Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Monday, 21 Mar 2005
Case Presentation and Mini-review
A Case with Infiltrating Perineal Mass Lin, Kuei-yu (Paul), National Taiwan University, School of Medicine Year VI Gillian Lieberman, M.D. Beth Israel Deaconess Medical Center
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
History • 43 year old woman, asymptomatic • 1998 : Bartholin’s gland enlargement, grape size • Aug 2000 : obvious growth noted Æ surgical excision + pathology exam : aggressive angiomyxoma • Sep 2000 : palpable spongy vulvar mass Æ re-excision Æ MR F/U • Dec 2001: post-operative scarring • Jun 2004 : tumor recurrence, observe • Mar 2005 : MR F/U 2
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR-T1WI
Well-defined mass, Iso-intense compared with muscle
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T2WI
Heterogeneously hyperintense mass, swirled appearance in the lesion
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T2
The lesion extending into the pelvis. It is 3.4 x 2x 8.7 cm
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T2 FS Signal of fat has been suppressed.
There is no obvious fatty tissue within the mass because the high signal is not suppressed.
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T1 post Gd Fat suppression
Vessels are enhanced.
This is a hypervascular lesion.
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T2 Local Invasion
Local infiltration of levator ani muscle
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T1 post Gd An evidence of tumor infiltration: levator ani muscle is abnormally enhanced.
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Discussion • Aggressive angiomyxoma (AAM) • Differential diagnosis
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Aggressive angiomyxoma • Rare benign soft-tissue tumor with myxoid and vascular components • Involving mainly the pelvis—particularly the perineum—in women of childbearing age • Propensity to grow to large sizes • Locally infiltrative, No metastases • Propensity to recur locally
From http://www.geocities.com/sampyroy2000/AgA1.jpg
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Image findings • CT – Well-defined mass within the pelvis or perineum that is iso- or hypoattenuated relative to muscle. – Moderately enhanced
From: Sarah T. Stewart, MD and Shirley M. McCarthy, MD, PhD, Case 77: Aggressive Angiomyxoma, Radiology 2004;233:697-700. 12
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Image findings • T1-weighted MR images: – Isointense when compared with muscle – Moderately enhanced by Gd – Lack of high fat content
• T2-weighted MR images – High signal intensity – Distinctive internal architecture composed of a swirled or layered appearance 13
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR-T1WI
Well-defined mass, Iso-intense compared with muscle
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T2WI
Heterogeneously hyperintense mass, swirled appearance in the lesion
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
MR T1 post Gd Fat suppression
Vessels are enhanced.
This is a hypervascular lesion.
From BIDMC PACS
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Pathology • Spindle cells separated by a myxoid stroma • Variably sized vessels. • Mitotic activity has been shown to be rare in most cases
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Pathology
MR T2WI showing hyperintensity caused by fluid-like myxoid stroma From Sarah T. Stewart, MD et al., Radiology 2004;233:697-700 (Right) & http://www.geocities.com/sampyroy2000/AgA1.jpg (left)
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Pathology
MR T1 post Gd, showing hetergeneously enhanced by hypervascularity From Sarah T. Stewart, MD et al., Radiology 2004;233:697-700 (Right) & http://www.telepathology.mds.qmw.ac.uk/ (left)
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Differential diagnosis • • • •
Myxoid Liposarcoma Myxoma Infiltrating angiolipoma Myxoid lipoma
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid liposarcoma • Malignant tumor • Most commonly occurs in lower extremities within the intramuscular fat • Post Gd: Homogenous enhancement • Some has high fat content
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid liposarcoma
Histology of Myxoid liposarcoma, showing plenty of adipose tissue From http://www.wheelessonline.com/image3/i1/lipm1.jpg 22
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid liposarcoma
Hyperintense fatty tissue at T1
Low signal intensity at FS
From Mark J. Kransdorf, MD, et al., Radiology 2002;224:99-104
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid lipoma • • • •
Morphologic variant of lipoma Benign counterpart to liposarcoma Predominantly myxoid in nature Mature adipose tissue
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid lipoma
Histology of Myxoid lipoma, showing plenty of adipose tissue From http://erl.pathology.iupui.edu/C603/IMAGES/5AF10.JPG 25
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoid lipoma
Hyperintense fatty tissue at T1
. From Mark J. Kransdorf, MD, et al. Imaging of Fatty Tumors:, Radiology 2002;224:99-104
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Infiltrating angiolipoma • • • • •
Benign soft-tissue mass Regional infiltration; No metastasis Hypervascular lesion similar to AAM Usually found in the thigh Composed of mature lipocytes
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Infiltrating angiolipoma
Histology of Infiltrating angiolipoma, showing adipose tissue From http://www.sapporo-dermpath.com/angilipo0204622/angiolipo.html 28
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Myxoma • Benign mesenchymal neoplasm • Lacks the vascular component • Mainly intramuscular
MR T1 post Gd, showing not enhanced centrally due to lack of vascular component From Mark D. Murphey, MD, Gina A. et al. Radiology 2002;225:215-224. 29
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Differential diagnosis
High Fat HyperContent vascul arity
Common location
Others
Aggressive Angiomyxoma
X
O
Pelvis Perineum
Whorledappearance Large and infiltrating
Myxoid liposarcoma
O
O
Lower ext. Intramuscular
Var-defined
Myxoid lipoma
O
X
Infiltrating angiolipoma
O
O
Intramuscular
Well-defined
Myxoma
X
X
Intramuscular
Well-defined
Well-defined
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
AAM Treatment • Primary treatment : Surgical excision • Recurrence rates of 36%–72% • Radiation therapy or chemotherapy is not helpful
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Conclusion • MR imaging shows the angiomatous and myxomatous natures of AAM well • MR images is valuable in diagnosis of this tumor, evaluation of tumor extent, and planning of surgery • Histology and radiology • Make use of different MR sequences 32
Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Reference • •
Mark D. Murphey, MD, Gina A. McRae, MD2, et al., Musculoskeletal Imaging Imaging of Soft-Tissue Myxoma with Emphasis on CT and MR and Comparison of Radiologic and Pathologic Findings Radiology 2002;225:215-224 Sarah T. Stewart, MD and Shirley M. McCarthy, MD, PhD, Case 77: Aggressive Angiomyxoma, Radiology 2004;233:697-700.
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Mark J. Kransdorf, MD, et al. Imaging of Fatty Tumors: Distinction of Lipoma and Well-differentiated Liposarcoma, Radiology 2002;224:99-104.
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Antonio Aversa do SoutoI; Flavio S. DominguesI; Leila ChimelliII; Armando M. LemosI Salvador, Bahia - Brazil, September 20 to 23, 2005 Outwater EK Marchetto BE Wagner BJ Siegelman ES, Aggressive angiomyxoma: findings on CT and MR imaging. AJR Am J Roentgenol (1999 Feb) 172(2):435-8 Chien AJ Freeby JA Win TT Gadwood KA Aggressive angiomyxoma of the female pelvis: sonographic, CT, and MR findings. AJR Am J Roentgenol (1998 Aug) 171(2):530-1 Davani M Chablani VN Saba PR, Aggressive angiomyxoma of pelvic soft tissues: MR imaging appearance. AJR Am J Roentgenol (1998 Apr) 170(4):1113-4 Destian S Ritchie WG Aggressive angiomyxoma: CT appearance. Am J Gastroenterol (1986 Aug) 81(8):711-3
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Acknowledgements • • • • • •
Gillian Lieberman, MD Pamela Lepkowski Larry Barbaras Ivan Pedrosa, MD Jesse Wei, MD Avneesh Gupta, MD
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Lin, Kuei-yu (Paul) Gillian Leiberman, MD
Thank you for your attention !!
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