Radiotherapy for glomus jugulare paraganglioma

Surgery has been long considered to be the treatment of choice for glomus jugulare paragangliomas, as it is the only modality able to totally eradicate the tumour. However, despite considerable progress in interventional radiology and nerve monitoring, surgery is associated with an unacceptably high complication rate for a benign tumour, explaining the growing place of radiotherapy in the management of these tumours. This review of the literature confirms the efficacy of conformal radiotherapy with or without intensity modulation and stereotactic radiotherapy, which both achieve tumour control rates ranging from 90% to almost 100% of cases, but for different tumour volumes, almost constant stabilization or even improvement of symptoms, and a considerably lower rate of adverse effects than with surgery. However, radiotherapy remains contraindicated in the presence of intracranial invasion or extensive osteomyelitis. In the light of these results, together with the improved quality of life and a better knowledge of the natural history of this disease, many authors propose radiotherapy as first-line treatment for all glomus jugulare paragangliomas regardless of their size, particularly in patients with no preoperative deficits.     

Paraganglioma of the Cauda Equina Region – Report of two Cases and Review of the Literature

Summary  A paraganglioma of cauda equina region is extremely rare and except for secreting tumour, the pre-operative diagnosis of paraganglioma is very difficult. Two cases of non-functional paragangliomas of the cauda equina region are reported, one was attached to the filum terminale and the other to a rootlet looking very much like a vascular neurinoma. Both were successfully removed by surgery. An extensive review of the literature permits one to find 77 other cases. The clinical, radiological, pathological (ultrastructural and immunohistochemical) features and surgical findings of all theses cases are discussed. Surgery remains the treatment of choice. No effect of radiotherapy on recurrence prevention has ever been demonstrated.     

Aortico-pulmonary paraganglioma showing remarkable regression with cyclophosphamide

The treatment of choice for mediastinal paraganglioma is complete surgical resection. Its local recurrence, however, is very difficult to treat surgically because it usually involves important structures of the mediastinum, such as the aorta and trachea. We report a 64-year-old woman with aortico-pulmonary paraganglioma who developed local recurrence after complete resection. The recurrent tumor responded dramatically to single-agent chemotherapy with cyclophosphamide, whereas it did not respond to radiotherapy or combination chemotherapy with mitomycin C, vindesine, and cisplatin. We therefore consider that single-agent chemotherapy with cyclophosphamide could be an alternative treatment for unresectable recurrent paraganglioma. Received: November 21, 1997 / Accepted: March 18, 1999     

腹膜后静止型副神经节细胞瘤的临床诊治

目的 提高腹膜后功能静止型副神经节细胞瘤的诊断和治疗水平.方法 分析1997年至今资料完整、病理证实的功能静止型副神经节细胞瘤10例.术前行内分泌、影像学及核医学等检查.4例术前服用2～4周α受体拮抗剂.3例接受腹腔镜手术,7例接受经腹开放手术.结果 功能静止型副神经节细胞瘤患者血压及尿儿茶酚胺通常为正常或稍高,131 IMIBG和奥曲肽显像定位准确、敏感性高.4例肿瘤位于肾门旁,6例位于肾下方腹主动脉旁,直径3～15 cm.术后病理结果:7例为副神经节细胞瘤,3例为恶性副神经节细胞瘤.免疫组化染色:cgA( ),syn( ),NSE( ),8.100( ).1例恶性副神经节细胞瘤于术后1年发生全身转移.结论 一旦确诊功能静止型副神经节细胞瘤,应该在充分药物准备的基础上,手术切除肿瘤,术后应密切随访.     

Polypoid nonchromaffin paraganglioma of the duodenum

Summary A polypoid tumor was surgically removed from the second part of the duodenum of a 56-year-old male. The main body consisted of large epithelioid cells arranged in an adenoma like pattern of strands and nests. These cells were argyrophil and had marked nonspecific esterase activity. Unmyelinated nerves with proliferated Schwann cells accompanied these epithelioid cells together with scattered gangliocyte like elements. Ultramicroscopically, the epithelioid cells were seen to contain round electron dense granules, 150 nm in diameter on average. The tumor is considered to be a nonchromaffin paraganglioma, as it probably developed from paraganglion cells associated with small arteries or branches of the vagus nerve, or from the undifferentiated pluripotent APUD cells of the duodenum.     

Paraganglioma of the Oropharynx

Paraganglioma is a rare tumor in head and neck region. A 35 years male presented with huge swelling of tonsillar region occupying a large portion of oropharynx. Tumor had been dissected out transorally. HPE showed extra-adrenal paraganglioma. It is being reported because of its rare clinical presentation and unusual surgical approach.     

The biology,function, and applications of exosomes in cancer

Exosomes are cell-derived nanovesicles with diameters from 30 to 150 nm, released upon fusion of multivesicular bodies with the cell surface. They can transport nucleic acids, proteins, and lipids for intercellular communication and activate signaling pathways in target cells. In cancers, exosomes may participate in growth and metastasis of tumors by regulating the immune response, blocking the epithelial–mesenchymal transition, and promoting angiogenesis. They are also involved in the development of resistance to chemotherapeutic drugs. Exosomes in liquid biopsies can be used as non-invasive biomarkers for early detection and diagnosis of cancers. Because of their amphipathic structure, exosomes are natural drug delivery vehicles for cancer therapy.     

Mutations of the <Emphasis Type="Italic">SDHB</Emphasis> and <Emphasis Type="Italic">SDHD</Emphasis> genes

The succinate dehydrogenase (SDH) is a mitochondrial enzyme complex with an important role in oxydative phosphorylation and intracellular oxygene sensing and signaling. Mutations in the SDHB (1p35–36) and SDHD subunits (11q23) give rise to the paraganglioma syndromes (PGL), namely PGL 4 and PGL 1, and generate paraganglioma and pheochromocytoma. For both genes mutations have been described that result in a loss of function of the gene products. SDHBmutations were found in five of eight exons and in two introns, SDHD mutations in all four exons and one intron. Phenotypes and rate of malignancy of SDHB and SDHD seem to be different, with a higher frequency of head-and-neck tumors in SDHD and indications of a higher risk of malignancy in SDHB mutations. As routine diagnostic procedure all SDH mutation carriers should have urine catecholamine analysis as well as pelvic, abdominal, thoracic and skull/neck MRI.