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Phosphoglyceride crystal deposition disease in the abdominal wall: a case report

机译:腹壁磷酸甘油酯晶体沉积病1例

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Abstract BackgroundPhosphoglyceride crystal deposition disease (PGDD) is characterized by phosphoglyceride crystal deposition that simulates neoplasia in soft tissue scars or bone. Reports of PGDDs are rare. Here, we present the case of a patient with PGDD in the abdominal wall.Case presentationA 57-year-old Japanese man with worsening right lower abdominal pain had no significant family or occupational history. Laboratory data showed elevated inflammatory markers with a white blood cell count of 14,400?×?109/L and C-reactive protein of 11.8?mg/L, but no other abnormalities. Helical computed tomography (CT) revealed a tumor in the abdominal wall (longest dimension, approximately 10?cm). Positron emission tomography–CT revealed fluorodeoxyglucose accumulation in the mass only (SUVmax, 41). Clinical and radiographic findings suggested malignant lymphoma, undifferentiated sarcoma, or liposarcoma. He underwent exploratory laparotomy and further treatment. At surgery, we found a huge milky-whitish mass with a rough surface in the transversus abdominis. Complete resection was performed and his postoperative recovery was good. Surprisingly, the final pathologic diagnosis was phosphoglyceride crystal deposition disease with the characteristic crystal deposition in a corolla shape, histiocytic reaction with abundant foreign-body-type giant cells, and no evidence of neoplasia. The patient remains asymptomatic with no disease recurrence.ConclusionAlthough phosphoglyceride crystal deposition disease in the abdominal wall is rarely encountered in clinical practice, its inclusion in differential diagnosis is important. Given the occurrence at sites of invasive procedures, we believe efforts to reduce invasiveness when performing surgery and follow-up for early detection of recurrence are important.
机译:摘要背景磷酸甘油酯晶体沉积病(PGDD)的特征是磷酸甘油酯晶体沉积,可模拟软组织疤痕或骨骼中的赘生物。关于PGDD的报道很少。在此,我们介绍了一名腹壁PGDD患者的病例。病例介绍一名57岁的日本男子右下腹疼痛加重,没有明显的家族或职业病史。实验室数据显示炎性标志物升高,白细胞计数为14400?×?109 / L,C反应蛋白为11.8?mg / L,但没有其他异常。螺旋计算机断层扫描(CT)显示腹壁有肿瘤(最长尺寸约为10?cm)。正电子发射断层扫描(CT)显示,氟脱氧葡萄糖仅在肿块中积聚(SUVmax,41)。临床和影像学检查结果提示恶性淋巴瘤,未分化肉瘤或脂肪肉瘤。他接受了探索性剖腹手术并接受了进一步的治疗。在手术中,我们发现腹横肌表面有粗糙的乳白色块状物。进行了完全切除,术后恢复良好。出乎意料的是,最终的病理学诊断为甘油三酸酯晶体沉积疾病,其特征是晶体呈花冠状沉积,与大量异物型巨细胞发生组织细胞反应,没有瘤形成的迹象。该患者无症状,无疾病复发。结论尽管在临床实践中腹壁磷酸甘油酯晶体沉积疾病很少见,但将其纳入鉴别诊断中仍很重要。鉴于在侵入性手术部位会发生这种情况,我们认为为减少手术时的侵入性和尽早发现复发而进行的随访很重要。

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