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

Walz, Martin K.; Iova, Laura D.; Deimel, Judith; Neumann, Hartmut P. H.; Bausch, Birke; Zschiedrich, Stefan; Groeben, Harald; Alesina, Pier Francesco

doi:10.1007/s00268-018-4488-y

Cited by 29 publications

(21 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Of note, endoscopic surgery is now also standard for extra-adrenal retroperitoneal pheochromocytoma and even a good option for pelvic and thoracic pheochromocytoma (Fig. 2) (Walz et al 2006(Walz et al , 2018.…”

Section: Answer 3: Treatment Optionsmentioning

confidence: 99%

65 YEARS OF THE DOUBLE HELIX: Genetics informs precision practice in the diagnosis and management of pheochromocytoma

Neumann¹,

Young²,

Krauß³

et al. 2018

Endocrine-Related Cancer

Self Cite

View full text Add to dashboard Cite

Although the authors of the present review have contributed to genetic discoveries in the field of pheochromocytoma research, we can legitimately ask whether these advances have led to improvements in the diagnosis and management of patients with pheochromocytoma. The answer to this question is an emphatic ! In the field of molecular genetics, the well-established axiom that familial (genetic) pheochromocytoma represents 10% of all cases has been overturned, with>35% of cases now attributable to germline disease-causing mutations. Furthermore, genetic pheochromocytoma can now be grouped into five different clinical presentation types in the context of the ten known susceptibility genes for pheochromocytoma-associated syndromes. We now have the tools to diagnose patients with genetic pheochromocytoma, identify germline mutation carriers and to offer gene-informed medical management including enhanced surveillance and prevention. Clinically, we now treat an entire family of tumors of the paraganglia, with the exact phenotype varying by specific gene. In terms of detection and classification, simultaneous advances in biochemical detection and imaging localization have taken place, and the histopathology of the paraganglioma tumor family has been revised by immunohistochemical-genetic classification by gene-specific antibody immunohistochemistry. Treatment options have also been substantially enriched by the application of minimally invasive and adrenal-sparing surgery. Finally and most importantly, it is now widely recognized that patients with genetic pheochromocytoma/paraganglioma syndromes should be treated in specialized centers dedicated to the diagnosis, treatment and surveillance of this rare neoplasm.

show abstract

Section: Answer 3: Treatment Optionsmentioning

confidence: 99%

65 YEARS OF THE DOUBLE HELIX: Genetics informs precision practice in the diagnosis and management of pheochromocytoma

Neumann¹,

Young²,

Krauß³

et al. 2018

Endocrine-Related Cancer

Self Cite

View full text Add to dashboard Cite

show abstract

“…The study by Amar et al (36) regarding the factors affecting the recurrences in pheochromocytoma shows that recurrence is impacted by the dimensions of the tumor, affected location (right side is more likely than the left side) and diagnosis age (recurrence is much more observed amongst the youth). Walz et al (37), in their study dated back to 2018 in which an assessment was made for the minimal invasive TA and PA for 70 adrenal masses observed on the patients involving 42 children and adolescents with pheochromocytoma and/or paraganlioma, (26 with pheochromocytoma, 11 PGL and 5 pheochromocytoma + PGL) reported that 31 of the patients with pheochromocytoma underwent PA while excision was made for the patients with PGL. During the follow up period of 8.5 years as an average, recurrence was observed in two patients with VHL syndrome in the case of PA and out of five PGL patients with recurrence, four patients had to undergo another operation.…”

Section: Oncologic Resultsmentioning

confidence: 99%

Contemporary Trends in Adrenal Sparing Surgery

Gökalp¹,

Akdogan²,

Bayazit³

2020

uob

View full text Add to dashboard Cite

Adrenal gland surgery has been practiced by the urologists ever increasingly in line with their interest in this particular field. Total adrenalectomy which is the classical approach for adrenal masses, is replaced by the organ-sparing surgery as soon as the significance of the organ sparing approach is understood as in the case of the renal masses. The partial adrenalectomy which has been in use through open surgery method since 1950's, was adapted to adrenal surgery by Gagner in 1991 and technical advancement was achieved since then with the introduction of the laparoscopic adrenalectomy by Janetschek six years later. Although it is increasingly being used, there still exist ambiguities concerning the technique and indications of the adrenal sparing surgery. In this review, the updated status of the adrenal sparing surgery is discussed with the current literature.

show abstract

“…26,27 A mini-invasive approach is feasible but it should be performed preferably in tertiary centers with high volumes of oncologic cases and laparoscopic procedures in the pediatric population. 24,25,28 This is justified in order to minimize the possible onset of severe intraoperative complications (such as severe bleeding) and to maximize the benefit of laparoscopic surgery. Moreover, the possible need of intensive postoperative monitoring should be taken into consideration.…”

Section: Discussionmentioning

confidence: 99%

Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP)

Virgone

Andreetta

Avanzini

et al. 2020

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Background: Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated. Materials and methods: Among over 1000 patients registered into the Tumori Rari in EtàPediatrica-rare tumors in pediatric age project-from 2000 to 2019, 50 were affected by PC/PGL. All clinical and therapeutic data were evaluated.Results: Twenty-eight patients had PC and 22 had PGL. Age at diagnosis ranged between 5 and 17 years. Thirty-five patients had symptoms related to catecholamine hypersecretion; in 7 of 50 patients, diagnosis was incidental or done during assessment of a familial syndrome. In all cases, conventional imaging was effective to assess the presence of a tumor. In addition, 18 of 38 functional imaging studies were positive (61%). Forty-eight patients were eligible for surgery: a complete resection was more frequently achieved in PC than in PGL (26/28 vs 11/22). All relapses were treated with surgery alone, surgery plus medical treatment, or chemotherapy alone; one PC with metastasis at diagnosis received radiotherapy only. Forty-four patients were in the first, second, or third complete remission (10/50 recurred; 8/10 carried a germline mutation). Five of 50 patients were alive with disease. One patient died of disease.Abbreviations: 18 F-DOPA, fluorine-18-L-dihydroxyphenylalanine; 18 F-FDG, fluorine-18-fluorodeoxyglucose; 123 I-MIBG, 123 iodium-metaiodobenzylguanidine; 177 Lu-DOTATATE, lutetium-177-DOTA 0 -Tyr 3 -octreotate; CT, computed tomography; EFS, event-free survival; Max, MYC-associated factor X; MRI, magnetic resonance imaging; NF1, neurofibromin 1; OS, overall survival; PC, pheochromocytoma; PET/CT, positron emission tomography/computed tomography; PGL, paraganglioma; Ret, rearranged during transfection; SDHA, succinate dehydrogenase complex flavoprotein subunit A; SDHAF2, succinate dehydrogenase complex assembly factor 2; SDHB, succinate dehydrogenase complex iron sulfur subunit B; SDHC, succinate dehydrogenase complex subunit C; SDHD, succinate dehydrogenase complex subunit D; TMEM127, transmembrane protein 127; TREP, Tumori Rari in Età Pediatrica-rare tumors in pediatric age; VHL, von Hippel-Lindau.

show abstract

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

Abstract: PH and PGL in children and adolescents should preferably be removed by minimally invasive surgery. Partial adrenalectomy provides long-term steroid independence in bilateral PH and a low rate of (ipsilateral) recurrence. α-Receptor blockade may not be necessary in these patients.

Cited by 29 publications

References 38 publications

65 YEARS OF THE DOUBLE HELIX: Genetics informs precision practice in the diagnosis and management of pheochromocytoma

65 YEARS OF THE DOUBLE HELIX: Genetics informs precision practice in the diagnosis and management of pheochromocytoma

Contemporary Trends in Adrenal Sparing Surgery

Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP)

Contact Info

Product

Resources

About