Diagnosis Of Esthesioneuroblastoma Research Articles

Esthesioneuroblastoma: Experience at the national institute of neurology and neurosurgery

IntroductionEsthesioneuroblastoma or olfactory neuroblastoma (ENB) is a neuroectodermic malignant tumor derived from the olfactory epithelium of the nasal cavity and paranasal sinuses mucosa. It can extend to the superior third of the nasal septum, anterior floor, and intracranial compartment. This article presents the institutional experience and the prognosis of patients with the diagnosis of esthesioneuroblastoma. Patient and MethodsA retrospective study that includes 24 patients with esthesioneuroblastoma. Treatment was based on surgery with later radiotherapy and/or chemotherapy in a period comprehended between October 2009 and September 2019 at our institute. Overall survival (OS) was compared with Kaplan-Meier plots and Log Rank test depending on the treatment, histologic grade, and staging. Results23 patients were included. Just one was excluded because of incomplete records. Mean age was 51.1 ± 14.3, 11 men (47.8%), 12 were female (52.2%). The most common initial symptom was a nasal obstruction in 8 patients, followed by epistaxis and rhinorrhea in 5 patients (21.7%). Ten patients had low-grade esthesioneuroblastoma (LGENB) (43.4%) and 13 high-grade esthesioneuroblastoma (HGENB) (56.5%). The mean accumulated OS was of 86.4 months. ConclusionENB is an infrequent malignant pathology with highly nonspecific symptomatology that delays diagnosis, usually achieved with advanced neuroimaging. Treatment is still controversial. Following international publications, total gross resection is beneficial. It is necessary to perform prospective analyses to establish the best standard of care for this entity.

Esthesioneuroblastoma: A Patterns-of-Care and Outcomes Analysis of the National Cancer Database.

The available literature to guide treatment decision making in esthesioneuroblastoma (ENB) is limited. To define treatment patterns and outcomes in ENB according to treatment modality using a large national cancer registry. 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. Log-rank statistics were used to compare overall survival by primary treatment modality. Logistic regression modeling was used to identify predictors of receipt of postoperative radiotherapy (PORT). Cox proportional hazards modeling was used to determine the survival benefit of PORT. Subgroup analyses identified subgroups that derived the greatest benefit of PORT. Primary surgery was the most common treatment modality (90%) and resulted in superior survival compared to radiation (P < .01) or chemotherapy (P < .01). On multivariate analysis, PORT was associated with decreased risk of death (hazard ratio [HR] 0.53, P < .01). PORT showed a survival benefit in Kadish stage C (HR 0.42, P < .01) and D (HR 0.09, P = .01), but not Kadish A (HR 1.17, P = .74) and B (HR 1.37, P = .80). Patients who received chemotherapy derived greater benefit from PORT (HR 0.22, P < .01) compared with those who did not (HR 0.68, P = .13). Predictors of PORT included stage, grade, extent of resection, and chemotherapy use. Best outcomes were obtained in patients undergoing primary surgery. The benefit of PORT was driven by patients with stages C and D disease, and by those also receiving chemotherapy.

Title-An Extrapolation to Small Round Blue Cell Tumor Diagnosed as Esthesioneuroblastoma –Acase Series

Small round blue cell tumor is a very common diagnostic finding after intraoperative diagnostic procedures such as squash cytology or frozen section. After proper biopsy techniques, histopathological diagnosis and immunohistochemistry confirmation, diagnosis of Esthesioneuroblastoma is established. Due to its aggressiveness and high recurrence rate, it is mandatory to diagnose this tumor as soon as possible. In our three year study period, only 3 cases of Esthesioneuroblastoma were reported in which extensive Immunohistochemistry panel played an important role to differentiate it from others and come to a conclusive diagnosis. These cases were first diagnosed through squash cytology as a small round blue cell and later confirmed as Esthesioneuroblastoma by histopathology and immunohistochemistry with synaptophysin, chromogranin, CD 56, CD 99. MIB-1 played an important role to grade the tumor according to Hyam’s histological grading.

Esthesioneuroblastoma Masquerading as Chronic Rhinosinusitis

Background: Esthesioneuroblastoma, a rare tumour of the nasal vault, can present with symptoms that can be mistaken for common conditions such as chronic rhinosinusitis or recurrent epistaxis. Case presentation: A 52-year-old, previously healthy, Caucasian female presented to the emergency room with two months of nasal congestion and a spontaneous, protracted nosebleed that required a nasal clamp to settle. In the following months she had another episode of epistaxis in addition to fatigue, headaches, anosmia, difficulty breathing, worsening vision, weight loss and behavioral changes. Subsequent investigations led to the diagnosis of esthesioneuroblastoma. Conclusion: When patients present with recurrent epistaxis or chronic rhinosinusitis that does not respond to a reasonable course of medical therapy and/or have atypical associated symptoms, further investigations should be considered. The presences of red flags, such as weight loss, severe headaches, vision changes or bizarre behavior are also clues that alternate diagnoses and further additional workup should be considered.

Ectopic olfactory neuroblastoma: case study and literature review

Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a slow-growing malignant tumor of that originates mainly in the neuroectodermal cells in the nasal cavity. The peak incidence of ENB is at age 53, with the majority of cases occurring between 40 and 70 years, and mostly among men. There are a few reports of ENB in the ethmoid, maxillary, and sphenoid sinuses, or in the pituitary and nasopharynx. This tumor usually presents as an invasive disease with a tendency for later recurrence. The most frequent dissemination of ENB is to the cervical lymph nodes, which represents a worse prognosis. Its symptoms are nonspecific and nasal obstruction is the most common complaint. Imaging tests are important for the diagnosis of ENB, along with histopathological and immunohistochemical tests to help in the differential diagnosis. Surgical treatment of craniofacial resection associated with radiotherapy can achieve the best results for survival. We present a discussion of an illustrative clinical case of the disease in a male patient aged eight months, an unusual age for ENB, brought to atten

The Added Value of 18F-FDG PET/CT for Evaluation of Patients with Esthesioneuroblastoma

The purpose of this study was to evaluate the clinical utility of (18)F-FDG PET/CT in esthesioneuroblastoma staging and restaging and quantify the additional benefit of PET/CT to conventional imaging. A retrospective review was performed with institutional review board approval for patients with a diagnosis of esthesioneuroblastoma who underwent PET/CT from 2000 to 2010. PET/CT results were retrospectively reviewed by 2 radiologists who were unaware of the clinical and imaging data. Positive imaging findings were classified into 3 categories: local disease, cervical nodal spread, and distant metastasis. All conventional imaging performed in the 6 mo preceding PET/CT, and the medical records, were reviewed to determine the potential added value. Twenty-eight patients (mean age, 52.3 ± 10 y; range, 23-81 y) were identified who underwent a total of 77 PET/CT examinations. Maximum standardized uptake value (SUVmax) was 8.68 ± 4.75 (range, 3.6-23.3) for the primary tumor and 8.57 ± 6.46 (range, 1.9-27.2) for the metastatic site. There was no clear association between primary tumor SUVmax and tumor grade (P = 0.30). Compared with conventional imaging, PET/CT changed disease stage or altered clinical management in 11 (39%) of 28 esthesioneuroblastoma patients. Of these, 10 (36%) of 28 were upstaged on the basis of their PET/CT studies. Cervical nodal metastases were found in 5 (18%) of 28, local recurrence in 2 (7%) of 28, cervical nodal and distant metastases in 2 (7%) of 28, and distant metastases in 1 (4%) of 28. One patient (4%) was downstaged after negative findings on PET/CT. PET/CT is a useful adjunct to conventional imaging in the initial staging and restaging of esthesioneuroblastoma by detecting nodal and distant metastatic disease not demonstrated by conventional imaging and identifying local recurrence hidden by treatment changes on conventional imaging.

Esthesioneuroblastoma Presenting with Complete External Ophthalmoplegia

An 85-year-old male presented with unilateral third, fourth, and sixth cranial nerve palsies. Neuroimaging revealed a mass involving the medial wall of the left cavernous sinus, without orbital involvement. Biopsy of the mass provided a pathologic diagnosis of esthesioneuroblastoma.

Esthesioneuroblastoma: A case report and review of the literature

Esthesioneuroblastoma (ENB) is an extremely rare malignant neoplasm of the nasal cavity. We report the case of a middle aged man who presented with a 6-months history of progressive nasal obstruction, watering of the eye, headache and occasional epistaxis. A diagnosis of esthesioneuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging the mass was classified as a kadish stage C tumor. The mass was excised via a craniofacial resection through a bi-coronal approach and the tumor mass was peeled away completely from the frontal lobe dura. The patient underwent postoperative radiation, and he was free of recurrence at follow-up 3 months later.

Langzeit-Erfahrungen in der Therapie des Ästhesioneuroblastoms

Esthesioneuroblastoma is a rare and clinically variable tumor of nasal sinus and skull base and challenging for modern multidisciplinary therapy. There are no generally known prognostic factors or generally accepted standard therapy regimens. Between 1975 and 2001 26 patients were treated after the diagnosis of esthesioneuroblastoma was established. The cases were evaluated retrospectively. According to the classification of Kadish 1 patient (4 %) had stage A, 16 patients (53 %) stage B and 11 cases (43 %) had stage C tumors. Hyams grading could be obtained in 22 cases (81 %). Tumors were in 12 patients (52 %) graded I or II and in 10 cases tumors had grade III or IV (48 %). Operative therapy was performed in 23 patients (88.5 %), being in 5 patients a single mode therapy. In 18 cases combined therapy was performed (surgery and radiotherapy or radiotherapy and chemotherapy). Actuarial survival was 61.5 % (16/26). Disease specific 10- and 15-year-survival according to the estimation of Kaplan-Meier was 76.2 %. Patients with small tumors (Kadish A/B) had a 15-year-survival of 86.7 % compared to 63.6 % in cases with advanced tumors (Kadish C). In 7 cases (26.9 %) recurrences developed. Salvage therapy was performed in 5 cases (71.4 %) with a success rate and a 15-year survival each of 60 %. Therapy of esthesioneuroblastoma is challenging because of rarity and biologic variability of the tumor and and because of lack of a standard therapy. An interdisciplinary multimodal therapeutic approach is necessary especially in case of advanced tumors with promising results. Histopathological grading according to Hyams and tumor stage are important factors for survival and prognosis. Although recurrence can occur with high frequency even after prolonged time interval, long time survival can be improved after aggressive salvage therapy. Therefore longterm follow up is mandatory.

Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region.

The sinonasal region is known to harbor several types of tumors that belong to the general category of "small" round cell tumors and offer considerable diagnostic challenges. This study evaluated 33 cases of such tumors by electron microscopy to characterize their ultrastructural features in conjunction with immunohistochemistry, in an attempt to define diagnostic criteria of various types. Electron microscopy was useful in the proper classification of tumors in 27 cases: esthesioneuroblastoma (EN), 12; undifferentiated carcinoma, 6; melanoma, 3; lymphoma, 3; melanotic neuroectodermal tumor, 1; rhabdomyosarcoma, 1; and pituitary adenoma, 1. In the remaining six cases, the ultrastructural features were those of poorly differentiated carcinomas. They usually exhibited some epithelial characteristics as well as neuroendocrine features by immunohistochemistry and electron microscopy. These tumors could be best described as poorly differentiated neuro-endocrine carcinomas (malignant neuroepitheliomas). The most controversial diagnostic problems existed between the tumors categorized as esthesioneuroblastomas and neuroendocrine (NE) carcinomas. Esthesioneuroblastomas were characterized by uniform round nucleated cells with variable amounts of dendritic processes containing numerous dense core granules ranging from 150 to 350 nm in the perikarya and dendritic processes. Dendritic processes contained longitudinally arranged neural tubules and revealed an occasional synaptic junction. In three of the 12 cases of EN, cells with the appearance of sustentacular cells were recognized by electron microscopy. The NE carcinomas usually consisted of closely packed round cells with scanty cytoplasm that lacked any feature of neuroblastic cells. The tumor cells in this category often were epithelioid in appearance and exhibited a varying degree of cytokeratin positivity. Neuron-specific enolase was also positive in all cases, further suggesting their neuroepithelial nature. The greatest difference between EN and NE carcinomas was the absence of sustentacular cells in NE carcinomas. Immunohistochemical and electron microscopic studies are essential in the differential diagnosis of EN and NE carcinomas, because their microscopic appearance is very similar. The study indicates that EM is useful in the diagnostic categorization of sinonasal tumors of uncertain nature, particularly when it is used in conjunction with immunohistochemistry.

Esthesioneuroblastoma. Intermediate filaments, neuroendocrine, and tissue-specific antigens.

Esthesioneuroblastoma (EN), a malignant neuroblastic tumor arising in the superior portion of the nasal cavity, shares histologic similarities with a number of primary malignant tumors that arise in this region, including rhabdomyosarcoma, lymphoepithelioma, and lymphoma. To establish an antigenic profile of EN as an aid in the differential diagnosis of these histologically similar nasal tumors, immunostaining was performed for the following intermediate filaments: keratin, neurofilament, glial fibrillary acidic protein, and desmin; neuron-specific enolase (NSE), S-100 protein, chromogranin, human common leukocyte antigen (HLE), epithelial membrane antigen (EMA), myoglobin, and carcinoembryonic antigen (CEA) on 21 primary nasal tumors: eight EN, five lymphoepitheliomas, two small cell carcinomas, three lymphomas, and three rhabdomyosarcomas. Keratin and CEA stained only the carcinomas (6/7+, 4/7+), respectively; desmin and myoglobin only rhabdomyosarcoma (3/3+, 1/3+); and HLE only lymphomas (3/3+). Chromogranin and neurofilament staining occurred exclusively in one case each of EN. S-100 and NSE commonly stained EN (5/8+, 6/8+), but carcinomas (1/7+, 2/7+) and rhabdomyosarcomas (1/3+, 3/3+) were also positive. Despite the apparent nonspecificity of NSE and S-100, an antigenic profile of positive NSE of S-100 staining with negative epithelial, muscle, and lymphoid antigens uniquely identified six of eight EN. Chromogranin and neurofilament positivity was further evidence for EN in two cases. This antigenic profile is a helpful adjunct in the diagnosis of EN and other primary malignant nasal tumors.