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Inverted Y incision and trans-sacral approach in retroperitoneal aggressive angiomyxoma: a case report
© Hong et al.; licensee BioMed Central Ltd. 2013
Received: 22 September 2012
Accepted: 29 April 2013
Published: 10 June 2013
Aggressive angiomyxoma is a rare myxedematous mesenchymal tumor that mainly occurs in the female pelvis and perineum. The principle of treatment for aggressive angiomyxoma is surgical excision. The tumor can be removed by local excision alone when it occurs locally on the perineum. However, it cannot be completely excised by a perineal approach alone when it passes through the perineum and pelvic bone to extend into the retroperitoneal space.
A 34-year-old Asian woman presented with a rapidly growing left perineal mass and swelling in the left gluteal region. The swelling was associated with a mild, dull pain in the left gluteal region. In the present case of bulky aggressive angiomyxoma extending to the perineum and retroperitoneal space, the authors made an inverted Y incision through the buttock, removed the coccyx and lower portion of the sacrum, and excised the retroperitoneal mass and perineal lesion through a perineal approach.
The inverted Y incision and trans-sacral approach can provide easy access to deep retroperitoneal aggressive angiomyxoma and reduce damage to neighboring organs.
Aggressive angiomyxoma (AAA) is a rare myxedematous mesenchymal tumor that mainly occurs in the female pelvis and perineum. AAA was first described by Steeper and Rosai in 1983 . The principle treatment for AAA is surgical excision. It is not easy to determine the tumor-free margin because of infiltration; thus, complete resection is difficult. The tumor can be removed by local excision alone when it occurs locally on the perineum. However, it cannot be completely excised by a perineal approach alone when it passes through the perineum and pelvic bone to extend into the retroperitoneal space [2, 3].
Given that AAA usually manifests in the second to fourth decades of life and frequently extends into the retroperitoneal space, appropriate surgical methods should be selected to ensure a cosmetic incision and effective mass removal. In the present case, involving a bulky AAA extending to the perineum and retroperitoneal space, the authors made an inverted Y incision through the buttock, removed the coccyx and lower portion of the sacrum, and excised the retroperitoneal mass and perineal lesion through a perineal approach without laparotomy.
A 34-year-old Asian woman presented with a six-month history of a rapidly growing left perineal mass and swelling in the left gluteal region. The swelling was associated with a mild, dull pain in the left gluteal region.
Macroscopically, the tumor had a smooth surface, well-encapsulated upper portion, and partially encapsulated portion in the buttock region. The well-encapsulated retroperitoneal mass showed a glistening, gelatinous appearance and was homogenous in consistency without nodularity. However, the partially encapsulated subcutaneous lesion in the buttock area contained nodular and satellite-like lesions.
Although AAA mostly occurs in women of reproductive age, it also occurs in men or children before puberty. It mainly occurs in urogenital organs such as the perineum, vagina, bladder, scrotum, and retroperitoneum, but may also occur in the larynx and orbit [4–6]. AAA is very difficult to diagnose before surgery. Radiological examinations such as ultrasonography, CT, and MRI are helpful in estimating the size of the tumor and degree of infiltration into the surrounding tissues and determining the range and method of surgery . On CT, the tumor has well-defined margins and less attenuation than that of muscle. On T2-weighted MRI, the tumor has high signal intensity . The best treatment of choice is surgical excision with tumor-free margins. However, the tumor recurs in approximately 70 percent of cases, even when the margin has been sufficiently excised . Postoperative adjuvant therapy is also necessary, especially in cases of recurrent or residual tumors. It has been suggested that the growth of AAA is associated with stimulation by sex hormones, especially estrogen. Based on these reports, GnRH analogs have been used in estrogen- and progesterone-receptor-positive recurrent or residual tumors [10, 11]. Radiotherapy has been also performed, but its effect is not clear .
There is no definite treatment modality with the exception of radical excision with tumor-free margins. The size of AAA varies from less than 5cm to 60cm, and large tumors frequently show retroperitoneal involvement. Cosmetic incisions are difficult to make in young women who undergo removal of bulky retroperitoneal masses via laparotomy. The patient in the present case was a 34-year-old woman, and she did not want to undergo laparotomy with a midline incision. If intra-abdominal organs are displaced and the peritoneum is dissected before the retroperitoneal mass removal, the risk of damage to adjacent organs and postoperative intraperitoneal adhesion will increase. In cases involving retroperitoneal masses close to the anus, the anal sphincter may be damaged; thus, local sphincter-preserving procedures are necessary. The trans-sacral approach to remove retroperitoneal masses was first proposed by Kraske in 1885 . This method has since been used to remove colorectal tumors, pelvic bone tumors, and retroperitoneal soft tissue tumors. In 2003, Sonoda et al.  used the trans-sacral approach to remove a remnant cervix in a 71-year-old woman with endometrial cancer who had undergone previous laparotomy. He suggested that if radical resection is needed in an unusual situation, adaptation of different surgical approaches may be required. The herein-described inverted Y incision and trans-sacral approach can provide easy access to deep retroperitoneal AAA and reduce damage to neighboring organs.
The inverted Y incision and trans-sacral approach performed by these authors is considered to result in minimal incision scars and provide easy access to tumors, avoiding damage to adjacent organs. These authors propose the above-mentioned procedure for bulky AAA involving the retroperitoneal space.
Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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