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

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.

Phosphoglyceride crystal deposition disease involving adnexa uteri: a case report with histogenetic consideration

“…These findings indicate that PG crystals separated from old hemorrhagic foci in the center of tumors, although it is not clear whether old hemorrhagic foci in the tumor are due to endometriosis or surgical procedures for total hysterectomy performed 44 years ago. Some authors speculated that local disturbance of PG metabolism within macrophages is initiated by local inflammation and undigested material accumulates and crystallizes 1 , 4 , 7 , 9 , whereas, in the present case, the foreign-body reaction seems to be a secondary change against the deposited PG crystals. In the previously reported cases also, the tumors showed cystic changes in the center in three cases 1 , 2 , 7 ; one contained old hematoma 2 and the other had necrotic tissue, fibrinous exudate and cholesterin crystals 7 .…”

“…PCDD is extremely rare; to the best of our knowledge, only 12 cases, all from Japan, have been reported to date 1-10 . In 10 of these cases, the lesions developed at the site of postoperative scar or repeated injections on the body surface (abdominal wall 1 , 3 , 9 , buttock 2 and upper arm 3 ) as well as in internal organs (peritoneum 4 , spine 5 , anterior mediastinum 6 , 8 , cardiac wall 10 , and pelvic wall 7 ) a long time (20-45 years) after operation/injection. These findings suggest that tissue injury may play an important role in PG deposition, while its developmental process is still largely unknown.…”

“…The PG crystals are characterized by refringence under polarized light and positive staining by the gold hydroxamic acid (GHA) method 11 . PCDD is extremely rare and usually develops at the site of surgical incision or that of repeated injections on the body surface 1-3 , 9 as well as in internal organs 4-8 , 10 . These facts suggest the role of tissue injury in the deposition and formation of PG crystals 1 .…”

Phosphoglyceride crystals deposition disease: a rare pseudosarcomatous growth with peculiar predilections

“…The mainstay treatment of PCDD is localised excision surgery. 5,6,11 However, some patients were subjected to more extensive surgery, as described by Miura et al 1 in which the patient underwent multiple resections and extensive surgeries, and Nakahara et al 11 where the patient underwent exploratory laparotomy instead of laparascopic surgery, mainly because a malignancy could not be safely ruled out clinically and radiologically.…”

Phosphoglyceride crystal deposition disease in the intestine

“…Phosphoglyceride crystal deposition has been noted at intramuscular injection sites or postoperative sites such as the gluteal muscles, scapula, spine, mediastinum, as well as soft tissue of the abdominal wall and abdominal cavity. [1][2][3][4][5] Only 16 cases have been reported (including our case), of which four reported phosphoglyceride crystal deposition in the pericardium after cardiac surgery (Supporting Information: Table S1). In our case, phosphoglyceride crystals were deposited at the anastomotic site after ileocecal resection 33 years previously.…”