A Case of Phosphoglyceride Crystal Deposition Disease in the Pelvic Soft Tissues Recurring after Initial Surgery

Abstract

Phosphoglyceride crystal deposition disease (PGDD) is a rare disease entity that is characterized by phosphoglyceride crystal deposition that stimulates the formation of masses in soft tissue scars or bones. We report a case of PGDD in the pelvic soft tissues that recurred after initial surgical treatment. A 50-year-old woman was referred to our hospital for the evaluation of pelvic masses that were observed on an abdominal ultrasound. Magnetic resonance imaging (MRI) revealed masses in the pelvic region, with the largest being 10 cm in diameter. The masses were diagnosed as ovarian malignant tumors, and an exploratory laparotomy was performed. Operative findings revealed them to be foreign body granulomas, and the patient was diagnosed with PGDD. The patient had a history of cesarean delivery at the age of 24 years. PGDD is extremely rare, but it should be considered in the differential diagnosis of abdominal masses in patients with a history of abdominal surgery.

Phosphoglyceride crystal deposition disease mimicking a malignant tumor

“…The histologic findings for phosphoglyceride crystal deposition disease are known to include clustering of macrophages around the crystals and formation of foreign body granulomas. The crystals are 50–150 μm in diameter, appearing as oval pink or blue aggregates on hematoxylin and eosin staining, arranged in corona-like circles [ 3 ]. On polarized light microscopy, the fibrillar crystals appear refractive [ 3 ].…”

“…The crystals are 50–150 μm in diameter, appearing as oval pink or blue aggregates on hematoxylin and eosin staining, arranged in corona-like circles [ 3 ]. On polarized light microscopy, the fibrillar crystals appear refractive [ 3 ]. The crystals do not dissolve in the usual specimen creation process, but characteristically dissolve in acetic acid, with oxygen gas formation.…”

“…The crystals do not dissolve in the usual specimen creation process, but characteristically dissolve in acetic acid, with oxygen gas formation. They also dissolve easily in high-pH solvents, and stain positively for phosphoglycerides in the gold hydroxamic acid method [ [1] , [2] , [3] ]. Immunohistochemistry shows macrophages accumulating around the numerous crystal deposits, which show weak focal positivity for pan-macrophage marker (CD68), and faint positivity for lysozymes and S-100 protein [ [1] , [2] , [4] ].…”

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

“…To our knowledge, only 10 cases of PGDD have been reported previously, including our case. The available clinical information on these cases is summarized in Table 1 [ 1 – 7 ]. The mean age of patients is 57 (range, 37–76) years.…”

“…Postoperative follow-up has not revealed any findings suspicious of recurrence in our case. However, there are reports in the literature of recurrence at 4 years after surgery [ 7 ]. Few studies have reported on the process of crystallization and deposition of phosphoglycerides and so the mechanism remains unclear.…”