Vulva, vagina & female urethra
Mesenchymal neoplasms
Aggressive angiomyxoma


Topic Completed: 17 November 2020

Minor changes: 17 November 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Aggressive angiomyxoma vulva[TI]

David B. Chapel, M.D.
Jennifer A. Bennett, M.D.
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Cite this page: Chapel DB, Bennett J. Aggressive angiomyxoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvaaggressiveangiomyxoma.html. Accessed November 25th, 2020.
Definition / general
  • Aggressive angiomyxoma is an infiltrative spindle cell neoplasm arising in the soft tissues of the lower genital tract, perineum and pelvis; approximately 30% recur locally but distant metastasis is exceptionally rare
Essential features
  • Unique to the soft tissues of the lower genital tract, pelvis and perineum
  • Infiltrative hypocellular myxoid lesion with bland spindle cells and prominent variably sized vessels
  • HMGA2 overexpressed by immunohistochemistry in > 90%
  • Local recurrence in ~ 30% but distant metastasis and death from disease are exceptionally rare
ICD coding
  • ICD-10: D48.1 - neoplasm of uncertain behavior of connective and other soft tissue
Sites
Pathophysiology
  • Histogenesis uncertain
  • May arise from site specific stromal cells with capacity for fibroblastic, myofibroblastic and smooth muscle differentiation (Cancer 1996;78:79)
Clinical features
Diagnosis
Radiology description
Radiology images

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Ultrasound and MRI Ultrasound and MRI Ultrasound and MRI Ultrasound and MRI Ultrasound and MRI Ultrasound and MRI

Ultrasound and MRI


MRI

MRI

Perineal, MRI

Perineal, MRI

Prognostic factors
Case reports
Treatment
Clinical images

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Paravaginal, intraoperative

Paravaginal, intraoperative

Perineal, intraoperative

Perineal, intraoperative

Vulvar, preoperative

Vulvar, preoperative

Vulvar, pre and postoperative

Vulvar, pre and postoperative

Gross description
Gross images

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Cut surface

Cut surface

Microscopic (histologic) description
  • Unencapsulated and locally infiltrative (Am J Surg Pathol 1983;7:463, Hum Pathol 1985;16:621, Am J Dermatopathol 1993;15:446, Histopathology 1997;30:3)
  • Hypocellular (though focally increased cellularity is common) (Histopathology 1997;30:3)
  • Tumor cells:
    • Spindle to stellate with delicate cytoplasmic processes
    • Bland chromatin with small nucleoli
    • Mitoses rare or absent
  • Tumor stroma:
    • Myxoid stroma with scattered delicate collagen fibers
    • Stromal mucin positive for Alcian blue
    • Stroma peripherally entraps fat, nerves and muscle
    • Extravasated red blood cells common (Histopathology 1997;30:3)
    • Necrosis absent
  • Vasculature:
    • Conspicuous haphazard dilated capillaries
    • Scattered large, thick walled (medial hypertrophy) or hyalinized vessels
    • Vessels are nonanastomosing but may cluster together
    • Stromal smooth muscle bundles cluster around tumor vessels (Cancer 1996;78:79, Histopathology 1997;30:3)
  • Recurrent lesions may show increased cellularity, increased vasculature and dense stromal collagen
  • Rare cases may show minor foci with morphologic features of angiomyofibroblastoma (Histopathology 1997;30:3)
Microscopic (histologic) images

Contributed by David B. Chapel, M.D.
Infiltrative growth

Infiltrative growth

Entrapped adipose tissue

Entrapped adipose tissue

Extravasated red blood cells

Extravasated red blood cells

Thick walled vessels

Thick walled vessels

Myoid bundles

Myoid bundles

Entrapped nerve

Entrapped nerve


Bland cytology

Bland cytology

HMGA2

HMGA2

Virtual slides

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Aggressive angiomyxoma

Aggressive angiomyxoma

32 year old woman with a 6 cm vulvar mass

32 year old woman with a 6 cm vulvar mass

Aggressive angiomyxoma

Aggressive angiomyxoma

Positive stains
Electron microscopy description
Molecular / cytogenetics description
Molecular / cytogenetics images

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FISH, karyotype

FISH, karyotype

Videos

Histopathology

Sample pathology report
  • Vulva, mass, wide local excision:
    • Aggressive angiomyoma with involvement of the deep margin (see comment)
    • Comment: Microscopic examination reveals a poorly circumscribed hypocellular lesion with myxoid stroma and abundant vasculature, infiltrating fibroadipose tissue. By immunohistochemistry, lesional stromal cells are positive for desmin, ER, PR and HMGA2. The morphologic and immunophenotypic findings are most consistent with aggressive angiomyxoma. Although distant metastasis is exceptionally rare, approximately 30% of such lesions recur locally. Clinical follow up is advised.
Differential diagnosis
Additional references
Board review style question #1

A 35 year old woman presented to her gynecologist with a complaint of pelvic discomfort and a slowly growing, ill defined vulvar mass. Radiology showed a vascular lesion extending from the vulva into deep pelvic soft tissues. An excision was performed. A representative photomicrograph of the lesion is shown. By immunohistochemistry, the lesional stromal cells were positive for desmin, ER and PR. Which of the following statements about this lesion is true?

  1. Approximately 50% of patients develop distant metastases
  2. FISH reveals a characteristic DDIT3 rearrangement
  3. HMGA2 is positive (overexpressed) by immunohistochemistry
  4. Immunohistochemistry for S100 is characteristically positive
  5. Less than 10% of patients experience local recurrence
Board review answer #1
C. HMGA2 is positive (overexpressed) by immunohistochemistry. This is an aggressive angiomyxoma.

Comment Here

Reference: Aggressive angiomyxoma
Board review style question #2
Which of the following most accurately describes the expected immunophenotype of aggressive angiomyxoma?

  1. Desmin, ER, PR, HMGA2 positive; S100 negative
  2. S100, EMA, NF positive; ER, PR negative
  3. SMA, CD34 positive; ER, PR negative
  4. SMA, desmin, caldesmon positive; S100 negative
  5. SMA, ER, PR positive; Rb negative (lost)
Board review answer #2
A. Desmin, ER, PR, HMGA2 positive; S100 negative

Comment Here

Reference: Aggressive angiomyxoma
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