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原发性甲状旁腺功能亢进-颌骨肿瘤综合征:一种容易混淆且被遗忘的诊断。

Primary hyperparathyroidism-jaw tumor syndrome: a confusing and forgotten diagnosis.

作者信息

Piciu Doina, Piciu Andra, Barbus Elena, Pestean Claudiu, Larg Maria Iulia, Fetica Bogdan

机构信息

Department of Nuclear Medicine and Endocrinology, Ion Chiricuta Institute of Oncology, Cluj-Napoca, Romania; Department of Nuclear Medicine and Endocrinology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.

Department of Medical Oncology, Ion Chiricuta Institute of Oncology, Cluj-Napoca, Romania; Department of Medical Oncology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.

出版信息

Clujul Med. 2016;89(4):555-558. doi: 10.15386/cjmed-638. Epub 2016 Oct 20.

Abstract

BACKGROUND

Primary hyperparathyroidism is caused by the excessive growth of parathormone secretion, its consequence being hypercalcemia. The parathyroid adenoma is responsible for over half of primary hyperparathyroidism cases. The mandibular tumor can be the initial sign in the case of primary hyperparathyroidism.

CASE PRESENTATION

We present the case of a 33 year old patient with history of a mandibular operated tumor, repetitive pathological fractures and hypercalcemia manifestations. The level of the parathormone at the first measurement indicated a very high value. The parathyroid scintigraphy with 99mTc-MIBI (methoxy-isobutyl-isonitrile) evidenced a high uptake of the tracerin the superior mediastinum, suggestive for an ectopic parathyroid adenoma. The histopathological examination after surgery leads to the diagnosis of parathyroid adenoma. The association between the primary hyperparathyroidism, the mandibular tumour, the clinical history and the nuclear imaging lead to the diagnosis of primary hyperparathyroidism - Jaw tumor syndrome.

CONCLUSION

The hyperparathyroidism - Jaw tumor syndrome has a special clinical importance because of the severe and progressive symptomatology, and because of the risk of developing neoplasia of parathyroid glands, which have a reserved prognosis.

摘要

背景

原发性甲状旁腺功能亢进症是由甲状旁腺激素分泌过度增加引起的,其结果是高钙血症。甲状旁腺腺瘤是原发性甲状旁腺功能亢进症病例中超过半数的病因。下颌肿瘤可能是原发性甲状旁腺功能亢进症的首发症状。

病例介绍

我们报告一例33岁患者,有下颌肿瘤手术史、反复病理性骨折及高钙血症表现。首次测量时甲状旁腺激素水平显示极高值。用99mTc - MIBI(甲氧基异丁基异腈)进行的甲状旁腺闪烁显像显示上纵隔对示踪剂摄取增高,提示异位甲状旁腺腺瘤。手术后的组织病理学检查确诊为甲状旁腺腺瘤。原发性甲状旁腺功能亢进症、下颌肿瘤、临床病史及核素显像之间的关联确诊为原发性甲状旁腺功能亢进症 - 颌骨肿瘤综合征。

结论

甲状旁腺功能亢进症 - 颌骨肿瘤综合征具有特殊的临床重要性,这是因为其症状严重且呈进行性,还因为存在甲状旁腺发生肿瘤的风险,而其预后不佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59c5/5111498/22e92efa78d9/cm-89-555f1.jpg

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