Proximal-type epithelioid sarcoma—unusual presentation: unilateral vulvar mass

Abstract

Epithelioid sarcoma of the vulva is a very rare malignant soft tissue tumour, usually initially asymptomatic, which simulates a variety of benign lesions, leading to diagnosis only at later stages. Prognosis is generally poor. We report a case of a 55-year-old postmenopausal woman, who presented with rapid growth of an asymptomatic mass located in the left labia majora. Biopsy of the lesion revealed features characteristic of proximal-type epithelioid sarcoma. Ganglionar, adrenal, pulmonary and hepatic metastases were found at thoracoabdominopelvic CT scan. During hospitalisation, the patient's clinical condition suffered rapid deterioration and she died on the 16th day of admission.

Successful management of vulvar proximal-type epithelioid sarcoma in pregnancy

“…Therefore, approximately one-third of these cases are initially misdiagnosed as a Bartholin’s gland cyst or abscess ( Matsuo et al, 2009 ), as observed in this case. Misdiagnosis often leads to tumor progression and worse prognosis due to delayed treatment ( Altundag et al, 2004 , Matsuo et al, 2009 , Rodrigues et al, 2015 , Tjalma et al, 1999 ). The mean age of PES occurrence is approximately 27.8–35 years, similar to that in patients with other sarcomas ( Altundag et al, 2004 , Matsuo et al, 2009 , Rodrigues et al, 2015 , Tjalma et al, 1999 ).…”

“…Malignancies of the vulva are rare ( Altundag et al, 2004 ). Particularly, proximal-type epithelioid sarcoma (PES) of the vulva is extremely rare; it accounts for only 1–3% of vulvar malignancies ( Rodrigues et al, 2015 , Tjalma et al, 1999 ). However, clear management guidelines and optimal therapies for this malignancy have not been established.…”

“…Overall, 27.0% died of the disease ( Han et al, 2016 ). When ES occurs in Bartholin’s gland, it can be misdiagnosed as benign tumor and lead to tumor advancement ( Matsuo et al, 2009 , Rodrigues et al, 2015 , Tjalma et al, 1999 ). Early diagnosis and appropriate treatment are essential to improve prognosis and survival in patients with ES.…”

An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures

“…The first case of PES of the vulva was described by Pier et al in 1972 [ 8 ]. It often affects the labia majora of young women, and its differential diagnoses include Bartholin’s cyst, lipoma, and genital warts [ 7 , 9 ]. Its benign appearance as an asymptomatic subcutaneous nodule poses diagnostic and therapeutic dilemmas for clinicians [ 8 - 10 ].…”

Clinical Features and Treatment Outcomes of Vulvar Sarcoma: A Single-Institution Review of 21 Cases

“…The histological type of VS is heterogeneous and mainly includes leiomyosarcoma, dermato brosarcoma protuberans, angiosarcoma, rhabdomyosarcoma and epithelioid sarcoma [3,4]. Unlike other vulvar malignancies, VS usually grows with benign appearance and non-speci c manifestation, which increases the di culty of early diagnosis [5]. The biological behavior of VS is featured by frequent recurrence and high potential to metastasize, for which the initial treatment is quite important [6].…”