Immunoglobulin G4‐related disease presenting as bilateral ovarian masses and mimicking advanced ovarian cancer

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive infiltration of IgG4(+) plasma cells and fibrosis in various organs. However, the involvement of the ovary in IgG4-RD has never been reported. A 59-year-old woman presented with urinary retention. Magnetic resonance imaging and computed tomography revealed a huge multinodular pelvic mass and common iliac/para-aortic lymph node swelling. A laparotomy was performed under the suspicion of advanced ovarian cancer, and the pelvic mass was identified as ovary in origin. Histopathology of the excised tumor revealed massive lymphoplasmacytic infiltration (>90% were IgG4(+) plasma cells), storiform fibrosis, and obliterative phlebitis; thus leading to a diagnosis of IgG4-RD. We conclude that IgG4-RD can present as a bilateral ovarian mass along with lymphadenopathy, therefore mimicking ovarian cancer.

Igg4-related disease of the ovary

“…In a case reported by Sekulic, the ovarian involvement was unilateral (11). With these clinical findings, the preliminary diagnosis was advanced ovarian malignancy in both our case and Maruyama's case (10).…”

“…Later, lesions similar to those of the pancreas were defined in many organs, including the bile ducts, gallbladder, lymph nodes, retroperitoneum, mesentery, breasts, prostate gland, and skin (3)(4)(5)(6)(7)(8). However, IgG4-RD in the female genital tract has been reported in very few cases (9)(10)(11).…”

A 47-Year-Old Woman with Immunoglobulin G4 (IgG4)-Related Disease Involving the Right Ovary

“…Two cases of IgG4-related disease involving the ovaries have been reported in the literature [ 8 , 9 ]. One, which was reported by Maruyama et al, had a presentation similar to our case, with an ovarian mass mimicking a malignant process [ 8 ], while the second case was an incidental finding after a total abdominal hysterectomy and bilateral salpingo-oophorectomy for symptoms related to leiomyoma [ 9 ].…”

“…Two cases of IgG4-related disease involving the ovaries have been reported in the literature [ 8 , 9 ]. One, which was reported by Maruyama et al, had a presentation similar to our case, with an ovarian mass mimicking a malignant process [ 8 ], while the second case was an incidental finding after a total abdominal hysterectomy and bilateral salpingo-oophorectomy for symptoms related to leiomyoma [ 9 ]. Compared with our case, however, the first case relied mainly on the percent of IgG4-positive plasma cells in relation to the total number of lymphocytes and plasma cells in the inflammatory infiltrate to establish the diagnosis, a method which was criticized by Sekulic et al, who reported the second case, for not being a diagnostic component of the Boston consensus statement criteria.…”

“…To our knowledge, IgG4-related disease involving the ovaries has been reported only twice in the literature [ 8 , 9 ]. The presentation varied between the 2 cases as 1 patient, similar to the patient in our case, presented with an ovarian mass suspicious for malignancy [ 8 ] and the other case was an incidental finding in a patient who had undergone a total abdominal hysterectomy with salpingo-oophorectomy performed as part of management for leiomyoma [ 9 ]. The present report is of a case of IgG4-related disease involving the right ovary.…”