PMID- 23771922
OWN - NLM
STAT- MEDLINE
DCOM- 20131104
LR  - 20211021
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 98
IP  - 8
DP  - 2013 Aug
TI  - Multiplicity of hormone-secreting tumors: common themes about cause, expression, 
      and management.
PG  - 3139-48
LID - 10.1210/jc.2013-1511 [doi]
AB  - CONTEXT: Multiplicity of hormone-secreting tumors occurs in a substantial portion 
      of hormone-excess states. Multiplicity increases the difficulty of management and 
      drives the selection of special strategies. EVIDENCE ACQUISITION: This is a 
      synthesis from publications about tumor development and expression, and also 
      about types of clinical strategy for hormone-secreting tumors. EVIDENCE 
      SYNTHESIS: Comparisons were made between patient groups with solitary tumors vs 
      those with multiple tumors. Major themes with clinical relevance emerged. 
      Usually, tumor multiplicity develops from a genetic susceptibility in all cells 
      of a tissue. This applies to hormone-secreting tumors that begin as either 
      polyclonal (such as in the parathyroids of familial hypocalciuric hypercalcemia) 
      or monoclonal tumors (such as in the parathyroids of multiple endocrine neoplasia 
      type 1 [MEN1]). High penetrance of a hereditary tumor frequently results in 
      bilaterality and in several other types of multiplicity. Managements are better 
      for the hormone excess than for the associated cancers. Management strategies can 
      be categorized broadly as ablation that is total, subtotal, or zero. Examples are 
      discussed for each category, and 1 example of each category is named here: 1) 
      total ablation of the entire tissue with effort to replace ablated functions (for 
      example, in C-cell neoplasia of multiple endocrine neoplasia type 2); 2) subtotal 
      ablation with increased likelihood of persistent disease or recurrent disease 
      (for example, in the parathyroid tumors of MEN1); or 3) no ablation of tissue 
      with or without the use of pharmacotherapy (for example, with blockers for 
      secretion of stomach acid in gastrinomas of MEN1). CONCLUSIONS: Tumor 
      multiplicity usually arises from defects in all cells of the precursor tissue. 
      Even the optimized managements involve compromises. Still, an understanding of 
      pathophysiology and of therapeutic options should guide optimized management.
FAU - Marx, Stephen J
AU  - Marx SJ
AD  - Genetics and Endocrinology Section, Metabolic Diseases Branch, National Institute 
      of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, 
      Bethesda, Maryland 20892, USA. marxs@mail.nih.gov
LA  - eng
GR  - Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PT  - Review
DEP - 20130614
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (Hormones)
SB  - IM
MH  - Cell Proliferation
MH  - Hormones/*metabolism
MH  - Humans
MH  - Hyperplasia
MH  - Multiple Endocrine Neoplasia/etiology/genetics/metabolism/*therapy
MH  - Organ Specificity
PMC - PMC3733851
EDAT- 2013/06/19 06:00
MHDA- 2013/11/05 06:00
PMCR- 2014/08/01
CRDT- 2013/06/18 06:00
PHST- 2013/06/18 06:00 [entrez]
PHST- 2013/06/19 06:00 [pubmed]
PHST- 2013/11/05 06:00 [medline]
PHST- 2014/08/01 00:00 [pmc-release]
AID - jc.2013-1511 [pii]
AID - 13-1511 [pii]
AID - 10.1210/jc.2013-1511 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2013 Aug;98(8):3139-48. doi: 10.1210/jc.2013-1511. Epub 
      2013 Jun 14.