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

Abstract

A primary ovarian leiomyosarcoma (POLMS) is a rare malignant tumor of the ovary. Herein, we report a patient with an early‐stage POLMS who underwent unilateral adnexectomy in our department with a subsequent pregnancy, delivery, and tumor recurrence. In this case, the patient was a 29‐year‐old female with a complaint of abdominal distention who underwent a right adnexectomy for a solid tumor in the right ovary. The pathological diagnosis was a stage IA leiomyosarcoma of the right ovary. One month later, a recurrence in the left ovary tumor was diagnosed. The patient did not agree to further treatment. She subsequently achieved a spontaneous pregnancy and had an uneventful pregnancy and delivery at term. During the puerperium, she underwent radical surgery and chemotherapy with the recommended follow‐up evaluation. The literature review shows the pathogenesis of POLMS has not been established. There are no specific tumor markers, and it is difficult to distinguish the nature and origin of the tumor with imaging modalities, thus, early diagnosis is difficult. The malignancy rate is high, and the prognosis of advanced tumors is poor. To properly counsel patients to optimize treatment, more reports of cases, the disease course, treatments, and outcomes are needed.

Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature

“…Table 2 shows the main clinical features of all 113 cases of POLMS reported in the literature. [6,10,15,16,20,44,56,57,62,65], anorexia weight loss was reported in 7/52 (13.5%) patients [6,10,15,16,20,44,56,57,62,65], urinary disorders were reported in 6/50 (12%) patients [4,19,27,47,53,61], weakness was reported in 5/52 (11.5%) patients [34,35,37,46,65], constipation was reported in 3/52 (5.8%) [5,28,38], fever was reported in 3/50 (6%) patients [37,46,65], rectal hemorrhage was reported in 1/52 (1.9%) patients [65], metrorrhagia was reported in 2/52 (3.8%) patients [33,63] and vaginal prolapse was reported in 1/50 (2%) patients [67]. A total of 3/52 (5.8%) patients were asymptomatic [15,40,59].…”

“…Information about POLMS treatment was reported in 80/113 (70.8%) patients [1,2,[4][5][6]9,10,15,16,[19][20][21][22][25][26][27][28][29][30][31][32][33]. POLMS was treated by surgical resection in 76/80 (95%) patients [1,2,[4][5][6]9,10,15,16,19,20,22,[25][26][27][28][29][30][31][32][33]. A total of 34/80 (42.5%) patients received surgery without lymphadenectomy [2,9,13,14,16,19,20,…”

“…POLMS was treated by surgical resection in 76/80 (95%) patients [1,2,[4][5][6]9,10,15,16,19,20,22,[25][26][27][28][29][30][31][32][33]. A total of 34/80 (42.5%) patients received surgery without lymphadenectomy [2,9,13,14,16,19,20,22,26,[28][29][30][34][35][36][37]39,40,43,[57][58][59][60]64,65]…”

“…The tumor size was available in 73/113 (64.6%) patients [1,5,6,10,13,15,16,19-22,25-28, 31,34,36-41,44,46,48,51-65], ranging from 33 to 350 mm, with a mean size of 151.2 mm (SD +/− 68.5). CA 125 was reported in 32/113 (28.3%) patients [1,[4][5][6]10,[13][14][15][16]34,38,40,41,[47][48][49][50][51][52][53][54][55][56][57]59,[61][62][63][65][66][67], 19/32 (59.4%) patients had a normal value [4][5][6]14,15,34,38,40,47,49,52,53,56,57,59,61,62,…”

“…As it is a very rare tumor, it is not possible to identify clear guidelines regarding staging, treatment and prognosis. Recently, some literature reviews have tried to clarify the treatment and prognosis of patients with POLMS, though including only a part of the cases reported in the literature and analyzing only a part of the known potential prognostic factors [2,3,16]. Here, we reviewed the English literature since 1951, trying to obtain the largest possible sample of patients and analyzing all known prognostic factors, from clinical to histopathological, in order to identify the best treatment and clarify the outcome of POLMS patients.…”