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Background <p>Myoepithelioma-like tumours of the vulvar region (MELTVR) are a rare mesenchymal neoplasm of the vulvar area. Histologically MELTVRs are usually similar to soft tissue myoepithelioma; however, they have characteristic immunohistochemistry phenotype including positivity for oestrogen receptor, negativity for S100 protein and glial fibrillary acidic protein and loss of INI 1/SMARCB1 expression.</p> Purpose <p>The intention is to highlight the need to include MELTVR in the differential diagnosis of vulvar tumours and to understand morphology, clinical course and treatment of MELTVR.</p> Material and methods <p>We are reporting a case of a 47-year-old female who presented with complaint of swelling in perineal area in right labia majora since 6&#xa0;months. The patient underwent wide local excision taking adequate margin. Inguinal lymph node dissection was planned as second-stage procedure after reviewing final histopathology report. Since histopathology was reported as myoepithelioma-like tumour of vulvar region (MELTVR) and evidence of lymph node metastasis was not known and not yet reported in the literature, inguinal lymph node dissection was deferred. No adjuvant therapy was given. The patient is free from local recurrence and distant metastasis in follow-up period of 1&#xa0;year after the initial excision.</p> Conclusion <p>Based on the literature and our report, these tumours have low-grade malignant features with no evidence of distant metastasis. Wide local excision seems to be an adequate treatment for it with no need of inguinal lymph node dissection. No adjuvant therapy is required after excision. Due to the rarity of the tumour, further investigations are required to clearly determine the pathological and immunohistochemical features of this tumour.</p>

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Myoepithelioma-Like Tumour of the Vulvar Region: A Case Report and Review of Literature

  • 期刊论文

  • 发表日期：

    2025年4月19日

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  • Meena Parihar,
  • Prerna Chadha,
  • Anila Sharma,
  • Zeba Naaz,
  • Vandana Jain

摘要

Background

Myoepithelioma-like tumours of the vulvar region (MELTVR) are a rare mesenchymal neoplasm of the vulvar area. Histologically MELTVRs are usually similar to soft tissue myoepithelioma; however, they have characteristic immunohistochemistry phenotype including positivity for oestrogen receptor, negativity for S100 protein and glial fibrillary acidic protein and loss of INI 1/SMARCB1 expression.

Purpose

The intention is to highlight the need to include MELTVR in the differential diagnosis of vulvar tumours and to understand morphology, clinical course and treatment of MELTVR.

Material and methods

We are reporting a case of a 47-year-old female who presented with complaint of swelling in perineal area in right labia majora since 6 months. The patient underwent wide local excision taking adequate margin. Inguinal lymph node dissection was planned as second-stage procedure after reviewing final histopathology report. Since histopathology was reported as myoepithelioma-like tumour of vulvar region (MELTVR) and evidence of lymph node metastasis was not known and not yet reported in the literature, inguinal lymph node dissection was deferred. No adjuvant therapy was given. The patient is free from local recurrence and distant metastasis in follow-up period of 1 year after the initial excision.

Conclusion

Based on the literature and our report, these tumours have low-grade malignant features with no evidence of distant metastasis. Wide local excision seems to be an adequate treatment for it with no need of inguinal lymph node dissection. No adjuvant therapy is required after excision. Due to the rarity of the tumour, further investigations are required to clearly determine the pathological and immunohistochemical features of this tumour.