Clinical management and outcomes of primary ovarian leiomyosarcoma – Experience from a sarcoma specialist unit

Abstract

Ovarian sarcomas account for 1% of all ovarian malignancies and amongst these, primary ovarian leiomyosarcoma is the rarest subtype. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. Only a few cases have been published in the literature and there is very limited knowledge on the clinical behaviour and optimal management of these tumours. We have performed a retrospective analysis of a prospectively maintained database to identify all primary ovarian leiomyosarcoma diagnosed and treated at the Royal Marsden NHS Foundation Trust between 1998 and 2020. Sixteen patients were identified from our database and fifteen were eligible for the analysis. Twelve patients presented with localized disease and underwent initial surgery and three patients had metastatic disease at presentation. Recurrence-free survival post-surgery was 16 months. Eight patients received first-line chemotherapy and four patients received second-line chemotherapy. Two patients had indolent metastatic disease and benefited from local therapies only. The median overall survival in the metastatic setting in our cohort was 51 months, which is consistent with previously published cases. Primary ovarian leiomyosarcoma is an extremely rare malignancy with a poor prognosis. This study is the largest case series of primary ovarian leiomyosarcoma published to date, providing clinically important information regarding survival and metastatic rate as well as treatment outcomes in the metastatic setting.

Recurrent primary ovarian leiomyosarcoma preconception, pregnancy, delivery, and puerperal management: A case report and literature review

“…Desmin, SMA, vimentin, P53, and h‐caldesmon are diffusely and strongly positive based on immunohistochemistry staining 12 . In the current case, desmin, SMA, and vimentin were positive, while S‐100 was negative, and there were ≥10 mitotic figures/hpf, which is consistent with reports in the literature 1,12 …”

“…An ovarian sarcoma is a type of nonspecific stromal cell tumor, including a pure ovarian sarcoma and malignant mixed Mullerian tumors (MMMTs) 5–7 . Pure ovarian sarcoma includes ovarian leiomyosarcoma, fibrosarcoma, and angiosarcoma, etc 1,5,6 . MMMTs can be divided into homologous and heterologous components according to differences in tissue composition 5,7 .…”

“…The main symptoms include lower abdominal distension, abdominal pain, abdominal masses, changes in urination and defecation, and may be accompanied by weight loss and anemia 2,6 . Multiple retrospective analyses have shown that the tumors are mostly unilateral (bilaterality is rare) with more tumors on the right than on the left 1,11 . The tumor marker, CA‐125, is slightly elevated in most patients 2 .…”

“…Most of the tumors are >10 cm in diameter, and the cut surface is fish‐like, accompanied by hemorrhagic necrosis and cystic degeneration 12 . The diagnosis of POLMS mainly depends on ultrasound and MRI findings, and pathological immunohistochemistry 1,6,12,13 . The tumor cells are arranged in a spiral, woven, and fence‐like shape, with multinucleated giant cells, medium‐severe atypia, and ≥10 mitotic figures/hpf 6,12 .…”

“…POLMS is asymptomatic in the early stage, disease progression is rapid, and symptoms are non‐specific in the advanced stage with a high degree of malignancy 2 . Indeed, most patients with POLMS are advanced when diagnosed 1,2 . Herein we report, for the first time, a patient with POLMS who underwent unilateral adnexectomy, and subsequently achieved pregnancy and delivered with a known contralateral tumor.…”