A proximal type epithelioid sarcoma of the vulva with multiple distant metastases: A case report and review of the literature

Chung, Hyewon; Jang, Tae-Kyu; Kwon, Sun Young; Ha, Jinkyeong; Shin, So Jin

doi:10.1016/j.gore.2021.100835

Cited by 4 publications

(4 citation statements)

References 15 publications

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“…ES of the vulva is extremely rare and comprises approximately 1% of soft tissue sarcomas. To the best of our knowledge, 40 cases have been reported till date ( Chung et al, 2021 ). The median age of such patients is 34 years (range 17 to 80), similar to other vulvar sarcomas, including the present study (median = 32 years) ( Table 1 ) ( Noh et al, 2021 ).…”

Section: Discussionmentioning

confidence: 99%

Proximal-type epithelioid sarcoma of vulva – Case series of a rare tumor

Dash

Rekhi

Shylasree

et al. 2022

Gynecologic Oncology Reports

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Highlights Proximal-type Epithelioid sarcoma of the vulva is a rare, aggressive soft tissue neoplasm seen in young and middle-aged females. Biopsy of the lesion with immunohistochemistry aids in diagnosis and differentiating from benign mimics. Loss of INI1 is characteristic and seen in the majority. Surgery is the mainstay of management in localized disease. Recently, Tazemetostat has obtained accelerated FDA approval for use in locally advanced and metastatic settings.

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Section: Discussionmentioning

confidence: 99%

Proximal-type epithelioid sarcoma of vulva – Case series of a rare tumor

Dash

Rekhi

Shylasree

et al. 2022

Gynecologic Oncology Reports

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“…This tumor is characterized by aggressive behavior and poor prognosis [ 8 , 41 , 43 , 45 , 46 , 48 ]. According to studies, poor prognostic factors are regional lymph node involvement, early metastasis, high-grade tumors, vascular invasion, tumor size greater than 2 cm, deep location, and presence of necrosis [ 3 , 18 , 47 ].…”

Section: Discussionmentioning

confidence: 99%

“…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...…”

Section: Authormentioning

confidence: 99%

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

Buitron¹,

Panduro²,

Morales³

2022

Cureus

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Epithelioid sarcoma is a rare entity that shows a predilection for the young and middle-aged population. There are two subtypes, i.e., the distal or conventional type, which is located in distal extremities, and the proximal type, located in proximal areas of the extremities, pelvis, perineal, and genital region. The latter is characterized by more aggressive behavior, a higher recurrence rate, and poor prognosis. Histopathological and immunohistochemical diagnoses are key to correct and timely treatment and a higher survival rate. We report a case of a 41-year-old man who presented a palpable progressive growth mass in the hypogastrium. The disease time was of nine months, and the tumor was resected, but it recurred a few months later in the same location. Computed tomography (CT) scans showed images suggestive of lung metastasis and the patient had to undergo a second surgery. He received eight cycles of chemotherapy and a subsequent CT scan control showed the progression of the disease, so a new chemotherapy regimen was established. The patient received three cycles of chemotherapy without improvement, so he decided to discontinue treatment. His last outpatient medical consultation was in January 2022. A systematic review of the studies published in PubMed and Google Scholar was performed. We identified 291 articles, but only 41 reports and case series were included, with a total of 55 patients. It is important to include this type of tumor in the differential diagnosis of epithelial tumors due to its aggressive behavior. Correct and timely diagnosis is crucial to obtain lower recurrence rates, lower mortality, and higher survival rates in these patients.

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Proximal-type epithelioid sarcoma of the vulva initially misdiagnosed as Bartholin cyst: A case report

Costa

Barros²,

Júnior³

et al. 2022

gocm

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A proximal type epithelioid sarcoma of the vulva with multiple distant metastases: A case report and review of the literature

Abstract: Highlights Epithelioid sarcomas (ESs) are rare and aggressive malignancy of a poor prognosis. Radiotherapy may be an alternative treatment to decrease the size of the lesion. Targeted therapy with pazopanib may be a treatment option for soft tissue sarcomas.

Cited by 4 publications

References 15 publications

Proximal-type epithelioid sarcoma of vulva – Case series of a rare tumor

Proximal-type epithelioid sarcoma of vulva – Case series of a rare tumor

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

Proximal-type epithelioid sarcoma of the vulva initially misdiagnosed as Bartholin cyst: A case report

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