Successful management of vulvar proximal-type epithelioid sarcoma in pregnancy

Abstract

Highlights Vulvar proximal-type epithelioid sarcoma during pregnancy is extremely rare. Immediate and agressive treatment is essential even in pregnancy, and vulvectomy during pregnancy is considered safe. Further reserch is required to discuss about the effectiveness of adjuvant therapy.

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

“…A total of 55 patients were included, in whom the age range fluctuated between nine and 87 years, of which 18 patients (32.73%) were men and 37 (67.27%) were women, and two were pregnant [ 15 , 16 ]. The time from the onset of signs and symptoms and diagnosis ranged from seven days to 36 months.…”

“…Also, it frequently presents rhabdoid differentiation, cellular atypia, and the absence of a granulomatous pattern [ 5 , 9 , 11 , 25 , 35 , 40 ]. Occasionally, it can present pseudoangiomatous architecture and spindle cells [ 12 , 15 ]. The presence of foci of necrosis and mitotic activity, which is usually low (less than five per high-power field), are unfavorable histological features [ 3 , 10 , 24 ].…”

“…Regarding treatment, 19 patients were treated surgically by wide local excision [1,4,11,12,19,[25][26][27][28][29][30][31][32][33][34], five with surgery plus lymphadenectomy [18,24,[35][36][37], seven underwent surgery and adjuvant radiotherapy [17,20,21,36,38,39], one underwent surgery and chemotherapy [26], six had surgery, chemotherapy, and adjuvant radiotherapy [13,16,17,[40][41][42], four underwent surgery, lymphadenectomy, and radiotherapy [23,36,43,44], one had surgery, lymphadenectomy, and chemotherapy [15], two underwent surgery, lymphadenectomy, radiotherapy, and chemotherapy, three received palliative radiotherapy and chemotherapy [36,45,46], and four underwent treatment with chemotherapy alone [9,10,33]. However, one patient, with metastases at presentation, received only palliative care [3] and two patients refused treatment...…”

“…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].…”