PDF(Pubmed)
Abstract：
Brown tumors, an exceptional bone complication of severe primary (PHP) or renal (secondary) hyperparathyroidism (RHP), are caused by long-standing, elevated parathormone (PTH)-induced osteoclast activation causing multinucleated giant cell conglomerates with hemosiderin deposits in addition to the local production of cytokines and growth factors. We aim to present an adult case series including two females displaying this complication as part of a multidisciplinary complex panel in high PTH-related ailments. The approach was different since they had distinct medical backgrounds and posed a wide area of challenges amid real-life settings, namely, a 38-year-old lady with PHP and long-term uncontrolled hypercalcemia (with a history of pregnancy-associated PHP, the removal of a cystic jaw tumor, as well as a family and personal positive diagnosis of polycystic kidney disease, probably a PHP-jaw tumor syndrome), as well as, a 26-year-old woman with congenital single kidney and chronic renal disease-associated RHP who was poorly controlled under dialysis and developed severe anemia and episodes of metabolic acidosis (including one presentation that required emergency hemodialysis and was complicated with convulsive seizures, followed by resuscitated respiratory arrest). Both subjects displayed a severe picture of PHP/RHP with PTH levels of >1000 pg/mL and >2000 pg/mL and elevated serum bone turnover markers. Additionally, they had multiple brown tumors at the level of the ribs and pelvis (asymptomatically) and the spine, skull, and pelvis (complicated with a spontaneous cervical fracture). As an endocrine approach, the control of the underlying parathyroid disease was provided via surgery in PHP (for the postparathyroidectomy hungry bone syndrome) via medical intervention (with vitamin D analogs) in RHP. Additionally, in this case, since the diagnosis was not clear, a multidisciplinary decision to perform a biopsy was taken (which proved inconclusive), and the resection of the skull tumor to confirm the histological traits. This series highlights the importance of addressing the entire multidisciplinary panel of co-morbidities for a better outcome in patients with PHP/RHP-related brown tumors. However, in the instance of real-life medicine, poor compliance and reduced adherence to recommendations might impair the overall health status. Thus, sometimes, a direct approach at the level of cystic lesion is taken into consideration; this stands for a narrow frame of decision, and it is a matter of personalized decision. As seen here, brown tumors represent the hidden face of PHP/RHP, primarily the complex and severe forms, and awareness is essential even in the modern era.
摘要：
棕色肿瘤,严重原发性（PHP）或肾脏（继发性）甲状旁腺功能亢进（RHP）的特殊骨并发症，是由长期存在的，甲状旁腺激素（PTH）诱导的破骨细胞活化，除了局部产生细胞因子和生长因子外，还导致多核巨细胞团和铁血黄素沉积。我们的目标是提供一个成人病例系列，其中包括两名女性，这些女性表现出这种并发症，作为高PTH相关疾病的多学科复杂小组的一部分。这种方法是不同的，因为他们有不同的医学背景，并在现实生活中提出了广泛的挑战，即,一位38岁的女性患有PHP和长期不受控制的高钙血症（有怀孕相关的PHP病史，切除囊性颌骨肿瘤，以及多囊肾病的家庭和个人阳性诊断，可能是PHP-颌骨肿瘤综合征)，还有,一名患有先天性单肾和慢性肾病相关RHP的26岁女性，透析控制不佳，出现严重贫血和代谢性酸中毒发作（包括一次需要紧急血液透析并并发惊厥性癫痫发作，随后复苏呼吸停止)。两名受试者均表现出严重的PHP/RHP图片，PTH水平>1000pg/mL和>2000pg/mL，血清骨转换标志物升高。此外,他们在肋骨和骨盆（无症状）和脊柱水平有多个棕色肿瘤，头骨,和骨盆（并发自发性颈椎骨折）。作为一种内分泌方法，通过RHP中的医学干预(使用维生素D类似物),通过PHP中的手术(用于甲状旁腺切除术后饥饿骨综合征)控制了基础甲状旁腺疾病.此外,在这种情况下,由于诊断不清楚，采取了多学科的决定进行活检（事实证明没有定论），切除颅骨肿瘤以确认组织学特征。本系列强调了解决整个多学科合并症小组的重要性，以改善PHP/RHP相关棕色肿瘤患者的预后。然而,在现实生活中的医学中，依从性差和对建议的依从性降低可能会损害整体健康状况.因此，有时,考虑到囊性病变级别的直接方法；这代表狭窄的决策框架，这是一个个性化的决定。正如在这里看到的，棕色肿瘤代表PHP/RHP的隐藏面，主要是复杂和严重的形式，即使在现代，意识也是必不可少的。
