DataSheet_1_Case Report and Systematic Review: Sarcomatoid Parathyroid Carcinoma—A Rare, Highly Malignant Subtype.pdf

BackgroundParathyroid carcinoma (PC) is a rare malignancy, the incidence of which is less than 1/1 million per year. Sarcomatoid parathyroid carcinoma (SaPC) is an extremely peculiar subtype; only three cases have been reported internationally. It consists of both malignant epithelial components and sarcomatoid components (mesenchymal origin) simultaneously. This “confusing” cancer exhibits higher invasiveness, and traditional surgery does not appear to achieve the expectation, which differs significantly from that of general PC.
ObjectiveTo characterize the clinicopathologic features of SaPC and explore similarities and differences between SaPC and general PC.
Materials and MethodsWe collected clinical data of SaPC cases from our center and literature. The SaPC case in our center was presented. To better understand the characteristics of SaPC, we also reviewed clinical information in general PC cases from our center and literature within the last 5 years, and a systematic review was performed for further comparison.
ResultsA 60-year-old woman was admitted for a neck mass and hoarseness. After the surgery, she was confirmed as SaPC and ultimately developed local recurrence at 3 months. Together with the reported cases from literature, four cases of SaPC (three cases from literature) and 203 cases of general PC (200 cases from literature) were reviewed. Both tumors showed obvious abnormalities in parathormone (PTH) level and gland size. Compared to general PC, SaPC has a later age of onset (60.50 ± 7.42 vs. 51.50 ± 8.29), relatively low levels of PTH (110.28 ± 59.32 vs. 1,156.07 ± 858.18), and a larger tumor size (6.00 ± 1.63 vs. 3.14 ± 0.70). For SaPC, all four cases were initially misdiagnosed as thyroid tumors (4/4). Spindle cell areas or transitional zones were common pathological features in SaPC cases (3/4).
ConclusionSaPC is a very rare pathologic subtype of PC and appears to be much more easily misdiagnosed as a thyroid tumor. Spindle cell areas or transitional zones are highly possible to be pathological features in its sarcomatoid components. Despite many similarities, there are some differences between SaPC and general PC—SaPC does not show the obvious endocrine feature but stronger aggressiveness. Surgical treatment of SaPC does relieve life-threatening symptoms and improve quality of life even with recurrence in the short term.

背景：甲状旁腺癌（Parathyroid carcinoma, PC）是一种罕见的恶性肿瘤，年发病率不足1/100万。肉瘤样甲状旁腺癌（Sarcomatoid parathyroid carcinoma, SaPC）是一种极为特殊的亚型，目前国际上仅报道过3例。该肿瘤同时包含恶性上皮成分与肉瘤样间叶起源成分。这种“易混淆”的癌症侵袭性更强，传统手术疗效未达预期，与普通型甲状旁腺癌存在显著差异。
目的：明确肉瘤样甲状旁腺癌的临床病理特征，并探讨其与普通型甲状旁腺癌的异同。
材料与方法：我们从本中心及文献中收集肉瘤样甲状旁腺癌病例的临床资料，对本中心的肉瘤样甲状旁腺癌病例进行了详细呈现。为更全面地了解该亚型的特征，我们同时回顾了本中心及近5年文献报道的普通型甲状旁腺癌病例的临床信息，并开展系统综述以进行进一步对比分析。
结果：本次纳入的1例患者为60岁女性，因颈部肿块伴声音嘶哑入院。术后病理确诊为肉瘤样甲状旁腺癌，术后3个月出现局部复发。结合文献报道的病例，本次研究共回顾了4例肉瘤样甲状旁腺癌病例（其中3例来自文献）以及203例普通型甲状旁腺癌病例（其中200例来自文献）。两类肿瘤均表现为甲状旁腺激素（parathormone, PTH）水平及腺体体积的明显异常。与普通型甲状旁腺癌相比，SaPC患者的发病年龄更晚（60.50±7.42 vs 51.50±8.29），PTH水平相对更低（110.28±59.32 vs 1156.07±858.18），肿瘤体积更大（6.00±1.63 vs 3.14±0.70）。在肉瘤样甲状旁腺癌病例中，所有4例患者初始均被误诊为甲状腺肿瘤（4/4）；梭形细胞区域或移行带是其常见的病理特征（3/4）。
结论：肉瘤样甲状旁腺癌是甲状旁腺癌中极为罕见的病理亚型，且更易被误诊为甲状腺肿瘤。梭形细胞区域或移行带极有可能是其肉瘤样成分的典型病理特征。尽管二者存在诸多相似之处，但肉瘤样甲状旁腺癌与普通型甲状旁腺癌仍存在部分差异：肉瘤样甲状旁腺癌无明显内分泌特征，但侵袭性更强。即便短期内出现复发，外科手术治疗仍可缓解危及生命的症状并改善患者的生活质量。