Audiological, vestibular and radiological abnormalities in Kallman's syndrome.

Kallman's syndrome is a multifaceted congenital disorder with predominantly endocrine abnormalities. We have characterized the associated mixed hearing loss and identified consistent radiological evidence of abnormal temporal bone anatomy. Abnormal labyrinthine morphology is accompanied by a complete absence of response to vestibular stimulation with caloric or rotational chair testing. The endocrine abnormalities are correctable and Kallman's syndrome is a diagnosis worthy of consideration when assessing children with congenital hearing loss.     

Retropharyngeal lymph node metastasis from olfactory neuroblastoma: a report of two cases

Olfactory neuroblastoma is a rare, malignant neoplasm arising from the olfactory epithelium. It has an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis. The incidence of cervical lymph node metastasis in olfactory neuroblastoma is variable, and treatment modalities are controversial. Moreover, few reports have been published concerning retropharyngeal lymph node metastasis from olfactory neuroblastoma. We present two cases of olfactory neuroblastoma with retropharyngeal lymph node metastasis. In addition, we provided a review of the current literature regarding olfactory neuroblastoma and retropharyngeal lymph node metastasis from olfactory neuroblastoma.     

Ectopic olfactory neuroblastoma: report of four cases and a review of the literature

Our objective is to present a short series of four rare cases of ectopic olfactory neuroblastoma. Our methods present four case reports of ectopic olfactory neuroblastoma and a review of the literature for management and treatment of this disease. The results indicate short case series reports of ectopic olfactory neuroblastoma arising from the anterior ethmoidal sinuses, the nasopharynx, the lateral nasal wall and the floor of the nose. The discussion focuses on likely origins of ectopic olfactory neuroblastoma, its clinical features and management. We conclude that ectopic olfactory neuroblastoma is a rare disease. Treatment principles are the same for non-ectopic disease and guided by extension into adjacent structures such as the orbit or anterior cranial fossa and usually involves surgery with or without adjuvant radiotherapy.     

Olfactory neuroblastoma metastatic to the breast

Olfactory neuroblastoma (Esthesioneuroblastoma) is a rare malignant tumour arising from olfactory epithelium. It has a predilection for cervical lymph node metastasis and also has potential for distant metastasis to unusual sites like scalp, face, aorta, spleen, liver, adrenal gland and ovary. We report here a rare case of olfactory neuroblastoma in an adolescent girl with metastatic deposits in the breast. A poor prognosis due to rapidly progressive disseminated disease was observed. The relevant literature regarding metastatic olfactory neuroblastoma and metastasis in the breast from non-mammary malignant neoplasms is reviewed.     

Primary olfactory neuroblastoma originating from the inferior meatus of the nasal cavity

Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity arising from the olfactory neuroepithelium. It usually presents as a polypoid mass high in the nasal vault including the cribriform plate, superior turbinate, and superior portion of nasal septum. An 89-year-old man visited our office with symptoms of occasional left nasal bleeding and obstruction. On the endoscopic examinations, polypoid mass with bleeding tendency was observed in the left inferior meatus. Biopsy was performed, and the diagnosis of olfactory neuroblastoma was confirmed by histopathologic examination and immunohistochemical staining. The tumor was resected via medial maxillectomy, and the final pathologic report established that it was primary olfactory neuroblastoma that occurred from the inferior meatus. In this report, a literature review was performed on the pathologic characteristics and prognosis of the tumor, and possible hypotheses that olfactory neuroblastoma has originated from the inferior meatus were discussed.     

Hyponatremia secondary to olfactory neuroblastoma

A patient with a four-year history of unexplained hyponatremia was seen with recurrent nasal discharge and was found to have a typical olfactory neuroblastoma. The clinical laboratory diagnostic studies suggested that the patient's sodium deficiency was secondary to the syndrome of inappropriate antidiuretic hormone. Subsequent resection of the neoplasm led to resolution of the hyponatremia, suggesting that a (tumor-associated) humoral factor, such as vasopressin or a vasopressinlike substance, was responsible for the electrolyte disturbance. A search of the literature disclosed one previous case of vasopressin-secreting nasal neuroblastoma.     

Olfactory neuroblastoma: long-term clinical outcome at a single institute between 1979 and 2003

CONCLUSIONS: The progression of olfactory neuroblastoma showed a biphasic pattern. As well as Hyams' histopathological grading and neck metastasis at presentation, early phase recurrence should be regarded as an important prognosticator. A high local failure rate suggests that craniofacial resection followed by postoperative radiotherapy should still be the standard treatment for olfactory neuroblastoma. OBJECTIVE: The aim of this study was to evaluate factors associated with survival and local control of olfactory neuroblastoma in the long run and to estimate treatment strategies. PATIENTS AND METHODS: Twelve patients (seven men and five women) who had undergone initial curative treatment for olfactory neuroblastoma were retrospectively analyzed. RESULTS: Cause-specific 10-year survival was 64.8%, while disease-free 10-year survival remained 28.6%. Local failure was found in half of the patients. All of the three patients who did not receive radiotherapy developed local failure. A biphasic pattern of recurrence was observed. The early phase recurrence group showed a significantly poorer survival than the late phase recurrence group. Hyams' histopathological grading and neck metastasis at presentation were also correlated with survival.     

诊治7例鼻腔嗅神经母细胞瘤的体会

目的：提高临床对鼻腔嗅神经母细胞瘤的认识及治疗效果。方法：７例患者按Ｋａｄｉｓｈ分期，Ｂ期２例，Ｃ期５例，单纯放疗３例，术前放疗４例，放射剂量为５０００￣７０００ｃＧｙ。均随访至１９９７年１２月，结果：存活５例，存活时间分别为２０，２２，２３，２９及７６个月；结论：放疗对该肿瘤有效；放疗与化疗结合的方法值得探讨，该瘤病理上可能存在不同的亚型。     

Expression of somatostatin receptors in arginine vasopressin hormone-secreting olfactory neuroblastoma--report of two cases

OBJECTIVE: Arginine vasopressin hormone-secreting olfactory neuroblastomas are extremely rare, with fewer than twenty cases reported in the literature. Two of these cases, both initially presenting with the syndrome of inappropriate antidiuretic hormone, are presented. The second tumour was successfully identified using somatostatin receptor (octreotide) radiographic scintography. METHOD: The pathological specimens from both cases were examined immunohistochemically for somatostatin receptors. RESULTS: Samples from both cases demonstrated positivity for somatostatin receptors. CONCLUSIONS: This report demonstrates the potential use of somatastatin analogues in the investigation, follow-up and treatment of patients with olfactory neuroblastoma.     

Magnetic resonance imaging of olfactory neuroblastoma

Olfactory neuroblastoma is an uncommon intranasal tumor originating from olfactory neuroepithelium. Despite the development of electron microscopy and immunohistochemical testing, the pathological diagnosis of this tumor is still difficult because of the wide range of histological features. Magnetic resonance imaging (MR) of this tumor and the pattern of contrast enhancement have not been well described. The purpose of this report was to analyze the MR characteristics of olfactory neuroblastomas. The MR signal, pattern of contrast enhancement, and correlation with high-resolution computed tomography (CT) imaging were examined. Seventeen patients with olfactory neuroblastoma were treated at Hokkaido University Hospital and a related hospital during the past 25 years. MR images taken in 12 patients and CT images taken in 9 patients with histologically confirmed olfactory neuroblastoma were retrospectively reviewed. Compared with brain gray matter, 11 tumors were hypointense on T1-weighted images, 9 homogeneously and 2 heterogenously. Eight tumors were hyperintense on T2-weighted images, 3 homogeneously and 5 heterogeneously, although their appearance was less intense than that of sinusitis. Gadolinium enhancement was moderate in one case and marked in 10 of the 11 cases, 9 homogeneously and 2 heterogeneously. Nine of the 11 tumors showed smooth regular shaped margins; 2 of these tumors exhibited irregular infiltrating margins on gadolinium-enhanced images, compared to the pre-contrast T1-weighted images. Eight of the 11 tumors had clearly demarcated margins, while 3 of the 11 tumors did not exhibit gadolinium enhancement. Six of the 12 cases (50%) exhibited intracranial cysts on the gadolinium-enhanced images. T2-weighted or gadolinium-enhanced images successfully distinguished sinusitis from tumors in 4 cases whereas the CT images failed. Gadolinium enhancement, particularly in the tangential plane, demonstrated intracranial extension not apparent on the CT images in one case. In most cases, olfactory neuroblastomas are hypointense on T1-weighted images, hyperintense on T2-weighted images, and show marked homogeneous enhancement with well-demarcated regular margins upon gadolinium enhancement. Although the definite diagnosis is based on histopathology findings and MR features are nonspecific, they may suggest an imaging diagnosis of olfactory neuroblastoma when seen in the superior nasal cavity. MR is superior to CT both in delineating the extent of the tumor and in making an imaging diagnosis.     

Computed tomography and magnetic resonance imaging features of olfactory neuroblastoma: an analysis of 22 cases

The computed tomography (CT) and magnetic resonance imaging (MRI) studies of 22 patients with a histologically proven olfactory neuroblastoma were retrospectively reviewed. The tumours displayed a variety of imaging characteristics and aggressiveness. The expansile tendency of olfactory neuroblastoma is characterised by bowing of the sinus walls. The destructive aspect is manifested as tumour replacing the turbinates, septum, and sinus walls with extension into contiguous areas. The density/signal and enhancement characteristics are non-specific. Olfactory neuroblastoma should be suspected in all ages following identification of a mass in the superior nasal cavity demonstrating both expansile and destructive growth patterns. The otorhinolaryngologist and the radiologist should be aware of this tumour entity, as early diagnosis appropriately guides therapy and predicts survival.     

Computed tomography and magnetic resonance imaging features of olfactory neuroblastoma: an analysis of 22 cases.

The computed tomography (CT) and magnetic resonance imaging (MRI) studies of 22 patients with a histologically proven olfactory neuroblastoma were retrospectively reviewed. The tumours displayed a variety of imaging characteristics and aggressiveness. The expansile tendency of olfactory neuroblastoma is characterised by bowing of the sinus walls. The destructive aspect is manifested as tumour replacing the turbinates, septum, and sinus walls with extension into contiguous areas. The density/signal and enhancement characteristics are non-specific. Olfactory neuroblastoma should be suspected in all ages following identification of a mass in the superior nasal cavity demonstrating both expansile and destructive growth patterns. The otorhinolaryngologist and the radiologist should be aware of this tumour entity, as early diagnosis appropriately guides therapy and predicts survival.     

嗅神经母细胞瘤外科治疗分析

目的总结嗅神经母细胞瘤外科治疗经验。方法回顾性分析2001年1月～2012年3月在我院接受手术治疗的49例嗅神经母细胞瘤病例资料,对预后因素及手术疗效进行研究。结果随访时间6～130个月,中位随访时间44个月。单纯鼻内镜手术31例,鼻内镜辅助手术4例,开放性手术14例。3、5年总体生存率分别为77.8%和69.2%,3、5年无病生存率分别为66.9%和63.2%。颅内受侵和切缘阳性分别为预后不良和复发的独立危险因素。结论鼻内镜手术适用于绝大多数嗅神经母细胞瘤病例,其远期疗效仍需进一步观察。     

Olfactory neuroblastoma: Clinicopathologic and immunohistochemical characterization of four representative cases

Olfactory neuroblastomas are rare tumors whose clinical prognosis is not predictable by assessment of initial stage or grade. The pathologic diagnosis is often difficult because of the wide range of the patients' age and histologies. In this report, we document that the diagnosis of olfactory neuroblastoma can be clarified by immunohistochemical demonstration of a unique antigenic profile that can be obtained in routinely processed biopsies. We describe four cases of olfactory neuroblastoma diagnosed and treated from 1979 to 1989, each confirmed by im-munohistology. One of our patients was misdiagnosed twice at an outside institution, first as having nasopharyngeal carcinoma and then as having small-cell, undifferentiated “oat cell” carcinoma. Despite accurate tumor diagnosis and appropriate therapy, we found that there was no apparent correlation of clinical outcome with Kadish clinical stage or histologic grade of tumor.     

颅内外沟通性嗅神经母细胞瘤8例显微手术治疗分析

目的讨论颅内外沟通性嗅神经母细胞瘤（ONB）的临床特点及显微手术策略。 方法湘雅医院神经外科2013年1月—2019年6月收治的颅内外沟通性ONB患者8例,男5例,女3例,年龄13~65岁;病程7 d至5年,平均12个月。其中手术选择双侧扩大经额底入路5例,联合经鼻内镜3例。全部患者术后行放疗,2例辅助化疗。观察患者的手术策略和临床疗效。结果8例患者颅内外沟通性ONB全切除,术后头痛及鼻腔疼痛全部改善,随访7个月至6年,存活4例,失访2例,2例术后4~10个月肿瘤广泛复发导致死亡。结论双侧扩大经额底入路及联合经鼻内镜显微手术有助于颅内外沟通性ONB的全切除,可提高患者生存率,值得临床推广。     

Infantile olfactory neuroblastoma. A clinicopathological study with review of the literature.

A case of olfactory neuroblastoma occurring in a 3-year-old girl is reported. The rarity of the lesion in early childhood is stressed and discussed with the clinico-pathological characteristics of the tumour, which are apparently more aggressive the younger the patient. Olfactory neuroblastoma should be considered as a possible diagnosis regardless of the age of the patient.     

Olfactory neuroblastoma.

A case of olfactory neuroblastoma, type 1 (olfactory neurocytoma), in a 44-year-old man is described. The tumour grew extensively in the right nasal cavity with involvement of the maxillary sinus and ethmoidal region, but was radically removed at operation. The prognosis and treatment are discussed in the light of earlier literature.     

Olfactory neuroblastoma. Chemotherapy and radiotherapy for extensive disease

A patient with olfactory neuroblastoma had extensive involvement of the nasopharynx, sinuses and orbit, and cervical metastasis. After surgical biopsy and excision of metastatic disease, she was treated with chemotherapy: three cycles of high-dose cisplatin (100 mg/sq m) and infusion of fluorouracil (1 g/sq m/day for five days). Subsequent treatment included definitive radiation therapy followed by three additional cycles of chemotherapy. The patient is free of disease three years after initiating therapy. This case report demonstrates the potential efficacy of planned combined modality therapy, including early chemotherapy in the control of locally advanced, unresectable olfactory neuroblastoma.     

鼻内镜微创手术治疗鼻颅底肿瘤36例临床分析

目的 探讨鼻内镜微创外科技术应用于鼻颅底肿瘤的适应证、疗效及安全性.方法 回顾性分析2000年1月至2004年6月鼻内镜下行鼻颅底肿瘤切除术36例,其中鼻咽血管纤维瘤16例、鼻窦骨化纤维瘤8例、垂体瘤8例、嗅神经母细胞瘤4例.患者术后均行病理检查证实.鼻咽血管纤维瘤患者术前行供血动脉介入栓塞术;嗅神经母细胞瘤患者术后给予放疗;2例较大的垂体瘤患者手术未能完全切除,术后给予放疗.结果 36例患者均行肿瘤全部或者大部分切除.3例患者出现脑脊液鼻漏并发症,其中2例分别采用中鼻甲黏膜和肌肉组织修补成功,另1例仪给予降颅压、抗炎处理后自愈.术后随访4～8年均无复发.结论 在正确选择适应证的前提下,鼻内镜微创手术能够安全有效地处理鼻颅底肿瘤.     

Olfactory neuroblastoma: imaging by magnetic resonance,CT and conventional techniques

Twenty-four patients with the histological diagnosis of olfactory neuroblastoma have been treated at the Royal National Throat, Nose and Ear Hospital since 1975. The tumour showed a bimodal age distribution and 30% of the patients were under 30 years of age. The imaging characteristics on plain film, computed tomography and magnetic resonance combined with intravenous gadolinium DTPA are detailed. None of the changes described is wholly specific. However, a tumour in the ethmoids and upper part of the nasal cavity, which expands into the orbit and erodes the roof of the fronto-ethmoid complex or cribriform plate unilaterally in a young patient, is highly suggestive of olfactory neuroblastoma, particularly if this is combined with the magnetic resonance signal characteristics of a vascular tumour. The typical MR features are those of an intense signal on pre-contrast T2 weighted spin echo sequences and strong enhancement after gadolinium on Tl weighted sequences. A characteristic feature of the response to gadolinium is an enhancement of tumour higher than that of turbinate mucosa on inversion recovery and less than that of mucosa when Tl weighted spin echo sequences are employed. The extent of tumour in the paranasal sinuses and the anterior cranial fossa is best demonstrated after magnetic resonance with intravenous gadolinium and this is now regarded as the most accurate method of preoperative assessment of these patients prior to craniofacial surgery.