Aggressive angiomyxoma: a second case of metastasis with patient’s death

Blandamura, Stella; Cruz, Julia; Vergara, Lourdes Faure; Puerto, Isidro Machado; Ninfo, Vito

doi:10.1053/s0046-8177(03)00419-2

Cited by 201 publications

(140 citation statements)

References 5 publications

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“…The patient succumbed to lung metastases. A case of Aggressive angiomyxoma with lung and mediastinal metastases has also been described in a postmenopausal woman (Blandamura et al, 2003). Our Patient has not developed any local recurrence or distant metastasis so far.…”

Section: Discussionsupporting

confidence: 53%

Rare Case of Aggressive Angiomyxoma of the Vulva: A Case Report

shanmugasundaram

Maharajan

2016

CCO

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Aggressive Angiomyxoma (AA) is a rare locally aggressive soft tissue tumor with high chance of local recurrence occuring in young women of reproductive age group. These neoplasms are characterized by a mixture of spindle or stellate cells in a loosely myxoid stroma with prominent vascular component containing large, thickwalled blood vessels. A 35 year old multiparous lady presented with right vulval swelling which was rapidly progressing in size. Local examination revealed a soft fleshy mass occupying whole of the right labia majora. In view of the rapidly enlarging lesion over a short duration, we suspected a soft tissue neoplasm and preceded with wide local excision of the mass with adequate margins in all dimensions, eventhough we did not have a preoperative tissue diagnosis. To our surprise, final histopathology was Aggressive Angiomyxoma (AA). The treatment of choice for Aggressive Angiomyxoma is wide local excision with negative margins and the same was done in our case. Recurrence of the tumor may be avoided by wide local excision with adequate margins. It is possibly a hormonally responsive neoplasm. Treatment with Gonadotropin-releasing hormone is an emerging therapy. Overall prognosis of AA is good.

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Section: Discussionsupporting

confidence: 53%

Rare Case of Aggressive Angiomyxoma of the Vulva: A Case Report

shanmugasundaram

Maharajan

2016

CCO

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“…Tumor recurrence is a significant cause of patient morbidity, and recurrence rates from the largest case series 2,4,7 range from 25% to 47%. Although 2 cases of metastasis have been reported, 8,9 AAM is considered an indolent neoplasm with locally aggressive behavior.…”

mentioning

confidence: 99%

Aggressive Angiomyxoma

Sutton¹,

Laudadio²

2012

Archives of Pathology &Amp; Laboratory Medicine

135

155

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Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum, and pelvis of women. The term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Patients often present with nonspecific symptoms which are frequently misdiagnosed with more common entities, such as a Bartholin cyst, lipoma, or hernia. Histologic examination reveals a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindled cells. The tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor. Chromosomal translocation of the 12q13-15 band involving the HMGA2 gene has been described. Surgical excision is the treatment of choice, although treatment with gonadotropin-releasing hormone agonists is an emerging therapy. Metastases are exceedingly rare, and overall, the prognosis is good.

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“…The incidence rate between male and female is about 1:6 [2,3]. Though in most cases it looks like a benign tumor, there are still cases of metastasis and led to the death of the patients reported in articles [4,5]. Because of the low speed of growth and untypical symptoms, this tumor is easily misdiagnosed as Bartholin cyst, hernia or vaginal cyst [3].…”

Section: Discussionmentioning

confidence: 99%

Aggressive Angiomyxoma: Two Case Reports and Review of the Literature

Cao¹

2017

Int J Radiol Imaging Technol

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Aggressive Angiomyxoma (AA) is a kind of rare, low grade myofibroblastic neoplasm with high risk of local recurrence mainly for the character of locally aggressive and misdiagnosis as other soft tissue mass in the pelvis. The "laminated or swirled sign" of swirled strands of low-signal intensities in T2WI is the diagnostic sign for the layered configuration of AA. We report two cases with contrast enhanced CT and Dynamic Contrast Enhancement (DCE) MRI to show the characteristic imaging features and our findings about AA in enhanced CT and DCE MRI. KeywordsAggressive Angiomyxoma (AA), Laminated or Swirled Sign, Magnetic Resonance Imaging (MRI), X-ray Computed Tomography (X-ray CT), Multiplanner Reformation (MPR), Diffusion Weighted Imaging (DWI), Apparent Diffusion Coefficient (ADC) Case RepoRt Case OneA 29-year-old woman was incidentally found a mass in her vagina. She was asymptomatic and was diagnosed as fibroma by biopsy. The CTs can show a well-limited mass with low density compared to muscle located in the left pelvic cavity (Figure 1a). After iodine contrast injection, the mass showed a progressive and heterogeneous enhancement without any layered character (Figure 1b). MRI showed a big mass extending into the obturator foramen and ischiorectal fossa, displacing the gluteus, uterus and vagina without invading (Figure 2a). The mass showed ho-

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Aggressive angiomyxoma: a second case of metastasis with patient’s death

Cited by 201 publications

References 5 publications

Rare Case of Aggressive Angiomyxoma of the Vulva: A Case Report

Rare Case of Aggressive Angiomyxoma of the Vulva: A Case Report

Aggressive Angiomyxoma

Aggressive Angiomyxoma: Two Case Reports and Review of the Literature

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