Furuzono Nozomi, Togami Shinichi, Komazaki Hiromi, Nagata Chikako, Mizuno Mika, Yanazume Shintaro, Kobayashi Hiroaki
Department of Obstetrics and Gynecology, Faculty of Medicine, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520 Japan.
Int Cancer Conf J. 2024 Jul 31;13(4):440-444. doi: 10.1007/s13691-024-00706-1. eCollection 2024 Oct.
Phosphoglyceride crystal deposition disease is a rare condition occurring in soft tissues, resulting in scarring and affecting the bones, making preoperative differentiation from malignant tumors challenging. Here, we describe a case of phosphoglyceride crystal deposition disease initially suspected to be a malignant ovarian tumor before surgery. A 50-year-old woman with a history of three cesarean sections presented with lower abdominal pain. Transvaginal ultrasonography revealed a 54 × 58 mm tumor in the lower right abdomen. Pelvic contrast-enhanced magnetic resonance imaging showed a thickened cystic wall with diffusion restriction, a low signal intensity region on T1-weighted images, and a slightly high signal intensity region on T2-weighted images. The tumor markers, cancer antigen 125 and carbohydrate antigen 19-9 levels, were within normal ranges; however, positron emitting tomography-computed tomography revealed fluorodeoxyglucose accumulation (SUVmax 31.28) in the tumor wall. Suspecting a malignant ovarian tumor, a laparoscopy was performed to observe the abdominal cavity. A 10 cm white solid tumor was identified in the midline of the lower abdominal wall, leading to an open surgery recommendation. The tumor, adhering to the pubic bone, was excised. The tumor measured 9 × 7 cm, with the cut surface showing a yellow brownish solid periphery and central region with liquefied degeneration. Histologically, radial basophilic deposits, dense infiltration of macrophages, multinucleated giant cells, and foam-like tissue spheres were observed. The central region exhibited cholesterol clefts, fibrin exudation, and necrosis, leading to a diagnosis of phosphoglyceride crystal deposition disease. This disease is rare, occurring in patients with atypical fluorodeoxyglucose accumulation on positron emission tomography-computed tomography or with a history of tissue damage, such as abdominal surgery.
磷酸甘油酯晶体沉积病是一种罕见的发生于软组织的疾病，可导致瘢痕形成并累及骨骼，使得术前与恶性肿瘤进行鉴别具有挑战性。在此，我们描述一例术前最初被怀疑为恶性卵巢肿瘤的磷酸甘油酯晶体沉积病病例。一名有三次剖宫产史的50岁女性因下腹部疼痛就诊。经阴道超声检查发现右下腹有一个54×58mm的肿瘤。盆腔增强磁共振成像显示囊壁增厚且有弥散受限，在T1加权图像上为低信号强度区域，在T2加权图像上为稍高信号强度区域。肿瘤标志物癌抗原125和糖类抗原19-9水平在正常范围内；然而，正电子发射断层扫描-计算机断层扫描显示肿瘤壁内有氟脱氧葡萄糖积聚（最大标准摄取值31.28）。怀疑为恶性卵巢肿瘤，遂行腹腔镜检查以观察腹腔。在下腹壁中线处发现一个10cm的白色实性肿瘤，因此建议行开放手术。该肿瘤与耻骨相连，予以切除。肿瘤大小为9×7cm，切面显示外周为黄棕色实性，中央区域有液化变性。组织学检查可见放射状嗜碱性沉积物、巨噬细胞密集浸润、多核巨细胞以及泡沫样组织球。中央区域可见胆固醇裂隙、纤维蛋白渗出和坏死，最终诊断为磷酸甘油酯晶体沉积病。这种疾病很罕见，见于正电子发射断层扫描-计算机断层扫描显示氟脱氧葡萄糖异常积聚或有组织损伤史（如腹部手术）的患者。
