同侧双侧甲状旁腺腺瘤，其中一例为多生腺瘤，一例为异位腺瘤：病例报告
抽象的
双腺瘤 (DA) 是原发性甲状旁腺功能亢进症 (PHPT) 的一个独特临床表现。DA 可能遵循各种胚胎分布模式，并且可能是多余的和/或异位的。
我们描述了第一例 PHPT 病例，其是由同侧双腺瘤引起的，其中一个是异位的和多余的。一名 45 岁的希腊男性患者因右下甲状旁腺腺瘤而被诊断为 PHPT，他被送入我们科室接受手术治疗。术前检查（颈部超声、Sestamibi 扫描）确诊为单腺疾病。患者接受了重点甲状旁腺切除术。冰冻切片显示甲状旁腺腺瘤，有轻微的甲状旁腺癌可能性。切除后 10 分钟，完整 PTH (iPTH) 与术前值相比下降了 50% 以上，并在正常范围内。由于存在甲状旁腺癌的可能性，进行了右半甲状腺切除术和额外的同侧中央颈部解剖术。最终病理报告显示，切除的第一个组织被证实为甲状旁腺腺瘤，同时还发现了第二个包膜下组织和正常的右上甲状旁腺。
术前使用常规影像学检查对 DA 进行定位以及术中甲状旁腺激素测定在检测多个腺瘤方面仍然不可靠。此外，可能存在第二个同时存在多余和异位腺瘤的可能性很小。因此，当怀疑有 DA 时，建议使用更先进的影像学检查（例如 4D-CT、4D-MRI）或其他诊断工具。
背景
原发性甲状旁腺功能亢进症（PHPT）是一种常见的内分泌疾病，据估计美国每年有10万例新发病例。PHPT的特征是甲状旁腺激素（PTH）分泌过多，导致钙和磷酸盐异常。大多数PHPT病例由单个良性腺瘤引起（85%-90%），少数病例（15%-20%）由多个腺瘤或增生引起，极少数病例由甲状旁腺癌引起（1%）[1,2,3]。近年来，越来越多的共识认为双腺瘤（DA）是一种独特的临床疾病，不应与非同步型四腺体增生相混淆。据报道，在所有PHPT患者中，DA的发病率高达15%[4,5,6,7]。
异位或多余的甲状旁腺是由于甲状旁腺细胞从第三和第四鳃囊异常移位到最终位置而引起的。此外，一个（或多个）异位甲状旁腺腺瘤的发生率为 6% 至 16%，而多余的甲状旁腺腺瘤的发生率甚至更低，约为 5% [8,9,10]。但是，异位和多余的功能亢进腺的发生率仅为 0.69% 左右 [11]。Mila 等对 127 名 DA 患者进行了调查，发现 DA 与多余的或异位的甲状旁腺共存的情况很少，但正常（127 名患者中分别为 4 名和 3 名）[7]。本文，我们报道了第一例因同侧 DA 导致的 PHPT 病例，其中一名患者同时存在异位和多余的甲状旁腺。
病例介绍
A 45 year-old Greek male with PHPT admitted to our department for parathyroidectomy due to diagnosed PHPT caused by benign parathyroid adenoma. The patient was an Officer in Hellenic Air Force, married without children. On admission, he reported fatigue as the only symptom he experienced and the vital signs were normal (blood pressure: 135/65 mmHg, heart rate: 72 pulses/min, temperature: 36.8 °C). Our findings during the physical examination were the following: 1. head: eyes, nose, mouth, ears were normal, 2. neck: without any swelling area and trachea in the central position, 3. anterior torso: thorax and abdomen without abnormalities during observation, normal breath sounds, normal heart sounds and normal abdominal sounds, 4. posterior torso: without any spinal curvature or lung deformity, 5. pelvic and rectal examination: normal, 6. upper and lower extremities: without any abnormalities. Moreover, the results of our neurological examination were: 1. normal mental status, 2. cranial nerves evaluation: normal findings, 3. normal reflexes, 4. motor function and balance: normal findings, 5. sensory examination: normal findings, 6. coordination examination: normal findings. His medical history was free and there was not any family history for hypercalcemia or multiple endocrine neoplasia syndromes. The patient was not taking any medication before diagnosis. Besides, no allergies were reported and the patient did not smoke or consume alcohol. Laboratory tests revealed a serum total calcium of 2.8 mmol/l (normal range 2.05–2.55 mmol/l) with an elevated PTH of 204.70 pg/ml (normal range 15.00–68.30 pg/ml). Serum phosphate was 3.2 mg/dl (normal range: 2.5–4.5 mg/dl), 25 hydroxyvitamin-D level was 27.2 ng/ml (normal range: 20–50 ng/ml) and 24-h urine calcium level was 202.1 mg/24 h (normal range: 100–300 mg/24 h). Thyroid function tests were normal. The detailed laboratory results are reported in Table1. Neck ultrasound revealed that the thyroid gland had normal dimensions and there was an inferior right-sided 23 x 11 x 13 mm extrathyroid nodule, indicative of parathyroid adenoma (Fig.1). Furthermore, the patient underwent99mTc–Sestamibi scintigraphy (MIBI) and the result was in accordance with the neck ultrasound (Fig.2). Under the diagnosis of PHPT and with a CaPTHUS score of 4 [Intact PTH level ≥ 2 times upper limit of normal PTH level, Sestamibi scan results positive for 1 enlarged parathyroid gland, Neck ultrasound results positive for 1 enlarged thyroid gland and concordant sestamibi and neck ultrasound study results (identifying 1 enlarged gland on the same side of the neck)] indicative for single gland disease, the patient was admitted for focused parathyroidectomy [12]. However, intraoperatively, we did not find any parathyroid gland at the lower pole of the right thyroid lobe. Therefore, we continued with a right neck exploration and only a normal parathyroid gland was recognized on the superior right pole of the thyroid. Thus, we decided to proceed on exploring the left side of the neck which revealed two normal parathyroid glands. Nevertheless, after repeated attempts to discover the pathologic right parathyroid gland, we noticed a small bulging at the right lower thyroid pole (Fig.3: blue arrow) suspicious for an intrathyroidal parathyroid adenoma. Prior to the attempt to dissect this bulging, we finally found an enlarged nodule, morphologically similar to parathyroid adenoma. This was at the right side of the neck in connection with the carotid sheath, far away from the thyroid gland [Type C based on the nomenclature of parathyroid classification (Table2) [13], which was excised and sent for frozen section (Fig.4). Ten minutes after the excision of the suspected adenoma, a blood sample was taken and the intact PTH level dropped and was within normal range (49.30 pg/ml), indicating a reduction of more than 50% from the preoperative level. The frozen section report showed a “parathyroid neoplasm, with a low possibility of parathyroid carcinoma, due to the presence of fibrous bands”. Based on these findings, we decided to perform right hemithyroidectomy with additional ipsilateral central neck dissection (Fig.3). In the immediate postoperative period and until discharge, paracetamol (1000 mg, four times per day), nonsteroidal anti-inflammatory drugs (lornoxicam 4 mg, twice per day) and omeprazole (20 mg, twice per day) were administered intravenously. The patient received and tolerated well liquid and solid food six hours after the procedure. In the first postoperative day, serum calcium, phosphate and PTH levels were 2.175 mmol/l, 1.04 mmol/l (normal range: 0.74–1.52 mmol/L) and 45.80 pg/ml respectively. Τhe patient discharged without any complications, in the second postoperative day. To our surprise, the final pathology report revealed that: (1) the first dissected “parathyroid neoplasm” was a parathyroid adenoma and not a carcinoma, with 2.7 cm maximum diameter, (2) there was one smaller subcapsular parathyroid adenoma, with 1.1 cm maximum diameter, located at the lower pole of the right thyroid lobe (Fig.5) and (3) a normal parathyroid gland was found at the inferior border of the right central neck dissection specimen. Finally, eighteen months after surgery, patient’s serum calcium, serum phosphate and PTH levels were still within normal range (2.425 mmol/l, 1.06 mmol/l and 55.0 pg/ml respectively), confirming a successful, curative parathyroidectomy for PHPT. Furthermore, the patient did not report any symptom and physical and neurological examination did not reveal any pathological signs.
图 1
甲状腺超声检查显示右侧下方有结节，提示为甲状旁腺腺瘤
图 2
99mTc–Sestamibi 闪烁显像显示右侧下方甲状旁腺腺瘤
图 3
右半甲状腺切除术标本，其中切除了右侧中央区域的其他淋巴结（箭头显示右下甲状腺极的小凸起）
图 4
切除异位甲状旁腺
图 5
右甲状腺叶下极包膜下甲状旁腺腺瘤病理图像
讨论
在我们的研究中，我们介绍了首例由同侧 DA 引起的 PHPT 病例，其中一只为异位且多生的 DA，另一只为包膜下 DA。我们表明 DA 是一种真正独立的临床实体，它可能导致 PHPT 患者的治疗和手术计划出现困难或陷阱。尽管人们已经做了很多工作来查明腺瘤的发病机制以及腺瘤与甲状旁腺增生的区别，但腺瘤是仅通过克隆还是通过多克隆机制产生仍然存在争议，即使大多数腺瘤都是单克隆增殖的 [14]。然而，临床证明 DA 代表了一种独特的临床情况，应该与增生区分开来。医学文献报道，PHPT 患者切除两个腺瘤后长期治愈率很高，这是 DA 是一种独立疾病的最好证据 [4]。此外，外科医生对该疾病的认识不断提高，以及有助于发现腺瘤和终止手术

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