Vulva
Malignant neoplasms
Aggressive angiomyxoma

Author: Priya Nagarajan, M.D., Ph.D. (see Authors page)
Editor: Sara Peters, Ph.D., M.D.

Revised: 5 October 2017, last major update January 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Aggressive angiomyxoma [title] vulva

Cite this page: Nagarajan, P. Aggressive angiomyxoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvaaggressiveangiomyxoma.html. Accessed November 25th, 2017.
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

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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

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Images contributed by Dr. Mark R. Wick:


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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

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Images contributed by Dr. Mark R. Wick:
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Aggressive angiomyxoma

Ectatic blood vessel

Prominent myoid differentiation

Myoid differentiation


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)