Genital Prolapse in Pregnant Woman as a Presentation of Aggressive Angiomyxoma: Case Report and Literature Review

Background: Aggressive angiomyxoma is a rare entity within mesenchymal cell neoplasms, especially in pregnant women. Its main characteristic is the ability to infiltrate neighboring structures and to recur. Case Presentation: We present the case of a pregnant woman who debuted with a genital prolapse in the second trimester of pregnancy. She was diagnosed with bilateral ovarian teratomas and a pelvic mass of which the diagnosis could not be established until delivery. The route of delivery used was cesarean section since the genital prolapse behaved as a previous tumor. After the puerperium, the patient was referred for consultation to complete the study of the mass. The extension study was carried out with a negative result. The patient underwent surgery for tumor exeresis. Hormonal treatment was not administered according to the patient’s preferences. Conclusions: Aggressive angiomyxoma is a benign neoplasm that should be considered in the differential diagnosis of pelvic tumors in women. In pregnant women, the vaginal route of delivery is not contraindicated as long as the tumor does not obstruct the birth canal. The definitive treatment is surgery, preferably performed in a second stage after delivery.

Radiologic imaging findings of pelvic aggressive angiomyxoma correlated with surgical and pathological features

Background Aggressive angiomyxoma is an infrequent benign tumor that usually occurs in the pelvic region. Pelvic masses have variety of differential diagnosis but some featured findings should prompt the diagnosis of aggressive angiomyxoma by the radiologist. Case presentation A 40-year-old female patient presented with a two-year history of perineal swelling. Radiological examination including gray scale and color Doppler ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) was consistent with the diagnosis of aggressive angiomyxoma. The patient underwent surgical operation that ensures total resection of the tumor. Conclusion In the case of extensive pelvic soft tissue mass with characteristic imaging findings, the radiologists should take the diagnosis of aggressive angiomyxoma into consideration.

Radio-pathological and Clinical Correlation of Aggressive Angiomyxoma: Experience of a Tertiary Care Cancer Hospital

Background Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor predominantly affecting the pelvis and perineum of females. Due to its variable clinical presentation, it is often misinterpreted and treated as other pathologies like Bartholin gland cyst, vulvar abscess, and hernia. The tumor is locally infiltrative, making its complete surgical removal difficult. Aim The aim of the study is to highlight and illustrate the imaging and pathological features of AAM. Materials and Methods After obtaining clearance from the Institutional Ethics Committee, a retrospective study was conducted on patients with histopathological diagnosis of AAM, presenting to the dedicated sarcoma clinic between 2016 and 2019. The demographic, clinical, radiological, pathological, and treatment details were obtained from the institute's database. The magnetic resonance imaging (MRI) and computed tomography images were reviewed to assess different imaging parameters. Follow-up imaging was assessed for residual or recurrent mass in the pelvis or perineum and distant metastasis. Results A total of four patients were included with an age range of 28 to 50 years. The baseline MRI of these four patients shows pelvic mass extending into the perineum, infiltrating along the surrounding fascial planes with the characteristic laminated appearance on T2-weighted image and gradual intense enhancement following contrast administration. All patients had residual disease post-surgery and were put on hormonal therapy. Conclusion AAMs are locally aggressive, rarely metastasizing mesenchymal tumor that has a specific predilection for the perineum and pelvis of females. MRI features like laminated or striated appearance, post-contrast enhancement, and finger-like infiltrating projections should raise the suspicion of the diagnosis on baseline imaging.

Aggressive angiomyxoma

Angiomyxoma is a rare slow-growing, benign low-grade tumor occurring in women of reproductive age group which is known for its recurrence. The symptoms are variable. Mrs. K. aged 33 years, reached our outpatient department (OPD) with complaints of painless swelling on the right labial region which she was feeling uncomfortable while sitting. Mass was found to be mobile with no evidence of inflammatory change. The location of the mass made us think about, Bartholin’s cyst and hence surgical excision of the mass was done. The whole mass was found to be lying below the fat in the right labial region and wide excision was completed. The histopathology of the mass was reported as angiomyxoma. Angiomyxoma arises from the mesenchymal tissue and it is locally invasive with high recurrence rate. It is more common in females. Mostly seen in the vulvovaginal, pelvic cavity and perineum. The lesion can reach huge size. It has to be differentiated from gynecologic malignancies, cyst, abscess and hernia. Histology along with immune-histochemistry can confirm the lesion. Wide excision is the mode of treatment. Incomplete excision can lead to relapse. Relapse can happen in 35-72% of the cases. Local recurrence may occur between 2 months to 15 years following initial diagnosis. Hence, follow up is essential. Angiomyxoma is a very rare condition and only around 250 cases have been reported in the world literature. It has to be differentiated from other benign conditions. Diagnosed by non-invasive techniques like ultrasound sonography (USG), magnetic resonance imaging (MRI) and computed tomography (CT). Wide excision is the treatment of choice. Long term follow up is needed as recurrences are high.

Vulvar aggressive angiomyxoma: a surgical challenge

Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed to the infiltrative nature and frequent local recurrences. They arise commonly from the vulvovaginal region, perineum or pelvis and are usually misdiagnosed as other common entities in these regions. Radiological investigations aid in the diagnosis and planning of surgery. However, the final diagnosis in most of the cases is established by histopathological examination. We herein report a case of a middle-aged woman presenting with recurrent large right vulvar mass highlighting the surgical challenges posed by its intrapelvic extension.

The Wide Morphological Spectrum of Deep (Aggressive) Angiomyxoma of the Vulvo-Vaginal Region: A Clinicopathologic Study of 36 Cases, Including Recurrent Tumors

Background: Deep angiomyxoma (DAM) is currently included in the category of “specific stromal tumors of the lower female genital tract”, along with angiomyofibroblastoma, cellular angiofibroma and myofibroblastoma. Given the high rate of local recurrences, it is crucial to recognize DAM from other tumors that possess indolent behaviour. In the present paper, we analyzed the morphological and immunohistochemical features of 42 surgically-resected vulvo-vaginal DAMs (36 primary and 6 recurrent lesions) in order to widen the morphological spectrum of this uncommon tumor. Methods: A series of 36 cases of surgically-resected primary vulvo-vaginal DAMs were retrospectively collected. Locally recurrent tumors were also available for six of these cases. Results: Out of the primary tumors, 25 out of 36 exhibited the classic-type morphology of DAM. In the remaining cases (11/36 cases), the following uncommon features, which sometimes coexist with one another, were observed: (i) alternating myxoid and collagenized/fibrous areas; (ii) hypercellular areas; (iii) neurofibroma-like appearance; (iv) perivascular hyalinization; (v) microcystic/reticular stromal changes; (vi) “microvascular growth pattern”; (vii) perivascular cuffing; (viii) nodular leiomyomatous differentiation; (ix) hypocellular and fibro-sclerotic stroma. Among the six locally recurrent tumors the following features were observed: (i) classic-type morphology; (ii) hypocellular fibro-sclerotic stroma; (iii) extensive perivascular hyalinization, lumen obliteration and formation of confluent nodular sclerotic masses; (iv) hypercellularity. Immunohistochemically, the neoplastic cells of classic-type DAM in both primary and recurrent tumors were diffusely stained with desmin, suggesting a myofibroblastic nature; in contrast, the neoplastic cells showing elongated fibroblastic-like morphology and set in collagenized/fibrosclerotic stroma in both primary and recurrent tumors were negative or only focally stained with desmin, which is consistent with a fibroblastic profile. Conclusion: Although diagnosis of DAM is usually straightforward if typical morphology is encountered, diagnostic problems may arise when a pathologist is dealing with unusual morphological features, especially hypercellularity, extensive collagenous/fibrosclerotic stroma or neurofibroma-like appearance.

Aggressive angiomyxoma of left buttock

Aggressive angiomyxoma (AA) is a rare mesenchymal tumour that is characterised by increased incidence in women compared with men, local invasion to the surrounding tissue and high recurrence rate. A premenopausal woman presented to clinic with pelvic pressure, intermittent tingling in the thigh and pressure emptying the bladder. CT scan, vaginal and gluteal biopsies, and MRI scan were performed to conclude a final diagnosis of AA. The patient underwent complete resection of the mass. The mass tested positive for oestrogen receptor and progesterone receptor. The patient received leuprolide postoperatively to prevent recurrence. AA should be considered as a differential diagnosis for a pelvic and perineal mass. Patients should be warned of high recurrence rate, necessity of surgical removal and long-term hormonal treatment.