Paraparesis As a Rare First Presentation Of Primary Hyperparathyroidism-Related Brown Tumor in The Thoracic Spine: Case report and literature review

Jarrar, Sultan; Daoud, S; Jbarah, Omar F.; Albustami, Iyad S

doi:10.1016/j.amsu.2021.102197

Cited by 2 publications

(1 citation statement)

References 29 publications

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“…При этом в структуре кости могут быть выявлены зоны некроза с образова-нием кист с определяющимся уровнем жидкости, кровоизлияний и обызвествлений [25]. Гистологическая структура кистозно-фиброзных изменений при ПГПТ своим строением и клеточным составом может напоминать гигантоклеточные опухоли и некоторые виды сарком [24,26], поэтому обязательна дифференциальная диагностика с другими литическими поражениями скелета, в первую очередь со злокачественными опухолями. В представленном клиническом случае первоначально предполагалось вторичное поражение костей вследствие паранеопластического процесса, в связи с чем проводилась сцинтиграфия костей.…”

Section: Discussionunclassified

Severe bone complications of primary hyperparathyroidism in a young patient with the rare verified mutation of <i>MEN1</i>

Eremkina

Sazonova

Bibik

et al. 2022

Probl Endokrinol (Mosk)

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Multiple endocrine neoplasia type 1 syndrome (MEN1) is a rare inherited disorder that can include combinations of more than 20 endocrine and non-endocrine tumors. Unfortunately, none of the described MEN1 mutations has been associated with a peculiar clinical phenotype, even within members of the same family, thus a genotype-to-phenotype correlation does not exist. MEN1 syndrome is the most common cause of hereditary primary hyperparathyroidism (PHPT), the disease penetrance of which exceeds 50% by the age of 20 and reaches 95% by the age of 40. At the same time, PHPT with hyperplasia or adenomas of the parathyroid glands (PTG) is the most distinctive manifestation of the MEN1 syndrome. One of the main symptoms of PHPT, both in sporadic and hereditary forms of the disease, is bone damage. At the time of diagnosis in PHPT/MEN1, the bone mineral density is generally lower in comparison with the sporadic form of PHPT. This may be due to excessive secretion of parathyroid hormone during the period of peak bone mass, concomitant components of the syndrome, extended surgical treatment, and the direct effect of a mutation in the menin gene on bone remodeling. This clinical case describes a young patient with severe bone complications of PHPT and uncertain rare MEN1 mutation. PHPT was diagnosed five years later from the first onset of bone complications and repeated orthopedic operations. There was the «hungry bones» syndrome after successful surgery of PHPT, which was managed with vitamin D and calcium carbonate supplementation and there is a positive dynamic in increased bone mineral density in the main skeleton after 6 months.

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Section: Discussionunclassified