Liebisch M, El Hamrawi N, Dufour M, Nöllner F, Krenn V
Medizinische Fakultät, Sigmund Freud PrivatUniversität, Wien, Österreich.
MVZ für Histologie, Zytologie und Molekulare Diagnostik Trier GmbH, Max-Planck-Str. 5, 54296, Trier, Deutschland.
Z Rheumatol. 2024 May;83(4):277-282. doi: 10.1007/s00393-023-01402-w. Epub 2023 Aug 24.
The tenosynovial giant cell tumor (pigmented villonodular synovitis) is a proliferative, mainly benign soft tissue tumor of the tendon sheaths, bursae and joints arising from the synovia. It can be divided into circumscribed localized and destructive diffuse types. Approximately 1% of all joint diseases are due to this entity. The tumor is considered as a rarity. Mostly case studies exist. For this study the focus was set on the localized type (L-TSRZT), which accounts for 90% of the diagnoses of this tumor. Given its rarity, data are limited. Therefore, the research aim was to provide data on prevalence, primary location and sensitivity of clinical versus histopathological diagnosis in a German sample.
Based on the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology, the data of the L‑TSRZT were retrospectively analyzed (time frame 1 January 2018-28 December 2020).
This database contained N = 7595 cases of arthropathy. A total of n = 45 patients with the diagnosis L‑TSRZT were identified. The prevalence of the tumor was 0.6%, 95% CI [0.4%, 0.8%], or 5.9 cases per 1000. The primary location involved the finger (48.9%). In 14 of 45 cases the diagnosis was correctly determined from the clinical side, corresponding to a sensitivity of 31.1%, 95% CI [18.2%, 46.7%].
For the first time, this paper was able to provide data on a large sample for Germany. Notably, the low sensitivity of the clinical diagnosis confirms the importance of histopathology for diagnosing L‑TSRZT.
腱鞘巨细胞瘤（色素沉着绒毛结节性滑膜炎）是一种起源于滑膜的、主要为良性的腱鞘、滑囊和关节的增殖性软组织肿瘤。它可分为局限性和弥漫性破坏型。所有关节疾病中约1% 由该病变引起。该肿瘤被认为较为罕见。大多是病例研究。本研究聚焦于局限性类型（L-TSRZT），其占该肿瘤诊断的90%。鉴于其罕见性，数据有限。因此，研究目的是提供德国样本中该肿瘤的患病率、原发部位以及临床诊断与组织病理学诊断的敏感性数据。
基于德国骨科学风湿病学会的组织病理学关节炎登记册，对L-TSRZT的数据进行回顾性分析（时间范围为2018年1月1日至2020年12月28日）。
该数据库包含N = 7595例关节病病例。共确定了n = 45例诊断为L-TSRZT的患者。该肿瘤的患病率为0.6%，95%置信区间[0.4%，0.8%]，即每1000人中有5.9例。原发部位累及手指（48.9%）。45例中有14例从临床方面正确确诊，对应敏感性为31.1%，95%置信区间[18.2%，46.7%]。
本文首次能够提供德国大样本的数据。值得注意的是，临床诊断的低敏感性证实了组织病理学对诊断L-TSRZT的重要性。
