Management and follow up of extraadrenal phaeochromocytoma

Abstract

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.

Diagnosis and surgical treatment of retroperitoneal paraganglioma: A single-institution experience of 34 cases

“…None of patients included exhibited a family history of paraganglioma, which is distinct from previous studies that have determined the association of paraganglioma with a family history (10,11). This distinction may be due to the inclusion of different ethnicities between studies.…”

Gestion péri opératoire d’un paragangliome rétro-péritonéal

“…Les paragangliomes ou phéochromocytomes extrasurrénaliens sont des tumeurs développées aux dépens de cellules neuroectodermiques du système nerveux autonome [ 1 ]. La localisation retro péritonéale est rare et n'est retrouvée que dans 2% des cas [ 1 , 2 ]. Sur le plan fonctionnel les céphalées, palpitations, sueurs abondantes sont retrouvée dans près de 90% des cas avec une hypertension artérielle [ 3 , 4 ].…”

“…La prise en charge d'un paragangliome nécessite une préparation préopératoire minutieuse pour éviter les complications cardiovasculaires pouvant survenir en per et postopératoire. Une résection chirurgicale complète constitue le seul garant de guérison avec des taux de survie de 75 et 45% respectivement à cinq et dix ans [ 1 , 2 ]. Une évaluation de la fonction cardiaque par une échographie, s'avère nécessaire pour apprécier le retentissement de la sécrétion des catécholamines.…”

“…Le paragangliome (PG) est une tumeur endocrinienne développée aux dépens des cellules chromaffines extra surrénaliennes. Les formes rétro péritonéales sont moins fréquentes que les autres localisations [ 1 , 2 ]. Nous rapportons une observation d'un paragangliome rétro péritonéal avec des particularités révélatrices du diagnostic, et de prise en charge périopératoire.…”

Preliminary experience of the robot-assisted laparoscopic excision of a retroperitoneal mass: A case report

“…Complete surgical resection is the first choice approach. Radioactive isotope treatment with [ 131 I]-MIBG, systemic chemotherapeutic intervention and genetic analysis under development ( 3 , 4 ). Recently, laparoscopic surgery has become the standard treatment for benign and malignant PHEO.…”