scholarly journals Parathyroid carcinoma in multiple endocrine neoplasia type 1. Case report and review of the literature

HORMONES ◽  
2011 ◽  
Vol 10 (4) ◽  
pp. 326-331 ◽  
Author(s):  
Carlos del Pozo ◽  
Luis García-Pascual ◽  
Montserrat Balsells ◽  
María-José Barahona ◽  
Enrique Veloso ◽  
...  
Keyword(s):  
Case Report ◽  
Parathyroid Carcinoma ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Review Of The Literature ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type

Breast cancer in a patient with multiple endocrine neoplasia type 1 (MEN 1): A case report and review of the literature.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e21136-e21136
Author(s):  
Zorka Momcilo Inic ◽  
Momcilo Inic ◽  
Radan Dzodic ◽  
Gordana Pupic ◽  
Svetozar Damjanovic
Keyword(s):  
Breast Cancer ◽  
Case Report ◽  
Serum Concentration ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
The Body ◽  
Men 1 ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type

e21136 Background: We treated a patient with breast cancer associated with multiple endocrine neoplasia type 1 (MEN 1). There are only few studies of the development of breast cancer in patients diagnosed with MEN1 syndrome. Methods: We found that patient had a pituitary macroadenoma that secretes prolactin (prolactinomas), increased serum concentration of calcium, 4.09 mmol/l and increased serum concentration of PTH, 212 pg/ml. Neck ultrasound and scintigrafy showed suspicious adenoma in lower left parathyroid gland. A computed tomography scan of the abdomen revealed a tumor mass in the body of the pancreas and adenoma in the left suprarenal gland. We diagnosed multiple endocrine neoplasia type 1 (MEN 1). Results: MEN1 occurs as a result of inactivating mutations of the MEN1 gene (MEN1), located on chromosome11q13. Results indicate that menin is a direct activator of ERalpha function. In a clinical study, in Kagawa, with 65 ER-positive breast cancer samples-all of which had been treated with tamoxifen for 2-5 years as adjuvant therapies-menin-positive tumors had a worse outcome than menin-negative ones. This indicated that menin can function as a transcriptional regulator of ERalpha and is a possible predictive factor for tamoxifen resistance.These results demonstrate that allelic deletions of the 13q12-14 region occur in some pituitary adenomas and 16% of parathyroid adenomas. Conclusions: As recent molecular studies have suggested genetic mutations in multiple endocrine neoplasia type 1, this syndrome may possibly predispose patients to breast cancer.This possibility cannot be evaluated definitively on the basis of a single case report; additional observations and studies are necessary to investigate this hypothesis further.

A complex case of Multiple Endocrine Neoplasia type 1 with Metastatic Parathyroid Carcinoma

Open Medicine ◽  
2010 ◽  
Vol 5 (1) ◽  
pp. 53-58 ◽  
Author(s):  
Shyam Kalavalapalli ◽  
Indrajit Talapatra ◽  
Ian Connell
Keyword(s):  
Clinical Features ◽  
Parathyroid Carcinoma ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Somatostatin Analogues ◽  
Complex Case ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Gut Hormone

AbstractWe describe below a patient with Multiple Endocrine Neoplasia type 1 (MEN type 1) who presented with features of Primary Hyperparathyroidism. However, the actual diagnosis of Parathyroid Carcinoma was delayed until metastases to the lung were discovered. She was also found to have Pituitary Macro adenoma with Silent Acromegaly, with no clinical features whatsoever. She underwent transphenoidal hypophysectomy with postoperative radiotherapy. However, the disease process remained biochemically active necessitating commencement of somatostatin analogues. There is also a tumour at the head of the pancreas which at present is non functional with normal gut hormone profile and normal 24 hour urinary 5-hydroxyl indole acetic acid (5-HIAA) excretion assay. Our case highlights the pitfalls in diagnosing the parathyroid carcinoma due to lack of initial proper histological features. The diagnosis of parathyroid carcinoma was based on histologically confirmed metastases to the lungs. We also discuss below the entity called Silent Acromegaly where patients have biochemically and/or histologically confirmed growth Hormone (GH) excess with no clinical features suggesting Acromegaly. We discuss the benefits of somatostatin analogues in indirectly controlling the rest of the tumours in MEN1 and hypothesise the same for metastatic parathyroid carcinoma. Metastatic parathyroid carcinoma with multiple endocrine neoplasia type 1 is extremely rare. Our case highlights the complexities of managing MEN1 with metastatic parathyroid carcinoma and the dilemmas in dealing with the various aspects of the care.

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