Oncogenic Osteomalacia Associated with Phosphaturic Mesenchymal Tumor of the Knee: Case Presentation and Review of the Literature

Oncogenic osteomalacia (OOM) is an uncommon metabolic and bone disease caused by fibroblast growth factor 23 (FGF23), a phosphaturic factor produced by phosphaturic mesenchymal tumors (mixed connective tissue variant, PMTMCTV) characterized by phosphate leakage from kidneys and subsequent hypophosphatemia. In this paper, we present the case of a patient, 42-year-old woman affected by left side limp and pain involving lumbar spine, pelvis and hip joints, referred to the Rheumatology Department of our Hospital for the treatment of a suspected sero-negative spondilo-arthritis. During hospitalization patient began an immuno-suppressive therapy with TNF-alpha inhibitors associated with Pamidornate, Indometacin, Esomeprazole and vitamin D3. Nevertheless pain did not decrease and a new examination found a worst hypophosphatemia (1 mg/dl) with normal Ca and PTH’s plasma values. During the same check-up a painful bulge on the anterior part of the right knee was observed and the Magnetic Resonance Imaging scan revealed an ovular solid lesion in the soft tissue closed to the upper part of the patella. Histological analysis identified the lesion as a PMTMCTV. After surgical removal patient got complete recovery. We will discuss about diagnostic evaluation, differential diagnosis and treatment.

良性肝脏肿瘤肝移植相关进展

肝脏肿瘤分为良性和恶性,其中良性肝脏肿瘤较为常见,治疗以随访观察、介入治疗为主,仅少数情况下需要进行外科手术切除治疗。肝移植可有效治疗终末期肝病,但通常不作为良性肝脏肿瘤的治疗手段,仅存在巨大占位压迫、合并肝衰竭、肿瘤破裂出血风险或恶变风险的良性肝脏肿瘤才考虑进行肝移植治疗。与恶性肿瘤及慢性肝衰竭肝移植相比,良性肝脏肿瘤肝移植手术风险相当,预后及远期生存较为理想。但良性肝脏肿瘤肝移植术前应充分谨慎评估,同时注意新形势下供肝匹配难度。本文对良性肝脏肿瘤包括肝血管瘤、多囊肝、肝细胞腺瘤、肝脏间叶错构瘤等肝移植相关研究进展进行总结,旨在为肝移植治疗良性肝脏肿瘤提供参考。

原发性肾脏血管母细胞瘤临床病理特征1例

原发肾脏血管母细胞瘤属于肾脏良性间叶源性肿瘤,发病罕见。大多数为散发病例,部分病例报道与VHL综合征相关。本文报道1例散发性原发肾脏血管母细胞瘤,镜下见瘤细胞胞浆丰富,呈上皮样形态,网状排列,血管丰富,免疫组化表达a-inhibin、VIM及NSE。同时复习整理相关文献资料,增加医师对原发肾脏血管母细胞瘤临床病理特点的了解,从而为患者选择更优更合适的治疗方案。临床疑似病例可行术前穿刺活检明确诊断,优先选择部分肾切除术,以保留更多肾功能。