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.     

Radiosurgery for paraganglioma of the temporal bone

PURPOSE: To report the outcome of patients with paraganglioma of the temporal bone treated with stereotactic radiosurgery at the University of Florida. METHODS AND MATERIALS: Between January 1997 and June 1999, five patients with paraganglioma of the temporal bone were treated with Linac-based stereotactic radiosurgery at the University of Florida. The ages of the three female and two male patients were between 40 and 88 years (median, 49 years). Four patients were treated at initial presentation, and one had recurrent disease. Treatment volumes ranged from 4.9 cm3 to 18.4 cm3, with a mean of 10.84 cm3. The dose applied to the margin of the tumor varied from 12.5 to 15 Gy (median, 15 Gy). The treatment dose was specified to the 80% isodose shell in two cases and to the 70% isodose shell in three cases. The median follow-up time was 27 months, ranging from 14 to 50 months. RESULTS: One of four previously untreated patients had a relapse at the primary tumor site. Treatment failure occurred at the field margin 6 months after radiosurgery; the patient was subsequently treated with fractionated stereotactic radiotherapy and at the time of analysis had no evidence of disease, 21 months after initiation of salvage therapy. The patient treated at the time of recurrence after conventional radiotherapy had a local recurrence 40 months after radiosurgery. At the time of this recurrence, the patient had biopsy-proven metastatic disease in two cervical lymph nodes, and no salvage therapy was performed. All patients were alive at the time of the analysis, one with disease present. Presenting symptoms improved in two patients and stabilized in one. The two patients who had local recurrence develop had worsening of their symptoms. One patient had a cranial nerve V palsy develop 6 months after treatment, which resolved after a few months. CONCLUSIONS: In this series, the results with stereotactic radiosurgery are discouraging compared with our results with conventional fractionated radiotherapy in patients with paraganglioma of the temporal bone.     

Combined endovascular and surgical treatment of head and neck paragangliomas--a team approach

BACKGROUND: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. METHODS: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. RESULTS: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. CONCLUSIONS: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen.     

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。     

A case of pelvic malignant paraganglioma

We report a rare case of pelvic malignant paraganglioma that was treated with surgery, combination chemotherapy and radiation. A 47-year-old man was diagnosed with pelvic malignant paraganglioma that had metastasised to the thoracic vertebrae. The pelvic mass, which was 6 cm in size, was on the posterior side of the bladder and had invaded the prostate, seminal vesicle and bladder neck. We resected the intrapelvic tumor and lymph nodes using cystoprostatectomy. Metastases to bilateral obturator lymph nodes and the right internal iliac lymph node were shown by pathology. Adjuvant therapies included six courses of the combination chemotherapy (cyclophosphamide, vincristine and dacarbazine), and 12 courses of VP-16 therapy. Radiation therapy was done for metastasis of the thoracic vertebrae. Local recurrence, progression of bone metastasis and new metastasis have not been detected since these treatments. The patient has been clinically stable during 20 months of follow-up. Chemotherapy of cyclophosphamide, vincristine and dacarbazine and VP-16 with radiation appears to be effective in treating advanced malignant paraganglioma.     

腹膜后副神经节瘤25例临床分析

目的 探讨腹膜后副神经节瘤的诊断和治疗,提高其早期诊断及手术切除率.方法 对25例原发性腹膜后副神经节瘤患者行手术治疗.结果 25例中术后病理诊断良性肿瘤19例,恶性肿瘤为6例,完全切除19例,大部切除3例,其中围手术期死亡l例,无法切除3例,术后复发3例中再手术2例肿瘤完全切除,1例放弃手术.结论 腹膜后副神经节瘤早期临床表现缺乏特异性,诊断目前主要靠影像学检查,围手术期处理至关重要,手术完整切除仍是治疗的主要手段,对复发性肿瘤应争取再次手术.     

三维可视化重建技术在机器人辅助巨大嗜铬细胞瘤/副节瘤切除术中的应用价值(附11例报告)

目的:探讨三维可视化重建技术在机器人辅助腹腔镜下巨大嗜铬细胞瘤/副节瘤切除术中的应用价值.方法:回顾性分析2019年7月-2020年1月我院收治的11例嗜铬细胞瘤/副节瘤患者的临床资料.其中男5例,女6例,中位年龄44(21～68)岁;肿瘤最大径中位值7.1(6.2～10.2)cm;病变位于右肾上腺3例,左肾上腺5例,...     

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.     

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.