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aggressive angiomyxoma

Tuesday 7 February 2006

AAMs, ’aggressive’ angiomyxoma; deep aggressive angiomyxoma

PO

Definition: Aggressive angiomyxoma (AA) is a distinctive, locally aggressive, fibromyxoid tumor of the pelvic and genital soft tissues.

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

Digital case

- JRC:1443 : Aggresive angiomyxoma of pelvic soft tissues.
- JRC:14570 : Aggresive angiomyxoma of peritoneum (ischiorectal fossa).

Images

- Deep (agressive) angiomyxoma. Young women vulvar tumor

AA is of unknown histogenesis but the cytologically bland spindled tumor cells, which surround characteristic variegated blood vessels, show fibroblastic or myofibroblastic features.

AA may be related to angiomyofibroblastoma (AMF), another cytologically bland fibromyxoid genital spindle cell tumor with variable myoid differentiation that does not, as a rule, recur.

The lesion formerly known as ’aggressive angiomyxoma’, which shows a predilection for the pelviperineal region of women, has been redesignated as deep ’aggressive’ angiomyxoma in order to de-emphasize the perception of aggression.

It is now appreciated that no more than 30% of these lesions recur locally and the majority of such recurrences are cured by one further re-excision.

Original perceptions concerning the more aggressive nature of this tumour type were likely to have been influenced by the inevitably skewed nature of consultation material.

Definition: Aggressive angiomyxoma is a large invasive vulvar lesion, with hypocellularity, bland stellate and spindle cells, prominent vessels and myxoid stroma.

Aggressive angiomyxoma is an invasive deep soft tissue lesion, most frequently found in the vulva; it is rarely found in the scrotum in men. It is commonly larger than 10 cm in greatest dimension, and is non-circumscribed. It is hypocellular, has bland stellate and spindle cells, prominent vessels and myxoid stroma. It is positive for desmin, vimentin, ER and PR.

Digital cases

- JRC:1443 : Aggressive angiomyxoma of pelvic soft tissues
- JRC:3175 : Aggressive angiomyxoma of pelvic soft tissues in a 40 y-o female.
- JRC:14570 : Aggressive angiomyxoma of the ischiorectal fossa.
- JRC:91 : Angiomyofibroblastoma or Aggressive angiomyxoma - rectovaginal septum.
- JRC:14781 : Aggressive angiomyxoma of the perirectal space.

Differential diagnosis

- myxoid leiomyoma
- myxoid neurofibroma
- myxolipoma
- intramuscular myxoma
- angiomyofibroblastoma

See also

- angiomyxomas

  • deep aggresive angiomyxoma

Synopsis

- tumoral cells: wavy and fibrillar, spindled, stellate
- polygonal fibroblast-like CD34+ dendritic cells
- +/- nuclear and cytoplasmic staining for FXIIIa

Differential diagnosis

- angiomyofibroblastoma (AMF)

  • AA and AMF are myxoid fibrohistiocytic tumors composed of CD34+ fibroblasts.
  • Neoplastic CD34+ dendritic fibroblasts showed predominantly myxo-collagenous differentiation.
  • Both AMF and AA are part of a morphologic and histogenetic continuum of myxofibrous and myoid tumors that may arise due to interactions between microvascular CD34+ fibroblasts and FXIIIa+ histiocytes.

- myxoid tumors

Localization

- vulva, vagina (vulvovaginal aggressive angiomyxoma)
- pelvis soft tissues (aggresive angiomyxoma of pelvic soft tissues)
- hypodermis (hypodermal aggressive angiomyxoma)
- larynx (laryngeal aggressive angiomyxoma) (8868184)

Cytogenetics

- 12q13-15 rearrangement with HMGA2 rearrangement and abnormal expression in aggressive angiomyxoma

Molecular biology

- HMGA2 rearrangements in 33% of AAMs (17654722)

See also

- CD34+ primitive fibroblasts
- factor XIIIa+ dendritic histiocytes
- fibrovascular tumors
- fibrohistiocytic tumors
- myxoid soft tissue tumors
- microvascular unit (tissue responsible for stromal repair and remodeling and angiogenesis)
- CD34+ fibroblasts
- FXIIIa+ histiocytes (FXIIIa+ dendritic cells or FXIIIa+ dendritic histiocytes)

- angiomyxomas

  • superfical angiomyxoma (cutaneous myxoma)
  • deep angiomyxoma

- myxoid tumors

References

- Rawlinson NJ, West WW, Nelson M, Bridge JA. Aggressive angiomyxoma with t(12;21) and HMGA2 rearrangement: report of a case and review of the literature. Cancer Genet Cytogenet. 2008 Mar;181(2):119-24. PMID: 18295664

- The evolving classification of soft tissue tumours: an update based on the new WHO classification. Fletcher CD. Histopathology. 2006 Jan;48(1):3-12. PMID: 16359532

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