WHEN EVEN THE CLASSICS REQUIRE CLOSE SURVEILLANCE – A TYPICAL CASE OF MEN SYNDROME

The genetic syndrome known in literature as the Multiple Endocrine Neoplasia type 1 (MEN 1) is transmitted in a dominant manner. The primary discovery of more than two endocrine tumor formations representative for MEN 1 should guide the doctor to the suspicion and subsequent confirmation of the diagnosis. Statistics state a long association of the MEN 1 syndrome with the tumors discovered in the small intestine and the pancreas with the adenomas encountered in the parathyroid and pituitary glandular tissueWhile in postmenopausal women, the primary hyperparathyroidism is diagnosed mostly with abnormal levels of parathyroid hormone and serum calcium specifically. The most useful preoperative imaging tools are neck ultrasound scanning and technetium-99m sestamibi scintigraphy. Using bilateral neck exploration, subtotal, and parathyroidectomy are proposed. Our case consists of a 54 years old female, diagnosed with right inferior parathyroid adenoma. With three first degree relatives operated for neuroendocrine pancreatic tumors, her medical history includes distal corporeal-caudal pancreatectomy for a neuroendocrine tumor four years ago. Laboratory tests unveil increased calcium (12, 41 mg/dl) and glucose (150 mg/dl) levels. During hospitalization, the patient’s representative parameters for monitoring hyperparathyroidism maintained at high levels. Parathyroid ultrasound and the 99mTctetrofosmin/99mTc-pertechnetate parathyroid scintigraphy sustained the diagnosis. Consequently, the patient was proposed for a subtotal parathyroidectomy with a pathology examination. It is highly recommended the interdisciplinary cooperation for the monitoring and management of patients with suspicion of MEN 1. The annual assessment of serum calcium level, together with the parathyroid hormones, is proposed as a screening tool in early diagnosis.

[1]  E. Grubbs,et al.  Surgical Management of Multiple Endocrine Neoplasia 1 and Multiple Endocrine Neoplasia 2. , 2019, The Surgical clinics of North America.

[2]  D. Shoback,et al.  Applying the Guidelines for Primary Hyperparathyroidism: The Path Not Taken. , 2019, JAMA internal medicine.

[3]  C. Stratakis,et al.  Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis , 2019, Front. Endocrinol..

[4]  V. Mai,et al.  Octreotide Use in a Patient with MEN-1 Syndrome and Multifocal Pancreatic Neuroendocrine Tumors: A Case Report and Review of the Literature , 2019, Case reports in gastrointestinal medicine.

[5]  D. Lourenço,et al.  Could the Less-Than Subtotal Parathyroidectomy Be an Option for Treating Young Patients With Multiple Endocrine Neoplasia Type 1-Related Hyperparathyroidism? , 2019, Front. Endocrinol..

[6]  D. Goltzman,et al.  Evolution of Our Understanding of the Hyperparathyroid Syndromes: A Historical Perspective , 2018, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

[7]  C. Marcocci,et al.  Familial and Hereditary Forms of Primary Hyperparathyroidism. , 2018, Frontiers of hormone research.

[8]  D. Scott-Coombes,et al.  The surgical management of sporadic primary hyperparathyroidism. , 2018, Best practice & research. Clinical endocrinology & metabolism.

[9]  Herbert Chen,et al.  Can imaging studies be omitted in patients with sporadic primary hyperparathyroidism? , 2018, The Journal of surgical research.

[10]  N. Tolley,et al.  Morbidity in parathyroid surgery for renal disease is under reported: a comparison of outcomes with primary hyperparathyroidism , 2018, Annals of the Royal College of Surgeons of England.

[11]  V. Mathew,et al.  Five-year Retrospective Study on Primary Hyperparathyroidism in South India: Emerging Roles of Minimally Invasive Parathyroidectomy and Preoperative Localization with Methionine Positron Emission Tomography-Computed Tomography Scan , 2018, Indian journal of endocrinology and metabolism.

[12]  G. Thompson,et al.  Long‐term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours , 2017, Clinical endocrinology.

[13]  R. Doi Determinants of surgical resection for pancreatic neuroendocrine tumors , 2015, Journal of hepato-biliary-pancreatic sciences.

[14]  J. Bilezikian,et al.  Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). , 2012, The Journal of clinical endocrinology and metabolism.

[15]  J. Bilezikian,et al.  Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 ( MEN 1 ) , 2012 .

[16]  C. Stratakis,et al.  Solid tumors associated with multiple endocrine neoplasias. , 2010, Cancer Genetics and Cytogenetics.

[17]  D. Figarella-Branger,et al.  Pituitary Tumors and Hyperplasia in Multiple Endocrine Neoplasia Type 1 Syndrome (MEN1): A Case-Control Study in a Series of 77 Patients Versus 2509 Non-MEN1 Patients , 2008, The American journal of surgical pathology.

[18]  E. Blind,et al.  [Asymptomatic primary hyperparathyroidism]. , 1991, Deutsche medizinische Wochenschrift.

[19]  F. Kuhlencordt,et al.  [Primary hyperparathyroidism]. , 1979, Deutsche medizinische Wochenschrift.