Jugular Foramen Lesions and their Management
John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed in Paediatrics, The Ear, Skull Base, 2018
The term paraganglioma is derived from the site of origin of the tumour. In the temporal bone, paraganglia are present in the adventitia of the jugular bulb and along the IXth and Xth cranial nerves, and in the middle ear in association with Jacobsen’s plexus. Chromaffin and non-chromaffin paragangliomas have been distinguished according to their intrinsic ability to take up and stain with chromium salts. In the past, a chromaffin paraganglioma was considered capable of producing adrenalin, whereas its non-chromaffin counterpart was not. This distinction between chromaffin and non-chromaffin paragangliomas has since become outdated, as both tumours are now known to synthesize all types of catecholamines. The term chemodectoma should be reserved for tumours that originate from the chemoreceptor system. The paraganglia in the temporal bone do not belong to this histological entity, in contrast with those in the carotid sinus. Therefore, the term chemodectoma is less appropriate for lesions in the jugular foramen and in other parts of the temporal bone.
Pheochromocytoma and Paraganglioma
Giuseppe Mancia, Guido Grassi, Konstantinos P. Tsioufis, Anna F. Dominiczak, Enrico Agabiti Rosei in Manual of Hypertension of the European Society of Hypertension, 2019
The European Society of Endocrinology Clinical Practice Guideline proposed a standardized long-term follow-up in all patients operated for PPGLs (54). The key recommendations for the duration of follow-up and monitoring methods are as follows (54): Annual follow-up is suggested for at least 10 years in all patients operated on for a PPGL, while high-risk patients (young age, with a genetic disease, with a large tumour, with a paraganglioma) should be offered lifelong annual follow-up.Measurement of plasma or urinary metanephrines and MTY every year to screen for local or metastatic recurrences or new tumours.Perform imaging tests every 1–2 years in patients with a biochemically inactive PPGL.
Pharynx, Larynx and Neck
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
This is a rare tumour that has a higher incidence in areas where people live at high altitudes because of chronic hypoxia leading to carotid body hyperplasia. The tumours most commonly present in the fifth decade and approximately 10% of patients have a family history. There is an association with phaeochromocytoma. The tumours arise from the chemo- receptor cells on the medial side of the carotid bulb and, at this point, the tumour is adherent to the carotid wall. The cells of the chemodectoma are not hormonally active and the tumours are usually benign with only a small number of cases producing proven metastases (Figure47.55).
Pheochromocytoma with Acute Non-cardiac Pulmonary Edema: A Report of One Case and the Review of Literature
Published in Cancer Investigation, 2021
Yuan Liu, Ning Wang, Shi Li, Ling Jiang, Chunfang Liu, Jian Xu, Huadong He
Pheochromocytoma is a tumor originated from adrenal medulla, sympathetic ganglion or other chromaffin tissues. Among them, tumors originated from sympathetic ganglion, parasympathetic ganglion or other parts of chromaffin tissue, also known as paraganglioma or extraadrenal pheochromocytoma. Pheochromocytoma and paraganglioma (ppgl) occur in adrenal medulla and extraadrenal. At present, adrenal pheochromocytoma and extraadrenal pheochromocytoma (paraganglioma) are collectively referred to as pheochromocytoma. It can lead to sustained or paroxysm hypertension, multiple organ dysfunction and metabolic disorders due to sustained or intermittent releasing of catecholamine (1). pheochromocytoma is mainly found in adrenal gland, also in retroperitoneal paraaortic tissue. Therefore, it indicates that the occurrence of pheochromocytoma with acute non-cardiac pulmonary edema is relatively rare. Although previous studies have reported the clinic characteristics of patients with pheochromocytoma, few studies have reported the clinic characteristics of patients with pheochromocytoma and acute non-cardiac pulmonary edema. In the present study, we reported a case of pheochromocytoma combined with acute non-cardiac pulmonary edema and reviewed literatures in an attempt to improve the recognition of the disease in order to strive for early diagnosis and therapy.
Advancing the Care of Pediatric Rare Solid Tumors: A virtual rare tumor board
Published in Pediatric Hematology and Oncology, 2022
Priya Mahajan, Shoba Navai, Valeria Smith, Samara Potter, Rajkumar Venkatramani
To date 86 pediatric patients with rare solid tumors with 70 distinct diagnoses (Table 1) have been presented. Diagnoses presented at this forum include adenoid cystic carcinoma, esthesioneuroblastoma, adrenocortical carcinoma, inflammatory myofibroblastic tumor, DICER related sarcoma, angiomatoid fibrous histiocytoma, synovial sarcoma, malignant ectomesenchymoma, paraganglioma, neuroendocrine tumor, renal cell carcinoma, and alveolar soft part sarcoma. Sixty-three physicians from 43 institutions across the United States and Canada have presented clinical cases at this rare tumor board since its inception. Participants include pediatric oncologists, surgeons, pathologists, radiologists, geneticists, and medical trainees. Two to four cases are discussed at each 60-minute tumor board, and the tumor board is open to all institutions. All tumor board presentations, classified by diagnosis, are made available online for future reference (https://bcm.box.com/v/raretumorboard).3
Para-aortic paraganglioma mimicking lymph node metastasis in an ovarian carcinoma: a case report
Published in Journal of Obstetrics and Gynaecology, 2018
Gozde Sahin, Ceyhan Ugurluoglu, Serra Akar, Ayhan Gul, Aysegul Kebapcilar, Cetin Celik
Typical clinical manifestations, including paroxysmal hypertension, headache, excessive perspiration and palpitations, are well-described. In the absence of this complex of symptoms, an extra-adrenal paraganglioma may be incidentally discovered during unrelated surgery, as in our patient; at autopsy or during abdominal imaging for abdominal pain or mass (Krane 1986). The preoperative diagnosis of asymptomatic paraganglioma is difficult due to the lack of specific symptoms. Unfortunately, when diagnosed during surgery, patients have high morbidity and mortality rates. Unless patients who harbour this uncommon tumour are correctly prepared and protected from the effects of excessive catecholamine release, they are at risk when undergoing any kind of surgical procedure (Hull 1986). A life-threatening reaction may be induced via the physical manipulation of the tumour or the utilisation of drugs that induce free catecholamine release. The risk of a hypertensive crisis due to surgical excision can be reduced via an alpha and beta adrenergic blockade. Once the diagnosis and drug preparation have been accomplished, a complete surgical resection can be performed. Resection is often challenging because these highly vascular tumours are located near vital blood vessels (Young 2006; Wen et al. 2010).
Related Knowledge Centers
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- Sdha
- Succinate Dehydrogenase Complex Subunit C
- Sdhb