Parathyroid cancer: An update

Parathyroid cancer (PC) is a rare malignant tumor which comprises 0.5–5% of patients with primary hyperparathyroidism (PHPT). Most of these cancers are sporadic, although it may also occur as a feature of various genetic syndromes including hyperparathyroidism-jaw tumor syndrome (HPT-JT) and multiple endocrine neoplasia (MEN) types 1 and 2A. Although PC is characterized by high levels of serum ionized calcium (Ca) and parathyroid hormone (PTH), the challenge to the clinician is to distinguish PC from the far more common entities of parathyroid adenoma (PA) or hyperplasia, as there are no specific clinical, biochemical, or radiological characteristic of PC. Complete surgical resection is the only known curative treatment for PC with the surgical approach during initial surgery strongly influencing the outcome. In order to avoid local recurrence, the lesion must be removed en-bloc with clear margins. PC has high recurrence rates of up to 50% but with favorable long-term survival rates (10-year overall survival of 60–70%) due to its slow-growing nature. Most patients die not from tumor burden directly but from uncontrolled severe hypercalcemia. In this article we have updated the information on PC by reviewing the literature over the past 10 years and summarizing the findings of the largest series published in this period.     

原发性甲状旁腺功能亢进的骨骼X线分析(附36例报告)

本文分析了36例原发性甲状旁腺功能亢进的骨骼X线改变,认为主要的骨病征象可概括为指骨的骨膜下骨吸收、颅骨的磨玻璃样及颗粒状改变、骨皮质下的囊肿样改变和棕色瘤,骨的突起或肌腱韧带附着处的骨吸收,以及软骨下骨吸收。骨皮质下囊变的征象对鉴别诊断很有帮助。作者认为骨质疏松和反复泌尿系结石可提示甲状旁腺功能亢进的早期表现,应及时检查血钙磷以确诊。     

Hypercalcemic hyperparathyroidism and hypophosphatemic osteomalacia complicating neurofibromatosis

Summary Neurofibromatosis is sometimes complicated by impaired renal tubular reabsorption of phosphate, hypophosphatemia, and osteomalacia. Hyperparathyroidism has also been reported in patients with neurofibromatosis. When hypercalcemia and elevated levels of parathyroid hormone are found in osteomalacia, however, it may be difficult to determine if the hyperparathyroidism was primary or tertiary. We describe a patient with neurofibromatosis, hypercalcemic hyperparathyroidism, hypophosphatemic osteomalacia, vitamin D deficiency, and clear-cell hyperplasia of all four parathyroid glands. Serial biomechanical, bone biopsy, and densitometric studies confirmed that treatment with ergocalciferol, calcium, and phosphate supplements significantly improved the osteomalacia but caused increased parathyroid overactivity. After subtotal parathyroidectomy, the parathyroid hormone concentration became normal and the bone mineral content increased at the spine and hip, but inappropriate phosphaturia persisted. The findings indicate that hyperparathyroidism, osteomalacia, and vitamin D deficiency adversely affect each other.     

A case of polycythemia vera complicated by chronic renal failure under hemodialysis requiring parathyroidectomy for secondary hyperparathyroidisim

A case of polycythemia vera complicated by chronic renal failure under maintenance hemodialysis requiring parathyroidectory (PTH) for secondary hyperparathyroidism (2° HPT) is reported. A 62 year old female presented with 75000 white blood cells (WBC)/μl, 703×10⁴ red blood cells (RBC)/μl, 23×10⁴ platelets (PLT)/μl, hyperuricemia and hypertension in 1970 and the diagnosis of polycythemia vera was made. Hemodialysis was started in October 1974 for chronic renal failure. Blood cells in peripheral blood rapidly decreased in number after the beginning of dialysis, reaching the level of 10000∼20000 WBC/μl, and 150∼250×10⁴RBC/μl. In August 1988, marked bone resorption in X-ray picture and high serum alkaline phosphatase and parathyroid hormone (PTH) noted along with 17400 WBC/μl, 370×10⁴RBC/μl and 35.9×10⁴PLT/μl. After subtotal PTX removing 3.21g parathyroid gland, serum PTH rapidly fell. At 3 months after PTX, WBC rose to 23600/μl, RBC 372×10⁴/μl and PLT 94.0×10⁴/μl. At 6 months, WBC was to 31000/μl, RBC 429×10⁴/μl and PLT 78.0×10⁴/μl, suggesting an inhibitory action of PTH on not only RBC, but also WBC and PLT.     

B超和彩色多普勒超声对原发性甲状旁腺功能亢进的诊断价值

报道应用Ｂ型超声及彩色多普勒超声对７６例原发性甲状旁腺功能亢进症进行系统研究的结果。总的诊断效率：敏感性７２％，特异性９２％，正确率８７％，阳性预测值６９％，阴性预测值９２％，以正常位置腺瘤的诊断效率最高，Ｂ型超声及彩色多普勒超声的诊断效率进行比较，敏感性有显著差异（Ｐ＜０．０５），正确率有非常显著差异（Ｐ＜０．０１），说明彩色多普勒超声的诊断正确率较高。本文还讨论了Ｂ型超声对甲状旁腺病变的定位价值、各类甲状旁腺病变的鉴别；同时对异位甲状旁腺病变、原发性甲旁亢危象、各种影像诊断的比较等也进行了讨论。     

原发性甲状腺机能亢进症的手术探查技巧

目的探讨原发性甲状腺机能亢进症的手术探查技巧和经验教训。方法对50例原发性甲状腺机能亢进症患者临床资料进行回顾性总结分析。结果48例患者手术治疗，术后38例诊断为甲状旁腺瘤，6例为甲状旁腺增生，4例为甲状旁腺癌。48例中44例第一次手术探查成功（占88％），2例行2次手术，2例行3次手术。46例术后痊愈，2例并发多处肋骨骨折发生呼吸衰竭而死亡。结论确诊为原发性甲状腺机能亢进症伴有临床征象者均应手术治疗。术前病灶定位明确者手术较容易，定位不明确者应在“热区内”重点探查。     

Multiple brown tumors in a patient with nutritional secondary hyperparathyroidism

Summary For years, brown tumors have been considered to be a characteristic of primary hyperparathyroidism. However, since 1963 several reports indicate the incidence of brown tumors in patients with renal secondary hyperparathyroidism to be 1.5%–1.7%. The appearance of multiple brown tumor lesions is rather uncommon in secondary hyperparathyroidism which is also true for malabsorption as its cause. We report on a 56-year-old man presenting with pain in the bones and multiple osteolyses. A bone biopsy specimen and the laboratory examinations were indicative of secondary hyperparathyroidism caused by malabsorption most likely due to Billroth's II/I gastric resection. Thus, the patient's osteolyses represent brown tumors which have been induced by nutritional secondary hyperparathyroidism.     

慢性肾功能衰竭患者血清C—PTH变化与并发症的关系探讨

探讨慢性肾功能衰竭(CRF)患者血清羧基端甲状旁腺素(C-PTH)的变化及与钙磷代谢、高血压和贫血的关系。方法44例CRF患者用放免法测定血清C-PTH,血清尿素氮(BUN)、血清肌酐(Scr)、血清钙(Ca     

肾性骨病的骨膜下骨吸收

笔者采用手骨直接Ｘ线放大摄影和双手Ｘ线平片来研究慢性肾衰血透病人５１例。特征性的指骨骨膜下吸收放大摄影发现２５例占４９％;平片为１１例占２１．６％,结果表明,放大摄影的阳性率明显高于平片（Ｐ＜０．００５）,骨膜下吸收的二,三指中间指骨桡侧及指骨末端是肾性骨病的“靶区”,认为二者无显著差异（Ｐ＞０．５０）。骨膜下吸收还与血透时间,血磷,血钙和碱性磷酸酶水平相关。     

Alphacalcidol oral pulses normalize uremic hyperparathyroidism prior to dialysis

Alphacalcidol oral pulse therapy was given for secondary hyperparathyroidism to 22 children (mean age of 5.6 years) with renal insufficiency. At the beginning of the study, the glomerular filtration rate was <50% of normal, serum intact parathyroid hormone (PTH) was >100 ng/l and the serum phosphate and calcium concentrations were within the normal range. Alphacalcidol (0.5–3.0 g) was given orally thrice weekly in the evening and adjusted according to PTH, ionized calcium and phosphate concentrations. Serum PTH (mean ± SEM) decreased significantly from a pre-treatment level of 393±81 ng/l to 122±34 ng/l after 12 months, and stabilized at this level. Mean vitamin D metabolite concentrations were within the normal range. 1,25-Dihydroxyvitamin D did not increase during therapy, while PTH decreased. The estimated creatinine clearance remained almost unchanged (20±3 and 21±6 ml/min per 1.73 m²). Growth remained low normal (height standard deviation score –1.8±0.3 initially and –1.7±0.4 12 months later) and bone mineral density did not decrease. We concluded that feedback regulation of PTH with oral alphacalcidol pulse therapy is effective in the treatment of hyperparathyroidism in children with renal failure prior to dialysis.