背景:磷酸间充质肿瘤(PMT)是罕见的间充质肿瘤,与长期存在的,在受影响的患者中,非特异性但通常使人衰弱的症状。这些肿瘤表现出特征性的组织病理学特征,及时识别,可以为患者带来福音,因为切除是完全治愈的。
目的:评估10例PMT的临床和组织病理学特征,在我们的机构诊断,以及这些患者的临床结果。
方法:这是一项回顾性研究,其中10个PMT,2013年1月至2022年7月诊断,包括在内。
结果:诊断时的平均年龄为40岁,M:F比为4:1。临床特征包括肿块,弱点,骨痛,移动和行走困难,和病理性骨折。生化分析显示血清钙水平正常(平均=9.5mg/dL),低血清磷(平均=2.2mg/dL)和升高的血清成纤维细胞生长因子23(FGF23)水平,在所有情况下,无论哪里可用。在组织病理学上,所有肿瘤都显示细胞排列为血管外皮细胞瘤,包括椭圆形到短的主轴形式。多核巨细胞存在于九个肿瘤中,在八个肿瘤中观察到特征性的“粗糙钙化”。在八个肿瘤中可见明显的假性囊性间隙。在任何肿瘤中均未见到大量有丝分裂图和肿瘤坏死。在五个可以进行随访的案例中,手术切除后症状完全缓解,无复发或转移.所有这些患者在最后一次随访之前都没有疾病。
结论:这是对我国这些罕见肿瘤的首次最大的综合性研究。可以根据某些组织病理学特征以及与临床放射和生化发现的相关性来诊断PMT。这些总是良性肿瘤。充分手术切除肿瘤后,患者的衰弱症状得到缓解。因此,他们正确及时的诊断至关重要。
BACKGROUND: Phosphaturic mesenchymal tumors (PMTs) are rare mesenchymal tumors, associated with long-standing, non-specific but often debilitating symptoms in the affected patients. These tumors display characteristic histopathological features and in case, identified timely, can be a boon for patients, given an excision is completely curative.
OBJECTIVE: To evaluate the clinical and histopathological features of 10 PMTs, diagnosed at our institution, along with clinical outcomes in those patients.
METHODS: This was a retrospective study, wherein 10 PMTs, diagnosed from January 2013 to July 2022, were included.
RESULTS: The average age at the time of diagnosis was 40 years with an M:F ratio of 4:1. Clinical features included lumps, weakness, bone pain, difficulty in moving and walking, and pathologic fractures. The biochemical analysis showed normal serum calcium levels (average = 9.5 mg/dL), with low serum phosphorus (average = 2.2 mg/dL) and raised serum fibroblast growth factor 23 (FGF23) levels, in all the cases, wherever available. On histopathology, all tumors showed cells arranged in a hemangiopericytomatous pattern, including oval to short spindle forms. Multinucleate giant cells were present in nine tumors, and characteristic \"grungy calcifications\" was observed in eight tumors. Prominent pseudo cystic spaces were seen in eight tumors. A significant number of mitotic figures and tumor necrosis were not seen in any tumor. In five cases where follow-up was available, there was complete resolution of symptoms post-resection with no recurrence or metastasis. All those patients were free of disease until the last follow-up.
CONCLUSIONS: This constitutes the first largest comprehensive study on these rare tumors from our country. PMTs can be diagnosed based on certain histopathological features and correlation with clinicoradiological and biochemical findings. These are invariably benign neoplasms. Patients are relieved of their debilitating symptoms after adequate surgical tumor resection. Therefore, their correct and timely diagnosis is crucial.