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Rare disease
CASE REPORT
Rare paratesticular aggressive angiomyxoma with negative oestrogen and progesterone receptors in a male patient
  1. Azfar Neyaz,
  2. Nuzhat Husain,
  3. Nidhi Anand,
  4. Pallavi Srivastava
  1. Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
  1. Correspondence to Professor Nuzhat Husain, drnuzhathusain{at}hotmail.com

Summary

Aggressive angiomyxoma (AAM) is a rare mesenchymal myxoid tumour localised to the pelvis and/or perineum in adult females in reproductive age group. AAM is very rare in males, with <50 cases described in literature, and involves scrotum, spermatic cord and perineum. It is slow growing, with a marked tendency for local recurrence after excision, but without metastatic potential. We present a rare case of a paratesticular AAM in a man aged 53 years. Tumour cells were immunoreactive for desmin, smooth muscle actin (SMA), vimentin, CD34 and were negative for S100. Unlike AAMs in females which express oestrogen receptor (ER) and/or progesterone receptor (PR) in >90% cases, the tumour cells in our case were negative for ER and PR, suggesting that the hypothesis that these markers play a role in tumour development and pathogenesis, does not apply in males. Androgen receptor positivity was noted in 2%–5% tumour cells.

  • pathology
  • urology
  • urological surgery
  • urological cancer

https://doi.org/10.1136/bcr-2017-222164

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    Footnotes

    • Contributors All authors made a significant contribution to this study. AN: manuscript preparation, documentation, acquisition of data. NH: compilation and manuscript editing. NA: conception and design. PS: acquisition of data and reports. This manuscript has not been submitted for publication; it has not been accepted for publication and has not been published in any other journal. All authors have read and approved the final version of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.