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Annals of Medicine and Surgery (2012) May 2023Esthesioneuroblastoma (ENB) is a rare tumor, arising from the olfactory epithelium. It manifests as an aggressive tumor in the superior aspect of the nasal cavity....
UNLABELLED
Esthesioneuroblastoma (ENB) is a rare tumor, arising from the olfactory epithelium. It manifests as an aggressive tumor in the superior aspect of the nasal cavity. Sinonasal symptoms are the most common. The cervical lymph nodes ensue in nearly 10% of cases and hematogenous metastases are rare. The diagnosis is histological. This tumor is staged using the Kadish et al System. The imaging techniques, using both computed tomography (CT) and MRI provides all the important information required for treatment modality. Today, the standard multimodal treatment combining external craniofacial resection, radiotherapy, and chemotherapy has improved long-term survival.
CASE PRESENTATION
A 27-year-old male patient with no medical history, complained of a headache, a unilateral right nasal obstruction, epistaxis, and anosmia for 2 months. Nasal endoscopy showed a pinkish-gray mass filling the right nasal cavity. An enhanced-contrast CT scan was performed and objectified a mildly enhancing extensive mass of the sphenoid sinus with bone erosion of the left wall of the sinus and intracranial involvement. An intranasal biopsy was performed, resulting in a histopathological diagnosis of olfactory neuroblastoma. Our case was staged as stage C according to the Kadish staging. The tumor was inoperable, the patient had chemotherapy, radiotherapy, and pain management.
CLINICAL DISCUSSION
ENB is an aggressive malignant tumor derived from the specialized olfactory neuroepithelium of the upper nasal cavity. Several published reports confirm ectopic cases of ENB throughout the nasal cavity and the central nervous system. Because sinonasal malignant lesions are rare and difficult to distinguish from their benign counterparts. ENBs appears as a soft, glistening, polypoidal, or nodular mass covered by intact mucosa or as friable masses with ulceration and granulation tissue. A radiological, CT scan through the skull base and paranasal sinuses with intravenous contrast should be performed. ENBs are solid, enhancing nasal cavity masses that may manifest erosion into nearby osseous. MRI provides better discrimination between tumor and secretions and optimal assessment of orbital, intracranial, or brain parenchymal involvement. The biopsy is the next important step in securing a diagnosis. Classic treatment strategies of ENB are based on surgery or radiotherapy as unique modalities or a combination of surgery and radiation therapy. More recently, chemotherapy has been introduced in the therapeutic armamentarium since ENB has proven to be chemosensitive. The elective neck dissection remains controversial. Long-term follow-up is mandatory for patients with ENB.
CONCLUSION
While most ENBs originate in the superior nasal vault and present with typical symptoms of nasal obstruction and epistaxis in the late stages of the disease, uncommon manifestations should be considered as well. Adjuvant therapy should be considered in patients with advanced disease and unresectable disease. A continuing follow-up period is needed.
PubMed: 37228980
DOI: 10.1097/MS9.0000000000000532 -
Head and Neck Pathology Jun 2023Although the definitions of sinonasal neuroendocrine and neuroectodermal neoplasms did not change substantially in the 5th edition WHO Classification of Head and Neck... (Review)
Review
Proceedings of the 2023 North American Society of Head and Neck Pathology Companion Meeting, New Orleans, LA, March 12, 2023: Navigating New Developments in High Grade Sinonasal Neuroendocrine and Neuroectodermal Neoplasms.
Although the definitions of sinonasal neuroendocrine and neuroectodermal neoplasms did not change substantially in the 5th edition WHO Classification of Head and Neck Tumours, the diagnosis of olfactory neuroblastoma (ONB), small cell neuroendocrine carcinoma, and large cell neuroendocrine carcinoma remains quite challenging in practice. Ambiguities surrounding the amount of keratin expression allowable in ONB and the amount of neuroendocrine differentiation seen in sinonasal undifferentiated carcinoma (SNUC) lead to significant diagnostic discrepancies at the high grade end of this tumor spectrum. Furthermore, a group of problematic neuroepithelial tumors that show overlapping features of ONB and neuroendocrine carcinoma have never been recognized in formal classification schemes. Since publication of the 5th edition WHO, two new tumor entities have been proposed that help resolve these problems. Olfactory carcinoma is defined by high grade keratin-positive neuroectodermal cells with frequent intermixed glands and shows recurrent Wnt pathway, ARID1A, and RUNX1 alterations. IDH2-mutant sinonasal carcinoma is a molecularly-defined category that encompasses tumors with undifferentiated (SNUC), large cell neuroendocrine, and neuroepithelial phenotypes. This review will provide a practical overview of these emerging entities and their application to diagnostic challenges in the post-WHO sinonasal neuroendocrine and neuroectodermal tumor classification.
Topics: Humans; New Orleans; Carcinoma, Neuroendocrine; Paranasal Sinus Neoplasms; Maxillary Sinus Neoplasms; Esthesioneuroblastoma, Olfactory; Keratins; Nose Neoplasms; Nasal Cavity
PubMed: 37184733
DOI: 10.1007/s12105-023-01548-8 -
BMC Endocrine Disorders May 2023Giant prolactinoma (> 4 cm in dimension) is a rare disorder. Invasive macroprolactinoma has the potential to cause base of skull erosion and extend into the nasal... (Review)
Review
BACKGROUND
Giant prolactinoma (> 4 cm in dimension) is a rare disorder. Invasive macroprolactinoma has the potential to cause base of skull erosion and extend into the nasal cavity or even the sphenoid sinus. Nasal bleeding caused by intranasal tumor extension is a rare complication associated with invasive giant prolactinoma. We report a case of giant invasive macroprolactinoma with repeated nasal bleeding as the initial symptom.
CASE PRESENTATION
A 24-year-old man with an invasive giant prolactinoma in the nasal cavity and sellar region who presented with nasal bleeding as the initial symptom, misdiagnosed as olfactory neuroblastoma. However, markedly elevated serum prolactin levels (4700 ng/mL), and a 7.8-cm invasive sellar mass confirmed the diagnosis of invasive giant prolactinoma. He was treated with oral bromocriptine. Serum prolactin was reduced to near normal after 6 months of treatment. Follow-up magnetic resonance imaging showed that the sellar lesion had disappeared completely and the skull base lesions were reduced.
CONCLUSION
This case is notable in demonstrating the aggressive nature of untreated invasive giant prolactinomas which can cause a diagnostic difficulty with potential serious consequences. Early detection of hormonal levels can avoid unnecessary nasal biopsy. Early identification of pituitary adenoma with nasal bleeding as the first symptom is particularly important.
Topics: Male; Humans; Young Adult; Adult; Prolactinoma; Epistaxis; Prolactin; Pituitary Neoplasms; Bromocriptine; Magnetic Resonance Imaging
PubMed: 37173679
DOI: 10.1186/s12902-023-01345-y -
Medicina (Kaunas, Lithuania) Apr 2023Developing in a limited space, rare tumors located at the nose and paranasal sinuses are sometimes difficult to diagnose due to their modest clinical presentation, which...
Developing in a limited space, rare tumors located at the nose and paranasal sinuses are sometimes difficult to diagnose due to their modest clinical presentation, which is uncorrelated with anatomopathological diversity. This limits the preoperative diagnosis without added immune histochemical study; for that reason, we present our experience with these tumors with the intention of raising awareness. The patient included in our study was investigated by our department through clinical and endoscopic examination, imaging investigations, and an anatomic-pathological study. The selected patient gave consent for participation and inclusion in this research study in compliance with the 1964 Declaration of Helsinki.
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Paranasal Sinuses; Nasal Cavity; Hematology
PubMed: 37109689
DOI: 10.3390/medicina59040731 -
Indian Journal of Pathology &... 2023Herein 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... (Review)
Review
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.
Topics: Male; Humans; Middle Aged; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Tomography, X-Ray Computed; Nasal Cavity
PubMed: 37077088
DOI: 10.4103/ijpm.ijpm_209_21 -
Surgical Neurology International 2023Esthesioneuroblastomas (ENBs) are rare malignancies of the upper digestive tract, often demonstrating local metastasis to the intracranial space through the cribriform...
BACKGROUND
Esthesioneuroblastomas (ENBs) are rare malignancies of the upper digestive tract, often demonstrating local metastasis to the intracranial space through the cribriform plate. These tumors show high rates of recurrence locally following treatment. Here, we report a patient with advanced recurrent ENB 2 years following initial treatment, affecting both the spine and intracranial space without evidence of local recurrence or contiguous extension from the initial tumor site.
CASE DESCRIPTION
A 32-year-old male presents with a 2 month history of neurological symptoms 2 years following treatment of Kadish C/AJCC stage IVB (T4a, N3, M0) ENB. No evidence of locoregional recurrent disease was observed prior with intermittent imaging. Imaging revealed a large ventral epidural tumor invading multiple levels of the thoracic spine as well as a ring enhancing lesion in the right parietal lobe. The patient was treated surgically with debridement, decompression, and posterior stabilization of the thoracic spine followed by radiotherapy to the spinal and parietal lesions. Chemotherapy was also initiated. Despite treatment, the patient passed away 6 months after surgery.
CONCLUSION
We report a case of delayed recurrent ENB with widespread metastases to the central nervous system without evidence of local disease or contiguous extension from initial tumor site. This represents a highly aggressive form of this tumor as recurrences are primarily locoregional. In follow-up of ENB treatment, clinicians must be cognizant of these tumors demonstrated ability to spread to distal regions. All new onset neurological symptoms should be investigated fully even if no local recurrence is observed.
PubMed: 37025527
DOI: 10.25259/SNI_1137_2022 -
Head and Neck Pathology Mar 2023Nested is defined as "cellular clusters arranged in small groupings with intervening vascular or stromal networks, lacking lumens or glandular formation." Using this... (Review)
Review
BACKGROUND
Nested is defined as "cellular clusters arranged in small groupings with intervening vascular or stromal networks, lacking lumens or glandular formation." Using this definition, multiple neoplastic and non-neoplastic lesions of the head and neck come into the differential. We have broadly organized the differential diagnosis of "nested" tumors into entities with neuroendocrine differentiation, squamous differentiation, thyroid follicular cell differentiation, and other lesions.
METHODS
Review.
RESULTS
Many different entities have a nested appearance and the morphologic, immunohistochemical, clinical, and radiographic features contribute to the differential diagnosis. The different tumors covered in this review include neuroendocrine neoplasms, paraganglioma, middle ear neuroendocrine tumor (formerly known as middle ear adenoma), medullary thyroid carcinoma, poorly differentiated thyroid carcinoma, olfactory neuroblastoma, ectopic pituitary neuroendocrine tumor, hyalinizing trabecular tumor, solid subtype of papillary thyroid carcinoma, solid cell nests/C-cell hyperplasia, necrotizing sialometaplasia, and meningioma.
CONCLUSION
In this review, we discuss the morphologic and immunohistochemical features of the covered entities as a guide to differential diagnosis when nested-patterned head and neck lesions are encountered.
Topics: Humans; Thyroid Neoplasms; Carcinoma, Neuroendocrine; Neuroendocrine Tumors; Neck; Pituitary Neoplasms; Ear Neoplasms; Diagnosis, Differential
PubMed: 36928740
DOI: 10.1007/s12105-023-01534-0 -
Journal of Clinical Medicine Mar 2023Non-squamous cell carcinoma-related malignant sinonasal tract tumors (non-SCC MSTT) are rare and diverse malignancies. In this study, we report our experience in the...
Results of Primary Treatment and Salvage Treatment in the Management of Patients with Non-Squamous Cell Malignant Tumors of the Sinonasal Region: Single Institution Experience.
Non-squamous cell carcinoma-related malignant sinonasal tract tumors (non-SCC MSTT) are rare and diverse malignancies. In this study, we report our experience in the management of this group of patients. The treatment outcome has been presented, involving both primary treatment and salvage approaches. Data from 61 patients treated radically due to non-SCC MSTT between 2000 and 2016 at the National Cancer Research Institute, Gliwice branch, were analyzed. The group consisted of the following pathological subtypes of MSTT: adenoid cystic carcinoma (ACC), undifferentiated sinonasal carcinoma (USC), sarcoma, olfactory neuroblastoma (ONB), adenocarcinoma, small cell neuroendocrine carcinoma (SNC), mucoepidermic carcinoma (MEC), and acinic cell carcinoma, which were found in nineteen (31%), seventeen (28%), seven (11.5%), seven (11.5%), five (8%), three (5%), two (3%) and one (2%) of patients, respectively. There were 28 (46%) males and 33 (54%) females at the median age of 51 years. Maxilla was the primary tumor localization followed by the nasal cavity and ethmoid sinus in thirty-one (51%), twenty (32.5%), and seven (11.5%) patients, respectively. In 46 (74%) patients, an advanced tumor stage (T3 or T4) was diagnosed. Primary nodal involvement (N) was found in three (5%) cases, and all patients underwent radical treatment. The combined treatment consisted of surgery and radiotherapy (RT) and was given to 52 (85%) patients. The probabilities of overall survival (OS), locoregional control (LRC), metastases-free survival (MFS), and disease-free survival (DFS) were assessed in pathological subtypes and grouped together, along with the ratio and effectiveness of salvage. Locoregional treatment failure was seen in 21 (34%) patients. Salvage treatment was performed in fifteen (71%) patients and was effective in nine (60%) cases. There was a significant difference in OS between patients who underwent salvage and those who did not (median: 40 months vs. 7 months, = 0.01). In the group of patients who underwent salvage, OS was significantly longer when the procedure was effective (median: 80.5 months) than if it failed (median: 20.5 months), < 0.0001. OS in patients after effective salvage was the same as in patients who were primary cured (median: 80.5 months vs. 88 months, = 0.8). Distant metastases developed in ten (16%) patients. Five and ten year LRC, MFS, DFS, and OS were 69%, 83%, 60%, 70%, and 58%, 83%, 47%, 49%, respectively. The best treatment results were observed for patients with adenocarcinoma and sarcoma, while USC gave the poorest results in our set of patients. In this study, we indicate that salvage is possible in most patients with non-SCC MSTT with locoregional failure and that it may significantly prolong their overall survival.
PubMed: 36902738
DOI: 10.3390/jcm12051953 -
Cancers Feb 2023Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported...
INTRODUCTION
Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported 5-year overall survival (OS) of 82%, recurrence is frequent and occurs in 40-50% of cases. This study investigates the characteristics of ENB recurrence and the subsequent prognosis of patients with recurrence.
METHODS
The clinical records of all patients diagnosed as having ENB with subsequent recurrence at a tertiary hospital from 1 January 1960 to 1 January 2020 were retrospectively reviewed. Overall survival (OS) and progression-free survival (PFS) were reported.
RESULTS
A total of 64 out of 143 ENB patients had recurrences. In total, 45 out of 64 recurrences met the inclusion criteria and were included in this study. From these, 10 (22%) had a sinonasal recurrence, 14 (31%) had an intracranial recurrence, 15 (33%) had a regional recurrence, and 6 (13%) had a distal recurrence. The average interval from initial treatment to recurrence was 4.74 years. There were no differences in rates of recurrence with respect to age, sex, or types of surgery (endoscopic, transcranial, lateral rhinotomy, and combined). The time to recurrence was shorter for Hyams grades 3 and 4 compared to Hyams grades 1 and 2 (3.75 years vs. 5.70 years, < 0.05). Patients with recurrence limited to the sinonasal region had a lower overall primary Kadish stage compared to recurrences beyond the sinonasal region (2.60 vs. 3.03, < 0.05). A total of 9 (20%) out of 45 patients developed secondary recurrence. Following recurrence, the subsequent 5-year OS and PFS were 63 and 56%, respectively. The mean time to secondary recurrence after treatment of the primary recurrence was 32 months, which was significantly shorter than the time to primary recurrence (32 months vs. 57 months, = 0.048). The mean age of the secondary recurrence group is significantly older than the primary recurrence group (59.78 years vs. 50.31 years, = 0.02). No statistically significant differences were observed between the secondary recurrence group and the recurrence group in terms of their overall Kadish stages or Hyams grades.
CONCLUSIONS
Following an ENB recurrence, salvage therapy appears to be an effective therapeutic option with a subsequent 5-year OS of 63%. However, subsequent recurrences are not infrequent and may require additional therapy.
PubMed: 36900297
DOI: 10.3390/cancers15051506