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Cureus 2018 Nov 28;10(11):e3653. Epub 2018 Nov 28.

Radiology, Osceola Regional Medical Center, Kissimmee, USA.

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a malignant tumor of the upper nasal cavity. This report illustrates the case of a 63-year-old woman who presented with intractable headaches. Subsequent radiologic evaluation and correlation with histopathologic analysis confirmed esthesioneuroblastoma. Read More

J Neurosurg 2019 Feb;130(2):337-346

2Otorhinolaryngology, NewYork-Presbyterian Hospital/Weill Cornell Medicine, New York; and.

OBJECTIVEEndoscopic skull base surgery (ESBS) is a relatively recent addition to the neurosurgical armamentarium. As with many new approaches, there has been significant controversy regarding its value compared with more traditional approaches to ventral skull base pathology. Although early enthusiasm for new approaches that appear less invasive is usually high, these new techniques require rigorous study to ensure that widespread implementation is in the best interest of patients. Read More

World Neurosurg 2019 02;122:707

Department of ENT and Head and Neck Surgery, All India Institute of Medical Sciences, Ansari Nagar East, New Delhi, India.

Head Neck 2018 Dec 10. Epub 2018 Dec 10.

Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota.

Background: Past research established that surgery plus adjuvant radiotherapy (S + AR) improves overall survival (OS) in esthesioneuroblastoma (ENB). However, it is unknown if the addition of adjuvant chemotherapy (AC) further improves survival. The primary objective of this study was to compare survival among patients treated with S + AR alone to patients who underwent S + AR + AC. Read More

J Surg Oncol 2019 Jan 22;119(1):130-142. Epub 2018 Nov 22.

Department of Head and Neck Surgery, Memorial Sloan Kettering Cancer Center, New York, New York.

Background: Esthesioneuroblastoma (ENB) is a rare neuroendocrine tumor. The purpose of this study was to compare the Kadish, tumor-node-metastasis (TNM), and Dulguerov's modified TNM staging in order to determine the impact of the stage on primary surgical treatment selection, margin status, and survival.

Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with ENB between 2004 to 2015. Read More

Cancers (Basel) 2018 Nov 20;10(11). Epub 2018 Nov 20.

Department of Radiation Oncology, Heidelberg University Hospital, 69120 Heidelberg, Germany.

(1) Background: Esthesioneuroblastoma (ENB) is a rare tumor entity originating from the olfactory neuroepithelium. There is a scarcity of data about different treatment strategies. Intensity modulated radiotherapy (IMRT) and carbon ion radiotherapy (CIRT) are advanced radiation techniques that might improve local tumor control. Read More

Int Forum Allergy Rhinol 2019 Feb 9;9(2):212-219. Epub 2018 Nov 9.

Division of Head and Neck Surgery & Communication Sciences, Department of Surgery, Duke University, Durham, NC.

Background: Cancers of the paranasal sinuses are rare tumors that tend to be aggressive and usually are diagnosed at an advanced stage. Despite being rare, these tumors include a wide spectrum of histological subtypes with different biological behaviors. Choosing the optimal treatment modalities and analyzing the different oncological outcomes is therefore challenging. Read More

J Int Med Res 2018 Nov;46(11):4760-4768

1 Department of Internal Medicine, Division of Diabetes, Endocrinology and Metabolism, Hyogo College of Medicine, Mukogawa-cho, Nishinomiya, Hyogo, Japan.

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a condition of endogenous hypercortisolism sustained by an extrapituitary ACTH-secreting tumor. Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of the sinonasal tract and is derived from the olfactory epithelium. Because the paranasal sinus is not a common site of EAS, the development of ONB in patients with EAS is rare. Read More

J Clin Neurosci 2018 Dec 16;58:117-123. Epub 2018 Oct 16.

Division of Neurosurgery, Toronto Western Hospital, University Health Network, Toronto, ON, Canada.

During the last two decades evolution of the endoscopic endonasal approaches (EEA) has offered the option of minimally invasive techniques in the management of esthesioneuroblastomas (ENB), either as endoscope assisted cranial resection or as pure endoscopic procedures. This study presents the use of pure EEA in the management of ENB in our center, along with a literature review. We retrospectively reviewed the clinical, radiology and pathology records of patients with ENB treated during the period July 2006 to January 2016. Read More

Cell Rep 2018 Oct;25(3):811-821.e5

Department of Medical Oncology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique Hôpitaux de Paris, Sorbonnes-Universités, University Pierre and Marie Curie, Paris, France; Department of Hematology and Medical Oncology, CHRU Strasbourg, Hôpital Hautepierre, Strasbourg, France; Institut Génomique et de Biologie Moléculaire et Cellulaire (IGBMC), Illkirch-Graffenstaden, France. Electronic address:

Esthesioneuroblastoma (ENB) is a rare cancer of the olfactory mucosa, with no established molecular stratification to date. We report similarities of ENB with tumors arising in the neural crest and perform integrative analysis of these tumors. We propose a molecular-based subtype classification of ENB as basal or neural, both of which have distinct pathological, transcriptomic, proteomic, and immune features. Read More

World Neurosurg 2018 Dec 24;120:426-429. Epub 2018 Sep 24.

Department of Neurology & Neurosurgery, University of Miami Health System, Miami, Florida, USA. Electronic address:

Background: The use of extracorporeal membrane oxygenation (ECMO) for cardiopulmonary support is indicated for refractory respiratory failure but carries a high morbidity and mortality in the neurosurgical setting due to associated risks of intracranial hemorrhage.

Case Description: We describe the case of a 62-year-old man who underwent craniotomy for resection of an esthesioneuroblastoma involving the anterior skull base and extending intracranially, through the cribriform plate into the right epidural space. He developed refractory hypoxemic and hypercapnic respiratory failure and circulatory shock in the immediate postoperative period. Read More

J Neurol Surg B Skull Base 2018 Oct 22;79(5):495-500. Epub 2018 Feb 22.

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United States.

 Neck metastases in patients with esthesioneuroblastoma (ENB) constitute the most significant predictor of poor long-term survival. Recently, researchers discovered the existence of dural lymphatic channels that drain to the cervical lymph nodes. From this physiologic basis, we hypothesized that patients with ENB who develop dural invasion (DI) would exhibit a proclivity for neck metastases. Read More

J Neurol Surg B Skull Base 2018 Oct 29;79(5):419-426. Epub 2017 Dec 29.

Department of Otolaryngology Head and Neck Surgery, University of Toronto, Toronto, Canada.

 (1) To determine the occult rate of dural invasion in patients with tumors extending to and/or eroding the bony anterior skull base but without evidence of dural invasion on preoperative imaging. (2) To determine the impact of dural resection and of skull base erosion on survival outcomes in this group of patients (without evidence of dural invasion upon preoperative imaging).  Retrospective study. Read More

Laryngoscope 2018 Sep 8. Epub 2018 Sep 8.

Department of Otolaryngology-Head and Neck Surgery, Rush University Medical Center, Chicago, Illinois, U.S.A.

Objective: Esthesioneuroblastoma is an uncommon malignancy of the sinonasal tract arising from the olfactory epithelium. Surgical management of the primary site, often via an endoscopic approach, with or without adjuvant radiation, is often curative. There is growing but ultimately limited data regarding management of the neck and the risk of nodal metastases. Read More

Int J Radiat Oncol Biol Phys 2018 10 5;102(2):353-361. Epub 2018 Jun 5.

Hospital of the National Institute of Radiological Sciences, National Institutes for Quantum and Radiological Sciences and Technology, Chiba, Japan.

Purpose: To evaluate the safety and efficacy of carbon-ion radiation therapy (C-ion RT) for locally advanced sinonasal malignant tumors in a multicenter retrospective study (J-CROS 1402 HN).

Methods And Materials: Clinical data were collected for patients who had sinonasal malignant tumors of stage N0-1M0 and received C-ion RT at 4 institutions in Japan between November 2003 and December 2014. Of the 458 patients, 393 had naïve tumors and 65 had recurrent tumors. Read More

APMIS 2018 Aug;126(8):663-666

Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.

The aim of this study was to report incidence and patient characteristics of olfactory neuroblastoma (ONB) at a tertiary cancer institution during a 16-year period. A retrospective review was conducted on patients with ONB treated at Rigshospitalet, Copenhagen from 2000 to 2016 covering Eastern Denmark. Patient demographics, symptoms, stage, pathology-reports, treatment, and outcome were extracted from the patient records and the Danish pathology register. Read More

BMC Pulm Med 2018 Aug 22;18(1):142. Epub 2018 Aug 22.

Division of Pulmonary Medicine, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo, 160-8582, Japan.

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. Read More

J Neurosurg 2018 Jul 1:1-7. Epub 2018 Jul 1.

Departments of1Neurosurgery.

OBJECTIVEOlfactory neuroblastoma (ONB) is a rare malignant neoplasm of the sinonasal cavity. Surgery has been and remains a mainstay of treatment for patients with this tumor. Open craniofacial resections have been the treatment of choice for many decades. Read More

Ear Nose Throat J 2018 Jun;97(6):E24-E30

Department of Otorhinolaryngology, Zhujiang Hospital of Southern Medical University, Guangzhou, China.

We retrospectively reviewed the cases of 85 patients with primary sinonasal malignancies who had undergone endoscopic surgery with curative intent achieved by "regional resection." Our goal was to assess the efficacy of endoscopic surgical treatment vis-à-vis traditional open surgery. Kaplan-Meier data analysis revealed that the 1-, 3-, and 5-year disease-specific survival rates were 82, 60, and 49%, respectively. Read More

Medicine (Baltimore) 2018 Jul;97(28):e11527

Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University.

Rationale: Primary intracranial immature teratoma accounts for majority of congenital central nervous system germ-cell tumors, but it is extremely rare in patients older than 15 years.

Patient Concerns: A 27-year-old woman was referred to our hospital for headache, nasal congestion, and decreased olfactory sensation. Imaging showed a mass measuring approximately 5 cm × 4 cm in the right frontal lobe, which also filled the right nasal cavity. Read More

Ear Nose Throat J 2018 Apr-May;97(4-5):E18-E21

Department of Internal Medicine, 500 W. Thomas Rd., Suite 900, Phoenix, AZ 85013, USA.

Esthesioneuroblastoma (ENB) is an uncommon sinonasal tract tumor, and it is even more uncommon among all neoplasms. Literature regarding the incidence and spread of the disease is limited. The prognosis of metastatic disease is poor. Read More

Pathol Res Pract 2018 Aug 10;214(8):1123-1129. Epub 2018 Jun 10.

Tianjin Key Laboratory on Technologies Enabling Development of Clinical Therapeutics and Diagnostics, School of Pharmacy, Tianjin Medical University, Tianjin 300070, China; Research Center of Basic Medical Sciences, Tianjin Medical University, Tianjin 300070, China. Electronic address:

Olfactory neuroblastoma (ONB) is a kind of rare and complex head and neck tumor. The reports on this field are very scarce due to the low morbidity. Here, we summarized the clinical features and prognosis of ONB through analysis of 10 cases, and determined the phosphorylation status of some molecules known to be involved in carcinogesis such as Akt, Erk, Stat3 and Stat5 in ONB tissue. Read More

Medicine (Baltimore) 2018 Jun;97(24):e10932

Department of Otolaryngology-Head and Neck Surgery, Kyorin University School of Medicine, Tokyo, Japan.

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. Read More

Cancer Manag Res 2018 29;10:1359-1364. Epub 2018 May 29.

Department of Radiation Oncology, Tianjin Medical University Cancer Institute and Hospital, Key Laboratory of Cancer Prevention and Therapy, National Clinical Research Center for Cancer, Tianjin, People's Republic of China.

Objective: The objective of the study was to describe the age distribution and to evaluate the role of prognostic value of age on survival in patients diagnosed with olfactory neuroblastoma (ONB). A population-based retrospective analysis was conducted.

Materials And Methods: The population-based study of patients in the Surveillance, Epidemiology, and End Results (SEER) tumor registry, who were diagnosed with ONB from 1973 to 2014, were retrospectively analyzed. Read More

J Neurol Surg B Skull Base 2018 Apr 28;79(2):184-188. Epub 2017 Aug 28.

Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, United States.

 Olfactory preservation after resection of esthesioneuroblastoma (ENB) has been reported, however, the ability to predict tumor involvement of the olfactory system is critical to this surgical strategy. This study aims to answer the question: Can a surgeon predict, based on preoperative imaging, whether there is unilateral involvement of the olfactory system allowing for safe attempt of olfactory preservation?  This is a retrospective review of post-resection ENB meeting inclusion criteria of having bilateral olfactory tracts and bulbs submitted at the time of primary resection for pathologic margins. Five board-certified skull base surgeons blinded to the pathology individually reviewed the preoperative MRI scans to predict degree of tumor involvement. Read More

Acta Otorrinolaringol Esp 2018 May 19. Epub 2018 May 19.

Hospital Complex of Santiago de Compostela, Primary care and Emergency Department, Spain.

Breast J 2018 Sep 20;24(5):836-837. Epub 2018 May 20.

Department of Radiology, Regional Hospital of Ben Arous, University el Manar, Tunis, Tunisia.

Ocul Oncol Pathol 2018 Apr 18;4(3):192-195. Epub 2017 Nov 18.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

Background: Esthesioneuroblastoma is a rare neuroepithelial tumor arising from the olfactory epithelium in the cribriform plate or nasal cavity. It accounts for 1-5% of cases of malignant neoplasms of the nasal cavity.

Methods: A 20-year-old pregnant female presented with painful loss of vision in both eyes, on imaging diagnosed as meningioma. Read More

J Neurol Surg B Skull Base 2018 Jun 1;79(3):282-288. Epub 2017 Nov 1.

Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota, United States.

 The objective of this study was to identify factors that may influence the rate and location of regional metastasis in sinonasal malignancies (SNMs).  This is a retrospective review.  This study was set at the single-institution tertiary referral center. Read More

J Neurol Surg B Skull Base 2018 Jun 1;79(3):262-268. Epub 2017 Nov 1.

Department of ENT Surgery, Beaumont Hospital, Dublin 9, Ireland.

 Evaluation of the changing trends in esthesioneuroblastoma in an Irish context and review of management options nationally to clarify the best current therapeutic approach by comparing with international research on this uncommon malignancy.  Retrospective review.  Tertiary referral center. Read More

Head Neck 2018 Oct 13;40(10):2295-2303. Epub 2018 May 13.

Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota.

Background: The purpose of this study was to determine the clinical outcomes and review the management strategies for metastatic esthesioneuroblastoma.

Methods: We conducted a systematic review and meta-analysis.

Results: Forty-eight studies totaling 118 patients met inclusion criteria. Read More

Otolaryngol Head Neck Surg 2018 Oct 8;159(4):643-649. Epub 2018 May 8.

2 Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.

Objectives Primary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL. Read More

Acta Neuropathol 2018 Aug 5;136(2):255-271. Epub 2018 May 5.

Center for Neuropathology, Ludwig-Maximilians-University, Munich, Germany.

Olfactory neuroblastoma/esthesioneuroblastoma (ONB) is an uncommon neuroectodermal neoplasm thought to arise from the olfactory epithelium. Little is known about its molecular pathogenesis. For this study, a retrospective cohort of n = 66 tumor samples with the institutional diagnosis of ONB was analyzed by immunohistochemistry, genome-wide DNA methylation profiling, copy number analysis, and in a subset, next-generation panel sequencing of 560 tumor-associated genes. Read More

Clin Nucl Med 2018 Jun;43(6):452-453

A 53-year-old woman with metastatic esthesioneuroblastoma was referred for FDG PET/CT imaging. FDG PET/CT showed symmetrical uptake in bilaterally enlarged adrenal glands, in addition to abnormal uptake in bone and lymph node metastases. In-pentetreotide scintigraphy was further performed and revealed the lack of adrenal uptake. Read More

Oper Neurosurg (Hagerstown) 2018 Dec;15(6):100-109

Department of Neurosurgery, Lenox Hill Hospital, Zucker School of Medicine at Hofstra/Northwell, New York, New York.

Background And Importance: Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a malignant neoplasm with an unpredictable behavior. Currently, the widely accepted treatment is inductive chemotherapy, with or without surgery, followed by radiotherapy. Since data on genetics and molecular alterations of ENB are lacking, there is no standard molecularly targeted therapy. Read More

BMJ Case Rep 2018 Mar 13;2018. Epub 2018 Mar 13.

Division of Metabolism, Endocrinology and Nutrition, Department of Medicine, University of Washington, Seattle, Washington, USA.

A 62-year-old woman presented with an 11-month history of worsening nasal symptoms of rhinorrhoea, anosmia, nasal congestion and intermittent epistaxis. MRI revealed a large mass in the upper nasal vault. Biopsy of the mass revealed an olfactory neuroblastoma. Read More

Case Rep Endocrinol 2018 4;2018:6389374. Epub 2018 Feb 4.

Vanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USA.

We present a case of a 52-year-old male who developed Cushing's Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Read More

Anticancer Res 2018 03;38(3):1665-1670

Hospital of the National Institute of Radiological Science, National Institutes for Quantum and Radiological Sciences and Technology, Chiba, Japan.

The purpose was to evaluate efficacy and safety of carbon ion radiotherapy (C-ion RT) in patients with locally advanced olfactory neuroblastomas (ONBs). This study was a sub-analysis of the Japan Carbon-Ion Radiation Oncology Study Group Study (1402 HN, UMIN000024473). Clinical data of T4 ONBs treated with C-ion RT at four Institutions between November 2003 and December 2014 were retrospectively reviewed. Read More

Neurosurgery 2018 Nov;83(5):940-947

Department of Radiation Oncology, University of Utah, Salt Lake City, Utah.

Background: The available literature to guide treatment decision making in esthesioneuroblastoma (ENB) is limited.

Objective: To define treatment patterns and outcomes in ENB according to treatment modality using a large national cancer registry.

Methods: This study is a retrospective cohort analysis of 931 patients with a diagnosis of ENB who were treated with surgery, radiation therapy, and/or chemotherapy in the United States between the years of 2004 and 2012. Read More

Auris Nasus Larynx 2018 Oct 21;45(5):1107-1112. Epub 2018 Feb 21.

Clinic of Otolaryngology, Head and Neck Surgery, Department of Translational Surgery and Medicine, University of Florence, Italy.

Olfactory neuroblastoma is a rare malignant tumor of neuroectodermal origin and represents the most common cancer of the nasal cavity in pediatric age. The gold standard of treatment consists of en bloc resection, numerous studies have shown as the endoscopic approaches permit good control of the disease improving the quality of life after the treatment. Herein we describe the case of a 13-year-old patient referred to our outpatient clinic with a polypoid multi-lobed lesion occupying the left nasal cavity and imaging that confirmed a left-sided nasal mass without cribriform plate involvement (Kadish B). Read More

Acta Otolaryngol 2018 Aug 22;138(8):750-758. Epub 2018 Feb 22.

a Miyakojima IGRT Clinic , Miyakojimahondori, Miyakojima-ku , Osaka , Japan.

Purpose: To analyze the outcomes following re-irradiation for local recurrence of rare head and neck tumors.

Material And Methods: We retrospectively analyzed 11 patients who had received intensity-modulated radiation therapy (IMRT) for recurrent tumors in the head and neck except for laryngopharynx.

Results: Primary tumor sites included the maxillary sinus, nasal cavity, and external ear canal in six, three, and two patients, respectively. Read More

Indian J Otolaryngol Head Neck Surg 2018 Mar 21;70(1):159-161. Epub 2017 Jul 21.

Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamilnadu India.

Esthesioneuroblastomas are rare neuroectodermally derived neoplasms occurring in the upper nasal septum or cribriform plate. They have been rarely reported in other sites, when they are called ectopic esthesioneuroblastomas. Due to the scarcity of reports, publications and molecular/genetic studies of these tumours, their diagnosis is likely to be missed when they are ectopic. Read More

Laryngoscope 2018 Oct 10;128(10):2226-2233. Epub 2018 Feb 10.

Department of Radiation Oncology, University of California-San Francisco, San Francisco, California, U.S.A.

Objective: To quantify the rate of late intracranial recurrences among esthesioneuroblastoma patients treated with surgical resection and postoperative radiation.

Study Design: Retrospective review.

Methods: All patients receiving definitive-intent therapy for esthesioneuroblastoma between March 1995 and September 2015 were reviewed. Read More

Head Neck Pathol 2018 Mar 9;12(1):22-30. Epub 2018 Feb 9.

Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA.

Sinonasal tumors with neuroendocrine differentiation form a group of rare heterogeneous neoplasms of neuroectodermal and epithelial origin, consisting of olfactory neuroblastomas and neuroendocrine carcinomas. Because the natural history and biological behavior of this group of tumors vary, the morphological diagnosis coupled with grading/staging is important for prognostication, and the approach to treatment and rehabilitation is multidisciplinary. The identification of molecular abnormalities underlying these tumors is critical to the development of specific targeted therapies and the design of clinical trials. Read More

Curr Oncol Rep 2018 02 7;20(1). Epub 2018 Feb 7.

Department of Head and Neck Surgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Pickens academic tower, unit 1445, Houston, TX, 77030, USA.

Purpose Of Review: To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma.

Recent Findings: Multimodality treatment is usually recommended for the majority of ONB cases. Recent advances in surgical approaches include the evolving role of endonasal endoscopic surgical resection and reconstruction. Read More

Pathologe 2018 May;39(3):255-263

Institut für Allgemeine Pathologie und Neuropathologie, Universitätsklinikum Tübingen, Liebermeisterstraße 8, 72076, Tübingen, Deutschland.

Neuroendocrine neoplasms (NENs) are infrequent in sensory organs. There are well-differentiated neuroendocrine neoplasms that should be classified as neuroendocrine tumors, in analogy to their gastrointestinal counterparts, however the nomenclature is inconsistent. The best defined entities are neuroendocrine tumors in the middle ear and ectopic pituitary adenoma in the sphenoid region. Read More