Fibromatosis and massive edema of the ovary, possibly related entities: a report of 14 cases of fibromatosis and 11 cases of massive edema.

Twenty-five cases of ovarian enlargement secondary to massive edema, to a hitherto undescribed lesion that we have designated fibromatosis, or to both processes were reviewed to explore the possibility of a relation between them. The patients ranged in age from 13 to 39 years. The 14 patients with pure or predominant fibromatosis usually presented because of menstrual abnormalities; two had evidence of androgen excess and three had abdominal pain. At laparotomy two involved ovaries were observed to have twisted on their pedicles. There was definite bilateral ovarian involvement in two cases and probable bilateral involvement in two others. The excised specimens measured up to 12 cm in diameter and typically had firm, white sectioned surfaces. Microscopic examination showed a proliferation of spindle cells usually separated by dense collagen, which surrounded normal follicular structures. Lutein cells were present in the fibromatous areas in one patient, who was masculinized, and in the adjacent ovarian stroma in another patient, who was hirsute; a proliferation of cells of sex cord type was observed in the fibromatous areas in three cases. Six of the specimens contained focal areas of edema similar in appearance to that seen in cases of massive edema. The features of the 11 cases of massive edema were, in general, similar to those of the 40 examples of this entity reported in the literature. Six of the 11 patients had menstrual abnormalities and/or evidence of androgen excess, but most of them presented because of abdominal pain. At operation an involved ovary was observed to have undergone torsion in five of the cases; bilateral ovarian enlargement due to massive edema was present in two cases. The excised specimens, which measured up to 15 cm in diameter, typically had a watery appearance on sectioning. Microscopic examination showed edematous stroma surrounding residual normal ovarian structures. Lutein cells were identified in the edematous stroma in four cases and in the adjacent ovarian stroma in three of these cases. One of these patients was masculinized and another was hirsute. Small foci of fibromatosis similar to that seen in cases of pure fibromatosis were present in eight cases. The similar age range and clinical manifestations of these two processes and the overlap in their histologic features suggest that they are closely related and may reflect differing morphologic expressions of the same underlying disorder. Some of the cases of massive edema, however, may result from the development of stromal edema in ovaries involved by hyperthecosis.