- Email: [email protected]
“Swirl sign” of aggressive angiomyxoma—a lesser known diagnostic sign
Swirl sign” of Aggressive angiomyxoma – A lesser known diagnostic sign Sivasubramanian Srinivasan, Vijay Krishnan, Syed Zama Ali, Natesan Chidambaranathan PII: DOI: Reference:
S0899-7071(14)00102-8 doi: 10.1016/j.clinimag.2014.04.001 JCT 7603
To appear in:
Journal of Clinical Imaging
Received date: Revised date: Accepted date:
25 November 2013 5 April 2014 8 April 2014
Please cite this article as: Srinivasan Sivasubramanian, Krishnan Vijay, Ali Syed Zama, Chidambaranathan Natesan, Swirl sign” of Aggressive angiomyxoma – A lesser known diagnostic sign, Journal of Clinical Imaging (2014), doi: 10.1016/j.clinimag.2014.04.001
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: “Swirl
sign” of Aggressive angiomyxoma – A lesser known diagnostic sign.
Running Head: Swirl sign of Aggressive angiomyxoma
Authors: Sivasubramanian Srinivasan, M.D.,D.N.B., F.R.C.R., 1
SC
Vijay Krishnan, M.D., F.R.C.R., 1
NU
Syed Zama Ali M.D., F.R.C.R., 1 Natesan Chidambaranathan M.D., 2 1
RI P
T
Category: Case report
MA
Department of Diagnostic Radiology, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828
ED
2 Department of Radiology, Apollo Hospitals, Chennai 600006
PT
Address corespondence and reprint requests to:
Sivasubramanian Srinivasan,
AC
CE
Department of Diagnostic Radiology, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828, Republic of Singapore. Tel: (65) 66022689 Fax: (65) 66023796 Email: [email protected] Source(s) of support: Nil. Conflict of interest :Nil
ACCEPTED MANUSCRIPT “Swirl sign” of Aggressive angiomyxoma – A lesser known diagnostic sign. Abstract
T
Aggressive angiomyxoma is a rare mesenchymal tumour with high rate of local recurrence,found
RI P
mainly in pelvis and perineum of women of reproductive age group. We present cases of two patients,with emphasis on the magnetic resonance(MR) imaging characteristics.The clinical presentation was nonspecific.MR imaging revealed characteristic"Swirl sign"in T2-weighted
SC
sequence.Pre-operative diagnosis could not be made in the first patient, due to lack of experience about the diagnosis. Pre-operative diagnosis was made in the second case and was helpful for the
NU
surgeon to excise the mass completely. Aggressive angiomyxoma should be on the top of a
MA
differential diagnosis in the appropriate clinical and imaging scenario. Keywords
ED
Pelvic neoplasm, aggressive angiomyxoma, swirl sign, magnetic resonance imaging Introduction:
PT
Aggressive angiomyxoma is an exceedingly rare neoplasm of mesenchymal origin. Although it
CE
is histologically benign, it is very vascular and locally aggressive with infiltration of structures such as the pelvic floor. Clinical diagnosis may be difficult, however MR imaging usually shows the swirled strands of low signal intensities in T2w sequence which is diagnostic of this
AC
neoplasm. We report two cases of aggressive angiomyxoma (from different hospitals) which had characteristic MR imaging features. Case 1: A 37-year-old woman presented with vague lower abdominal pain of 5-6 months duration. No significant abnormality was noted on clinical examination. Ultrasound showed a vague mass in right side of the uterus with mass effect over the right side of the urinary bladder. The mass could not be well characterized and the extent could not be assessed. Hence MR imaging was done, which revealed a fairly well defined mass in the pelvic side wall, on the right side of bladder and the uterus with involvement of the pelvic floor inferiorly. Medially the lesion was compressing and displacing the urinary bladder. The lesion was hypo-intense on T1w and on T2w sequence (Fig. 1a, b), the mass showed a whirled pattern of T2 hypo-intensity within the
ACCEPTED MANUSCRIPT lesion. The mass demonstrated enhancement in post-contrast T1w images. Pre-operative diagnosis could not be made due to lack of experience of the radiologists. Surgical excision of the mass was planned. However total excision was not possible because of significant vascularity
T
and extensive pelvic floor infiltration. Histopathology (Fig. 2a,b) showed loosely cellular
RI P
mesenchymal neoplasm with ectatic blood vessels. The cells were stellate shaped and had bland nuclear features and the cytoplasm was positive for desmin (Fig. 2c).
SC
Case 2:
NU
A 36-year-old woman presented with painless abdominal swelling which was progressively increasing in size for more than 15 years. Upon physical examination, her
MA
abdomen was soft and there was no tenderness. Vague mass was palpable in the lower abdomen and pelvis. The laboratory findings were within normal limits.MR imaging of pelvis was performed,which revealed a large abdomino- pelvic mass measuring around 30cm in height.
ED
Inferior extent was into the recto-uterine pouch with displacement of the uterus and urinary bladder and is involving the perineum and vulva on the left side. On the T1-weighted image, the
PT
tumor was of isointense to muscles (Fig. 3a), and on T2-w sequence, it displayed hyperintense signal with “swirled” areas of low signal intensity within. (Fig. 3b).Based on high signal with
CE
swirled strands of low signal intensity on T2w sequence, location of the lesion and age of the patient, the diagnosis of aggressive angiomyxoma was made.The tumor was excised with wide
AC
local excision and histo-pathological examination of the specimen revealed mixture of myxoid and vascular elements (Fig. 4) consistent with aggressive angiomyxoma.
Discusssion Aggressive angiomyxoma of the female pelvis is a highly rare neoplasm, which was first described in 1983 (1) and has been sporadically reported since then. These tumors occur in the lower pelvis, perineal or the genital area of females, usually in the third to fifth decade and are six times more common in females (2). Clinically they present as an ill-defined pelvic or perineal mass. They may be clinically confused with pelvic abscesses, bartholin gland cysts (3) or hernias or sometimes, as adnexal masses. The exact pathogenesis is not clearly understood. The predominant location of the mass in the pelvis and the female predilection suggest that
ACCEPTED MANUSCRIPT hormonal factors play an important role (4). These are not malignant neoplasms, however they can be locally aggressive and tend to infiltrate adjacent structures (5).
T
Histological examination usually shows myxoid background of collagen fibres with small
RI P
vessels and loosely arranged cells which have bland features and may have spindle or stellate shapes. Cellular atypia and mitotic figures are rare. The tumour is usually positive for vimentin and desmin on immunohistochemistry. Other important characteristic is positivity for estrogen
SC
and/or progesterone receptors (6).
NU
Although initial screening modality would be an ultrasonography (US) examination, MR imaging is the modality of choice in characterization of the mass as well as assessing the
MA
extent of the mass. US and computed tomographyCT) features of aggressive angiomyxoma has been described (7). On US, the mass may appear hypo-echoic or cystic (7). The CT features are variable and include hypo-attenuating or iso-attenuating mass involving the pelvis and perineum
ED
with enhancement in the contrast–enhanced scan, which may show the classical swirled appearance. In the series of six cases, reported by Outwater et al(2), only 50% of the cases
PT
showed the classical swirling architecture on CT. MR imaging is more reliable because of better soft tissue resolution and the
CE
characteristic appearance (7). On MR imaging, the mass is usually iso-intense to muscle on T1weighted (T1w) image and hyperintese on T2w image with „swirled‟ areas of low signal areas
AC
within, as in our cases. This morphology is characteristic of this tumour (2). The hyperintense signal in T2w sequences is due to the myxoid element and presence of water. The swirled appearance is likely due to fibrovascular stroma which gets stretched as they extend and involve the pelvic diaphragm. On post-contrast T1w images, the mass shows significant enhancement due to vascular component of the neoplasm. The characteristic swirled appearance can also be seen in post-contrast T1w images (7). Infiltration into the perineum or pelvic floor muscles are best seen with MR imaging. Accurate evaluation of the extension or infiltration into the pelvic floor is important for surgical planning.
The imaging differential diagnosis includes myxoma, infiltrating angiolipoma and infiltrating liposarcoma. Myxoma is a benign mesenchymal neoplasm that most frequently occurs in older patients. However, myxomas are mainly intramuscular and show no significant
ACCEPTED MANUSCRIPT vascularity (8). Infiltrating angiolipoma is usually found in the thigh region and as the name implies it infiltrates and replaces involved muscle and subcutaneous tissue (9). Lack of high fat content is a key feature in differentiating the angiomyxoma from the main differential
T
considerations, myxoid liposarcoma and infiltrating angiolipoma (10).
RI P
The definitive treatment is surgery with wide local excision. Complete surgical excision has been reported to be technically difficult secondary to the bulky and locally
SC
infiltrative nature of this tumor (11). In the first patient, the pre-operative diagnosis was not made (on imaging) and hence surgical planning was difficult because of the significant
NU
vascularity and infiltration of the pelvic floor. The local recurrence is common and the reported incidence is around 30% (12) and usually seen in the first three years. The role of chemotherapy
MA
and radiation therapy is not well established. Reports of successful radiotherapy for tumour recurrences have been described (13). Treatment with gonadotropin-releasing hormone (GnRH) agonist has been reported to have shown tumour shrinkage and this is likely because of the
ED
estrogen/progesterone receptors. However there is no evidence with larger number of patients
PT
(4).
In conclusion, if the characteristic "swirl sign" is seen on MR imaging, then aggressive
CE
angiomyxoma can be confidently offered as a primary differential consideration. This is
References: 1.
AC
important clinically for surgical planning or a trial of medical management.
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;7:463-475
2.
Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: findings on CT and MR imaging. AJR Am J Roentgenol 1999;172:435–438
3.
Huang CC, Sheu CY, Chen TY, Yang YC. Aggressive angiomyxoma: a small palpable vulvar lesion with a huge mass in the pelvis. J Low Genit Tract Dis. 2013;17:75-78
ACCEPTED MANUSCRIPT 4.
McCluggage WG, Jamieson T, Dobbs SP, Grey A . Aggressive angiomyxoma of the vulva: dramatic response to gonadotropin-releasing hormone agonist therapy. Gynecol
Catalano O. Case report: Aggressive angiomyxoma of the pelvic soft tissues: US and CT
RI P
5.
T
Oncol. 2006;100:623–625
SC
findings. Clin Radiol. 1998;53(10):782-783
Sutton BJ, Laudadio J. Aggressive angiomyxoma. Arch Pathol Lab Med. 2012;136:217-2
7.
Jeyadevan NN, Sohaib SA, Thomas JM, Jeyarajah A, Shepherd JH, Fisher C.
NU
6.
MA
Imaging features of aggressive angiomyxoma. Clin Radiol. 2003;58:157–162
Elchalal U, Lischitz-Mercer B, Dgani R, Zalel Y. Aggressive angiomyxoma of the vulva.
ED
8.
9.
PT
Gynecol Oncol 1992; 47:260–262.
Chew FS, Hudson TM, Hawkins IF Jr. Radiology of infiltrating angiolipoma. AJR Am J
Stewart ST, McCarthy SM. Case 77: aggressive angiomyxoma. Radiology.
AC
10.
CE
Roentgenol 1980; 135(4):781-7
2004;233:697-700.
11.
Smith HO, Worrell RV, Smith AY, Dorin MH, Rosenberg RD,Bartow SA. Aggressive angiomyxoma of the female pelvis and perineum: review of the literature. Gynecol Oncol 1991; 42:79–85
12.
Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer. 1996;78(1):79-90
ACCEPTED MANUSCRIPT 13.
Suleiman M, Duc C, Ritz S, Bieri S. Pelvic excision of large aggressive angiomyxoma in
T
a woman: irradiation for recurrent disease. Int J Gynecol Cancer. 2006;16:356-60
RI P
Legend for images:
Figure 1. (a) Coronal and (b) Sagittal T2w MR images showing pelvic mass in right side of the
SC
urinary bladder causing mass effect over the urinary bladder. Inferiorly the pelvic floor is
aggressive angiomyxoma(white arrows).
NU
involved. Swirls of T2 hypointensity is noted within the mass which is characteristic of
MA
(c) Post-contrast fat suppressed T1w coronal image showing significant post-contrast
ED
enhancement (black arrow) and again showing the characteristic swirled appearance.
Figure 2. Histopathological photomicrograph at low magnification (a) shows loosely cellular
PT
lesion with myxoid background and ectatic blood vessels. Higher magnification shows stellate
CE
shaped cells with bland nuclear features. (c) strong positivity of the cytoplasm for desmin.
AC
Figure 3: (a) Sagittal T1w and (b) Sagittal T2w MR images showing, showing mass lesion in pelvis displacing uterus and the urinary bladder anteriorly. Note the extension of tumor into vulva and perineum (arrowhead). Swirls of T2 hypointensity is noted within the mass which is characteristic of aggressive angiomyxoma (arrows). Figure 4.Histopathological photomicrograph showing loosely arranged cells with bland nuclear features, in a myxoid background. Multiple vessels are also seen.
AC
Fig. 1A
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Fig. 1B
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Fig. 1C
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ACCEPTED MANUSCRIPT
S0899-7071(14)00102-8 doi: 10.1016/j.clinimag.2014.04.001 JCT 7603
To appear in:
Journal of Clinical Imaging
Received date: Revised date: Accepted date:
25 November 2013 5 April 2014 8 April 2014
Please cite this article as: Srinivasan Sivasubramanian, Krishnan Vijay, Ali Syed Zama, Chidambaranathan Natesan, Swirl sign” of Aggressive angiomyxoma – A lesser known diagnostic sign, Journal of Clinical Imaging (2014), doi: 10.1016/j.clinimag.2014.04.001
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: “Swirl
sign” of Aggressive angiomyxoma – A lesser known diagnostic sign.
Running Head: Swirl sign of Aggressive angiomyxoma
Authors: Sivasubramanian Srinivasan, M.D.,D.N.B., F.R.C.R., 1
SC
Vijay Krishnan, M.D., F.R.C.R., 1
NU
Syed Zama Ali M.D., F.R.C.R., 1 Natesan Chidambaranathan M.D., 2 1
RI P
T
Category: Case report
MA
Department of Diagnostic Radiology, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828
ED
2 Department of Radiology, Apollo Hospitals, Chennai 600006
PT
Address corespondence and reprint requests to:
Sivasubramanian Srinivasan,
AC
CE
Department of Diagnostic Radiology, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828, Republic of Singapore. Tel: (65) 66022689 Fax: (65) 66023796 Email: [email protected] Source(s) of support: Nil. Conflict of interest :Nil
ACCEPTED MANUSCRIPT “Swirl sign” of Aggressive angiomyxoma – A lesser known diagnostic sign. Abstract
T
Aggressive angiomyxoma is a rare mesenchymal tumour with high rate of local recurrence,found
RI P
mainly in pelvis and perineum of women of reproductive age group. We present cases of two patients,with emphasis on the magnetic resonance(MR) imaging characteristics.The clinical presentation was nonspecific.MR imaging revealed characteristic"Swirl sign"in T2-weighted
SC
sequence.Pre-operative diagnosis could not be made in the first patient, due to lack of experience about the diagnosis. Pre-operative diagnosis was made in the second case and was helpful for the
NU
surgeon to excise the mass completely. Aggressive angiomyxoma should be on the top of a
MA
differential diagnosis in the appropriate clinical and imaging scenario. Keywords
ED
Pelvic neoplasm, aggressive angiomyxoma, swirl sign, magnetic resonance imaging Introduction:
PT
Aggressive angiomyxoma is an exceedingly rare neoplasm of mesenchymal origin. Although it
CE
is histologically benign, it is very vascular and locally aggressive with infiltration of structures such as the pelvic floor. Clinical diagnosis may be difficult, however MR imaging usually shows the swirled strands of low signal intensities in T2w sequence which is diagnostic of this
AC
neoplasm. We report two cases of aggressive angiomyxoma (from different hospitals) which had characteristic MR imaging features. Case 1: A 37-year-old woman presented with vague lower abdominal pain of 5-6 months duration. No significant abnormality was noted on clinical examination. Ultrasound showed a vague mass in right side of the uterus with mass effect over the right side of the urinary bladder. The mass could not be well characterized and the extent could not be assessed. Hence MR imaging was done, which revealed a fairly well defined mass in the pelvic side wall, on the right side of bladder and the uterus with involvement of the pelvic floor inferiorly. Medially the lesion was compressing and displacing the urinary bladder. The lesion was hypo-intense on T1w and on T2w sequence (Fig. 1a, b), the mass showed a whirled pattern of T2 hypo-intensity within the
ACCEPTED MANUSCRIPT lesion. The mass demonstrated enhancement in post-contrast T1w images. Pre-operative diagnosis could not be made due to lack of experience of the radiologists. Surgical excision of the mass was planned. However total excision was not possible because of significant vascularity
T
and extensive pelvic floor infiltration. Histopathology (Fig. 2a,b) showed loosely cellular
RI P
mesenchymal neoplasm with ectatic blood vessels. The cells were stellate shaped and had bland nuclear features and the cytoplasm was positive for desmin (Fig. 2c).
SC
Case 2:
NU
A 36-year-old woman presented with painless abdominal swelling which was progressively increasing in size for more than 15 years. Upon physical examination, her
MA
abdomen was soft and there was no tenderness. Vague mass was palpable in the lower abdomen and pelvis. The laboratory findings were within normal limits.MR imaging of pelvis was performed,which revealed a large abdomino- pelvic mass measuring around 30cm in height.
ED
Inferior extent was into the recto-uterine pouch with displacement of the uterus and urinary bladder and is involving the perineum and vulva on the left side. On the T1-weighted image, the
PT
tumor was of isointense to muscles (Fig. 3a), and on T2-w sequence, it displayed hyperintense signal with “swirled” areas of low signal intensity within. (Fig. 3b).Based on high signal with
CE
swirled strands of low signal intensity on T2w sequence, location of the lesion and age of the patient, the diagnosis of aggressive angiomyxoma was made.The tumor was excised with wide
AC
local excision and histo-pathological examination of the specimen revealed mixture of myxoid and vascular elements (Fig. 4) consistent with aggressive angiomyxoma.
Discusssion Aggressive angiomyxoma of the female pelvis is a highly rare neoplasm, which was first described in 1983 (1) and has been sporadically reported since then. These tumors occur in the lower pelvis, perineal or the genital area of females, usually in the third to fifth decade and are six times more common in females (2). Clinically they present as an ill-defined pelvic or perineal mass. They may be clinically confused with pelvic abscesses, bartholin gland cysts (3) or hernias or sometimes, as adnexal masses. The exact pathogenesis is not clearly understood. The predominant location of the mass in the pelvis and the female predilection suggest that
ACCEPTED MANUSCRIPT hormonal factors play an important role (4). These are not malignant neoplasms, however they can be locally aggressive and tend to infiltrate adjacent structures (5).
T
Histological examination usually shows myxoid background of collagen fibres with small
RI P
vessels and loosely arranged cells which have bland features and may have spindle or stellate shapes. Cellular atypia and mitotic figures are rare. The tumour is usually positive for vimentin and desmin on immunohistochemistry. Other important characteristic is positivity for estrogen
SC
and/or progesterone receptors (6).
NU
Although initial screening modality would be an ultrasonography (US) examination, MR imaging is the modality of choice in characterization of the mass as well as assessing the
MA
extent of the mass. US and computed tomographyCT) features of aggressive angiomyxoma has been described (7). On US, the mass may appear hypo-echoic or cystic (7). The CT features are variable and include hypo-attenuating or iso-attenuating mass involving the pelvis and perineum
ED
with enhancement in the contrast–enhanced scan, which may show the classical swirled appearance. In the series of six cases, reported by Outwater et al(2), only 50% of the cases
PT
showed the classical swirling architecture on CT. MR imaging is more reliable because of better soft tissue resolution and the
CE
characteristic appearance (7). On MR imaging, the mass is usually iso-intense to muscle on T1weighted (T1w) image and hyperintese on T2w image with „swirled‟ areas of low signal areas
AC
within, as in our cases. This morphology is characteristic of this tumour (2). The hyperintense signal in T2w sequences is due to the myxoid element and presence of water. The swirled appearance is likely due to fibrovascular stroma which gets stretched as they extend and involve the pelvic diaphragm. On post-contrast T1w images, the mass shows significant enhancement due to vascular component of the neoplasm. The characteristic swirled appearance can also be seen in post-contrast T1w images (7). Infiltration into the perineum or pelvic floor muscles are best seen with MR imaging. Accurate evaluation of the extension or infiltration into the pelvic floor is important for surgical planning.
The imaging differential diagnosis includes myxoma, infiltrating angiolipoma and infiltrating liposarcoma. Myxoma is a benign mesenchymal neoplasm that most frequently occurs in older patients. However, myxomas are mainly intramuscular and show no significant
ACCEPTED MANUSCRIPT vascularity (8). Infiltrating angiolipoma is usually found in the thigh region and as the name implies it infiltrates and replaces involved muscle and subcutaneous tissue (9). Lack of high fat content is a key feature in differentiating the angiomyxoma from the main differential
T
considerations, myxoid liposarcoma and infiltrating angiolipoma (10).
RI P
The definitive treatment is surgery with wide local excision. Complete surgical excision has been reported to be technically difficult secondary to the bulky and locally
SC
infiltrative nature of this tumor (11). In the first patient, the pre-operative diagnosis was not made (on imaging) and hence surgical planning was difficult because of the significant
NU
vascularity and infiltration of the pelvic floor. The local recurrence is common and the reported incidence is around 30% (12) and usually seen in the first three years. The role of chemotherapy
MA
and radiation therapy is not well established. Reports of successful radiotherapy for tumour recurrences have been described (13). Treatment with gonadotropin-releasing hormone (GnRH) agonist has been reported to have shown tumour shrinkage and this is likely because of the
ED
estrogen/progesterone receptors. However there is no evidence with larger number of patients
PT
(4).
In conclusion, if the characteristic "swirl sign" is seen on MR imaging, then aggressive
CE
angiomyxoma can be confidently offered as a primary differential consideration. This is
References: 1.
AC
important clinically for surgical planning or a trial of medical management.
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;7:463-475
2.
Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: findings on CT and MR imaging. AJR Am J Roentgenol 1999;172:435–438
3.
Huang CC, Sheu CY, Chen TY, Yang YC. Aggressive angiomyxoma: a small palpable vulvar lesion with a huge mass in the pelvis. J Low Genit Tract Dis. 2013;17:75-78
ACCEPTED MANUSCRIPT 4.
McCluggage WG, Jamieson T, Dobbs SP, Grey A . Aggressive angiomyxoma of the vulva: dramatic response to gonadotropin-releasing hormone agonist therapy. Gynecol
Catalano O. Case report: Aggressive angiomyxoma of the pelvic soft tissues: US and CT
RI P
5.
T
Oncol. 2006;100:623–625
SC
findings. Clin Radiol. 1998;53(10):782-783
Sutton BJ, Laudadio J. Aggressive angiomyxoma. Arch Pathol Lab Med. 2012;136:217-2
7.
Jeyadevan NN, Sohaib SA, Thomas JM, Jeyarajah A, Shepherd JH, Fisher C.
NU
6.
MA
Imaging features of aggressive angiomyxoma. Clin Radiol. 2003;58:157–162
Elchalal U, Lischitz-Mercer B, Dgani R, Zalel Y. Aggressive angiomyxoma of the vulva.
ED
8.
9.
PT
Gynecol Oncol 1992; 47:260–262.
Chew FS, Hudson TM, Hawkins IF Jr. Radiology of infiltrating angiolipoma. AJR Am J
Stewart ST, McCarthy SM. Case 77: aggressive angiomyxoma. Radiology.
AC
10.
CE
Roentgenol 1980; 135(4):781-7
2004;233:697-700.
11.
Smith HO, Worrell RV, Smith AY, Dorin MH, Rosenberg RD,Bartow SA. Aggressive angiomyxoma of the female pelvis and perineum: review of the literature. Gynecol Oncol 1991; 42:79–85
12.
Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer. 1996;78(1):79-90
ACCEPTED MANUSCRIPT 13.
Suleiman M, Duc C, Ritz S, Bieri S. Pelvic excision of large aggressive angiomyxoma in
T
a woman: irradiation for recurrent disease. Int J Gynecol Cancer. 2006;16:356-60
RI P
Legend for images:
Figure 1. (a) Coronal and (b) Sagittal T2w MR images showing pelvic mass in right side of the
SC
urinary bladder causing mass effect over the urinary bladder. Inferiorly the pelvic floor is
aggressive angiomyxoma(white arrows).
NU
involved. Swirls of T2 hypointensity is noted within the mass which is characteristic of
MA
(c) Post-contrast fat suppressed T1w coronal image showing significant post-contrast
ED
enhancement (black arrow) and again showing the characteristic swirled appearance.
Figure 2. Histopathological photomicrograph at low magnification (a) shows loosely cellular
PT
lesion with myxoid background and ectatic blood vessels. Higher magnification shows stellate
CE
shaped cells with bland nuclear features. (c) strong positivity of the cytoplasm for desmin.
AC
Figure 3: (a) Sagittal T1w and (b) Sagittal T2w MR images showing, showing mass lesion in pelvis displacing uterus and the urinary bladder anteriorly. Note the extension of tumor into vulva and perineum (arrowhead). Swirls of T2 hypointensity is noted within the mass which is characteristic of aggressive angiomyxoma (arrows). Figure 4.Histopathological photomicrograph showing loosely arranged cells with bland nuclear features, in a myxoid background. Multiple vessels are also seen.
AC
Fig. 1A
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Fig. 1B
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