Advertisement

Incidental resection of a scrotal aggressive angiomyxoma mimicking a spermatocele: a case report

  • Mona KafkaEmail author
  • Peter Rehder
  • Hans Maier
  • Wolfgang Horninger
Open Access
case report
  • 68 Downloads

Summary

Background

Aggressive angiomyxoma is a rare mesenchymal neoplasm, occurring mainly in females. It is located in the pelvis and perineum, with known metastasis and hormone sensitivity only in females. Local recurrence is relatively common. We describe the case of a 62-year-old man who presented with symptoms and signs of a spermatocele.

Methods

Scrotal exploration with surgical excision of the lesion was done. Because a benign setting was assumed, no radical inguinal orchiectomy was performed.

Results

The specimen sent to pathology confirmed an aggressive angiomyxoma with positive resection margins.

Conclusion

Despite the plurality of benign scrotal masses such as spermatoceles or hydroceles, rare neoplasms should always be kept in mind. Hence, complete excision should be performed whenever possible. We selected an active surveillance strategy despite positive margins, since there is no described case of metastasis in men to date. Therefore, regular scrotal ultrasound examinations every 3 months were arranged as follow-up.

Keywords

Aggressive angiomyxoma Scrotal Male Surveillance strategy Surgical resection 

Main novel aspects

  • Due to the fact that an ordinary benign setting was assumed, a scrotal exploration adequate for a spermatocele was performed, resulting in positive resection margins.

  • Nevertheless, we decided on an active surveillance strategy with fine-meshed ultrasound follow-up, performed by a dedicated uroradiologist.

  • However, no signs of local recurrence were detected in the following 6 months.

Background

Aggressive angiomyxoma (AAM) is a rare mesenchymal neoplasm, occurring in the pelvis and perineum mostly in females [1, 2]. It was first described by Steeper and Rosai as a distinctive type of gynecological soft tissue neoplasm [3]. Generally, cystic lesions in the scrotum are common and mostly benign. They may be located in the testis, epididymis, and spermatic cord or they may be a hydrocele. Possibilities in the epididymis include tubular ectasia, cysts in the head or appendix testis, appendix epididymis, or a spermatocele. Aggressive angiomyxomas in males are rare. Only a few cases in males have been described, with less than 20 of them mentioning a scrotal genesis [1, 2, 4]. Aggressive angiomyxoma is described as a locally aggressive neoplasm [5] with a high tendency to recur [6]. We describe the case of a man presenting clinically with a spermatocele, which even at surgical exploration was not obvious to be a neoplasm. Dealing with this kind of neoplasm is empirical, as currently no guidelines exist.

Case presentation

A healthy-looking non-smoking 62-year-old man presented with a painless, mobile, and soft mass cranial to the left testis. The clinical diagnosis of a spermatocele was made. On ultrasound, a cystic lesion was identified without any signs suggestive of malignancy. Magnetic resonance tomography showed a cystic lesion with some contrast enhancement to its lateral aspect, a finding that conforms to a spermatocele. No lymphadenopathy was shown. The size of the lesion disturbed the patient and therefore a surgical resection was desired (Fig. 1).
Fig. 1

Magnetic resonance tomography images of the scrotal mass. a T1-weighted image of the right testicle (A) and the scrotal mass with a length of 55 mm (B); b T2-weighted image of a different tomographic section

An exploration and macroscopically total surgical excision using a scrotal skin incision followed. Contrary to a spermatocele, the 4 × 5 × 7-cm big lesion had no connection to the testis or epididymis. The mass was removed in toto. Finally, the histological examination confirmed an AAM with a positive resection margin. This rare diagnosis was made as a result of the typical localization in the pelvic region combined with characteristic histological features such as fibrosclerotic vessel walls and bundles and fascicles of eosinophilic smooth muscle cells, the so-called Catherine wheel. Immunohistochemistry frequently shows actin and desmin reactivity (Fig. 2).
Fig. 2

Histological slice. Aggressive angiomyxoma. Tumor with low cellularity, composed of spindled and stellate-shaped cells within a myxoid-appearing, edematous stroma with a loose meshwork of collagen fibers and thin-walled vessels. H&E, ×200

Discussion

Aggressive angiomyxomas are mesenchymal neoplasms mainly in females arising from the soft tissues in the pelvis and perineum. They are locally infiltrative with recurrence rates varying from 36 to 72% [1, 7]. Two cases with metastasis in females have been described to date [1]. Sensitivity to neoadjuvant and adjuvant hormonal manipulation of estrogen and progesterone has been suggested [1, 8]. Contrary to expectations, a long-term follow-up study did not differentiate between outcomes after complete or incomplete primary resection [9]. As reliable follow-up is possible with our patient, we decided, after informed consent including radical orchiectomy, to perform check-ups every 3 months for the first year. Imaging is done by scrotal ultrasonography by a dedicated uroradiologist. Even a faint suspicion would lead to surgical exploration and radical orchiectomy to facilitate complete resection. If no recurrence is demonstrable, follow-up visits could be stretched out. The patient was free of recurrence with a follow-up of 6 months.

Conclusion

In summary, the etiology and pathophysiology of AAM are poorly understood. The literature suggests slightly different presentations with metastasis only described in females. It is important to realize that cystic lesions in the scrotum do not include only hydroceles or spermatoceles. A high index of suspicion should be maintained during scrotal exploration, demanding histological analysis to confirm rare findings. Furthermore, complete excisions should be done whenever possible, to attain tumor-free resection margins during primary resection.

Notes

Author contributions

M Kafka, P. Rehder, manuscript preparation; H. Maier, pathology and image; W. Horninger, supervision. The authors M. Kafka and P. Rehder contributed equally to the manuscript.

Funding

Open access funding provided by University of Innsbruck and Medical University of Innsbruck.

Compliance with ethical guidelines

Conflict of interest

M. Kafka, P. Rehder, H. Maier, and W. Horninger declare that they have no competing interests.

Ethical standards

A written consent for publication was received.

References

  1. 1.
    Aydin AM, Katipoglu K, Baydar DE, Bilen CY. Long-standing aggressive angiomyxoma as a paratesticular mass: a case report and review of literature. SAGE Open Med Case Rep. 2017;  https://doi.org/10.1177/2050313x17712090. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5459349/.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Morag R, Fridman E, Mor Y. Aggressive angiomyxoma of the scrotum mimicking huge hydrocele: case report and literature review. Case Rep Med. 2009;  https://doi.org/10.1155/2009/157624. https://www.hindawi.com/journals/crim/2009/157624/.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol. 1983;7(5):463–75.CrossRefGoogle Scholar
  4. 4.
    Coppola S, Desai A, Tzanis D, Honoré C, Bitsakou G, Le Péchoux C, et al. Conservative en bloc surgery for aggressive angiomyxoma achieves good local control: analysis of 14 patients from a single institution. Int J Gynecol Cancer. 2013;23(3):540–5.CrossRefGoogle Scholar
  5. 5.
    Umranikar S, Ubee S, Williams G. Aggressive angiomyxoma of the perineum: a rare presentation in a male with 4 years follow up. J Surg Case Rep. 2017;  https://doi.org/10.1093/jscr/rjx086.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Gaunay GS, Barazani Y, Kagen AC, Stember DS. Aggressive angiomyxoma of the scrotum. Clin Imaging. 2013;37(6):1122–4.CrossRefGoogle Scholar
  7. 7.
    Draeger DL, Protzel C, Hakenberg OW. Aggressive angiomyxoma as a rare differential diagnosis of enlargement of the scrotum. Clin Genitourin Cancer. 2016;14(2):e237–e9.CrossRefGoogle Scholar
  8. 8.
    Bigby SM, Symmans PJ, Miller MV, Dray MS, Jones RW. Aggressive angiomyxoma [corrected] of the female genital tract and pelvis—clinicopathologic features with immunohistochemical analysis. Int J Gynecol Pathol. 2011;30(5):505–13.CrossRefGoogle Scholar
  9. 9.
    Chan YM, Hon E, Ngai SW, Ng TY, Wong LC, Chan IM. Aggressive angiomyxoma in females: is radical resection the only option? Acta Obstet Gynecol Scand. 2000;79(3):216–20.CrossRefGoogle Scholar

Copyright information

© The Author(s) 2018

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Authors and Affiliations

  1. 1.Department of UrologyMedical University InnsbruckInnsbruckAustria
  2. 2.Department of Clinical Pathology and CytodiagnosticsTyrolean State Hospital Ltd.InnsbruckAustria

Personalised recommendations