Vulva & female urethra
Other tumors
Aggressive angiomyxoma


Topic Completed: 1 January 2014

Revised: 6 August 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Aggressive angiomyxoma [title] vulva

Priya Nagarajan, M.D., Ph.D.
Sara B. Peters, M.D., Ph.D.
Page views in 2018: 9,191
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Cite this page: Nagarajan P. Aggressive angiomyxoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvaaggressiveangiomyxoma.html. Accessed August 24th, 2019.
Definition / general
  • Rare, locally infiltrative mesenchymal tumor of women of reproductive age and infrequently men (see Testis chapter), usually arising in perineal region
  • Originally described by Steeper and Rosai in 1983 (Am J Surg Pathol 1983;7:463)
  • > 250 cases reported to date
Epidemiology
  • Women: predominantly in early reproductive years
  • Adult men: less commonly affected
  • Female to male ratio ~6:1
  • Rare in children (Pediatr Surg Int 2005;21:563)
Sites
Pathophysiology
  • Unclear but tumor is mesenchymal in origin
  • Hormonal (estrogen and progesterone) influence may also play a role
Clinical features
  • May be an incidental finding
  • Slow growing palpable or visible tumor of vulva, gluteal region or suprapubic region
  • Imaging frequently reveals pelvic extension of huge mass
  • Patients often complain of mass, dull aching pain, urinary and gastrointestinal symptoms such as dysuria, urinary retention, dyspareunia (Can J Urol 2011;18:5908)
Diagnosis
  • Histologic examination of core biopsies or more commonly the resection specimen
Radiology description
  • Extensive imaging to assess extent of tumor is necessary prior to surgery
  • Ultrasound (abdominal, endorectal and scrotal) demonstrates a soft, hypoechoic, solid mass (Can J Urol 2011;18:5908)
  • CT scan: (Abdom Imaging 2011;36:739)
    • Unenhanced CT scan shows a low density mass
    • Contrast enhanced CT scan shows a mildly enhancing mass with an internal swirling pattern
  • MRI: (Case Rep Oncol 2013;6:373)
    • Shows bulky perineal and intrapelvic tumor displacing rather than infiltrating surrounding structures
    • Diffusion weighted MRI has diagnostic and prognostic value
    • T1 weighted imaging: tumor is isointense to muscle
    • T2 weighted imaging: tumor shows high signal intensity with layered strands of lower signal intensity and swirled architecture
    • May have finger-like projections into surrounding fat
    • Followup MRI useful to detect recurrence after resection
Radiology images

Contributed by Dr. Mark R. Wick
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CT scan



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Axial noncontrast CT

Prognostic factors
  • Recurrence is common due to incomplete excision
  • Involvement close to urethra, vagina, rectum and anal sphincter as well as extension through pelvic diaphragm is associated with incomplete resection
  • Vascular invasion is associated with distant metastasis to lungs and mediastinum, leading to death (Korean J Radiol 2012;13:90, Hum Pathol 2003;34:1072, N Engl J Med 1999;341:1772)
Case reports
Treatment
  • Wide surgical resection as an en bloc specimen is usually accomplished by a combined transperineal and abdominopelvic approach, with preservation of surrounding structures
  • Preoperative vascular embolization may decrease the tumor size
  • Lifetime followup to monitor for recurrence is mandatory
  • Chemotherapy and radiotherapy are ineffective due to the low proliferative potential of the lesional cells but high dose radiotherapy has been used successfully to treat recurrent disease (Int J Gynecol Cancer 2006;16:356)
  • Hormonal therapy with gonadotrophin releasing hormone (GnRH) inhibitors such as leuprolide is useful for recurrent tumors that are positive for estrogen and progesterone receptors (J Low Genit Tract Dis 2014;18:E55)
  • Nonsteroidal selective estrogen receptor modulators (SERM) such as raloxifene have also been used (Gynecol Oncol 2011;123:172)
Clinical images

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Well defined polypoidal pedunculated

Pedunculated mass

Intraoperative

Gross description
  • Typically a pedunculated vulvar mass
  • Usually large, partly encapsulated, relatively circumscribed and soft to firm in consistency
  • Cut surface is homogenous, gelatinous and usually glistening with no obvious hemorrhage or necrosis
Gross images

Contributed by Dr. Mark R. Wick
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Aggressive angiomyxoma



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Aggressive angiomyxoma

Well circumscribed fleshy tan gelatinous mass

Specimen cut open

Microscopic (histologic) description
  • Monotonous and hypocellular, composed of small spindled and stellate fibroblasts with no atypia, extremely rare mitoses and no atypical mitotic figures
  • Stroma is myxoid with collagen fibers and prominent, dilated, thick walled vessels, which may be hyalinized with a prominent vascular smooth muscle layer
  • Peripheral infiltrative margins with extension into surrounding muscles, nerves, adipose tissue and rarely vascular structures
  • Red blood cell extravasation may be present (J Clin Pathol 2000;53:798)
  • Some tumors have a component of admixed adipose tissue, unclear if due to extensive infiltration (Can J Plast Surg 2007;15:163)
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick
Missing Image Missing Image Missing Image Missing Image

Various images



Contributed by Dr. Pryia Nagarajan

Aggressive angiomyxoma

Ectatic blood vessel

Prominent myoid differentiation

Myoid differentiation




Images hosted on other servers:

Various images

Thick walled vessels: H&E and trichrome


CD34+

ER+

Vimentin+

Cytology description
  • Smears show arborizing capillaries in a loose myxoid background containing dispersed cells
  • Cells are spindled or stellate with delicate elongated cytoplasm and bland vesicular nuclei with fine chromatin and inconspicuous nucleoli (Cytopathology 2010;21:207)
Positive stains
Electron microscopy description
  • Lesional cells show myofibroblastic differentiation consisting of irregular but delicate cellular processes, prominent golgi apparatus, dilated rough endoplasmic reticulum
  • Cytoplasm has several intermediate filaments, while the stroma contains aggregates of collagen fibers and fine granular material (Hum Pathol 1985;16:621)
Molecular / cytogenetics description
Molecular / cytogenetics images

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t(12;21)

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