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* May present as Cushing syndrome
* May present as Cushing syndrome
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| colspan="2" |Pheochromocytoma
| colspan="2" |Pheochromocytoma<ref name="pmid24636754">{{cite journal |vauthors=Martucci VL, Pacak K |title=Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment |journal=Curr Probl Cancer |volume=38 |issue=1 |pages=7–41 |date=2014 |pmid=24636754 |pmc=3992879 |doi=10.1016/j.currproblcancer.2014.01.001 |url=}}</ref>
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* Headaches
* Headaches

Revision as of 17:05, 22 January 2019

Neuroendocrine tumor Clinical manifestations Dianosis Gold

standard

Other features
Symptoms Signs Blood & Urine Gross Histology Immunohistochemistry Imaging Others
Medullary thyroid carcinoma[1][2][3]
  • Dysphagia
  • Hoarseness
  • Respiratory difficulty
  • Flushing
  • Diarrhea
  • Weight loss
  • Palpable neck mass
  • Signs of Cushing syndrome
  • ↑ Calcitonin
  • ↑ Serum calcium
  • ↑ or - Cortisol
  • ↑ CEA level
  • White or gray in color
  • Firm to palpation
  • Nests of uniform cells
  • Deposition of stromal amyloid
  • Granular chromatin
  • C-cell hyperplasia
  • Calcitonin
  • Chromogranin A
  • Carcinoembryonic antigen (CEA)
  • Solid thyroid nodule (US)
  • CT scan/MRI and PET scan for metastatis
  • Genetic testing
  • FNA cytology with immunohisto-

chemistry and

calcitonin levels

  • Associated with MEN 2A and 2B
  • Familial association with RET mutations
  • May present as Cushing syndrome
Pheochromocytoma[4]
  • Headaches
  • Palpitations
  • Excessive sweating
  • Anxiety
  • Pallor
  • Pain in chest/abdomen
  • Weakness, fatigue
  • Nausea/vomiting
  • Dizziness
  • Paresthesias
  • Constipation (rarely diarrhea)
  • Visual disturbance
  • Hypertension
  • Postural hypotension
  • Tachycardia or reflex bradycardia
  • Tremulousness
  • Pallor
  • Flushing (rare)
  • Weight loss
  • Fasting hyperglycaemia
  • Decreased GI motility
  • Pallor
  • ↑ Respiratory rate
  • Psychosis
  • ↑ Plasma and urine catecholamines
  • ↑ Plasma and urine metanephrines
  • ↑ Chromogranin A
  • ↑ Plasma methoxytyramine
  • N/A
  • Loosely cohesive clusters
  • Scattered tumor cells with prominent anisokaryosis, abundant eosinophilic granular cytoplasm and indistinct cell borders
  • Occasional binucleate cells
  • Chromogranin A (CGA)
  • Protein gene product (PGP) 9.5
  • Synaptophysin (SYN)
  • CD56 (also known as neural cell adhesion molecule or N-CAM)
  • Glial fibrillary acidic protein (GFAP)
  • Heterogeneous appearance, often with some cystic areas. Calcifications or hemorrhage may also be present (CT)
  • T2-bright lesions, with/without cystic or necrotic components (MRI)
  • Cystic or solid with necrotic areas or hemorrhages (U/S)
  • Genetic testing
  • Provacative glucagon test
  • Clonidine suppression test
  • Metaiodobenzyl-guanidine scintigraphy
  • PET scan
  • Octereoscan
  • Plasma and urine catecholamines & metanephrines combined with clinical findings
  • May mimic panic attack
  • May be associated with Von Hippel-Lindau disease, MEN type 2 and Neurofibromatosis type 1.
  • Arise from the chromaffin cells
Merkel cell carcinoma
Parathyroid adenoma
Paraganglioma
Pituitary gland tumors
Neuroendocrine tumors of the ovaries or testicles
Thymic neuroendocrine cancer
Lung neuroendocrine tumors Typical carcinoid tumours
Atypical carcinoid tumours
Large cell lung neuroendocrine carcinomas
Small cell lung neuroendocrine carcinomas
Pancreatic neuroendocrine tumours Gastrinoma
Insulinoma
Glucagonoma
Somatostatinoma
VIPoma
ACTHoma
Gastric neuroendocrine tumors Type I GNET
Type II GNET
Type III GNET
Type IV GNET
Duodenal neuroendocrine tumors Gastrinomas
Somatostatinomas
Gangliocytic paraganglionomas
Nonfunctioning NET
neuroendocrine carcinomas
Jejuno-Ileal neuroendocrine tumors
Appendix neuroendocrine tumors
Colon neuroendocrine tumors
Rectum neuroendocrine tumors

References

  1. ↑ Segura S, Ramos-Rivera G, Suhrland M (2018). "Educational Case: Endocrine Neoplasm: Medullary Thyroid Carcinoma". Acad Pathol. 5: 2374289518775722. doi:10.1177/2374289518775722. PMC 6024338. PMID 29978018.
  2. ↑ Roy M, Chen H, Sippel RS (2013). "Current understanding and management of medullary thyroid cancer". Oncologist. 18 (10): 1093–100. doi:10.1634/theoncologist.2013-0053. PMC 3805151. PMID 24037980.
  3. ↑ Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K (August 2010). "The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer". Pancreas. 39 (6): 775–83. doi:10.1097/MPA.0b013e3181ebb4f0. PMC 3419007. PMID 20664475.
  4. ↑ Martucci VL, Pacak K (2014). "Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment". Curr Probl Cancer. 38 (1): 7–41. doi:10.1016/j.currproblcancer.2014.01.001. PMC 3992879. PMID 24636754.
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