Gastroenteropankreatik nöroendokrin tümörleri
Lancet Oncol. 2008 Jan;9(1):61-72.
Gastroenteropancreatic neuroendocrine tumours.
Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, Caplin M,
Delle Fave G, Kaltsas GA, Krenning EP, Moss SF, Nilsson O, Rindi G, Salazar R,
Ruszniewski P, Sundin A.
Department of Gastroenterological Surgery, Yale University, New Haven, CT
06520-8062, USA. imodlin@optonline.net
Comment in
Lancet Oncol. 2008 Mar;9(3):202-3.
Lancet Oncol. 2008 Mar;9(3):203.
Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare
neoplasms that present many clinical challenges. They secrete peptides and
neuroamines that cause distinct clinical syndromes, including carcinoid syndrome.
However, many are clinically silent until late presentation with mass effects.
Investigation and management should be highly individualised for a patient,
taking into consideration the likely natural history of the tumour and general
health of the patient. Management strategies include surgery for cure (which is
achieved rarely) or for cytoreduction, radiological intervention (by
chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin
analogues to control symptoms that result from release of peptides and
neuroamines. New biological agents and somatostatin-tagged radionuclides are
under investigation. The complexity, heterogeneity, and rarity of GEP NETs have
contributed to a paucity of relevant randomised trials and little or no survival
increase over the past 30 years. To improve outcome from GEP NETs, a better
understanding of their biology is needed, with emphasis on molecular genetics and
disease modeling. More-reliable serum markers, better tumour localisation and
identification of small lesions, and histological grading systems and
classifications with prognostic application are needed. Comparison between
treatments is currently very difficult. Progress is unlikely to occur without
development of centers of excellence, with dedicated combined clinical teams to
coordinate multicentre studies, maintain clinical and tissue databases, and
refine molecularly targeted therapeutics.
J Clin Endocrinol Metab. 2001 Dec;86(12):5658-71.
Guidelines for diagnosis and therapy of MEN type 1 and type 2.
Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C,
Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli
M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson
NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ.
Department of Internal Medicine, University of Florence, 50139 Florence, Italy.
m.brandi@dmi.unifi.it
Comment in
J Clin Endocrinol Metab. 2002 Jun;87(6):2994.
This is a consensus statement from an international group, mostly of clinical
endocrinologists. MEN1 and MEN2 are hereditary cancer syndromes. The commonest
tumors secrete PTH or gastrin in MEN1, and calcitonin or catecholamines in MEN2.
Management strategies improved after the discoveries of their genes. MEN1 has no
clear syndromic variants. Tumor monitoring in MEN1 carriers includes biochemical
tests yearly and imaging tests less often. Neck surgery includes subtotal or
total parathyroidectomy, parathyroid cryopreservation, and thymectomy. Proton
pump inhibitors or somatostatin analogs are the main management for oversecretion
of entero-pancreatic hormones, except insulin. The roles for surgery of most
entero-pancreatic tumors present several controversies: exclusion of most
operations on gastrinomas and indications for surgery on other tumors. Each MEN1
family probably has an inactivating MEN1 germline mutation. Testing for a
germline MEN1 mutation gives useful information, but rarely mandates an
intervention. The most distinctive MEN2 variants are MEN2A, MEN2B, and familial
medullary thyroid cancer (MTC). They vary in aggressiveness of MTC and spectrum
of disturbed organs. Mortality in MEN2 is greater from MTC than from
pheochromocytoma. Thyroidectomy, during childhood if possible, is the goal in all
MEN2 carriers to prevent or cure MTC. Each MEN2 index case probably has an
activating germline RET mutation. RET testing has replaced calcitonin testing to
diagnose the MEN2 carrier state. The specific RET codon mutation correlates with
the MEN2 syndromic variant, the age of onset of MTC, and the aggressiveness of
MTC; consequently, that mutation should guide major management decisions, such as
whether and when to perform thyroidectomy.
Surg Clin North Am. 2009 Oct;89(5):1105-21.
Insulinoma.
Mathur A, Gorden P, Libutti SK.
Surgery Branch, National Cancer Institute, National Institutes of Health,
Bethesda, MD 20892, USA.
Insulinoma is a rare neuroendocrine tumor with an incidence of 4 per 1 million
persons per year, which may occur as a unifocal sporadic event in patients
without an inherited syndrome or as a part of multiple endocrine neoplasia type
1. Key neuroglycopenic and hypoglycemic symptoms in conjunction with biochemical
proof establish the diagnosis. Once the diagnosis is established, the insulinoma
is preoperatively localized within the pancreas with the goal of surgical
excision for cure. This review discusses the historical background, diagnosis,
and management of sporadic insulinoma.
Curr Probl Surg. 2006 Oct;43(10):685-765.
Islet cell tumors.
Mittendorf EA, Shifrin AL, Inabnet WB, Libutti SK, McHenry CR, Demeure MJ.
University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.
Surg Clin North Am. 2009 Feb;89(1):249-66, x.
The surgical and systemic management of neuroendocrine tumors of the pancreas.
Abood GJ, Go A, Malhotra D, Shoup M.
Department of General Surgery, Loyola University Medical Center, 2160 South First
Avenue, Maywood, IL 60153, USA.
Neuroendocrine tumors of the pancreas comprise a class of rare tumors that can be
associated with symptoms of hormone overproduction. Five distinct clinical
endocrinopathies are associated with neuroendocrine tumors; however, most of
these tumors remain asymptomatic and follow an indolent course. Complete surgical
resection offers the only hope for cure, but understanding the basic biology of
the tumors has advanced the medical management in metastatic disease. Surgical
resection of hepatic metastases offers survival advantage and should be performed
when feasible. Although hepatic artery embolization is currently the preferred
mode of nonsurgical palliation for pain and hormonal symptoms, other modalities
may play a role in metastatic disease.
