A Case with Infiltrating Perineal Mass

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1 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

2 History 43 year old woman, asymptomatic 1998 : Bartholin s gland enlargement, grape size Aug 2000 : obvious growth noted surgical excision + pathology exam : aggressive angiomyxoma Sep 2000 : palpable spongy vulvar mass re-excision MR F/U Dec 2001: post-operative scarring Jun 2004 : tumor recurrence, observe Mar 2005 : MR F/U 2

3 MR-T1WI Well-defined mass, Iso-intense compared with muscle From BIDMC PACS 3

4 MR T2WI Heterogeneously hyperintense mass, swirled appearance in the lesion From BIDMC PACS 4

5 MR T2 The lesion extending into the pelvis. It is 3.4 x 2x 8.7 cm From BIDMC PACS 5

6 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 6

7 MR T1 post Gd Fat suppression Vessels are enhanced. This is a hypervascular lesion. From BIDMC PACS 7

8 MR T2 Local Invasion Local infiltration of levator ani muscle From BIDMC PACS 8

9 MR T1 post Gd An evidence of tumor infiltration: levator ani muscle is abnormally enhanced. From BIDMC PACS 9

10 Discussion Aggressive angiomyxoma (AAM) Differential diagnosis 10

11 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 11

12 CT AAM Image findings 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:

13 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

14 MR-T1WI Well-defined mass, Iso-intense compared with muscle From BIDMC PACS 14

15 MR T2WI Heterogeneously hyperintense mass, swirled appearance in the lesion From BIDMC PACS 15

16 MR T1 post Gd Fat suppression Vessels are enhanced. This is a hypervascular lesion. From BIDMC PACS 16

17 AAM Pathology Spindle cells separated by a myxoid stroma Variably sized vessels. Mitotic activity has been shown to be rare in most cases 17

18 AAM Pathology MR T2WI showing hyperintensity caused by fluid-like myxoid stroma From Sarah T. Stewart, MD et al., Radiology 2004;233: (Right) & (left) 18

19 AAM Pathology MR T1 post Gd, showing hetergeneously enhanced by hypervascularity From Sarah T. Stewart, MD et al., Radiology 2004;233: (Right) & (left) 19

20 Differential diagnosis Myxoid Liposarcoma Myxoma Infiltrating angiolipoma Myxoid lipoma 20

21 Myxoid liposarcoma Malignant tumor Most commonly occurs in lower extremities within the intramuscular fat Post Gd: Homogenous enhancement Some has high fat content 21

22 Myxoid liposarcoma Histology of Myxoid liposarcoma, showing plenty of adipose tissue From 22

23 Myxoid liposarcoma Hyperintense fatty tissue at T1 Low signal intensity at FS From Mark J. Kransdorf, MD, et al., Radiology 2002;224:

24 Myxoid lipoma Morphologic variant of lipoma Benign counterpart to liposarcoma Predominantly myxoid in nature Mature adipose tissue 24

25 Myxoid lipoma Histology of Myxoid lipoma, showing plenty of adipose tissue From 25

26 Myxoid lipoma Hyperintense fatty tissue at T1. From Mark J. Kransdorf, MD, et al. Imaging of Fatty Tumors:, Radiology 2002;224:

27 Infiltrating angiolipoma Benign soft-tissue mass Regional infiltration; No metastasis Hypervascular lesion similar to AAM Usually found in the thigh Composed of mature lipocytes 27

28 Infiltrating angiolipoma Histology of Infiltrating angiolipoma, showing adipose tissue From 28

29 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:

30 Differential diagnosis High Fat Content Hypervascul arity Common location Others Aggressive Angiomyxoma X O Pelvis Perineum Whorledappearance Large and infiltrating Var-defined Myxoid liposarcoma O O Lower ext. Intramuscular Myxoid lipoma O X Well-defined Infiltrating angiolipoma O O Intramuscular Well-defined Myxoma X X Intramuscular Well-defined 30

31 AAM Treatment Primary treatment : Surgical excision Recurrence rates of 36% 72% Radiation therapy or chemotherapy is not helpful 31

32 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

33 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: Sarah T. Stewart, MD and Shirley M. McCarthy, MD, PhD, Case 77: Aggressive Angiomyxoma, Radiology 2004;233: Mark J. Kransdorf, MD, et al. Imaging of Fatty Tumors: Distinction of Lipoma and Well-differentiated Liposarcoma, Radiology 2002;224: 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): Destian S Ritchie WG Aggressive angiomyxoma: CT appearance. Am J Gastroenterol (1986 Aug) 81(8):

34 Acknowledgements Gillian Lieberman, MD Pamela Lepkowski Larry Barbaras Ivan Pedrosa, MD Jesse Wei, MD Avneesh Gupta, MD 34

35 Thank you for your attention!! 35

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