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Carcinoid tumor

MedGen UID:
2838
Concept ID:
C0007095
Neoplastic Process
Synonyms: Carcinoid; Carcinoid tumors
SNOMED CT: Carcinoid tumor (443492008); Carcinoid tumor (189607006); Carcinoid (189607006); Carcinoid tumor - morphology (189607006)
 

Definition

A slow growing neuroendocrine tumor, composed of uniform, round, or polygonal cells having monotonous, centrally located nuclei and small nucleoli, infrequent mitoses, and no necrosis. The tumor may show a variety of patterns, such as solid, trabecular, and acinar. Electron microscopy shows small secretory granules. Immunohistochemical studies reveal NSE, as well as chromogranin immunoreactivity. Malignant histology (cellular pleomorphism, hyperchromatic nuclei, prominent nucleoli, necrosis, and mitoses) can occasionally be seen. Such cases may have an aggressive clinical course. Gastrointestinal tract and lung are common sites of involvement. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCarcinoid tumor

Conditions with this feature

Multiple endocrine neoplasia, type 1
MedGen UID:
9957
Concept ID:
C0025267
Neoplastic Process
Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of more than 20 endocrine and non-endocrine tumors. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself. Parathyroid tumors are the main MEN1-associated endocrinopathy; onset in 90% of individuals is between ages 20 and 25 years with hypercalcemia evident by age 50 years; hypercalcemia causes lethargy, depression, confusion, anorexia, constipation, nausea, vomiting, diuresis, dehydration, hypercalciuria, kidney stones, increased bone resorption/fracture risk, hypertension, and shortened QT interval. Pituitary tumors include prolactinoma (the most common), which manifests as oligomenorrhea/amenorrhea and galactorrhea in females and sexual dysfunction in males. Well-differentiated endocrine tumors of the gastro-entero-pancreatic (GEP) tract can manifest as Zollinger-Ellison syndrome (gastrinoma); hypoglycemia (insulinoma); hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, and skin rash (glucagonoma); and watery diarrhea, hypokalemia, and achlorhydria syndrome (vasoactive intestinal peptide [VIP]-secreting tumor). Carcinoid tumors are non-hormone-secreting and can manifest as a large mass after age 50 years. Adrenocortical tumors can be associated with primary hypercortisolism or hyperaldosteronism. Non-endocrine tumors include facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, and leiomyomas.
Multiple endocrine neoplasia, type 4
MedGen UID:
373469
Concept ID:
C1970712
Neoplastic Process
Multiple endocrine neoplasia is a group of disorders that affect the body's network of hormone-producing glands called the endocrine system. Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple endocrine neoplasia typically involves tumors (neoplasia) in at least two endocrine glands; tumors can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumors become cancerous, the condition can be life-threatening.The major forms of multiple endocrine neoplasia are called type 1, type 2, and type 4. These types are distinguished by the genes involved, the types of hormones made, and the characteristic signs and symptoms.Many different types of tumors are associated with multiple endocrine neoplasia. Type 1 frequently involves tumors of the parathyroid glands, the pituitary gland, and the pancreas. Tumors in these glands can lead to the overproduction of hormones. The most common sign of multiple endocrine neoplasia type 1 is overactivity of the parathyroid glands (hyperparathyroidism). Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of bones, nausea and vomiting, high blood pressure (hypertension), weakness, and fatigue.The most common sign of multiple endocrine neoplasia type 2 is a form of thyroid cancer called medullary thyroid carcinoma. Some people with this disorder also develop a pheochromocytoma, which is an adrenal gland tumor that can cause dangerously high blood pressure. Multiple endocrine neoplasia type 2 is divided into three subtypes: type 2A, type 2B (formerly called type 3), and familial medullary thyroid carcinoma (FMTC). These subtypes differ in their characteristic signs and symptoms and risk of specific tumors; for example, hyperparathyroidism occurs only in type 2A, and medullary thyroid carcinoma is the only feature of FMTC. The signs and symptoms of multiple endocrine neoplasia type 2 are relatively consistent within any one family.Multiple endocrine neoplasia type 4 appears to have signs and symptoms similar to those of type 1, although it is caused by mutations in a different gene. Hyperparathyroidism is the most common feature, followed by tumors of the pituitary gland, additional endocrine glands, and other organs.

Recent clinical studies

Etiology

Herde RF, Kokeny KE, Reddy CB, Akerley WL, Hu N, Boltax JP, Hitchcock YJ
Am J Clin Oncol 2018 Jan;41(1):24-29. doi: 10.1097/COC.0000000000000221. PMID: 26270444Free PMC Article
Pikin O, Ryabov A, Sokolov V, Glushko V, Kolbanov K, Telegina L, Amiraliev A, Barmin V
Ann Thorac Surg 2017 Dec;104(6):1846-1851. Epub 2017 Oct 17 doi: 10.1016/j.athoracsur.2017.06.050. PMID: 29054306
Biçer EN, Öztürk AB, Ozyigit LP, Erus S, Tanju S, Dilege Ş, Tabak L
J Asthma 2015;52(10):1095-8. Epub 2015 Aug 17 doi: 10.3109/02770903.2015.1054402. PMID: 26291136
Mun MJ, Lee JC, Lee BJ
Auris Nasus Larynx 2015 Feb;42(1):59-62. Epub 2014 Sep 6 doi: 10.1016/j.anl.2014.08.015. PMID: 25199739
Lee SP, Sung IK, Kim JH, Lee SY, Park HS, Shim CS
J Korean Med Sci 2014 Apr;29(4):512-8. Epub 2014 Apr 1 doi: 10.3346/jkms.2014.29.4.512. PMID: 24753698Free PMC Article

Diagnosis

Herde RF, Kokeny KE, Reddy CB, Akerley WL, Hu N, Boltax JP, Hitchcock YJ
Am J Clin Oncol 2018 Jan;41(1):24-29. doi: 10.1097/COC.0000000000000221. PMID: 26270444Free PMC Article
Koustais S, O'Halloran PJ, Hassan A, Brett F, Young S
World Neurosurg 2017 Sep;105:1042.e11-1042.e14. Epub 2017 Jul 11 doi: 10.1016/j.wneu.2017.06.179. PMID: 28705700
Audlin JR, Khullar G, Deshaies EM, Kurra S, Lavelle WF
World Neurosurg 2016 Dec;96:607.e1-607.e6. Epub 2016 Oct 3 doi: 10.1016/j.wneu.2016.09.088. PMID: 27713062
Kim HS, Yoon G, Jang HI, Song SY, Kim BG
Int J Clin Exp Pathol 2015;8(5):5749-52. Epub 2015 May 1 PMID: 26191292Free PMC Article
Yagi M, Abe Y, Sasaki Y, Nomura E, Sato T, Iwano D, Yoshizawa K, Sakuta K, Kanno N, Nishise S, Ueno Y
Dig Endosc 2015 May;27(4):527-30. Epub 2014 Nov 16 doi: 10.1111/den.12385. PMID: 25283957

Therapy

Nakajima M, Uchiyama N, Shigemasa R, Matsumura T, Matsuoka R, Nomura A
Intern Med 2016;55(21):3151-3153. Epub 2016 Nov 1 doi: 10.2169/internalmedicine.55.6738. PMID: 27803410Free PMC Article
Erim T, Colak Y, Szomstein S
Surg Obes Relat Dis 2015 Nov-Dec;11(6):e51-2. Epub 2015 Jun 25 doi: 10.1016/j.soard.2015.06.016. PMID: 26507937
Kiratli H, Uzun S, Tarlan B, Ateş D, Baydar DE, Söylemezoğlu F
Ophthalmic Plast Reconstr Surg 2015 Jul-Aug;31(4):e91-3. doi: 10.1097/IOP.0000000000000112. PMID: 24777267
Pleşa A, Sarca E, Maxim R
Rev Med Chir Soc Med Nat Iasi 2014 Oct-Dec;118(4):1018-23. PMID: 25581963
Ordóñez NG
Appl Immunohistochem Mol Morphol 2012 Jul;20(4):421-6. doi: 10.1097/PAI.0b013e318238bb8f. PMID: 22710819

Prognosis

Herde RF, Kokeny KE, Reddy CB, Akerley WL, Hu N, Boltax JP, Hitchcock YJ
Am J Clin Oncol 2018 Jan;41(1):24-29. doi: 10.1097/COC.0000000000000221. PMID: 26270444Free PMC Article
Kim JY
World J Surg Oncol 2016 Apr 21;14:120. doi: 10.1186/s12957-016-0867-8. PMID: 27098182Free PMC Article
Harada H, Suehiro S, Shimizu T, Katsuyama Y, Hayasaka K, Ito H
World J Gastroenterol 2015 Sep 14;21(34):10041-4. doi: 10.3748/wjg.v21.i34.10041. PMID: 26379409Free PMC Article
Wang Q, Chen H, Zhou S
Int J Clin Exp Pathol 2014;7(12):9028-31. Epub 2014 Dec 1 PMID: 25674282Free PMC Article
Lee SP, Sung IK, Kim JH, Lee SY, Park HS, Shim CS
J Korean Med Sci 2014 Apr;29(4):512-8. Epub 2014 Apr 1 doi: 10.3346/jkms.2014.29.4.512. PMID: 24753698Free PMC Article

Clinical prediction guides

Pikin O, Ryabov A, Sokolov V, Glushko V, Kolbanov K, Telegina L, Amiraliev A, Barmin V
Ann Thorac Surg 2017 Dec;104(6):1846-1851. Epub 2017 Oct 17 doi: 10.1016/j.athoracsur.2017.06.050. PMID: 29054306
Nakajima M, Uchiyama N, Shigemasa R, Matsumura T, Matsuoka R, Nomura A
Intern Med 2016;55(21):3151-3153. Epub 2016 Nov 1 doi: 10.2169/internalmedicine.55.6738. PMID: 27803410Free PMC Article
Audlin JR, Khullar G, Deshaies EM, Kurra S, Lavelle WF
World Neurosurg 2016 Dec;96:607.e1-607.e6. Epub 2016 Oct 3 doi: 10.1016/j.wneu.2016.09.088. PMID: 27713062
Kim SS, Choi C, Kang TW, Choi YD
Pathol Int 2016 Jan;66(1):42-6. Epub 2015 Dec 8 doi: 10.1111/pin.12367. PMID: 26644387
Steuer CE, Behera M, Kim S, Chen Z, Saba NF, Pillai RN, Owonikoko TK, Khuri FR, Ramalingam SS
J Thorac Oncol 2015 Mar;10(3):479-85. doi: 10.1097/JTO.0000000000000419. PMID: 25371080

Recent systematic reviews

Pikin O, Ryabov A, Sokolov V, Glushko V, Kolbanov K, Telegina L, Amiraliev A, Barmin V
Ann Thorac Surg 2017 Dec;104(6):1846-1851. Epub 2017 Oct 17 doi: 10.1016/j.athoracsur.2017.06.050. PMID: 29054306
McCrary HC, Faucett EA, Reghunathan S, Aly FZ, Khan R, Carmody RF, Jacob A
Otol Neurotol 2017 Jan;38(1):114-117. doi: 10.1097/MAO.0000000000001228. PMID: 27749753
Kuroda N, Tanaka A, Ohe C, Mikami S, Nagashima Y, Inoue K, Shuin T, Taguchi T, Tominaga A, Alvarado-Cabrero I, Petersson F, Brunelli M, Martignoni G, Michal M, Hes O
Histol Histopathol 2013 Jan;28(1):15-21. doi: 10.14670/HH-28.15. PMID: 23233056
Demirci I, Herold S, Kopp A, Flaßhove M, Klosterhalfen B, Janßen H
World J Surg Oncol 2012 Jan 23;10:19. doi: 10.1186/1477-7819-10-19. PMID: 22269186Free PMC Article
Ferlito A, Devaney KO, Rinaldo A
Acta Otolaryngol 2006 Mar;126(3):228-31. doi: 10.1080/00016480500388901. PMID: 16618645

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