Pheochromocytoma: A three-decade clinical experience in a multicenter study

Iglesias, Pedro; Santacruz, Elisa; Garcia-Sancho, Paula; Marengo, Agustina Pia; Guerrero-Pérez, Fernando; Pián, Héctor; Fajardo, Carmen; Villabona, Carles; Díez, Juan J.

doi:10.1016/j.rceng.2019.12.011

Cited by 6 publications

(5 citation statements)

References 23 publications

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“…Similarly to other studies, we found germline pathogenic variants predominantly linked to MEN2A (RET pathogenic variants) [29,30]. However, in this study, hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes (SDHx pathogenic variants) were found more frequently than neuro bromatosis type 1 and Von Hippel-Lindau disease.…”

Section: Discussionsupporting

confidence: 83%

“…Quanti cation of 24h urinary fractionated metanephrines demonstrate high sensitivity, a rming their diagnostic value [13,14]. After the biochemical diagnosis, abdominal CT scan emerges as the costeffective imaging modality of choice, because of its excellent sensitivity and speci city [3,29]. In this study, CT scan was the most frequently imaging exam performed (n = 40; 90,91%).…”

Section: Discussionmentioning

confidence: 75%

“…As described in the literature, the prevalence of metastatic disease usually varies between 10-20% [29]. This study identi ed only 3 patients with metastatic disease, all of them exhibiting related genetic pathogenic variants.…”

Section: Discussionmentioning

confidence: 91%

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The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

Carvalho,

Machado,

Morais

et al. 2024

Preprint

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Purpose Pheochromocytoma is a rare neuroendocrine tumor. Despite the low incidence, these tumors are of indisputable importance. This study aimed to analyze the management of pheochromocytoma in a referral center, with an emphasis on the minimally invasive adrenalectomy, which is the preferred therapeutic approach. Methods A retrospective analysis was performed on a cohort of patients diagnosed with pheochromocytoma who underwent adrenalectomy between January 2013 and December 2022. Clinical data including demographics, timelines, symptomatology, comorbidities, biochemical markers, genetic testing, surgical details, and follow-up outcomes, were collected and analyzed. Results The cohort included 44 patients, predominantly women (52,27%), with a median age of 53,39 years (range 13–83). Most of patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86,36%), along with glucose anomalies (40,01%) and anxiety disorder (31,82%). Genetic testing was performed in 36 (81,81%) patients and 14 (38,88%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (79,07%) patients, showing significantly shorter operative time (2,5 hours vs. 4,25 hours, t-test p < 0,001) and fewer complications (23,53% vs 77,78%, p = 0,008). Postoperative complications occurred in 36,36% of the patients, mostly mild (grade I, 56,25%), with no mortality. SDHB pathogenic variant correlated with both recurrent and metastatic disease (p = 0.006). One-year follow-up reported 9.09% recurrence and 6.82% metastasis. Conclusions Adrenalectomy demonstrated a high safety and effectiveness. This study exhibited a higher rate of genetic testing referral than other studies. Despite past advances, there is still a need for further studies to establish protocols and evaluate new techniques.

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Section: Discussionsupporting

confidence: 83%

Section: Discussionmentioning

confidence: 75%

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The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

Carvalho,

Machado,

Morais

et al. 2024

Preprint

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“…11 La importancia del diagnóstico oportuno radica en la alta morbilidad y mortalidad cardiovascular asociada a los efectos de las catecolaminas, así como en el potencial maligno del 10% al 17% relacionado con esta patología. [12][13][14] En conclusión, el POCUS puede ser una herramienta diagnóstica clave sumado a la clínica del paciente para el diagnóstico del feocromocitoma la disponibilidad de este recurso está sujeta el entrenamiento previo del médico que la utiliza, en este caso fue una herramienta clave que permitió el diagnóstico rápido y preciso aun sin contar con las metanefrinas en sangre o en orina. Es importante destacar las características ecográficas de estas lesiones aumentando la precisión diagnóstica como así lo respalda el colegio estadounidense de médicos de emergencias y los estudios previamente mencionados.…”

Section: A Bunclassified

Abordaje ecográfico en el paciente críticamente enfermo con feocromocitoma

Serna-Trejos,

Castro-Galvis,

Castaño-Vargas

et al. 2024

spmi

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Varón de 73 años de edad quien consultó por dolor torácico, cefalea global, náuseas, emesis más diaforesis y cifras tensionales elevadas de cuatro horas de evolución. El paciente presentó alternancia de sus cifras tensionales en ciclos continuos de hipertensión e hipotensión de aproximadamente 11 min, en los cuales alcanzaba presiones arteriales sistólicas mayores de 300 mm Hg y variaba hasta tensiones arteriales sistólicas de 40 mm Hg, sin variaciones enla frecuencia cardiaca. En el Servicio de Urgencias se realizó una ecografía hallaándose una lesión homogénea, isoecogénica, de bordes definidos en la glándula suprarrenal izquierda, de aproximadamente 4,8 cm x 4,5 cm, hallazgo que fue comprobado con el estudio tomográfico, y que resultó ser un feocrocitoma. El abordaje ecográfico (POCUS) en suprarrenales en el paciente con crisis hiperensiva suele revelar hallazgos de alto valor diagnostico, toma de decisiones o direccionamiento de conductas terapéuticas.

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“…Несмотря на то что прошло почти 100 лет со дня первого проведения подобной операции, методика применяется до сих пор, однако в настоящее время к ней прибегают лишь при невозможности выполнить операцию малоинвазивными техниками, что обычно связано с крупными размерами опухоли, спаянностью с окружающими органами, инвазией в соседние органы и ткани или иными причинами. Наибольшим размером феохромоцитомы для использования эндоскопических методик чаще всего называли 5-6 см, однако на данный момент имеется множество публикаций, подтверждающих безопасность и эффективность эндоскопических методик в удалении опухолей и большего размера [19][20][21]. Одним из значимых факторов является личный опыт хирурга и операционной бригады в целом.…”

Section: выбор хирургического доступа к надпочечникуunclassified

Surgical treatment of pheochromocytoma

Shikhmagomedov,

Rebrova,

Krasnov

et al. 2023

Probl Endokrinol (Mosk)

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This review article contains a summary of modern aspects of preoperative preparation, surgical treatment, and follow-up of patients with adrenal pheochromocytomas. The main component of preoperative preparation is the use of alpha-blockers. The need to prescribe them to all patients is increasingly disputed, especially for patients without severe hypertension. An increasing number of publications demonstrate positive results of treatment without the use of alpha-blockers, advocating an individual approach and the use of the drug according to certain indications. Minimally invasive endoscopic techniques of adrenalectomy have become widespread in surgical treatment. They are represented by laparoscopic and retroperitonescopic technic, including using their single-port modifications. The earliest possible intersection of the central vein in the past was considered the most important aspect of adrenalectomy for pheochromocytoma, currently, due to the development of surgical techniques and anesthesiological manuals, this has ceased to be a mandatory rule of successful surgery. Despite the significant influence of the intersection of this vessel on intraoperative hemodynamics, surgical tactics with its later intersection have their own justifications and do not lead to a deterioration in treatment results. The standard volume of surgical intervention for pheochromocytomas is total adrenalectomy, however, in the presence of hereditary syndromes, such as multiple endocrine neoplasia type 2 syndrome, neurofibomatosis type 1, von Hippel-Lindau syndrome, it is possible to perform cortical-sparing adrenalectomy.

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Pheochromocytoma: A three-decade clinical experience in a multicenter study

Cited by 6 publications

References 23 publications

The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

Abordaje ecográfico en el paciente críticamente enfermo con feocromocitoma

Surgical treatment of pheochromocytoma

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