Extraskeletal Chondrosarcoma of Labium Majus

Khan, Arshad S.; Bakhshi, Girish D.; Shaikh, Aftab; Khan, Ashraf; Khan, Adil Aziz; Chitale, Arun R.

doi:10.1155/2011/429562

Cited by 10 publications

(10 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The differential diagnosis of MELTVR also includes extraskeletal myxoid chondrosarcoma (EMC) due to its uniform, loosely cohesive tumor cells in a myxoid matrix. EMC is an extremely rare subtype of vulvar sarcoma that is consistently positive for vimentin, variable positivity for S100 protein, neuron-specific enolase and synaptophysin, completely negative for CK [10][11][12][13][14][15][16], and harhor EWSR1-NR4A3 fusion in about 65% of cases [17]. However, the present case showed S100 negativity, EMA, ER positivity, and EWSR1 was intact showed by FISH assay.…”

Section: Discussioncontrasting

confidence: 49%

Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

Gao

2020

Diagn Pathol

View full text Add to dashboard Cite

Background: Myoepithelioma-like tumor of the vulvar region (MELTVR) is a recently described mesenchymal neoplasm which typically arising in vulvar regions of adult women. Case presentation: Here we report a case of a 65-year-old woman who presented with a 6-year history of subcutaneous mass in the vulvar region. The mass had recently increased in size continuously. Histologically, the tumor cells had an epithelioid to spindled shape. Epithelioid tumor cells proliferated singly or in a loosely cohesive manner with myxoid areas, while spindled tumor cells grew in diffuse sheets or storiform arrangements mainly in nonmyxoid areas. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, calponin, and were partially mild to moderate positive for estrogen receptor, but completely negative for S100 protein, glial fibrillary acidic protein, CD34, desmin, SMA and cytokeratin. INI1/SMARCB1 expression was deficient. EWSR1 and FUS genes were intact tested by fluorescence in situ hybridization analysis. Based on these findings, we diagnose this case as MELTVR. The patient remained relapse-free after the lesion was widely excised during 8 months follow-up. Conclusions: This disease should be included in the differential diagnostic list of vulvar tumors with epithelioid to spindled morphology. Recognition of its histopathological features and immunohistochemical reactivity will help to understand the tumor better.

show abstract

Section: Discussioncontrasting

confidence: 49%

Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

Gao

2020

Diagn Pathol

View full text Add to dashboard Cite

show abstract

“…ESMC of the vulva is an extremely rare malignant neoplasm. Only 4 clinical cases have been reported in the literature to date ( 38 – 41 ). The first clinical case was described in 1996, where the diagnosis of ESMC was established in a 40-year-old female with a tumor in the left labium majus.…”

Section: Discussionmentioning

confidence: 99%

Extraskeletal myxoid chondrosarcoma of the vulva: A case report

Виллерт¹,

Kolomiets²,

Vasilyev³

et al. 2015

Oncology Letters

View full text Add to dashboard Cite

Extraskeletal myxoid chondrosarcoma (ESMC) of the vulva is an extremely rare tumor and currently, there is little available information on its biological behavior and treatment strategy. The present study reports a case of recurrent ESMC of the vulva in a 32-year-old female. The patient presented with an increasingly painful mass of the right vulva, at the site of an exision which had been performed 7-months previously. The tumor mass was histopathologically diagnosed as primary ESMC of the vulva and subsequently, vulvectomy was performed. Cytological examination showed negative surgical margins. Intraoperative radiation therapy at a single dose of 10 Gy was administered to the bed of the removed tumor. The patient refused chemotherapy and five months after surgery, a new lesion was identified in the inguinal region. Bilateral inguinal-femoral lymph node dissection was performed and external beam radiation therapy at a dose of 40 Gy was administered to the inguinal region. Follow-up examination seven months after surgery revealed no evidence of disease progression and at present, the patient remains alive. This study highlights the importance of analyzing each clinical case of ESMC as this may lead to the development of guidelines for the optimal treatment of this rare tumor.

show abstract

“…To our knowledge, only 5 cases of sarcomas described as primary EMC of the vulva have been published in the English literature and all were among non-Caucasian women, [3][4][5][6] (Table 1). To our knowledge, only 5 cases of sarcomas described as primary EMC of the vulva have been published in the English literature and all were among non-Caucasian women, [3][4][5][6] (Table 1).…”

Section: Discussionmentioning

confidence: 99%

Extraskeletal Myxoid Chondrosarcoma of the Vulva With PLAG1 Gene Activation

Dotlić¹,

Gatalica

Wen

et al. 2014

Applied Immunohistochemistry &Amp; Molecular Morphology

View full text Add to dashboard Cite

Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal neoplasm, rarely reported in the genitourinary tract with only 5 cases reported in the vulva. We investigated 2 cases of vulvar sarcomas whose morphologic appearance and immunohistochemical profiles were consistent with EMC using fluorescence in situ hybridization (FISH), reverse-transcription polymerase chain reaction, and a whole genome expression array. FISH and reverse-transcription polymerase chain reaction assays showed no EWSR1 and NR4A3 loci rearrangements. Microarray-based analysis also revealed no changes in NR4A3 and EWSR1 gene transcription levels. Microarray data showed a significant downregulation of the muscle-related genes (eg, myosin heavy chain family, actins, myoglobin, desmin, creatine kinase, troponins) and cytokeratins (KRT6A, 6B, 13, 14, and 78), upregulation of several neuron-specific genes [neural cell adhesion molecule 1 (NCAM-1/CD56), neurofilament (NEFH)], along with some well-characterized tumor biomarkers [carbonic anhydrase IX (CA-9), topoisomerase IIα (TOP2A), matrix metalloproteinases (MMP-7, MMP-9), CDKN2 gene (p16-INK4a), checkpoint homolog 2 (CHEK2)]. Notably, both tumors showed upregulation of the pleomorphic adenoma gene 1 (PLAG1), and in 1 case PLAG1 gene rearrangement was detected by break-apart FISH. Some vulvar tumors with morphologic and immunohistochemical characteristics of EMC may represent a molecular genetic entity separate from EMCs arising in other locations. PLAG1 gene activation appears to be involved in the development of these neoplasms.

show abstract

Extraskeletal Chondrosarcoma of Labium Majus

Cited by 10 publications

References 9 publications

Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

Extraskeletal myxoid chondrosarcoma of the vulva: A case report

Extraskeletal Myxoid Chondrosarcoma of the Vulva With PLAG1 Gene Activation

Contact Info

Product

Resources

About