2018
DOI: 10.1016/j.ijscr.2018.06.016
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Myoepithelial carcinoma or epithelioid sarcoma – A rare diagnosis with poor prognosis. A case report and review of literature

Abstract: HighlightsMyoepithelial carcinoma of the vulva and epithelioid sarcoma has overlapping histological, immunohistochemical and genetic features.Similarities between two diagnoses in aggressive vulvar cancer should be noted as a sarcoma-based chemotherapy regimen should be considered.We recommend immediate surgical resection with bilateral superficial and deep inguinal lymph node dissections in these aggressive cancers.Identification of SMARCB1-deficiency trials of targeted therapies such as EZH2 inhibitors which… Show more

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Cited by 5 publications
(6 citation statements)
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“…The most recent case was reported by Khazeni et al and discussed a 33-year-old female that presented with pelvic pain ( Khazeni et al, 2018 ). A CT scan of the abdomen and pelvis reveled a 3.6 × 3.1 cm right inguinal mass and the patient underwent an excision and biopsy.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…The most recent case was reported by Khazeni et al and discussed a 33-year-old female that presented with pelvic pain ( Khazeni et al, 2018 ). A CT scan of the abdomen and pelvis reveled a 3.6 × 3.1 cm right inguinal mass and the patient underwent an excision and biopsy.…”
Section: Discussionmentioning
confidence: 99%
“…The most common location for myoepithelial carcinoma is the salivary glands, namely the parotid gland, but tumors have also been found in the skin, breast, lung and other soft tissues ( Frost et al, 2014 ). To our knowledge, only seven cases of primary myoepithelial carcinoma of the vulva have been reported ( Miyata et al, 2011 , Fukunaga, 2003 , Hinze et al, 1999 , Khazeni et al, 2018 , Meenakshi and McCluggage, 2009 , Noronha et al, 2006 ). These tumors often present with lobulated, firm, yellow/white or tan masses that are most often painless ( Hornick and Fletcher, 2003 ).…”
Section: Introductionmentioning
confidence: 99%
“…On the genetic level myoepithelial carcinoma is characterized by various abnormalities related to the predominant cell subtype with an EWSR-PBX1 fusion known for the clear cell variant and the EWSR-ZNF444 translocation known for the epithelioid cell variant [12]. In the rhabdoid variant of myoepithelial carcinoma of the soft tissue of the neck for example, FISH analysis revealed an EWSR Translocation without any other genetic fusions characteristic of EWSR, including EWSR1-NR4A3, TAF15-NR4A3, EWSR1-FLI1, EWSR1-ERG, EWSR1-WT1, EWSR1-ATF1, EWSR1-CREB1 or EWSR1-POU5F1 [13].…”
Section: Discussionmentioning
confidence: 99%
“…Radical resection with enough margin is a guarantee of oncological control for VS [6,21]. Unfortunately, all patients in our cohort were initially treated at community and none of them received wide resection at primary surgery.…”
Section: Discussionmentioning
confidence: 99%
“…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].…”
Section: Introductionmentioning
confidence: 99%