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心包型1型甲状旁腺瘤病:原发性甲状旁腺功能亢进的一种极为罕见的病因。

Pericardial Type 1 Parathyromatosis: A very rare cause of primary hyperparathyroidism.

作者信息

Altin O, Sari R

机构信息

Istanbul Kartal "Dr. Lutfi Kirdar" City Hospital - General Surgery, Istanbul, Turkey.

出版信息

Acta Endocrinol (Buchar). 2020 Oct-Dec;16(4):505-507. doi: 10.4183/aeb.2020.505.

DOI:10.4183/aeb.2020.505
PMID:34084244
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8126381/
Abstract

BACKGROUND

Parathyromatosis is a rare clinical condition where hyperfunctioning parathyroid tissue is scattered in the neck and the mediastinum. It is difficult to diagnose and manage due to recurrent or persistent hyperparathyroidism.

CASE REPORT

We present a peculiar case of hyperparathyroidism as the parathyroid tissue was in unusual location. Scintigraphy done revealed a lesion suspicious for ectopic parathyroid tissue in the retrosternum in a 58 years old female patient with primary hyperparathyroidism. No pathologic lesions were found on neck exploration, subsequently, sternotomy was performed and suspicious lesion removed but microscopic evaluation of the frozen section found no parathyroid tissue in the resected specimen. A decision to perform thymectomy and total resection of pericardial fatty tissue was made. Final histopathology revealed parathyromatosis and confirmed the first case of pericardial type 1 parathyromatosis.

CONCLUSION

Long-term remission is rare in these patients, sometimes needing medical treatment and multiple surgeries. In this patient, there was no recurrence during the four years follow-up. In patients with no history of neck surgery, primary parathyromatosis should be considered in the differential diagnosis of hyperparathyroidism.

摘要

背景

甲状旁腺瘤病是一种罕见的临床病症,其中功能亢进的甲状旁腺组织散布于颈部和纵隔。由于复发性或持续性甲状旁腺功能亢进,其诊断和治疗都很困难。

病例报告

我们呈现了一例特殊的甲状旁腺功能亢进病例,因为甲状旁腺组织位于不寻常的位置。对一名患有原发性甲状旁腺功能亢进的58岁女性患者进行的闪烁扫描显示,在胸骨后有一个可疑的异位甲状旁腺组织病变。颈部探查未发现病理病变,随后进行了胸骨切开术并切除了可疑病变,但冰冻切片的显微镜评估在切除标本中未发现甲状旁腺组织。于是决定进行胸腺切除术和心包脂肪组织全切术。最终组织病理学检查显示为甲状旁腺瘤病,并确诊为第一例心包型1甲状旁腺瘤病。

结论

这些患者很少能长期缓解,有时需要药物治疗和多次手术。在该患者中,四年随访期间未出现复发。在无颈部手术史的患者中,原发性甲状旁腺瘤病应被纳入甲状旁腺功能亢进的鉴别诊断中。

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Ann R Coll Surg Engl. 2019 Nov;101(8):e178-e183. doi: 10.1308/rcsann.2019.0105. Epub 2019 Sep 11.
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Atypical parathyroid adenomas: challenging lesions in the differential diagnosis of endocrine tumors.非典型甲状旁腺腺瘤:内分泌肿瘤鉴别诊断中的具有挑战性病变。
Endocr Relat Cancer. 2019 Jul;26(7):R441-R464. doi: 10.1530/ERC-19-0135.
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Recurrent primary hyperparathyroidism due to Type 1 parathyromatosis.1型甲状旁腺瘤病所致复发性原发性甲状旁腺功能亢进症。
Endocrine. 2017 Feb;55(2):643-650. doi: 10.1007/s12020-016-1139-7. Epub 2016 Oct 14.
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Surgical treatment of primary hyperparathyroidism: description of techniques and advances in the field.原发性甲状旁腺功能亢进症的外科治疗:技术描述及该领域的进展
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Parathyromatosis: a cause for recurrent hyperparathyroidism.甲状旁腺瘤病:复发性甲状旁腺功能亢进的一个病因。
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Surgery. 1994 Jul;116(1):111-5.
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