Low-grade Fibromyxoid Sarcoma Presenting Clinically as a Primary Ovarian Neoplasm

Abstract

Low-grade fibromyxoid sarcoma is an uncommon, deceptively bland mesenchymal neoplasm that typically occurs in the deep soft tissues of the proximal extremities of young to middle-aged patients. Intra-abdominal low-grade fibromyxoid sarcomas are distinctly rare. We describe the first reported example of this sarcoma involving the ovary. The 42-year-old patient presented with progressing abdominal pain and urinary frequency. Computed tomographic imaging of the abdomen and pelvis showed a 14-cm left-sided pelvic mass. The patient underwent surgical resection and, intraoperatively, a left ovarian mass was identified that extended to both the left hypogastric artery and the left ureter. The resected specimen consisted of a 17-cm tan, fleshy mass containing grossly recognizable nodules of gelatinous, myxoid tissue and a small rim of normal ovary. Microscopic examination of the neoplasm revealed a cytologically bland spindle cell proliferation set in a collagenized stroma that abruptly transitioned to nodules of a myxoid stroma with a well-formed capillary vasculature, features characteristic of low-grade fibromyxoid sarcoma. On the basis of the diagnosis, the patient was clinically staged as a retroperitoneal sarcoma with secondary ovarian involvement. The patient has stable residual disease 11 months postoperatively. This case adds to the literature of intra-abdominal low-grade fibromyxoid sarcoma and expands the list of malignant mesenchymal neoplasms that may involve the ovary.

Low Grade Fibromyxoid Sarcoma

“…[2][3][4][5] Increasingly, LGFMS is being reported at unusual sites, including inside solid organs. 5,[9][10][11][12] The tumor is well known for its propensity for local recurrence, with metastasis also seen in a subset of cases. 3,5 Surgical excision remains the treatment of choice, although some patients received additional chemotherapy and radiation therapy.…”

A huge low-grade fibromyxoid sarcoma of small bowel mesentery simulating hyper immune splenomegaly syndrome: A case report and review of literature

“…It is commonly reported to arise from the deep soft tissues of the lower extremities, particularly the thigh, but occasionally reported to arise also, from the chest wall, axilla, inguinal region, buttock, neck, the mediastinum and brain 2 . Abdominal LGFMS is extremely rare, it had been reported to arise from small bowel mesentery, colon, falciform ligament, retroperitoneum, and the ovary 3,4,5 .They are commonly seen in the young or middle-aged adult, although it can occur even at the extremes of life.The literature on LGFMS is still sparse, although it is growing. All the report on this disease comes from western country and Asia.…”

Rapidly enlarging low-grade fibromyxoid sarcoma with intracranial extension in a 5-year-old girl: case report

“…There is some suggestion that the published series with lower recurrence rates had higher rates of initial gross-total resection. 2,3,5,6,18 Surgery is the treatment of choice for local recurrence and for metastatic lesions. Other authors have reported treatment with chemotherapy or radiation therapy, but there is no convincing evidence that this alters the outcomes.…”

“…Chondrosarcoma tends to be positive for vimentin and S-100; this tumor was negative. [11][12][13][16][17][18] The tumor was negative for cytokeratin and smooth muscle actin which, along with vimentin and S-100, are suggestive of liposarcoma. Myxofibrosarcoma show much more cellular atypia than those in this tumor.…”

“…Myxofibrosarcoma show much more cellular atypia than those in this tumor. [11][12][13][16][17][18] Myxoma was also considered. Our patient's tumor had much less collagen and is less cellular than would be expected in a myxoma.…”