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| {{redirect|Angiomyxoma|the tumor sometimes called "Angiomyoma"|Angioleiomyoma}} | | {{Short description|A rare, slow-growing soft tissue tumor}} |
| {{Infobox medical condition (new)
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| | name = Angiomyxoma
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| | image = aggressive angiomyxoma - intermed mag.jpg
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| | caption = [[Micrograph]] of an aggressive angiomyxoma. [[Core biopsy]]. [[H&E stain]].
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| '''Angiomyxoma''' is a [[myxoid tumor]] involving the [[blood vessel]]s.
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| It can affect the [[vulva]]<ref name="pmid18603701">{{cite journal |vauthors=Mandal S, Dhingra K, Roy S, Khurana N |title=Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling |journal=Indian J Pathol Microbiol |volume=51 |issue=2 |pages=259–60 |year=2008 |pmid=18603701 |doi= 10.4103/0377-4929.41677}}</ref> and other parts of the [[pelvis]]. The characteristic feature of this tumor is its frequent local recurrence and it is currently regarded as a [[metastasis|non-metastasizing]] benign tumor.<ref name="pmid17568501">{{cite journal |vauthors=Mathieson A, Chandrakanth S, Yousef G, Wadden P |title=Aggressive angiomyxoma of the pelvis: a case report |journal=Can J Surg |volume=50 |issue=3 |pages=228–9 |date=June 2007 |pmid=17568501 |pmc=2384290 |doi= |url=http://www.cma.ca/multimedia/staticContent/HTML/N0/l2/cjs/vol-50/issue-3/pdf/pg228.pdf |access-date=2008-08-24 |archive-url=https://web.archive.org/web/20160805151812/https://www.cma.ca/multimedia/staticContent/HTML/N0/l2/cjs/vol-50/issue-3/pdf/pg228.pdf |archive-date=2016-08-05 |url-status=dead }}</ref>
| | '''Aggressive angiomyxoma''' is a rare type of [[soft tissue tumor]] that primarily affects the [[pelvic]] and [[perineal]] regions. It is characterized by its locally aggressive behavior and tendency to recur after surgical removal. Despite its name, aggressive angiomyxoma is generally considered a benign tumor because it does not metastasize to distant sites. |
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| == Genetics == | | ==Presentation== |
| * HMGA2 rearrangement
| | Aggressive angiomyxoma typically presents as a slow-growing mass in the [[pelvis]] or [[perineum]]. It is more common in [[women]] than in [[men]], with a higher incidence in women of reproductive age. Patients may experience symptoms such as a palpable mass, discomfort, or [[pain]] in the affected area. In some cases, the tumor may cause [[urinary]] or [[bowel]] symptoms due to its location and size. |
| ** by translocation t(12;21)(q15;q21.1)<ref>{{Cite journal | pmid = 18295664| year = 2008| last1 = Rawlinson| first1 = N. J.| title = Aggressive angiomyxoma with t(12;21) and HMGA2 rearrangement: Report of a case and review of the literature| journal = Cancer Genetics and Cytogenetics| volume = 181| issue = 2| pages = 119–24| last2 = West| first2 = W. W.| last3 = Nelson| first3 = M| last4 = Bridge| first4 = J. A.| doi = 10.1016/j.cancergencyto.2007.11.008| pmc = 2396496}}</ref><ref>{{Cite journal | pmid = 17654722| year = 2007| last1 = Medeiros| first1 = F| title = Frequency and characterization of HMGA2 and HMGA1 rearrangements in mesenchymal tumors of the lower genital tract| journal = Genes, Chromosomes and Cancer| volume = 46| issue = 11| pages = 981–90| last2 = Erickson-Johnson| first2 = M. R.| last3 = Keeney| first3 = G. L.| last4 = Clayton| first4 = A. C.| last5 = Nascimento| first5 = A. G.| last6 = Wang| first6 = X| last7 = Oliveira| first7 = A. M.| doi = 10.1002/gcc.20483}}</ref>
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| ** by translocation t(11;12)(q23;q15)<ref>{{Cite journal | pmid = 16568309| year = 2006| last1 = Micci| first1 = F| title = Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15)| journal = Virchows Archiv| volume = 448| issue = 6| pages = 838–42| last2 = Panagopoulos| first2 = I| last3 = Bjerkehagen| first3 = B| last4 = Heim| first4 = S| doi = 10.1007/s00428-006-0186-5}}</ref>
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| ** by translocation t(8;12)(p12;q15)<ref>{{Cite journal | pmid = 11550285| year = 2001| last1 = Nucci| first1 = M. R.| title = Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva| journal = Genes, Chromosomes and Cancer| volume = 32| issue = 2| pages = 172–6| last2 = Weremowicz| first2 = S| last3 = Neskey| first3 = D. M.| last4 = Sornberger| first4 = K| last5 = Tallini| first5 = G| last6 = Morton| first6 = C. C.| last7 = Quade| first7 = B. J.| doi = 10.1002/gcc.1179}}</ref>
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| * t(5;8)(p15;q22)<ref>{{Cite journal | pmid = 11550285| year = 2001| last1 = Nucci| first1 = M. R.| title = Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva| journal = Genes, Chromosomes and Cancer| volume = 32| issue = 2| pages = 172–6| last2 = Weremowicz| first2 = S| last3 = Neskey| first3 = D. M.| last4 = Sornberger| first4 = K| last5 = Tallini| first5 = G| last6 = Morton| first6 = C. C.| last7 = Quade| first7 = B. J.| doi = 10.1002/gcc.1179}}</ref>
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| == Pathology == | | ==Pathology== |
| === Microscopy ===
| | The tumor is composed of [[myxoid]] stroma with scattered [[blood vessels]] and [[spindle-shaped cells]]. The [[histological]] appearance is characterized by a loose, [[myxoid]] matrix with [[stellate]] and [[spindle cells]]. The [[vascular]] component is prominent, with numerous thin-walled vessels. Immunohistochemical staining often shows positivity for [[estrogen]] and [[progesterone receptors]], which suggests a hormonal influence on tumor growth. |
| * Vascular appearance of tumor<ref>{{Cite journal | pmid = 22247641| year = 2012| last1 = Geng| first1 = J| title = Aggressive angiomyxoma: An unusual presentation| journal = Korean Journal of Radiology| volume = 13| issue = 1| pages = 90–3| last2 = Cao| first2 = B| last3 = Wang| first3 = L| doi = 10.3348/kjr.2012.13.1.90| pmc = 3253408}}</ref>
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| * Hypocellular mesenchymal lesion
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| * Spindled and stellate cells with an ill-defined cytoplasm
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| * Cells loosely scattered in a myxoid stroma
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| * No evidence of nuclear atypia and mitosis
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| * Numerous, thin-to-thick wall vessels of different sizes
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| * Myxoid, hypocellular background
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| * Bland cytological appearance of spindle cells
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| === Microscopical views ===
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| <gallery>
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| File:Aggressive angiomyxoma.jpg
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| File:Aggressive angiomyxoma 2.jpg
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| File:Aggressive angiomyxoma 6.jpg
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| File:Aggressive angiomyxoma humpath 8.jpg
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| File:Aggressive angiomyxoma - high mag.jpg
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| </gallery>
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| === Immunochemistry ===
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| [[Immunohistochemical studies]] show strong staining for [[desmin]], [[estrogen receptor]]s, and [[progesterone receptor]]s. Staining for [[actin]], [[CD34]] and [[smooth muscle actin]] are intermediate. Staining for [[S-100 protein]] is negative. | |
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| ==Diagnosis== | | ==Diagnosis== |
| === Differential diagnosis ===
| | Diagnosis of aggressive angiomyxoma is typically made through a combination of [[imaging]] studies and [[biopsy]]. [[Magnetic resonance imaging]] (MRI) is the preferred imaging modality, as it provides detailed information about the extent and characteristics of the tumor. A [[biopsy]] is necessary to confirm the diagnosis and differentiate it from other soft tissue tumors. |
| * [[myxoid tumor]]s
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| ** [[Angiomyofibroblastoma]]
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| ==Treatment== | | ==Treatment== |
| {{Empty section|date=October 2017}}
| | The primary treatment for aggressive angiomyxoma is [[surgical excision]]. Due to the tumor's infiltrative nature, achieving clear surgical margins can be challenging, and there is a high risk of local recurrence. In some cases, [[hormonal therapy]] with agents such as [[gonadotropin-releasing hormone]] (GnRH) analogs may be used to reduce tumor size or as an adjunct to surgery. Regular follow-up with imaging is essential to monitor for recurrence. |
| == Prognosis ==
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| Although it is a [[benign]] tumour and does not 'invade' the neighbouring tissue, it has a tendency to recur after surgical excision so it is termed as aggressive. The recurrence can be as close as six months from initial resection. It will not be surprising to find cases coming to tertiary medical centers with history of having a [[Labium (genitalia)|labial]] mass (sometimes misdiagnosed as [[gartner's cyst]]) and have multiple surgical excisions from various doctors. There is no proven medical therapy and people have tried various sorts of chemotherapy like [[Tamoxifen]], [[Leuprolide]] and even full blown chemotherapy.
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| == History ==
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| Aggressive angiomyxoma was originally described in 1983,<ref name="pmid16749950">{{cite journal |vauthors=Adwan H, Patel B, Kamel D, Glazer G |title=A solitary encapsulated pelvic aggressive angiomyxoma |journal=Ann R Coll Surg Engl |volume=86 |issue=6 |pages=W1–3 |date=November 2004 |pmid=16749950 |pmc=1964274 |doi=10.1308/14787080465 }}</ref> but the term "angiomyxoma" dates back to at least 1952.<ref name="pmid13011222">{{cite journal |author =RAEBURN C |title=The histogenesis of four cases of angiomyxoma of the auricle |journal=J. Clin. Pathol. |volume=5 |issue=4 |pages=339–44 |date=November 1952 |pmid=13011222 |pmc=1023673 |doi= 10.1136/jcp.5.4.339|url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=13011222}}</ref>
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| ==See also==
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| * [[Myxoma]]
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| * [[Cutaneous myxoma]] (Superficial angiomyxoma)
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| ==References==
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| {{reflist}}
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| == External links ==
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| | ==Prognosis== |
| | The prognosis for patients with aggressive angiomyxoma is generally good, as the tumor is benign and does not metastasize. However, the high rate of local recurrence necessitates long-term follow-up. Recurrences can occur many years after the initial treatment, so ongoing surveillance is important. |
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| {{Soft tissue tumors and sarcomas}}
| | ==Related pages== |
| | * [[Soft tissue tumor]] |
| | * [[Pelvis]] |
| | * [[Perineum]] |
| | * [[Myxoid stroma]] |
| | * [[Estrogen receptor]] |
| | * [[Progesterone receptor]] |
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| [[Category:Vascular diseases]] | | [[Category:Soft tissue tumors]] |
| [[Category:Benign neoplasms]] | | [[Category:Rare diseases]] |
A rare, slow-growing soft tissue tumor
Aggressive angiomyxoma is a rare type of soft tissue tumor that primarily affects the pelvic and perineal regions. It is characterized by its locally aggressive behavior and tendency to recur after surgical removal. Despite its name, aggressive angiomyxoma is generally considered a benign tumor because it does not metastasize to distant sites.
Presentation
Aggressive angiomyxoma typically presents as a slow-growing mass in the pelvis or perineum. It is more common in women than in men, with a higher incidence in women of reproductive age. Patients may experience symptoms such as a palpable mass, discomfort, or pain in the affected area. In some cases, the tumor may cause urinary or bowel symptoms due to its location and size.
Pathology
The tumor is composed of myxoid stroma with scattered blood vessels and spindle-shaped cells. The histological appearance is characterized by a loose, myxoid matrix with stellate and spindle cells. The vascular component is prominent, with numerous thin-walled vessels. Immunohistochemical staining often shows positivity for estrogen and progesterone receptors, which suggests a hormonal influence on tumor growth.
Diagnosis
Diagnosis of aggressive angiomyxoma is typically made through a combination of imaging studies and biopsy. Magnetic resonance imaging (MRI) is the preferred imaging modality, as it provides detailed information about the extent and characteristics of the tumor. A biopsy is necessary to confirm the diagnosis and differentiate it from other soft tissue tumors.
Treatment
The primary treatment for aggressive angiomyxoma is surgical excision. Due to the tumor's infiltrative nature, achieving clear surgical margins can be challenging, and there is a high risk of local recurrence. In some cases, hormonal therapy with agents such as gonadotropin-releasing hormone (GnRH) analogs may be used to reduce tumor size or as an adjunct to surgery. Regular follow-up with imaging is essential to monitor for recurrence.
Prognosis
The prognosis for patients with aggressive angiomyxoma is generally good, as the tumor is benign and does not metastasize. However, the high rate of local recurrence necessitates long-term follow-up. Recurrences can occur many years after the initial treatment, so ongoing surveillance is important.
Related pages
