Recovery from severe osteoporosis following cure from ectopic ACTH syndrome caused by an appendix carcinoid

Abstract

It is well established that endogenous overproduction of glucocorticoids may cause osteoporosis. However, knowledge about potential recovery of the skeleton after cessation of corticosteroid excess is limited. We describe a 34-year-old female patient and discuss the complex diagnostic workup that led to the rare diagnosis of carcinoid of the appendix as the cause of Cushing's syndrome. The patient experienced multiple spontaneous rip fractures, and at that time, the bone mineral density of the lumbar spine was 4.3 SD below values for age-matched healthy controls. After removal of the tumour, the patient completely recovered. Measurements at the lumbar spine showed an increase in area bone density of 79% equivalent to 4 SD over a subsequent 3-year observation period. This first case of documented bone mass recovery in a patient cured of ectopic ACTH syndrome suggests a potential for excellent reversibility of steroid-induced osteoporosis and should be considered in the management of such patients.

Bone Metabolism and Vitamin D Implication in Gastroenteropancreatic Neuroendocrine Tumors

“…In addition to this direct effect on bone, glucocorticoid excess may also have a negative impact on vitamin D levels [52]. Different case reports describe the occurrence of osteoporosis in patients with GEP-NET and ECS (Table 1) [27,28]. Interestingly, a 34-year-old female patient with a history of multiple spontaneous rip fractures and osteoporosis, who had been diagnosed with NET of the appendix associated with ECS, reported an increase in BMD during the three years of follow-up after tumor resection [28].…”

“…Different case reports describe the occurrence of osteoporosis in patients with GEP-NET and ECS (Table 1) [27,28]. Interestingly, a 34-year-old female patient with a history of multiple spontaneous rip fractures and osteoporosis, who had been diagnosed with NET of the appendix associated with ECS, reported an increase in BMD during the three years of follow-up after tumor resection [28]. This study represents a potential reversibility of steroid-induced osteoporosis and, when possible, complete resection of the primary tumors should be considered in the management of such patients.…”

Glucocorticoid Excess During Adolescence Leads to a Major Persistent Deficit in Bone Mass and an Increase in Central Body Fat

“…Hypercortisolism during childhood and adolescence may have significant effects on peak bone mass accrual and body composition (14) . Only a small number of relatively short‐term prospective observations on areal bone mineral density (BMD) have been reported after the surgical cure of CS in children (14, 15) or in adults 15‐19) . Changes in total body and visceral abdominal fat in CS have been described in adults (20, 21) though the body composition changes in children after cure of CS have not been described.…”

Rare localization of an ACTH-producing tumour

“…В литературе описано не более 10 случаев обнаружения у больного АКТГ-эктопической опухоли указанной локализации и столько же работ с локализацией опухоли в ободоч-ной кишке [10][11][12][13][14][15].…”

“…Это осложнение встречается у 48-90% пациентов с синдромом Кушинга. Вос-становление массы костной ткани после оператив-ного лечения основного заболевания является весь-ма длительным процессом и может продолжаться 10 лет [11]. В работе H. Dobnig и соавт.…”