A spontaneous paraganglioma–pheochromocytoma syndrome

Toutounchi, Sadegh; Pogorzelski, Ryszard; Siński, Maciej; Łoń, Izabela; Zapała, Łukasz; Fiszer, Patryk; Krajewska, Ewa; Skórski, Maciej

doi:10.5173/ceju.2013.04.art12

Cited by 5 publications

(3 citation statements)

References 7 publications

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“…The bilateral adrenalectomy has been performed earlier and has lead to a life–long dependency from steroid and mineral corticoid replacement. Postoperatively, catecholamine normalization should be documented and a cortisol deficiency should be excluded if bilateral adrenal cortex–sparing surgery was performed [ 17 ].…”

Section: Resultsmentioning

confidence: 99%

Management and follow up of extraadrenal phaeochromocytoma

Barski¹

2014

CEJU

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IntroductionThe prevalence of phaeochromocytoma (PCC) in patients with hypertension is 0.1–0.6% and about 10% of PCCs are detected in extra–adrenal tissue. The diagnosis and therapy of this rare disease detected as a retroperitoneal tumor mass can be difficult for clinicians.Material and methodsA PubMed database was searched for the peer–reviewed articles, the listed articles until Dec 2012 were included. Following key words were used: “extra–adrenal phaeochromocytoma”, “paraganglioma”, “diagnosis”, “therapy”, “surgery”, “genetic analysis”, and “SDH mutation”.ResultsMagnetic Resonance Imaging (MRI) and Computed Tomography (CT) are first choice imaging tools for PCC (sensitivity 90–100%). For the validation of the diagnosis or follow up, the functional imaging 123I–metaiodobenzylguanidine (MIBG) or Fluorine–18–L–dihydroxyphenylalanine (18F–DOPA) positron emission tomography (excellent specificity and sensitivity of 90–100% in detection of small tumors >1–2 cm) are used. Laparoscopic surgery with complete resection is a safe and a first choice approach. The conversion (about 5%) to direct open operation was needed for large lesions (>8 cm) with the suspicion of malignancy. Currently, there are no histological criteria for distinguishing benign and malignant tumors. The genetic testing (Sanger DNA sequencing) for hereditary syndromes (von Hippel–Lindau, neurofibromatosis, etc.) is used for prediction of malignancy and recurrence. All patients should get individual and risk–adapted genetic analysis and consultation, including family members. The rate of malignancy in ePCC is about 30% (PCC about 5–10%). In patients with proven SDHB germline mutations, higher malignancy rate, multiple PCCs and recurrences are likely. A stringent lifelong clinical follow–up is recommended in these cases. Patients with syndromic hereditary forms should be screened for other often associated neoplasms.ConclusionsNew imaging tools and genetic analysis are crucial to improve the diagnosis and prognosis of phaeochromocytoma.

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Section: Resultsmentioning

confidence: 99%

Management and follow up of extraadrenal phaeochromocytoma

Barski¹

2014

CEJU

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“…They were diagnosed a few or many months after excision of the primary tumour. From other studies it is well known that metastasectomy from adrenal glands together with combined treatment prolong the life of the patients [18,19]. …”

Section: Rycina 2 Nowotwór Nadnerczy -Rezonans Magnetycznymentioning

confidence: 99%

The usefulness of laparoscopic adrenalectomy in the treatment of adrenal neoplasms — a single-centre experience

Pogorzelski

Toutounchi

Krajewska

et al. 2017

Endokrynologia Polska

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Introduction: Adrenal neoplasms comprise about 10% of all tumours affecting this organ and constitute a significant, at first diagnostic and subsequently therapeutic, problem, especially since a relatively high proportion of neoplastic lesions are asymptomatic. The number of diagnosed metastases to adrenal glands is increasing. Surgical treatment involves both open surgery as well as laparoscopy. Material and methods: There were 245 adrenalectomies performed at our centre due to various indications over the past four years. In 27 (11.5%) cases neoplasms were diagnosed in the final histopahtological examination. In 11 (40.7%) cases primary adrenal cortex tumours were diagnosed, metastases from other solid organ tumours were identified in another 12 (44.4%) patients, and rarer neoplasms were diagnosed in the remaning 4 (14.8%) subjects. Cases of malignant pheochromocytoma were not included in this report.

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“…Before surgery, the patient is conventionally prepared with α–adrenergic blockade (over 10–14 days) and subsequently, additional β–adrenergic blockade is required to treat any associated tachyarrhythmias [ 9 , 11 ]. A non–competitive, non–selective α–adrenergic blocker (phenoxybenzamine) is commonly used for α–receptor blocking.…”

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confidence: 99%

Adrenergic crisis due to pheochromocytoma – practical aspects. A short review

Juszczak¹,

Drewa²

2014

CEJU

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IntroductionThe definitive therapy in case of pheochromocytoma is complete surgical resection. Improper preoperative assessment and medical management generally places the patient at risk for complications, resulting from an adrenergic crisis. Therefore, it is crucial to adequately optimize these patients before surgery. Optimal preoperative medical management significantly decreases morbidity and mortality during the tumor resection.Material and methodsThis review addresses current knowledge in pre– and intraoperative assessment of a patient with pheochromocytoma.ResultsBefore surgery the patient is conventionally prepared with α–adrenergic blockade (over 10–14 days) and subsequently, additional β–adrenergic blockade is required to treat any associated tachyarrhythmias. In preoperative assessment, it is obligatory to monitor arterial blood pressure, heart rate, and arrhythmias and to restore the blood volume to normal.ConclusionsIn conclusion, due to the pathophysiological complexity of a pheochromocytoma, the strict cooperation between the cardiologist, endocrinologist, surgeon and the anaesthesiologist for an uneventful outcome should be achieved in patients qualified for the surgical removal of such a tumor.

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A spontaneous paraganglioma–pheochromocytoma syndrome

Cited by 5 publications

References 7 publications

Management and follow up of extraadrenal phaeochromocytoma

Management and follow up of extraadrenal phaeochromocytoma

The usefulness of laparoscopic adrenalectomy in the treatment of adrenal neoplasms — a single-centre experience

Adrenergic crisis due to pheochromocytoma – practical aspects. A short review

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