Altin O, Sari R
Istanbul Kartal "Dr. Lutfi Kirdar" City Hospital - General Surgery, Istanbul, Turkey.
Acta Endocrinol (Buchar). 2020 Oct-Dec;16(4):505-507. doi: 10.4183/aeb.2020.505.
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.
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.
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.
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。
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。
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。