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甲状旁腺功能亢进颌骨肿瘤综合征:一例放射学视角的病例报告

Hyperparathyroidism Jaw-Tumor Syndrome: A Case Report From a Radiological View.

作者信息

Khan Muhammad Mehraiz, Fazli Huda, Bilal Khan Touseef, Tehrany Pooya M, Bacani Niempa

机构信息

Radiology, Institute of Nuclear Medicine & Oncology Lahore, Lahore, PAK.

Radiology, Services Hospital, Lahore, PAK.

出版信息

Cureus. 2022 Aug 23;14(8):e28329. doi: 10.7759/cureus.28329. eCollection 2022 Aug.

Abstract

Limited formal guidelines, scarcity of cases, and variable manifestation forms all contribute to the challenges of diagnosing hyperparathyroidism-jaw tumor (HPT-JT) syndrome. This condition characterized by parathyroid tumors, fibro-osseous jaw tumors, and renal and gynecological pathologies results in significant morbidity, restricted functionality, and malignancy risk. Genetic testing is the gold standard investigation to evaluate for CDC73 mutations, that cause HPT-JT syndrome. Genetic testing for CDC73 mutations should be encouraged among family members of affected individuals. Surgery is the mainstay of treatment for many of the encountered pathologic entities. We report a 42-year-old female with a history of infertility and right subtrochanteric femoral fracture secondary to osteoporosis. The patient was suspected to have primary hyperparathyroidism secondary to parathyroid adenomas that were later biochemically and scintigraphically proved with subsequent partial parathyroidectomy. One and a half years following the initial presentation, the patient developed gradual swelling of the lower face with regional osseous involvement in addition to the clinical and radiological picture of recurrent parathyroid adenoma. We present this rare diagnosis of HPT-JT syndrome to promote awareness among physicians regarding this essential differential diagnosis. A low threshold for genetic testing and a high index of suspicion for HPT-JT syndrome must be kept in cases of patients presenting with high parathyroid hormone levels and masses. The screening must extend to all the family members as well. With this approach, the high morbidity, facial disfigurement, and significant malignancy risk can be lowered in the affected individuals improving their life expectancy.

摘要

有限的正式指南、病例稀缺以及表现形式多样,这些都给甲状旁腺功能亢进-颌骨肿瘤(HPT-JT)综合征的诊断带来了挑战。这种以甲状旁腺肿瘤、纤维性骨化性颌骨肿瘤以及肾脏和妇科病变为特征的疾病会导致严重的发病率、功能受限以及恶性肿瘤风险。基因检测是评估导致HPT-JT综合征的CDC73突变的金标准检查。对于受影响个体的家庭成员,应鼓励进行CDC73突变的基因检测。手术是许多所遇到的病理实体的主要治疗方法。我们报告了一名42岁女性,有不孕史,因骨质疏松继发右转子下股骨骨折。该患者疑似患有继发于甲状旁腺腺瘤的原发性甲状旁腺功能亢进,随后经生化和闪烁扫描证实,并进行了部分甲状旁腺切除术。初次就诊一年半后,患者除了出现复发性甲状旁腺腺瘤的临床和影像学表现外,还出现了面部下部逐渐肿胀并伴有局部骨质受累。我们呈现这例罕见的HPT-JT综合征诊断,以提高医生对这一重要鉴别诊断的认识。对于甲状旁腺激素水平升高和有肿块的患者,必须保持较低的基因检测阈值和对HPT-JT综合征的高度怀疑指数。筛查还必须扩展到所有家庭成员。通过这种方法,可以降低受影响个体的高发病率、面部畸形和重大恶性肿瘤风险,提高他们的预期寿命。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f3d/9500404/9feca77829b3/cureus-0014-00000028329-i01.jpg

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