PMID- 15233909
OWN - NLM
STAT- MEDLINE
DCOM- 20041027
LR  - 20220716
IS  - 1527-2729 (Print)
IS  - 1534-6277 (Linking)
VI  - 5
IP  - 4
DP  - 2004 Aug
TI  - Unusual functioning endocrine tumors.
PG  - 327-34
AB  - Endocrine surgeons should maintain a high index of suspicion when patients are 
      diagnosed with clinical signs or symptoms of parathyroid carcinoma. Although 
      rare, the best chance for cure of these patients is at the time of the initial 
      operation. Surgical resection of recurrent disease can provide effective 
      palliation and can sometimes be assisted using gamma-probe directed dissection of 
      sestamibi-labeled tumor tissue. Treatment of hyperparathyroidism in the setting 
      of multiple endocrine neoplasia type 1 (MEN-1), particularly in the reoperative 
      setting, can be aided by using the rapid intraoperative parathyroid hormone assay 
      to judge the adequacy of parathyroid debulking. In addition, in selected cases, 
      the gamma probe can assist in identifying the location of ectopic or autografted 
      sestamibi-labeled parathyroid tissue. Patients with incidental adrenal masses 
      rarely require fine needle aspiration to exclude metastatic cancer. Fine needle 
      aspiration, if performed, should never precede hormone evaluation to exclude 
      pheochromocytoma. Patients who are diagnosed with incidental adrenal masses in 
      the setting of a prior or concurrent cancer diagnosis are equally likely to have 
      a primary adrenal mass as they would be to have metastatic cancer in the adrenal 
      gland. Pheochromocytomas occasionally develop in patients with MEN-1. In 
      suspicious cases, molecular identification of an MEN-1 mutation can be used to 
      confirm the diagnosis. Preoperative hormone evaluation of a patient with an 
      adrenal incidentaloma should include evaluation for subclinical Cushing's 
      syndrome through an overnight 1-mg dexamethasone suppression test. Identification 
      of this condition allows for safe peri- and postoperative steroid hormone 
      replacement, with very slow withdrawal of exogenous steroids to allow the 
      opposite adrenal gland to recover and avoid postoperative Addisonian crisis. 
      Paragangliomas are more commonly multifocal and malignant compared to 
      pheochromocytomas. Evaluation of patients with paragangliomas should include 
      radiographic staging for multifocality and metastatic disease, and postoperative 
      hormone and radiographic follow-up evaluation should be performed. Consideration 
      should be given to genetic testing for von Hippel-Lindau and succinate 
      dehydrogenase mutations. Surgical treatment of rare functioning pancreatic and 
      duodenal endocrine tumors, such as metastatic sporadic insulinoma and 
      MEN-1-associated gastrinoma, can provide effective palliation. Surgical treatment 
      should be integrated into a comprehensive treatment scheme that recognizes the 
      natural history of the disease and incorporates appropriate adjunctive therapies 
      and follow-up strategies.
FAU - Raut, Chandrajit P
AU  - Raut CP
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1515 Holcombe Boulevard, Box 444, Houston, TX 77030, USA.
FAU - Lee, Jeffrey E
AU  - Lee JE
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Curr Treat Options Oncol
JT  - Current treatment options in oncology
JID - 100900946
SB  - IM
MH  - Adrenal Gland Neoplasms/pathology/*therapy
MH  - Adult
MH  - Aged
MH  - Combined Modality Therapy
MH  - Female
MH  - Humans
MH  - Hyperparathyroidism/diagnosis/*therapy
MH  - Insulinoma/pathology/*therapy
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/pathology/*therapy
MH  - Parathyroid Neoplasms/pathology/*therapy
MH  - Pheochromocytoma/pathology/*therapy
MH  - Prognosis
MH  - Risk Assessment
RF  - 16
EDAT- 2004/07/06 05:00
MHDA- 2004/10/28 09:00
CRDT- 2004/07/06 05:00
PHST- 2004/07/06 05:00 [pubmed]
PHST- 2004/10/28 09:00 [medline]
PHST- 2004/07/06 05:00 [entrez]
AID - 10.1007/s11864-004-0023-5 [doi]
PST - ppublish
SO  - Curr Treat Options Oncol. 2004 Aug;5(4):327-34. doi: 10.1007/s11864-004-0023-5.