Surgical analysis of pediatric and adolescent sporadic pheochromocytoma: single center experience

Osman, Yasser; Hussein, Naser; Sarhan, Osama; Shorrab, Ahmed A.; Dawaba, Mohamed; Ghoneim, Mohamed A.

doi:10.1007/s11255-011-9959-0

Cited by 4 publications

(3 citation statements)

References 18 publications

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“…The superiority of MIS compared to conventional open accesses for adrenalectomy could clearly be demonstrated (meanwhile in historical studies) without any need of prospective randomized trials [14, 15]. Up to now, endoscopic removal of PH in children has been published in case reports [16–19] or small series [20–23]. All these reports prove—as the data collected in the present study—safety of minimally invasive technology in children and adolescents.…”

Section: Discussionsupporting

confidence: 59%

Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients

et al. 2018

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Section: Discussionsupporting

confidence: 59%

Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients

et al. 2018

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“…In the large body of literature available, different authors report that significant improvement or complete recovery from hypertension and diabetes mellitus following surgical removal of phaeochromocytoma of up to 75–100% is observed in the majority of patients [ 6 , 18 – 21 ]. In the case of arterial hypertension, the underlying issue is follow–up, as in some patients hyperadrenalinemia overlaps primary hypertension.…”

Section: Discussionmentioning

confidence: 99%

The effect of surgical treatment of phaeochromocytoma on concomitant arterial hypertension and diabetes mellitus in a single-centre prospective study

Pogorzelski¹,

Toutounchi²,

Krajewska³

et al. 2014

CEJU

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IntroductionPhaeochromocytoma is one of the numerous causes of secondary hypertension. Furthermore, phaeochromocytoma may first present with type 2 diabetes mellitus. The objective of our study was to evaluate the effects of adrenalectomy on patient recovery with regards to normotension and well–controlled glycaemia.Material and methodsThe retrospective analysis involved 67 patients with phaeochromocytoma operated between 2006 and mid-2012. The pre–operative diagnoses were made in the departments of internal medicine and endocrinology. Based on laboratory tests and diagnostic imaging, we were able to confirm the diagnosis of phaeochromocytoma in 42 (62.7%) patients. We verified the influence of adrenalectomy on the level of patient recovery, with regards to normotension and glycaemic control: arterial pressure and fasting glycaemia levels were obtained on the day of hospital discharge, at follow–up 3 months post–operatively and 1 year after surgical intervention.ResultsOf the 67 patients operated for phaeochromocytoma, 48 (71.6%) were treated laparoscopically, whereas 19 (28.4%) underwent open adrenalectomy. Arterial hypertension was recorded in 53 (79.1%) cases. Furthermore, among this group, diabetes mellitus coexisted in 21 (31.3%) cases. Postoperatively, 70% of cases of arterial hypertension and 90% of type 2 diabetes mellitus were cured. Additionally, a high rate of patients reported a quantitative reduced use of antihypertensive medicines.ConclusionsIn the majority of patients, surgical treatment of symptomatic phaeochromocytoma leads to a regression of arterial hypertension, or a reduction of the number or doses of medicines taken in one's treatment, and glucose–intolerance symptoms.

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“…Therefore, the detection of genetic mutations may profoundly influence clinical outcomes and disease management. However, in clinical pediatric cases, only a small minority of patients have undergone genetic testing (10)(11)(12). Since the data for pediatric PCCs/PGLs are very limited, the clinical management of PCCs/PGLs in children or adolescents is usually extrapolated from the adult data (13), moreover, the clinical and genetic features of Han Chinese pediatric PCCs/PGLs patients have never been reported.…”

Section: Introductionmentioning

confidence: 99%

Clinical and genetic features of pediatric PCCs/PGLs patients: a single-center experience in China

Wang

Liu

et al. 2020

Transl Androl Urol

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Background: Although 40% to 80% of pediatric patients with pheochromocytoma (PCC) and paraganglioma (PGL) have been reported to carry germline mutations, the genetic and clinical features are poorly understood, and few such patients have undergone genetic testing. In this series, we aimed to investigate the clinical and genetic features of Han Chinese pediatric patients with PCC/PGL. Methods:The medical records of 15 pediatric patients with PCC/PGL who presented to our hospital between 2006 and 2018 were retrospectively studied. DNAs isolated from leukocytes of the patients were analyzed using whole-exome sequencing (WES).Results: The patients were nine girls and six boys with a mean age of 14.9 (range, 6-18) years. All were alive after a follow-up from 1 to 12 years, although two were diagnosed with pulmonary metastatic PGLs.Four patients were diagnosed with bilateral PCCs. Four patients were diagnosed with tumor syndromes.Among the 15 patients, nine were identified carrying germline mutations, of which seven were VHL and one each of RET and SDHB. In addition, a de novo mutation, VHL c.193T>A, was identified in a patient clinically diagnosed with a VHL syndrome.Conclusions: Among 15 pediatric patients studied, nine were identified carrying germline genetic mutations, four were diagnosed with bilateral PCCs, and four were diagnosed with other syndromic tumors in addition to PCC, which underscores the importance of genetic testing and managing treatment accordingly.

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Surgical analysis of pediatric and adolescent sporadic pheochromocytoma: single center experience

Cited by 4 publications

References 18 publications

Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients

Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients

The effect of surgical treatment of phaeochromocytoma on concomitant arterial hypertension and diabetes mellitus in a single-centre prospective study

Clinical and genetic features of pediatric PCCs/PGLs patients: a single-center experience in China

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