Proximal epithelioid sarcoma of the vulva: A case report and review of the literature

Abstract

Primary sarcomas of the vulva are rare, with an incidence of 1.5% to 5% of all vulvar malignancies. There are only 41 cases described in the English literature of proximal epithelioid sarcoma of the vulva, which also constitutes a highly aggressive neoplasm with a high rate of recurrence and metastasis. We are reporting the first case of vulvar epithelioid sarcoma reported in our country, Peru, and Latin America. We present the case of a 53-year-old woman with no significant history who presented a large tumor mass of 25 cm in the left hemivulva of 2 years of evolution, associated with intense pain. She underwent radical surgical resection with left inguinopelvic lymphadenectomy and reconstruction of Rectus Abdominal Muscle Myocutaneous Flaps (TRAM).

Clinicopathological Characteristics, Treatment, and Survival in Patients Diagnosed With Proximal-Type Epithelioid Sarcoma: A Case Report and Systematic Review

“…In the vulva, the presentation of each lesion was different; in one of the cases, it presented as an ulcerated and indurated lesion [ 23 ], while in other cases, the presence of painless masses of variable growth was observed [ 3 , 24 ]. The size of the tumors was variable according to the location, with a minimum of 1.1 cm to a maximum of 33 cm [ 22 , 24 ].…”

“…The presence of hemorrhage [ 15 , 17 , 22 , 33 , 40 , 42 ] and necrosis [ 10 , 15 - 19 , 21 - 23 , 25 - 27 , 32 - 34 , 36 , 37 , 40 - 42 , 47 ] were common findings present in eight and 23 cases, respectively. High mitotic activity was reported in 18 tumors [ 3 , 4 , 10 , 12 , 16 , 18 , 19 , 22 , 24 , 25 , 31 , 32 , 35 , 37 , 40 - 42 ], and in four of them, more than 20 mitoses were observed per 10 high-power fields [ 4 , 22 , 35 , 40 ].…”

“…High mitotic activity was reported in 18 tumors [ 3 , 4 , 10 , 12 , 16 , 18 , 19 , 22 , 24 , 25 , 31 , 32 , 35 , 37 , 40 - 42 ], and in four of them, more than 20 mitoses were observed per 10 high-power fields [ 4 , 22 , 35 , 40 ]. Vascular invasion was reported in four cases [ 22 , 32 , 36 ]. Other histologic features were spindle cell proliferation (14 cases) [ 16 , 19 , 22 , 23 , 26 , 27 , 29 , 30 , 32 , 33 , 35 , 37 , 41 , 42 ], presence of signet ring cells (one case) [ 4 ], anaplasia (two cases) [ 23 , 47 ], and pseudoangiosarcomatous features (one case) [ 35 ].…”

“…However, 17 studies did not include this variable [10,17]. Thirty-seven patients (67.28%) presented with an initial sign of a rapidly growing palpable mass, mostly painful [3,9,[18][19][20][21][22]. Other symptoms and signs were presented according to the affected area like headaches, neck pain, nausea, vomiting, abdominal pain, abdominal fullness, visual disturbances, palpebral ptosis, eye pain and protrusion, ectropion, epiphora, anorexia, weight loss, dyspnea, edema in the affected area, constipation, fever, rectal bleeding, vaginal bleeding, and epistaxis.…”

“…The presence of hemorrhage [15,17,22,33,40,42] and necrosis [10,[15][16][17][18][19][21][22][23][25][26][27][32][33][34]36,37,[40][41][42]47] were common findings present in eight and 23 cases, respectively. High mitotic activity was reported in 18 tumors [3,4,10,12,16,18,19,22,24,25,31,32,35,37,[40][41][42], and in four of them, more than 20 mitoses were observed per 10 high-power fields [4,22,35,40]. Vascular invasion was reported in four cases [22,32,…”