Incidental resection of a scrotal aggressive angiomyxoma mimicking a spermatocele: a case report
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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.
Scrotal exploration with surgical excision of the lesion was done. Because a benign setting was assumed, no radical inguinal orchiectomy was performed.
The specimen sent to pathology confirmed an aggressive angiomyxoma with positive resection margins.
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.
KeywordsAggressive 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.
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 . 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  with a high tendency to recur . 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.
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 . 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 . 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.
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.
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.
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.
A written consent for publication was received.
- 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
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