Abstract
The role of imaging in functioning endocrine tumours (FETs) is primarily to detect the tumour, that is, to verify lesion number and location. Radiological detection of carcinoid tumours is limited by typical tumour location throughout the gastrointestinal tract or appendix and is therefore dependent on the tumour being large enough to make it recognisable in that site. The most common FET is insulinoma, which is commonly characterised by the typical appearance of a hypervascular lesion at multidetector-row computed tomography and magnetic resonance imaging. A particularly important role is played by intraoperative ultrasound in defining the exact number of lesions, their relationship with adjacent vascular structures and the pancreatic duct for the purposes of correct surgical planning (enucleation or resection). In the setting of nonfunctioning endocrine tumours (NFETs), which manifest late as large masses causing compression symptoms or as incidental findings, imaging is not primarily aimed at tumour detection, as this is relatively easy given the large size of the lesions. Rather, its role is to characterise the tumour and, in particular, to differentiate pancreatic NFET from ductal adenocarcinoma, as in comparison, malignant NFETs have a more favourable prognosis (5-year survival rate 40% compared with 3%–5% for adenocarcinoma) and therefore require different treatment approaches. As NFET are often malignant, they also require accurate staging and appropriate follow-up. In 80% of cases, NFETs have a “typical” imaging appearance: location in the pancreatic head, large dimensions (diameter between 5 and 15 cm, >10 cm in 30% of cases), capsule, sharp and regular margins owing to the expansile and noninfiltrative growth pattern, solid density and arterial hypervascularity. Some 20% of NFETs display different imaging characteristics (“atypical” appearance) as a result of arterial hypovascularity due to the presence of abundant fibrous stroma. Lastly, a small percentage of NFETs has yet a different appearance (“unusual”) due to the cystic nature and/or diffuse location throughout the pancreatic parenchyma.
Riassunto
Nei tumori endocrini funzionanti (TEF), il ruolo dell’imaging è principalmente volto alla loro identificazione, intesa come precisazione del numero e della topografia delle lesioni. Nel carcinoide l’identificazione con imaging è limitata dalla sua tipica sede in corrispondenza della parete gastro-intestinale o appendicolare ed è pertanto vincolata a dimensioni sufficienti a renderlo riconoscibile in tali sedi. Il TEF più frequente è l’insulinoma, che comunemente si presenta con un aspetto tipico di lesione ipervascolarizzata alle indagini di tomografia computerizzata multidetettore (TCMS) e di risonanza magnetica (RM). Particolare importanza riveste l’indagine ecografica (US) intra-operatoria al fine di precisare l’esatto numero di lesioni, i loro rapporti vascolari e con il dotto pancreatico principale, per una corretta pianificazione della strategia chirurgica (enucleazione o resezione). Nei tumori endocrini non funzionanti (TENF), che si manifestano tardivamente come masse voluminose con sintomi compressivi, o come reperti occasionali, i quesiti all’imaging sono rappresentati non tanto dalla identificazione, agevole per le cospicue dimensioni, ma dalla tipizzazione, in particolare nelle forme pancreatiche nei confronti dell’adenocarcinoma duttale, rispetto al quale anche i TENF maligni hanno prognosi migliore (sopravvivenza a 5 anni del 40% versus quella dell’adenocarcinoma del 3%–5%), per cui richiedono strategie terapeutiche diverse; in queste lesioni è necessario inoltre un corretto bilancio, essendo spesso tumori maligni, ed infine il loro follow-up. Nell’80% le forme non funzionanti hanno un aspetto imaging peculiare (o tipico): sede cefalo-pancreatica, cospicue dimensioni (diametro tra 5 e 15 cm; nel 30% dei casi >10 cm), capsulate, a contorni netti e regolari per il tipo di crescita espansiva e non infiltrativa, densità solida, con ipervascolarizzazione arteriosa. Un 20% dei TENF mostrano caratteristiche imaging differenti (aspetto atipico) per una ipovascolarizzazione arteriosa dovuta alla presenza di abbondante stroma fibroso. In una minima percentuale i tumori neuroendocrini non funzionanti infine mostrano un aspetto ancora diverso (inusuale) per la loro natura cistica e/o diffusa a tutto il parenchima pancreatico.
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Graziani, R., Brandalise, A., Bellotti, M. et al. Imaging of neuroendocrine gastroenteropancreatic tumours. Radiol med 115, 1047–1064 (2010). https://doi.org/10.1007/s11547-010-0540-1
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DOI: https://doi.org/10.1007/s11547-010-0540-1