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Case Reports
. 2022 Jan 21:39:100933.
doi: 10.1016/j.gore.2022.100933. eCollection 2022 Feb.

Successful management of vulvar proximal-type epithelioid sarcoma in pregnancy

Yuji Orita  1   2 , Masaki Kamio  1 , Akio Tokudome  1   2 , Ikumi Kitazono  3 , Fumino Ichihara  1 , Hiroaki Kobayashi  1
Affiliations
Case Reports

Successful management of vulvar proximal-type epithelioid sarcoma in pregnancy

Yuji Orita et al. Gynecol Oncol Rep. .

Abstract

Vulvar proximal-type epithelioid sarcoma during pregnancy is extremely rare; only two reports are available to date. Herein, we describe a 36-year-old woman who presented with a pigeon-egg-sized solid mass with cystic component on the left labia majora at 18 weeks of gestation. The patient underwent tumor resection at 23 weeks of gestation and was diagnosed with epithelioid sarcoma, proximal-type. At 29 weeks of gestation, elective cesarean section, radical local resection of the vulva and vagina, and inguinal lymphadenectomy were performed. After surgery, she underwent six courses of adjuvant chemotherapy (doxorubicin 60 mg/m2 and cisplatin 50 mg/m2) every four weeks. The patient and her baby survived with neither recurrence nor complications until 5 years. Aggressive management for proximal-type epithelioid sarcoma, such as early termination of pregnancy and operation, can improve maternal outcomes.

Keywords: Long outcome; Pregnancy; Proximal-type epithelioid sarcoma; Vulvar.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1a
Fig. 1a
HE stain (×200). The tumor cells are epithelioid with abundant eosinophilic cytoplasm and round nuclei with prominent nucleoli.
Fig. 1b
Fig. 1b
Immunohistochemical staining of INI-1 (×200). Loss of INI-1 expression in nuclear of tumor cells; background inflammatory cells positive for INI-1.
See this image and copyright information in PMC

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