A case series of aggressive angiomyxoma: Using morphologic type and hormonal modification to tailor treatment

Kooy, Joni; Carlson, Vanessa; Šačiragić, Lana; Sawhney, Summit; Nelson, Gregg

doi:10.1016/j.gore.2021.100765

Cited by 6 publications

(6 citation statements)

References 15 publications

(8 reference statements)

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“…Among site-specific low-grade mesenchymal tumors of the vulva and vagina, myxoid features are especially common in aggressive angiomyxoma. As these tumors carry a 10% to 50% risk for local recurrence despite the lack of atypia, necrosis, or mitoses, they should be distinguished from IMT 36,37. As discussed, most aggressive angiomyxoma demonstrate HMGA2 immunoexpression, which was not present in the vulvar IMT we report.…”

Section: Discussionmentioning

confidence: 52%

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Bowman

Medeiros

Fadare

et al. 2022

International Journal of Gynecological Pathology

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Gynecologic tract origin of inflammatory myofibroblastic tumor (IMT), a receptor tyrosine kinase fusion driven tumor with malignant potential, is uncommon and mostly involves the uterine corpus where misclassification as a smooth muscle tumor may occur due to overlapping morphologic features. With rare exception, uterine IMT involves ALK rearrangements and exhibits ALK immunoexpression. Molecularly confirmed vulvovaginal IMT has not been reported, but several low-grade mesenchymal tumors in this region exhibit myxoid stroma and/or inflammatory infiltrates that may resemble IMT. The aims of this study were to define the diagnostic specificity of ALK immunoexpression for IMT among a broad spectrum (107 cases) of vulvovaginal mesenchymal tumors in the differential diagnosis of IMT and to report the clinicopathologic features of vulvovaginal IMT identified in our archives or via retrospective ALK staining of otherwise classified vulvovaginal tumors. Review of archives from 5 different centers revealed a single case of vulvar IMT in a 62-yr-old woman. The 2.5 cm well-circumscribed tumor exhibited the typical microscopic features of IMT, namely a loose fascicular distribution of bland spindle cells within a myxoid stroma, accompanied by an infiltrate of plasma cells, lymphocytes, and eosinophils. The tumor cells exhibited expression for smooth muscle actin, desmin, h-caldesmon, and ALK. Break-apart fluorescence in situ hybridization confirmed the presence of ALK rearrangement. The patient did not receive any treatment and is alive without disease 32 mo later. No evidence of ALK expression was detected in any of the other 107 vulvovaginal tumors, which included 14 aggressive angiomyxomas, 2 superficial angiomyxomas, 12 angiomyofibroblastomas, 8 cellular angiofibromas, 15 smooth muscle neoplasms, 10 peripheral nerve sheath tumors, 20 fibroepithelial polyps, and a variety of other low grade mesenchymal tumors. Although vulvovaginal ALK-rearranged IMT is exceedingly rare, the behavior remains to be fully understood. ALK immunohistochemistry, which appears specific for IMT in this anatomic site, is advised in the evaluation of vulvovaginal mesenchymal tumors exhibiting myxoid stroma and/or an inflammatory infiltrate.

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Section: Discussionmentioning

confidence: 52%

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Bowman

Medeiros

Fadare

et al. 2022

International Journal of Gynecological Pathology

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“… 4 , 11 , 12 Neoplastic cells in AA commonly express estrogen and progesterone receptor positivity, 9 in correlation with its prevalence in women of reproductive age. 13 …”

Section: Discussionmentioning

confidence: 99%

“…Recent research suggests that the gonadotropin-releasing hormone analog (GnRHa) may be a viable noninvasive alternative for primary cases of aggressive angiomyxoma with positive margins or recurrent AA. 7 , 13 , 14 In certain cases, treatment with adjuvant GnRHa has shown efficacy in reducing the risk of recurrence and treating residual disease. 13–16 In our case, the patient, who had negative margins after resection, adhered to the imaging follow-up plan without hormonal treatment.…”

Section: Discussionmentioning

confidence: 99%

Atypical Vaginal Aggressive Angiomyxoma in a Virgin: A Case Report from South Jordan

Al-Kharabsheh,

AlTarawneh,

Abufraijeh

et al. 2024

IJWH

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Aggressive angiomyxoma is a locally invasive, rarely diagnosed mesenchymal tumor that predominantly affects the perineal region of women in their reproductive years. It is typically found in areas such as the vagina, vulva, and other pelvic soft tissues, but its presentation can be highly variable. We describe a particularly unique case of aggressive angiomyxoma, presenting in an atypical manner within the vaginal region of a 31-year-old virgin woman. This case is noteworthy for the unusual growth pattern of the angiomyxoma—located strictly within the vaginal epithelium, showing no signs of invasion into surrounding tissues, which is not typical of the aggressive nature of this type of tumor. The patient presented with a mass that protruded through the hymen, an uncommon presentation that led to initial speculation about the nature of the mass. The definitive diagnosis of aggressive angiomyxoma was made by meticulous histopathological examination after surgical excision of the mass. This case emphasizes the critical importance of considering aggressive angiomyxoma in the differential diagnosis of vaginal masses, particularly those that do not exhibit invasive characteristics. The case also highlights the need for increased awareness among clinicians about the potential for atypical presentations of this rare tumor, to facilitate timely and accurate diagnosis, and to guide appropriate management strategies. This report contributes to the growing body of literature on aggressive angiomyxoma, highlighting the variability in its presentation and the need to heighten suspicion in atypical cases.

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“…Furthermore, hormone treatment, radiotherapy, and vascular embolization therapy have some curative effects on AAM. However, there is some uncertainty in the literature ( 12 – 14 ). As a result, we started an experimental therapy for AMM some years ago.…”

Section: Discussionmentioning

confidence: 99%

Aggressive angiomyxoma of the pelvis and abdominal wall: Dramatic response to chemical ablation therapy

Liu

et al. 2023

Front. Oncol.

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ObjectiveAggressive angiomyxoma (AAM) is a rare, locally aggressive soft tissue neoplasm with a marked tendency for local recurrence after surgery. Although hormone therapy, radiation therapy, and vascular embolization can be performed, we investigated the safety and efficacy of a new chemical ablation protocol for AAM.MethodsThis study included two female AAM patients from 2012 to 2016. The patients’ clinical and imaging data were collected. The amount of anhydrous ethanol and glacial acetic acid used for chemical ablation was documented, and the management of any complications was detailed.ResultsThe maximum dimensions of the residual tumor were 12.6 cm and 14.0 cm. In one case, the lesion was in the pelvis and protruded into the vulva. Eighty milliliters of liquid with a mixture of glacial acetic acid, anhydrous ethanol, and iohexol (10:9:1) was used for chemical ablation therapy via multipoint injections with a single needle. However, a pelvic fistula developed 1 month later. In another case, the lesion was located in the abdominal wall. The ablation procedure was improved by performing chemical ablation therapy with multiple needles for multi-point injections of smaller than 30 ml injections for each procedure. To date, no recurrence or metastasis has been observed in the two cases.ConclusionThe preferred treatment for AAM is complete resection. Chemical ablation therapy is a novel adjuvant therapy for AMM. Nonetheless, more research is needed to confirm these findings.

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A case series of aggressive angiomyxoma: Using morphologic type and hormonal modification to tailor treatment

Abstract: Highlights: Aggressive angiomyxoma tends toward two forms: deeply invasive and pedunculated. Anti-hormonal therapy can be an adjunct to aggressive angiomyxoma treatment. Pedunculated aggressive angiomyxoma can be considered for shorter follow-up.

Cited by 6 publications

References 15 publications

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Atypical Vaginal Aggressive Angiomyxoma in a Virgin: A Case Report from South Jordan

Aggressive angiomyxoma of the pelvis and abdominal wall: Dramatic response to chemical ablation therapy

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