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[Abstract]Deep aggressive angiomyxoma is an uncommon mesenchymal tumor， that is found predilection for the female pelvis at reproductive aye. We report CT appearances of a 42 year old woman with a deep aggressive angiomyxoma of the pelvis that was proven by preoperative biopsy. This article describes the imaging features of the tumor， which may help to know its characteristic of swirling or layering internal pattern. We further discuss disease characteristics， pathological characteristics， types， imaging features， differential diagnosis， treatment and prognosis.
[Key Words]：Tumor of the pelvis; deep aggressive angiomyxoma; CT; MRI; Pathology
【中圖分类号】R445.1 【文献标识码】A 【文章编号】1673-9026（2021）10--01
1.Introduction
Deep aggressive angiomyxoma is a rare， benign， mesenchymal tumor. It was first described by Steeper and Rosai in 1983. In 2013， WHO classified it as benign soft tissue tumor without well-defined differentiation. This tumor is called 'aggressive' due to frequent local recurrence rather than a propensity for distant metastatic disease [1]. Therefore， the main clinical features of this disease are high recurrence rate and strong invasion.
Most commonly presented in the female pelvis and perineum， but， there were also some cases occurring in the scrotum or spermatic cord in males[2]. The male to female ratio is 1：6[3]. This article reports a case of female pelvic deep aggressive angiomyxoma imaging features， and literature review， in order to deepen the understanding of this disease.
2.Case presentation
A 42-year-old woman， without a history of illness or drug use， presented to our radiology department with a large pelvic mass. No obvious inducement appeared in recent one month intermittent abdominal pain， accompanied by frequent urination， nearly 10 days conscious waist trapped.
After the hospital examination， the whole abdomen enhanced CT showed： irregular pelvic mass， about 19.6x8.3cm in size， with a CT value of about 18-30HU， and the lesion boundary was not clear. The enhanced scan showed progressive enhancement of solid parts， with a CT value of about 36HU in the arterial phase and 38HU in the venous phase and 7HU in the delayed phase. Finally， it was diagnosed as SBOT. Surgical findings： a soft， irregular mass， about 8X25x7cm in size in the pelvic and abdominal cavity， well demarcated from the surrounding organs， pedunculated， about 1.0cm in width， was located in the sigmoid colon—epiploic appendices. Pathological findings： a gray-yellow， gray-rad nodular mass， about 18x15x6cm in size， with a surface glossy smooth. The cut surface was grayish-yellow， some were gelatinous， and some areas were grayish-rad. Immunohistochemical：MDM2（-）， Ki-67（20%+）， CDK4（+）， S-100（-）， CD34（+）， CKpan（-）， Inhibin-a（-）， calretinin（-）， Desmin（+）， SMA（+）， CD163（+）， CD117（-）， DOG-1（-）， SOX-10（-）， Bcl-2（+）， STAT6（-）. Pathological diagnosis： according to the immunohistochemical results and clinical features， it was indicated that deep aggressive angiomyxoma （DAAM）. 3.Discussion
3.1 General characteristics
DAAM is a benign mesenchymal tumor that usually occurs in the pelvic cavity and perineum of a woman. It is common for premenopausal women. The peak age of DAAM is 30-40 years old[4]. It presents as a painless mass with local slow growth and is usually asymptomatic， the patient may touch or the huge tumor to the surrounding tissue organ compression， produces the local pain feeling， the lower abdomen is uncomfortable， the perineum drops pain and so on.
3.2 Pathological characteristics
The tumor volume is generally large， the largest one can reach 60 cm in the literature[5]. It is usually larger than 10 cm and has a soft mass with clear borders and a yellowish， gelatinous or fleshy cut surface. Microscopically， long rod-shaped or fusiform tumor cells are seen in the background of the mucinous stroma， interspersed with vessels of various sizes. Immunohistochemical： In Desmin， Vimentin， SMA， CD34， ER， PR is usually expressed in different degrees and S-100 is negative[6].
3.3 Types
There are two types of DAAM growth： pedunculated and infiltrative. Pedunculated growth is common in up to 1/3 of patients. The pedunculated is usually resection by local operation， the recurrence rate is low， but the initiative needs to expand the scope of operation， and local recurrence is easy[1].
3.4 Image characteristics
The CT appearance of DAAM was lower or equal density than that of muscle mass[7]. The inner density of DAAM was uneven. Plain CT scan showed low density area and cloud flocculent high density area. Irregular enhancement in arterial phase， gradual enhancement in venous phase and delayed phase， and sometimes typical spiral enhancement were seen. On MRI， the signal intensity on T1WI was almost equal to that on muscle， and the signal intensity on T2WI was high or mixed. Bands and swirls of low signal intensity were seen on T2WI. The low signal on T2-FS sequence showed more clearly， DWI showed limited diffusion of the mass， in contrast enhanced CT scan， there was obvious uneven enhancement with spiral or layered enhancement， which was the characteristic appearance of AAM reported in literature[8]. It may be related to the vascular maturity， the distribution and arrangement of the components of fibrous and vascular stroma[8]， and the high signal intensity of T2WI may be related to the water content and loose myxoid stroma of myxoma[9].
3.5 Identify with SBOT SBOT is an adnexal tumor with low malignant potential. It is common in 15-70 years of age， and is more common in young patients. It is generally cystic and papillary in shape with a diameter of 2.5～10cm. The cut surface is bright and soft. A complex branching papillary structure with small papillary projections is seen here. CT or MRI scan showed solid and cystic mass， the cystic fluid showed water like density or signal， the wall and division showed papillary protuberance， DWI showed no obvious diffusion limitation， and the wall nodules showed obvious enhancement.
3.6 Treatment and prognosis
Because DAAM is invasive， large in size and deep in location， surgical resection is still the primary treatment. Because of its rich blood supply， preoperative vascular embolization can reduce the tumor blood supply[10]， reduce the tumor， thereby reducing the difficulty of surgery. For patients with positive PR and ER， Sereda etc[11] proposed GnRH-a treatment， which is helpful to reduce the size of tumors. In addition， tumor mitosis is rare， preoperative radiotherapy is ineffective[12]， but postoperative radiotherapy can inhibit tumor growth[13].
When the tumor invaded adjacent tissues or organs， it was difficult to resect completely. The recurrence rate was 33%-83%[14]， and there is generally no distant metastasis. Therefore， long-term follow-up and imaging examinations were needed to monitor the tumor recurrence.
REFERENCES：
[1]Kooy Joni，Carlson Vanessa，?a?iragi? Lana，Sawhney Summit，Nelson Gregg. A case series of aggressive angiomyxoma： Using morphologic type and hormonal modification to tailor treatment.[J]. Gynecologic oncology reports，2021，36：100765
[2]Bensalah Abdellatif，Charifi Yahya，Ousrouti Laila Tahiri，Ters Jalila，Berrad Soumia，Haloua Meryem，Alami Badr，Boubbou Meryem，Chbani Laila，Benjelloun Lbachir，Arifi Samia，Maaroufi Mustapha，Lamrani Youssef Alaoui. Perineal and pelvic aggressive angiomyxoma： Imaging finding in an uncommon case report.[J]. Radiology case reports，2021，16（7）：1822-1827
[3]鄧文友，孙艳秋，黄晓姗.盆腔深部侵袭性血管黏液瘤合并会阴部疝一例[J].临床放射学杂志，2017，36（08）：1114-1115.
[4]刘玉芬，佟丽波，邢天容，王婧如.子宫侵袭性血管黏液瘤1例并文献复习[J].医学理论与实践，2020，33（07）：1149-1150.
[5]T. Giraudmaillet，F.Z. Mokrane，M.C. Delchier-Bellec，S. Motton，C. Cron，H. Rousseau. Aggressive angiomyxoma of the pelvis with inferior vena cava involvement： MR imaging features[J]. Diagnostic and Interventional Imaging，2015，96（1）：111-114.
[6]权继传，王锡山.盆腔侵袭性血管黏液瘤一例并文献复习[J].中华结直肠疾病电子杂志，2019，8（02）：192-194. [7]Xubin Li，Zhaoxiang Ye. Aggressive angiomyxoma of the pelvis and perineum： a case report and review of the literature[J]. Abdominal Imaging，2011，36（6）：739-741.
[8]Jeyadevan N N，Sohaib S A A，Thomas J M，Jeyarajah A，Shepherd J H，Fisher C. Imaging features of aggressive angiomyxoma.[J]. Clinical radiology，2003，58（2）：157-162.
[9]熊發奎，崔凤.侵袭性血管黏液瘤CT和MR影像学特征分析[J].浙江中西医结合杂志，2020，30（01）：65-67+93.
[10]I.J.M. Han-Geurts，A.N. van Geel，L. van Doorn，M. den Bakker，A.M.M. Eggermont，C. Verhoef. Aggressive angiomyxoma： multimodality treatments can avoid mutilating surgery[J]. European Journal of Surgical Oncology，2006，32（10）：1217-1221.
[11]Sereda Danya，Sauthier Philippe，Hadjeres Rachid，Funaro Deana. Aggressive angiomyxoma of the vulva： a case report and review of the literature.[J]. Journal of lower genital tract disease，2009，13（1）：46-50.
[12]Wang Zhaoling，Liu Ying，Yang Lu，Gu Ling，He Yuhong，Huang Diyan，Zhu Guoxiong. Maxillary aggressive angiomyxoma showing ineffective to radiotherapy： a rare case report and review of literature.[J]. International journal of clinical and experimental pathology，2015，8（1）：1063-1067.
[13]Sarah Coppola，Anant Desai，Dimitri Tzanis，Charles Honoré，Georgina Bitsakou，Cécile Le Péchoux，Philippe Terrier，Sylvie Bonvalot. Conservative En Bloc Surgery for Aggressive Angiomyxoma Achieves Good Local Control： Analysis of 14 Patients From a Single Institution[J]. International Journal of Gynecological Cancer，2013，23（3）：540-545.
[14]Toshio Nakamura，Katsutoshi Miura，Yuji Maruo，Kenichi Sunayama，Keiji Maruyama，Hidefumi Kashiwabara，Kou Ohata，Atsuko Fukazawa，Satoshi Nakamura. Aggressive angiomyxoma of the perineum originating from the rectal wall[J]. Journal of Gastroenterology，2002，37（4）：303-308.
Corresponding author： Xin Dou. E-mail： 2223169080@qq.com
[Key Words]：Tumor of the pelvis; deep aggressive angiomyxoma; CT; MRI; Pathology
【中圖分类号】R445.1 【文献标识码】A 【文章编号】1673-9026（2021）10--01
1.Introduction
Deep aggressive angiomyxoma is a rare， benign， mesenchymal tumor. It was first described by Steeper and Rosai in 1983. In 2013， WHO classified it as benign soft tissue tumor without well-defined differentiation. This tumor is called 'aggressive' due to frequent local recurrence rather than a propensity for distant metastatic disease [1]. Therefore， the main clinical features of this disease are high recurrence rate and strong invasion.
Most commonly presented in the female pelvis and perineum， but， there were also some cases occurring in the scrotum or spermatic cord in males[2]. The male to female ratio is 1：6[3]. This article reports a case of female pelvic deep aggressive angiomyxoma imaging features， and literature review， in order to deepen the understanding of this disease.
2.Case presentation
A 42-year-old woman， without a history of illness or drug use， presented to our radiology department with a large pelvic mass. No obvious inducement appeared in recent one month intermittent abdominal pain， accompanied by frequent urination， nearly 10 days conscious waist trapped.
After the hospital examination， the whole abdomen enhanced CT showed： irregular pelvic mass， about 19.6x8.3cm in size， with a CT value of about 18-30HU， and the lesion boundary was not clear. The enhanced scan showed progressive enhancement of solid parts， with a CT value of about 36HU in the arterial phase and 38HU in the venous phase and 7HU in the delayed phase. Finally， it was diagnosed as SBOT. Surgical findings： a soft， irregular mass， about 8X25x7cm in size in the pelvic and abdominal cavity， well demarcated from the surrounding organs， pedunculated， about 1.0cm in width， was located in the sigmoid colon—epiploic appendices. Pathological findings： a gray-yellow， gray-rad nodular mass， about 18x15x6cm in size， with a surface glossy smooth. The cut surface was grayish-yellow， some were gelatinous， and some areas were grayish-rad. Immunohistochemical：MDM2（-）， Ki-67（20%+）， CDK4（+）， S-100（-）， CD34（+）， CKpan（-）， Inhibin-a（-）， calretinin（-）， Desmin（+）， SMA（+）， CD163（+）， CD117（-）， DOG-1（-）， SOX-10（-）， Bcl-2（+）， STAT6（-）. Pathological diagnosis： according to the immunohistochemical results and clinical features， it was indicated that deep aggressive angiomyxoma （DAAM）. 3.Discussion
3.1 General characteristics
DAAM is a benign mesenchymal tumor that usually occurs in the pelvic cavity and perineum of a woman. It is common for premenopausal women. The peak age of DAAM is 30-40 years old[4]. It presents as a painless mass with local slow growth and is usually asymptomatic， the patient may touch or the huge tumor to the surrounding tissue organ compression， produces the local pain feeling， the lower abdomen is uncomfortable， the perineum drops pain and so on.
3.2 Pathological characteristics
The tumor volume is generally large， the largest one can reach 60 cm in the literature[5]. It is usually larger than 10 cm and has a soft mass with clear borders and a yellowish， gelatinous or fleshy cut surface. Microscopically， long rod-shaped or fusiform tumor cells are seen in the background of the mucinous stroma， interspersed with vessels of various sizes. Immunohistochemical： In Desmin， Vimentin， SMA， CD34， ER， PR is usually expressed in different degrees and S-100 is negative[6].
3.3 Types
There are two types of DAAM growth： pedunculated and infiltrative. Pedunculated growth is common in up to 1/3 of patients. The pedunculated is usually resection by local operation， the recurrence rate is low， but the initiative needs to expand the scope of operation， and local recurrence is easy[1].
3.4 Image characteristics
The CT appearance of DAAM was lower or equal density than that of muscle mass[7]. The inner density of DAAM was uneven. Plain CT scan showed low density area and cloud flocculent high density area. Irregular enhancement in arterial phase， gradual enhancement in venous phase and delayed phase， and sometimes typical spiral enhancement were seen. On MRI， the signal intensity on T1WI was almost equal to that on muscle， and the signal intensity on T2WI was high or mixed. Bands and swirls of low signal intensity were seen on T2WI. The low signal on T2-FS sequence showed more clearly， DWI showed limited diffusion of the mass， in contrast enhanced CT scan， there was obvious uneven enhancement with spiral or layered enhancement， which was the characteristic appearance of AAM reported in literature[8]. It may be related to the vascular maturity， the distribution and arrangement of the components of fibrous and vascular stroma[8]， and the high signal intensity of T2WI may be related to the water content and loose myxoid stroma of myxoma[9].
3.5 Identify with SBOT SBOT is an adnexal tumor with low malignant potential. It is common in 15-70 years of age， and is more common in young patients. It is generally cystic and papillary in shape with a diameter of 2.5～10cm. The cut surface is bright and soft. A complex branching papillary structure with small papillary projections is seen here. CT or MRI scan showed solid and cystic mass， the cystic fluid showed water like density or signal， the wall and division showed papillary protuberance， DWI showed no obvious diffusion limitation， and the wall nodules showed obvious enhancement.
3.6 Treatment and prognosis
Because DAAM is invasive， large in size and deep in location， surgical resection is still the primary treatment. Because of its rich blood supply， preoperative vascular embolization can reduce the tumor blood supply[10]， reduce the tumor， thereby reducing the difficulty of surgery. For patients with positive PR and ER， Sereda etc[11] proposed GnRH-a treatment， which is helpful to reduce the size of tumors. In addition， tumor mitosis is rare， preoperative radiotherapy is ineffective[12]， but postoperative radiotherapy can inhibit tumor growth[13].
When the tumor invaded adjacent tissues or organs， it was difficult to resect completely. The recurrence rate was 33%-83%[14]， and there is generally no distant metastasis. Therefore， long-term follow-up and imaging examinations were needed to monitor the tumor recurrence.
REFERENCES：
[1]Kooy Joni，Carlson Vanessa，?a?iragi? Lana，Sawhney Summit，Nelson Gregg. A case series of aggressive angiomyxoma： Using morphologic type and hormonal modification to tailor treatment.[J]. Gynecologic oncology reports，2021，36：100765
[2]Bensalah Abdellatif，Charifi Yahya，Ousrouti Laila Tahiri，Ters Jalila，Berrad Soumia，Haloua Meryem，Alami Badr，Boubbou Meryem，Chbani Laila，Benjelloun Lbachir，Arifi Samia，Maaroufi Mustapha，Lamrani Youssef Alaoui. Perineal and pelvic aggressive angiomyxoma： Imaging finding in an uncommon case report.[J]. Radiology case reports，2021，16（7）：1822-1827
[3]鄧文友，孙艳秋，黄晓姗.盆腔深部侵袭性血管黏液瘤合并会阴部疝一例[J].临床放射学杂志，2017，36（08）：1114-1115.
[4]刘玉芬，佟丽波，邢天容，王婧如.子宫侵袭性血管黏液瘤1例并文献复习[J].医学理论与实践，2020，33（07）：1149-1150.
[5]T. Giraudmaillet，F.Z. Mokrane，M.C. Delchier-Bellec，S. Motton，C. Cron，H. Rousseau. Aggressive angiomyxoma of the pelvis with inferior vena cava involvement： MR imaging features[J]. Diagnostic and Interventional Imaging，2015，96（1）：111-114.
[6]权继传，王锡山.盆腔侵袭性血管黏液瘤一例并文献复习[J].中华结直肠疾病电子杂志，2019，8（02）：192-194. [7]Xubin Li，Zhaoxiang Ye. Aggressive angiomyxoma of the pelvis and perineum： a case report and review of the literature[J]. Abdominal Imaging，2011，36（6）：739-741.
[8]Jeyadevan N N，Sohaib S A A，Thomas J M，Jeyarajah A，Shepherd J H，Fisher C. Imaging features of aggressive angiomyxoma.[J]. Clinical radiology，2003，58（2）：157-162.
[9]熊發奎，崔凤.侵袭性血管黏液瘤CT和MR影像学特征分析[J].浙江中西医结合杂志，2020，30（01）：65-67+93.
[10]I.J.M. Han-Geurts，A.N. van Geel，L. van Doorn，M. den Bakker，A.M.M. Eggermont，C. Verhoef. Aggressive angiomyxoma： multimodality treatments can avoid mutilating surgery[J]. European Journal of Surgical Oncology，2006，32（10）：1217-1221.
[11]Sereda Danya，Sauthier Philippe，Hadjeres Rachid，Funaro Deana. Aggressive angiomyxoma of the vulva： a case report and review of the literature.[J]. Journal of lower genital tract disease，2009，13（1）：46-50.
[12]Wang Zhaoling，Liu Ying，Yang Lu，Gu Ling，He Yuhong，Huang Diyan，Zhu Guoxiong. Maxillary aggressive angiomyxoma showing ineffective to radiotherapy： a rare case report and review of literature.[J]. International journal of clinical and experimental pathology，2015，8（1）：1063-1067.
[13]Sarah Coppola，Anant Desai，Dimitri Tzanis，Charles Honoré，Georgina Bitsakou，Cécile Le Péchoux，Philippe Terrier，Sylvie Bonvalot. Conservative En Bloc Surgery for Aggressive Angiomyxoma Achieves Good Local Control： Analysis of 14 Patients From a Single Institution[J]. International Journal of Gynecological Cancer，2013，23（3）：540-545.
[14]Toshio Nakamura，Katsutoshi Miura，Yuji Maruo，Kenichi Sunayama，Keiji Maruyama，Hidefumi Kashiwabara，Kou Ohata，Atsuko Fukazawa，Satoshi Nakamura. Aggressive angiomyxoma of the perineum originating from the rectal wall[J]. Journal of Gastroenterology，2002，37（4）：303-308.
Corresponding author： Xin Dou. E-mail： 2223169080@qq.com