Cauda equina paraganglioma with subsequent intracranial and intraspinal metastases

Summary A case of cauda equina paraganglioma is described; subsequent intracranial and intraspinal metastases occurred after partial resection and adjunctive radiotherapy. Cerebrospinal fluid dissemination is a rare complication of spinal paragangliomas. Factors predictive of this unusual biological behaviour are discussed.     

The value of magnetic resonance imaging in the differential diagnosis of parapharyngeal space tumours

Between 1987 and 1993 14 patients with a parapharyngeal space tumour were imaged by magnetic resonance imaging (MRI). The vagal body tumours, presenting in the poststyloid compartment, all showed flow voids with anterior and medial displacement of the internal carotid artery. None of the salivary gland tumours, all presenting in the prestyloid compartment with posterior displacement of the internal carotid artery, showed flow voids. MRI is superior compared with other modalities in evaluating the differential diagnosis, especially regarding vascular vs non-vascular tumours. It should encompass T₁ SE images to assess the presence or absence of flow voids. In vascular tumours angiography must be used to assess feeding vessels, multiplicity, and sides involved. T₁ GE images are useful as they allow superior identification of the internal carotid artery and its relation with the tumour accordingly. In addition to T₁ SE images, T₂ SE images may help in the evaluation of the differential diagnosis. In all non-vascular tumours aspiration cytology is required to differentiate between benign and malignant disease.     

Immunocytochemical characterization of neuroendocrine tumours of the larynx

Twenty-two neuroendocrine tumours of the larynx were investigated using a panel of immunocytochemical markers. Three were small cell carcinomas, eight were large cell neuroendocrine carcinomas and 11 were paragangliomas. Twenty were positive for protein gene product 9.5, 19 for neuron-specific enolase, 15 for chromogranin A, nine for bombesin, eight for substance P, eight for neuropeptide Y, eight for metenkephalin, seven for somatostatin, five for calcitonin, eight for calcitonin gene-related peptide and one for vasoactive intestinal polypeptide. Bombesin immunoreactivity was largely restricted to the small cell carcinomas and large cell neuroendocrine carcinomas and neuropeptide Y, metenkephalin and substance P to the parangangliomas. This comprehensive immunocytochemical analysis of neuroendocrine tumours of the larynx demonstrates that these tumours represent special entities but have similar patterns of immunostaining to those of neuroendocrine tumours in other sites.     

Combined paraganglioma and glioma of conus medullaris and cauda equina

A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.     

A case of spinal paraganglioma

Summary The neuroradiologic finding of a paraganglioma in the vertebral canal is described here for the first time. These tumors occur more frequently than was previously presumed. Similarities with paragangliomas in the craniocervical region are apparent on angiograms. We consider angiography to be indicated when, in association with a spinal tumor, urinary biogen amine levels are elevated. When necessary, embolization can be performed after angiography.This study is dedicated to Professor Dr. J. Wappenschmidt on the occasion of his 60th birthday     

Surgical management of carotid body tumours: a 24-year surgical experience

BACKGROUND: Carotid body tumours (CBT) are rare tumours, best treated by complete surgical resection. However, there is no uniform agreement on the method of resection. The aim of this study was to review our 24 years' experience of meticulous subadventitial excision of CBT. METHODS: A retrospective study, from March 1980 to September 2004 of patients with CBT was undertaken, detailing presentation, diagnosis and treatment and postoperative complications. RESULTS: Twenty-five patients (six men and 19 women) with an age range of 23-72 years had been operated on, and all were treated by surgical excision. All patients had neck mass. No patient had a positive family history. Angiography was the main method of diagnosis. All of the patients had unilateral tumours. There was no postoperative permanent neurological deficit. Temporary neurological problems developed in only four patients. External carotid artery was ligated in three patients to facilitate excision of the tumour. Surgical care limited blood loss to an average of 480 mL. CONCLUSIONS: Subadventitial excision, carried out meticulously, allowed complete resection to be achieved in all of the patients with minimal morbidity and no surgical mortalities. This method is therefore recommended. Facilities for shunting and arterial repair should always be available.     

Early and long-term results of the treatment of jugular paragangliomas using different ranges of surgical approach

Background and purposeThe applied approach to the jugular foramen is a combination of the juxtacondylar approach with the subtemporal fossa approach type A. The purpose of this study is to present our results of treatment of jugular paragangliomas using the aforementioned approach.Material and methodsTwenty-one patients (15 women, 6 men) with jugular paragangliomas were included in the study. The neurological status of the patients was assessed before and after surgery as well as at the conclusion of treatment. The approximate volume of the tumour, its relation to large blood vessels, cranial nerves and brainstem, as well as consistency and vascularity were also assessed.ResultsThe duration of symptoms ranged from 3 to 74 months. In 86% of patients hearing loss was the predominant symptom. The less frequent symptoms included pulsatile tinnitus in the head, dysphagia and dizziness. Approximate volume of the tumours ranged from 2 to 109 cm³. A gross total resection was achieved in 71.5% of patients. The postoperative performance status improved in 38% of patients, did not change in 38% and deteriorated in 24% of patients.ConclusionsA proper selection of the range of the approach to jugular foramen paragangliomas based on their topography and volume reduces perioperative injury without negative consequences for the radicality of the resection.     

Higher risk of phaeochromocytoma/paraganglioma (Phaeo‐Pgl) in SDHD than SDHB carriers: an Australian cohort study

Carriers of succinate dehydrogenase (SDHx) mutations are at risk of developing phaeochromocytomas, catecholamine secreting extra‐adrenal paragangliomas and non‐secretory head and neck paragangliomas and require lifelong surveillance. There is no current consensus on the optimal surveillance strategy. This study describes the outcomes of a cohort of 50 SDHx mutation carriers followed at a tertiary Australian hospital using a surveillance protocol involving annual clinical review with plasma/urine metanephrines and biennial magnetic resonance imaging from skull base to pelvis.     

恶性副神经节瘤的诊治探讨

目的：提高恶性副神经节瘤（MPGL）的诊治水平。方法：回顾性分析2003年4月～2011年1月诊治的12例MPGL患者的临床资料。高血压10例（83．3％）,腰腹部疼痛3例（25．0％）。血浆游离变肾上腺素类物质（MNs）和24h尿儿茶酚胺（CA）升高者分别为100．0％（12／12）和83．3％（10／12）。B超、CT、MRI和131I-间碘苄胍扫描（131I-MIBG）定位准确率分别为83．3％（10／12）、91．7％（11／12）、80．0％（4／5）和88．9％（8／9）。1例行放射治疗,11例行开放手术治疗。结果：原发性肿瘤9例,复发性肿瘤3例;单发性肿瘤8例,多发性肿瘤4例。根据肿瘤的中心定位：腹主动脉旁6例,下腔静脉后2例,膀胱2例,肾门2例。根据术中所见确诊恶性5例（41．7％）。病理检查11例,确诊恶性8例（72．7％）。肺转移1例,肝转移1例,骨转移1例,局部淋巴结转移3例．广泛淋巴结转移2例,2个或多个脏器同时发生转移2例。9例随访4～84个月,平均39个月;复发4例,再次手术;因多发浸润及远处转移死亡2例。结论：病理难以区分副神经节瘤的良恶性,需结合影像学检查、生化检查及术中所见。根治性肿瘤切除是治疗MPGL最有效的方法,肿瘤复发时再次手术仍然有效;无法手术者可用放化疗控制高血压及延缓疾病进展。长期随访观察肿瘤的转移情况是确诊疑似病例的重要方法。