Recognition and management of phaeochromocytoma

Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in preoperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before, during and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.     

Large gangliocytic paraganglioma of the duodenum: A rare entity

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.     

Microanatomy and surgical approaches to the infratemporal fossa: an anaglyphic three-dimensional stereoscopic printing study.

Objective: The infratemporal fossa (ITF) is a continuation of the temporal fossa between the internal surface of the zygoma and the external surface of the temporal bone and greater wing of the sphenoid bone that is sitting deep to the ramus of the mandible. The principal structure to understanding its relationships is the lateral pterygoid muscle. Other important structures are the medial pterygoid muscle, the maxillary artery, the pterygoid venous plexus, the otic ganglion, the chorda tympani nerve and the mandibular nerve. In this study, we describe the microsurgical anatomy of the ITF, as viewed by step-by-step anatomical dissection and also through the perspective of three lateral approaches and one anterior surgical approach. Methods: Eight cadaver specimens were dissected. In one side of all specimens, an anatomical dissection was done in which a wide preauricular incision from the neck on the anterior border of the sternoclidomastoid muscle at the level of the cricoid cartilage to the superior temporal line was made. The flap was displaced anteriorly and the structures of the neck were dissected followed by a zygomatic osteotomy and dissection of the ITF structures. On the other side were the surgical approaches to the ITF. The combined infratemporal and posterior fossa approach was done in two specimens, the subtemporal preauricular infratemporal fossa approach in two, the zygomatic approach in two, and the lateral transantral maxillotomy in two. The anatomical dissections were documented on the three-dimensional (3D) anaglyphic method to produce stereoscopic prints. Results: The lateral pterygoid muscle is one of the principal structures to enable understanding of the relationships into the ITF. The tendon of the temporal muscle inserts in the coronoid process at the ITF. The maxillary artery is the terminal branch of the external carotid artery that originates at the neck of the mandible and runs into the parotid gland. In our dissections the maxillary artery was lateral to the buccal, lingual, and inferior alveolar nerves. We found the second part of the maxillary artery superficial to the lateral pterygoid muscle in all specimens The anterior and posterior branches of the deep temporal artery supply the temporal muscle. In two cases we found a middle deep temporal artery. The different approaches that we used provided different views of the same anatomical landmarks and this provides not only safer surgery but also the best choice to approach the ITF according with the pathology extension. Conclusions: The ITF is a complex region on the skull base that is affected by benign and malignant tumors. The study through different routes is helpful to disclose the relationship among the anatomical structures. Although the authors have shown four approaches, there are a variety of approaches and even a combination of these can be used. This type of anatomical knowledge is essential to choosing the best approach to treat lesions in this area.     

颈静脉球瘤的MR诊断

目的探讨MR检查在颈静脉球瘤诊断中的应用价值。方法对15例病变进行回顾性分析,重点分析其部位、信号及其侵袭性表现等情况。结果15例患者中,右侧病变8个(8/15),左侧7个(7/15);肿瘤形态为类圆形或不规则形,12例较大病变不同程度侵犯颈静脉孔区、乳突、岩骨、岩尖、斜坡等处骨质;肿瘤信号欠均匀,12个(12/15)肿块在MR平扫出现“盐和胡椒征”,以“胡椒”表现最为显著;13个肿块(13/13)均有明显强化,其中10个肿块出现“盐和胡椒征”。9例行MRA检查,颈内动脉和颈内静脉不同程度移位和包绕。结论根据肿瘤的部位、“盐和胡椒征”等MR表现,可在手术前正确诊断颈静脉球瘤。     

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of 30 cases

Aims: Extra-adrenal paragliomas are neoplasms which have been the subject of much debate regarding parameters to establish their biological behaviour. This study describes the clinicopathological and immunohistochemical features of 30 cases of spinal paragliomas. Methods and results: There were 15 male and 15 female patients. The median age at diagnosis was 46 years (range 20–74 years). Fourteen patients presented with back pain, two with numbness of the lower extremities, one with difficulty in walking and one with spinal cord compression. Nineteen tumours were located in the lumbar region, six in the cauda equina, two in the filum terminale, two in the thoracic region and one in the cervical region. All patients underwent gross total excision. The size of the tumours ranged from 10 to 50 mm. Histologically, 18 neoplasms showed alveolar (Zellballen) pattern, seven a spindle component, two eosinophilic granular cells suggestive of oncocytic metaplasia, two melanin pigment and one ganglion cells. Positive immunohistochemical results include: neuron-specific enolase 23/23 (100%), synaptophysin 21/23 (91%), S100 protein 22/23 (95%, sustentacular cells), leu-enkephalin 11/23 (47%), somatostatin 8/23 (34%), focal glial fibrillary acidic protein 7/23 (30%), focal keratin 5/23 (21%), neurofilament proteins 3/23 (13%) and adrenocorticotrophic hormine (ACTH) 1/23 (4%). Follow-up information obtained in 20 patients show 17 patients alive over a period of 6–216 months. One patient had bone metastases. Two patients died of unrelated causes, including one of congestive heart failure and one of myocardial infarction. Conclusions: In our experience, spinal paragangliomas behave as slow-growing tumours susceptible to potential cure by total excision. We agree with the current World Health Organization (WHO) classification as grade I tumours. Less than 1% may be locally aggressive. Spinal paragangliomas immunoreact not only for conventional neuroendocrine markers but also for peptides including somatostatin and ACTH and focally for the epithelial marker keratin.     

Glomus jugulare tumours: A review of 61 cases

Summary A retrospective study of 61 patients with glomus jugulare tumours treated at the National Hospital for Neurology and Neurosurgery, and at the Royal National Throat, Nose and Ear Hospital, London. The average age at presentation was 41.7 years. The patients were mainly treated by a posterolateral combined otoneurosurgical approach. 42/61 of the patients had total or subtotal excision of their tumours, 7/61 had partial removal and the remaining 11/61 had no operation. Only one case required a 2-staged procedure. There were two deaths in the postoperative period, one from intracerebral haemorrhage and the other from the left hemisphere infarction.Postoperative radiotherapy was given to 5/7 of the patients who had partial removal. 3/40 of the patients with total removal had postoperative radiotherapy, and a further 3/40 had received radiotherapy pre-operatively.Of the 11 patients who did not undergo surgery, 7/11 were treated with radiotherapy and 4/11 had embolisation only.     

Case report: CT of a duodenal paraganglioma

Paraganglioma is a neoplasm of neuroectodermal origin that occurs rarely in the bowel. This case report illustrates the previously undescribed CT appearance of a duodenal paraganglioma. An intensely enhancing mass in the region of the pancreatic head was found. The intense enhancement ruled out adenocarcinoma and focal pancreatitis. An islet cell tumor of the pancreas was the major differential diagnostic consideration.     

CARNEY'S TRIAD

A 26 year old female presented with partial expression of Carney's triad. This is the first Australian report of the condition. The role of surgery and the need for vigilance to detect the occurrence of the complete triad are stressed.