PMID- 21550948
OWN - NLM
STAT- MEDLINE
DCOM- 20120130
LR  - 20210204
IS  - 1934-2403 (Electronic)
IS  - 1530-891X (Linking)
VI  - 17
IP  - 4
DP  - 2011 Jul-Aug
TI  - Cushing syndrome secondary to a thymic carcinoid tumor due to multiple endocrine 
      neoplasia type 1.
PG  - e92-6
LID - 10.4158/EP11038.CR [doi]
AB  - OBJECTIVE: To present an Iranian patient with a nonclassic form of multiple 
      endocrine neoplasia type 1 (MEN 1) who presented with ectopic Cushing syndrome 
      (CS) secondary to a corticotropin (ACTH)-producing thymic neuroendocrine tumor 
      (NET), recurrent renal stones, and a giant cell granuloma of the jaw due to 
      primary hyperparathyroidism (PHPT) without involvement of the pituitary or 
      pancreas. METHODS: Relevant imaging and hormonal evaluations were performed. The 
      patient was operated on 2 occasions for a thymic NET and on 3 occasions for PHPT. 
      DNA from a peripheral blood sample was extracted for sequencing of the MEN1 gene. 
      RESULT: Histopathologic evaluation of the thymic tumor removed during the first 
      surgery showed an atypical carcinoid tumor with a Ki-67 labeling index of 5%. 
      Evaluation after the second surgery revealed an invasive carcinoid tumor with a 
      Ki-67 labeling index of 30%. Parathyroid pathology was suggestive of glandular 
      hyperplasia. Menin gene sequencing revealed a novel frameshift mutation 
      c.1642_1648dup in exon 10. CONCLUSION: This case of MEN 1 is unusual because most 
      thymic NETs in MEN 1 are nonfunctional, and secretion of ACTH or other ectopic 
      hormones rarely occurs. In patients presenting with thymic NETs, the possibility 
      of MEN 1 should be considered, especially in the presence of hyperparathyroidism. 
      This case also demonstrates that the behavior of thymic NETs can change over time 
      from slow-growing tumors to highly invasive neoplasia, and that ectopic ACTH can 
      be produced by these tumors in the context of MEN 1.
FAU - Ghazi, Ali A
AU  - Ghazi AA
AD  - Endocrine Research Center, Research Institute for Endocrine Sciences, Shahid 
      Beheshti University of Medical Sciences, Tehran, Iran. ghazi@endocrine.ac.ir
FAU - Dezfooli, Azizollah Abbasi
AU  - Dezfooli AA
FAU - Mohamadi, Farzaneh
AU  - Mohamadi F
FAU - Yousefi, Seyed Vahid
AU  - Yousefi SV
FAU - Amirbaigloo, Alireza
AU  - Amirbaigloo A
FAU - Ghazi, Siavash
AU  - Ghazi S
FAU - Pourafkari, Marina
AU  - Pourafkari M
FAU - Berney, Dan
AU  - Berney D
FAU - Ellard, Sian
AU  - Ellard S
FAU - Grossman, Ashley B
AU  - Grossman AB
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Endocr Pract
JT  - Endocrine practice : official journal of the American College of Endocrinology 
      and the American Association of Clinical Endocrinologists
JID - 9607439
SB  - IM
MH  - Adult
MH  - Cushing Syndrome/*diagnosis/*etiology
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*complications
MH  - Thymus Neoplasms/*diagnosis/*etiology
EDAT- 2011/05/10 06:00
MHDA- 2012/01/31 06:00
CRDT- 2011/05/10 06:00
PHST- 2011/05/10 06:00 [entrez]
PHST- 2011/05/10 06:00 [pubmed]
PHST- 2012/01/31 06:00 [medline]
AID - S1530-891X(20)41770-7 [pii]
AID - 10.4158/EP11038.CR [doi]
PST - ppublish
SO  - Endocr Pract. 2011 Jul-Aug;17(4):e92-6. doi: 10.4158/EP11038.CR.