIADH in young patients: don't forget the nose.

Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine sinonasal cancer associated by many authors to ectopic production of several biologically active substances. We report a case of a 31-year-old male patient who presented with idiopathic syndrome of inappropriate secretion of antidiuretic hormone (SIADH). During diagnostic work-up, a CT scan of the head was performed and an ethmoidal ONB was detected. Endoscopical surgery followed by radiotherapy was carried out. Immediately after surgery natraemia levels normalised. Five years later the patient is disease-free. To our knowledge, 17 cases of SIADH associated to ONB have been published. In nine reports, idiopathic SIADH promptly led to the diagnosis of the sinonasal mass as in our clinical case, however, in many reports, correct diagnosis was accomplished months to years later. In young patients with idiopathic inappropriate antidiuretic hormone secretion, a neuroendocrine malignancy of the sinonasal area must be excluded.

Syndrome of inappropriate antidiuretic hormone secretion in a case of olfactory neuroblastoma without anti-diuretic hormone immunoreactivity: A case report and review of the literature.

Olfactory neuroblastoma (ONB) is a relatively rare nasal or paranasal malignant tumor. This tumor is rarely accompanied by paraneoplastic syndromes such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Here, we report a 31-year-old female with histologically confirmed ONB who had been diagnosed with SIADH three years prior. She was treated with surgery followed by concurrent chemoradiotherapy. SIADH resolved immediately after surgical tumor resection. Immunohistochemically, both biopsy and resected specimens from the nasal cavity had been negative for ADH. Although extremely rare, ONB may be associated with SIADH, and the possibility of this cancer should be taken into account during the follow-up of idiopathic SIADH.

Complicaciones en reconstrucción craneal con prótesis de polieteretercetona / Complications in cranial reconstruction using PEEK prosthesis

Introducción. Los defectos craneales tienen una repercusión importante en el paciente desde un punto de vista estético, psicológico y funcional. Actualmente no existe acuerdo sobre el material ideal para la reconstrucción de estos defectos. Material y métodos. Realizamos un estudio retrospectivo incluyendo a los pacientes con reconstrucción craneal con prótesis de polieteretercetona (PEEK) a medida entre los años 2008 y 2014 en el Servicio de Cirugía Oral y Maxilofacial del Hospital Universitario La Paz, Madrid. Las prótesis son diseñadas de manera específica para cada paciente con el sistema CAD-CAM. Resultados. Se reconstruyó a un total de 7 pacientes con defectos craneales con prótesis de PEEK. Solo en un caso, la resección y reconstrucción fueron realizadas en el mismo acto quirúrgico. En el resto de los casos se realizó una reconstrucción diferida. Las complicaciones que se presentaron fueron: un seroma y una fístula de LCR, que se resolvieron con tratamiento conservador; y una dehiscencia de la herida y un caso con episodios de infecciones repetidas, cuyo defecto estaba próximo al seno frontal, que terminaron con la retirada definitiva de la prótesis. Conclusión. El material ideal para la reconstrucción de defectos craneales no existe. Las prótesis de PEEK están obteniendo buenos resultados estética y funcionalmente. Cuando el hueso autógeno no esté disponible o en determinados casos con defectos grandes, las prótesis de PEEK pueden ser una buena opción. Sin embargo, si el defecto está en comunicación directa con los senos paranasales, debemos valorar bien la indicación (AU)

Introduction. Cranial defects tend to carry functional and aesthetic consequences for the patient. The most suitable material to be used remains controversial. Methods. We report a retrospective review of patients whose cranial defects were reconstructed using a computer designed polyetheretherketone (PEEK)-patient specific implant) between 2008 and 2014 at the Oral and Maxillofacial Surgery Department, in Hospital Universitario La Paz, Madrid. Implants are designed individually to each patient with CAD-CAM system. Results. Seven patients underwent cranial reconstruction using a PEEK-patient specific implant. One case involved a one-step primary reconstruction and the rest of cases underwent a delayed reconstruction. Complications were: one seroma and one CSF leak, resolved with conservative treatment, and a wound dehiscence and an infection case whose defect was near the frontal sinus, resolved with removal of the implant. Conclusion. The ideal material for reconstructing cranial defects does not exist, but PEEK has demonstrated good outcomes. When autologous bone is not available or in selected cases with large defects, PEEK is a good option to reconstruct these defects. However, if the defect is related with paranasal sinuses, indication must be evaluated (AU)

[Olfactory neuroblastoma with initial manifestations of hyponatremia: a case report].

Clinical records of a patient with olfactory neuroblastoma presented with hyponatremia as initial symptoms were analyzed and the literatures were reviewed. At initial onset, the patient presented with hyponatremia. After pathological examination, the diagnosis was olfactory neuroblastoma. The blood sodium has been normal after operation and radiotherapy. The incidence rate of olfactory neuroblastoma is low, and it is easily misdiagnosed. Its diagnosis relies on pathological examination. We should pay more attention to the unspecific symptoms of patients with hyponatremia, which can help to improve early diagnosis and the prognosis.