Angiomyofibroblastoma Research Articles

Angiomyofibroblastoma – A Rare Vulval Tumor

Angiomyofibroblastoma (AMFB) is a rare, benign, mesenchymal tumor occurring mostly in the genital tract in young to middle-aged women. Due to its presentation, it can be mistaken for Bartholin’s abscess, leiomyoma, or Gartner’s cyst. It has a low recurrence rate, but it has to be differentiated histopathologically from aggressive angiomyxoma. We present a case of AMFB of the vulva in a 40-year-old female who presented with swelling over the vulva.

Giant pedunculated angiomyofibroblastoma in young

Angiomyofibroblastoma (AMF) is an uncommon benign mesenchymal tumor that primarily affects women's vulvovaginal tissue but can sporadically affect male inguinoscrotal tissue. It is a well-circumscribed lesion that typically does not become a pedunculated hanging mass and is frequently believed clinically to be a Bartholin's gland cyst. A very few cases of vulvar AMF with pedunculated mass have been reported in the literature, and all cases involve the labia majora and middle-aged women. Here, we describe a case of a 22-year-old woman who experienced pedunculated mass in vulva involving the left labia majora. The differential diagnosis for the pedunculated vulvar mass in this young woman with a labial lesion was pedunculated AMF after excluding the most frequent illnesses.

Vulvovaginal Angiomyofibroblastoma: An Uncommon Benign Mesenchymal Tumour

The female genital tract angiomyofibroblastoma (AMFB) is a rare benign mesenchymal tumour that mostly affects premenopausal women. Although it is most commonly seen in the vulvovaginal region, it has also been documented in the inguinosacral region, pelvis, and retroperitoneum (1,2). Patients usually present with indolent, painless swelling in the vulvovaginal region, or a pedunculated lesion on rare occasions. Those that appeared in the vulva were frequently misdiagnosed as Bartholin cysts. Leiomyoma is a differential in the vaginal area. AMFB must be recognised from aggressive angiomyxoma (AA) due to the latter’s potential for recurrence with typical infiltrative boundaries. We discuss a case of a 46-year-old woman who had a slow-growing paravaginal swelling incidentally discovered during a total hysterectomy for leiomyoma five years prior.

Vulvar angiomyofibroblastoma is molecularly defined by recurrent MTG1-CYP2E1 fusions.

Angiomyofibroblastoma (AMF), a rare benign vulvovaginal mesenchymal tumour, poses a diagnostic challenge due to histologic and immunohistochemical overlap with other vulvar mesenchymal tumours. Recently, MTG1-CYP2E1 fusion transcripts were reported in 5/5 AMFs; no other genetic alterations have been described to date. Herein, we sought to investigate the frequency of the MTG1-CYP2E1 fusion and the presence of other potential genetic alterations in a cohort of AMFs (n = 7, patient age range: 28-49 years). Tumours demonstrated classic morphologic features including alternating hypo/hypercellular areas, capillary channels surrounded by epithelioid/spindled tumour cells, and variable amounts of mature adipose tissue. reverse transcription-polymerase chain reaction (RT-PCR) for MTG1-CYP2E1 fusion, performed in all seven cases, showed the fusion transcript in five of six cases (one case with technical failure). Two tumours, including the one lacking the fusion, were subjected to targeted next-generation sequencing (104 genes) and a sarcoma fusion assay (28 genes); the fusion negative AMF also underwent RNA sequencing. No additional mutations, copy number alterations, or fusion genes were identified with the assays employed. We conclude that the majority of AMFs harbour recurrent MTG1-CYP2E1 fusion transcripts and identification of this fusion may aid in the diagnosis.

Aggressive Angiomyxoma, Angiomyofibroblastoma, and Cellular Angiofibroma of the Lower Female Genital Tract: Related Entities With Different Outcomes.

IntroductionMesenchymal tumors of the lower female genital tract (FGT) are a miscellaneous group of tumors that include aggressive angiomyxoma (AAM), angiomyofibroblastoma (AMFB), cellular angiofibroma (CAF), and related entities. Histologically, these tumors are composed of stromal cells admixed with vessels, with some minor histological differences. An accurate diagnosis of these tumors is important owing to the differences in the outcome. In this study, we determined the clinicopathological characteristics of these tumors in our population and their association with recurrence. MethodsThis was a retrospective cross-sectional study conducted at the Department of Histopathology, Aga Khan University, from January 2005 to December 2019 over a period of 15 years. A total of 207 cases that were diagnosed as AAM, AMF, and CAF were selected for inclusion in the study. Clinical data, including age and location of the lesion, were obtained from histopathology referral forms. Tissue blocks of all cases were retrieved. Follow-up data were obtained from the patient files, and information regarding disease recurrence was recorded. One histological section from each tissue block was stained with hematoxylin and eosin stain, and histopathological findings were recorded. Additionally, immunohistochemical (IHC) studies, including vimentin, smooth muscle actin (SMA), and desmin were conducted on representative tissue blocks. Final histopathological diagnoses were rendered considering clinical, histopathological, and IHC findings.ResultsThe median age of patients involved in the study was 33 years and the median tumor size was 5 cm with a predilection for the vulva (47.3%). AAM showed a predilection for patients between the ages of 31-45 years, while AMFBs and CAFs were most common in younger age groups of less than 30 years. In 46.8% of cases, the tumor size of AAM was between 6 and 10 cm, while in all cases of CAF (100%) and the majority of AMFB cases (53.2%) the tumor size was smaller than 5 cm. Histologically, in all cases of AAM, the lesional cells were spindle (100%), whereas, in 13% of cases epitheloid cells were observed, with myxoid stroma in 92.2% cases. The presence of stromal smooth muscle was noted in 42.9% of cases, in 79.2% of cases the vessels were thick-walled, with 54.5% having hyalinized vessels, and most of the cases (77.9%) had ill-defined borders. Among IHC findings, AMFB was most frequently positive for actin (62.2%), while AAM and AMFB showed more frequent staining for desmin compared to CAF. A significantly higher recurrence rate was observed in AAM (27.3%), compared to AMFB and CAF. A significant association of recurrence was seen with tumor size. It was noted that the recurrence rate was directly proportional to the size of the tumor and was highest (60%) with a tumor size of more than 10 cm.ConclusionIn our study, we noted that AMFB was the most common among the three mesenchymal tumors of the lower FGT. In contrast, AAM had the highest recurrence rate, and recurrence was significantly associated with tumor size. Histological findings, especially the type of stromal cells and background vasculature, are of utmost importance for the correct recognition of these tumors, while the role of IHC studies is limited.

Practical Approach to the Diagnosis of the Vulvo-Vaginal Stromal Tumors: An Overview.

Background: The category of the “stromal tumors of the lower female genital tract” encompasses a wide spectrum of lesions with variable heterogeneity, which can be nosologically classified on the basis of their morphologic and immunohistochemical profiles as deep (aggressive) angiomyxoma (DAM), cellular angiofibroma (CAF), angiomyofibroblastoma (AMFB) or myofibroblastoma (MFB). Despite the differential diagnosis between these entities being usually straightforward, their increasingly recognized unusual morphological variants, along with the overlapping morphological and immunohistochemical features among these tumours, may raise serious differential diagnostic problems. Methods and Results: The data presented in the present paper have been retrieved from the entire published literature on the PubMed website about DAM, CAF, AFMB and MFB from 1984 to 2021. The selected articles are mainly represented by small-series, and, more rarely, single-case reports with unusual clinicopathologic features. The present review focuses on the diagnostic clues of the stromal tumours of the lower female genital tract to achieve a correct classification. The main clinicopathologic features of each single entity, emphasizing their differential diagnostic clues, are discussed and summarized in tables. Representative illustrations, including the unusual morphological variants, of each single tumour are also provided. Conclusion: Awareness by pathologists of the wide morphological and immunohistochemical spectrum exhibited by these tumours is crucial to achieve correct diagnoses and to avoid confusion with reactive conditions or other benign or malignant entities.

A Huge Vulvar Mass: An Angiomyofibroblastoma

Background: An angiomyofibroblastoma (AMF) is a mesenchymal tumor that affects mostly reproductive-age woman; very rare cases have been reported in men. An AMF is generally an asymptomatic mass tha...

Vaginal Angiomyofibroblastoma: A Case Report and Review of Diagnostic Imaging

Background Angiomyofibroblastoma (AMFB) is a benign mesenchymal tumor most commonly found in the female genital tract of premenopausal women. Although rare, AMFB is an important consideration in the differential diagnosis of vulvar and vaginal masses, as it must be distinguished from aggressive angiomyxoma (AA), a locally recurrent, invasive, and damaging tumor with similar clinical and pathologic findings. CaseWe describe a patient with a 4 cm vaginal AMFB and the relevant preoperative radiographic imaging findings. ConclusionPreoperative diagnosis of AMFB remains difficult. Common findings on magnetic resonance imaging and transvaginal sonography are described. We conclude that both transvaginal ultrasound and MRI are potentially useful imaging modalities in the preoperative assessment of vulvar and vaginal AMFB, with more data needed to determine superiority of one modality over the other.

Large-sized pedunculated and polypoidal angiomyofibroblastoma of the vulva: A case report and literature review

Angiomyofibroblastoma (AMF) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region, which may be misdiagnosed as aggressive angiomyxoma (AAM). Herein, we report a case of a 20-year-old nulliparous Chinese woman with a unique pedunculated and polypoidal mass, which had been developing within the previous 6 months in the left labium majus, exhibiting the AAM clinical impression but diagnosed as AMF. The mass measured 18 × 10 × 6 cm, and contained diffuse ulcerated areas and purulent discharge. A complete excision of the mass was performed. There was no subsequent evidence of recurrence, according to a 13-month follow-up. As a rare benign vulvovaginal tumor, AMF can present on patients of an early reproductive age with rapidly growing, polypoidal pattern. The whole exon sequencing analysis revealed the genomic alterations, which may contribute to the occurrence of AMF.

Angiomyofibroblastoma-like tumors

Angiomyofibroblastomas are rare, benign, slow-growing tumors, that occurring predominantly in vulvar lesions of premenopausal women. In male patients only few cases are reported (angiomyofibroblastoma-like tumors). They occur mostly in scrotal and inguinal regions. We report a case of left scrotal angiomyofibroblastoma-like tumor in a 52-years old man. After a biopsy for histological examination, a complete surgical excision was performed. At 12 months' follow-up the patient is asymptomatic, and no tumor signs were was found on by abdominal computed tomography.

Angiomyofibroblastoma of the spermatic funiculus. A case report of this lesion of the spermatic funiculus

The authors are hereby presenting a rare case of angiomyofibroblastoma of the funiculus in a 20-year-old patient, having a non-aching tumefaction at the left region of the scrotum. This tumefaction was solid and non homogeneous, both on ultrasonography and MRI, of about 5cm in diameter, fully separated from the testicle. The markers were all negative. We proceeded with surgical treatment of the neoformation. It is mandatory to include this lesion within the differential diagnosis of testicle masses. The case is presented for its absolute histopathologic rarity and for the difficulty in diagnosis.

A Rare Case of Intravesical Angiomyofibroblastoma

To study the first reported case of intravesical angiomyofibroblastoma (AMFB). AMFB is an uncommon benign mesenchymal lesion, mostly described in the lower female genital tract, especially in the vulva and less commonly in the vagina. It is rarely seen in the male genital tract. The importance of this rare neoplasm is related to its potential mimicry of more infiltrative and prognostically less favorable lesions, such as aggressive angiomyxomas, that are found in the same anatomic area. A 26-year-old man presented to us with hematuria. Investigations revealed an intraluminal polypoidal lesion of approximately 92 × 77 × 76 mm within the urinary bladder. Cystoscopy revealed a large well-circumscribed tumor with a pedicle, attached to the inferolateral wall of the bladder near the bladder neck. Open exploration and en bloc excision of the bladder mass was done. Histopathology and immunohistochemistry (IHC) confirmed the diagnosis of AMFB. AMFB presents as a well-circumscribed superficial mass. Complete excision of the tumor for histopathologic and IHC examination is essential for diagnosis. This rare tumor shows morphologic similarity to some more aggressive tumors with less favorable outcome; hence, it needs to be differentiated from those. IHC is helpful but not always conclusive in the diagnosis of this rare neoplasm. This being the first-ever reported case of intravesical AMFB bears a significant importance. Careful clinical assessment, operative management, and histopathologic examination are of utmost importance in making a precise diagnosis and management of the patient.

The Morphological and Immunohistochemical Features of a Pedunculated Vulvar Angiomyofibroblastoma: A Case with Review of Literature

Angiomyofibroblastoma (AMF) is a rare and benign soft tissue tumor that presents in the vulvovaginal area in women. Numerous entities such as Bartholin's cysts, aggressive angiomyxoma, cellular angiofibroma, fibroepithelial stromal polyps, and superficial myofibroblastoma can also develop in the female genital region and mimic AMF; distinguishing between these lesions can be difficult due to their morphological similarities. Among the tumors mentioned, histological similarities add strain to AMF diagnosis. Furthermore, the immunohistochemical variability that exists within each discrete tumor category makes differentiation arduous. While challenging, it is necessary to diagnose these lesions accurately and hastily as the prognosis for

Local recurrent vaginal aggressive angiomyxoma misdiagnosed as cellular angiomyofibroblastoma: A case report.

Aggressive angiomyxoma (AAM) and angiomyofibroblastoma (AMFB) are two rare types of mesenchymal tumors with overlapping clinicopathological features. In certain cases, the differential diagnosis between the two tumors is difficult even for experienced pathologists. The present study reported the case of a well-circumscribed soft tissue mass on the anterior wall of the vagina in a 25-year-old woman. The mass was initially removed without disturbance to the adjacent tissues. The histopathological features included spindle cells in inconspicuous myxoid stroma and a well-demarcated mass without evidence of invasion, which prompted the initial diagnosis of AMFB. After 2 years, a mass returned in the same area and a wide tumor excision was performed. The histopathological examination confirmed the final diagnosis of AAM upon review.

Mesenchymal Extratesticular Tumors and Tumorlike Conditions: From the Radiologic Pathology Archives.

Both benign and malignant tumors and tumorlike conditions can arise from the nonepididymal extratesticular tissues. Benign tumors are far more common than malignant tumors, with lipoma being the most common lesion found at this site. Several imaging features can help narrow the differential diagnosis of these lesions, including the presence of fat and imaging features suggestive of fibrous tissue. Lesions that contain fat represent lipoma, liposarcoma, or angiomyofibroblastoma (AMF)-like tumor. If enhancing soft tissue is present in conjunction with fat, the differential diagnosis narrows further to just liposarcoma and AMF-like tumor. Lesions that display magnetic resonance imaging characteristics compatible with fibrous tissue are likely to be fibrous pseudotumor. However, many of the lesions in this location have overlapping imaging findings, and surgical excision is most often necessary for accurate diagnosis. The ability to narrow the differential diagnosis with imaging, however, is helpful for the clinician for both treatment planning and patient counseling.

Kız çocuğunda vulvar anjiomyofibroblastoma: olgu sunumu

Introduction: Angiomyofibroblastoma (AMFB) is a rare seen mesenchymal tumor that is categorized as a genital stromal tumor. It is commonly seen in the middle-aged women usually affecting the vulva and rarely the vagina. A variant called AMFB-like tumors are also rarely seen in male patients. AMFB with its clinical presentation and location can be wrongly diagnosed as an aggressive angiomyxomas, bartholin cyst or lower genital tract lipomas. The treatment of this rare tumor is generally simple surgical excision. Case - Discussion: In the following case, angiomyofibroblastoma case that was seen in childhood for the first time according to the literature was explained.

Vulvar Anjiyomiyofibroblastom: Bir Olgu Sunumu

Angiomyofibroblastoma (AMFB) is a rare seen mesenchymal tumor that is categorized as a genital stromal tumor. It is commonly seen in the middle-aged women usually affecting the labia majora and rarely the vagina. A variant called AMFB-like tumors are also rarely seen in male patients. AMFB with its clinical presentation and location can be wrongly diagnosed as an aggressive angiomyxomas, bartholin cyst or lower genital tract lipomas. In the patients who are applying because of the vulvar mass, AMFB should not be forgotten for the pre-diagnosis. The treatment is generally simple surgical excision.In this case, AMFB was reported with a wrongly pre-diagnosis as a bartholin cyst to which a medical treatment had been given.

English

Angiomyofibroblastoma (AMF) is a rare soft tissue tumor with unique morphological features occurring in the vulvo vaginal region of women. Few reported cases of occurrence in men. The tumor is localized in the scrotum &in the inguinal area of male patients. It is often mistaken for Bartholin's gland cyst in women. We report a case of Angiomyofibroblastoma of vulva in a 46 yrs old woman. INTRODUCTION: Vulval tumors are rare, 1,2 usually distributed among Dermatology, Genito urinary medicine, Gynecology and Pathology. Due to complexity of vulvar diseases there is a necessity of multi-disciplinary approach.3 Angiomyofibroblastoma is a rare benign mesenchymal tumor occurring in middle aged women.4 Clinically it is misdiagnosed as a Bartholin's Gland Cyst. It is also reported in men in scrotal & inguinalregion. 5 We report a case of angiomyofibroblastoma in a 46 yr. old women in

Vulvovaginal angiomyofibroblastomas: morphologic, immunohistochemical, and fluorescence in situ hybridization analysis for deletion of 13q14 region.

Angiomyofibroblastoma (AMFB) is a benign tumor that belongs to the category of the "stromal tumors of the lower female genital tract," together with cellular angiofibroma and myofibroblastoma. Previous studies have shown overlapping morphologic and immunohistochemical features between these tumors and spindle cell lipoma, mammary-type myofibroblastoma, and vulvovaginal cellular angiofibroma and myofibroblastoma. In addition, typical loss of genetic material from the 13q14 region has been documented in all the above-mentioned tumors, suggesting that they are histogenetically related. We report the clinicopathologic features of 11 new cases of vulvovaginal AMFBs. Histologically, the basic common theme was a proliferation of bland-looking spindle to round-to-epithelioid cells set in an edematous to fibrous stroma, frequently arranged around thin-walled blood vessels. Two cases were composed of a prominent mature fatty component closely admixed with typical areas of AMFB, and thus, they were designated as "lipomatous AMFBs." Notably, 1 case was closely reminiscent of Sertoli cell tumor, sclerosing type, because of its predominant cord-like arrangement. Immunohistochemically, all tumors were diffusely positive for vimentin, whereas desmin and α-smooth muscle actin were expressed in a minority of cases, suggesting a fibroblastic rather than myofibroblastic differentiation. Most cases of AMFBs coexpressed Bcl-2 protein and CD99. Interestingly, all 5 cases of AMFB with evaluable signals failed to show monoallelic loss of FOXO1 loci (13q14) by fluorescence in situ hybridization. These cytogenetic findings suggest that vulvovaginal AMFB is not genetically related to cellular angiofibroma and myofibroblastoma of the lower female genital tract.

Angiomyofibroblastoma-like tumor of the scrotum: A case report and literature review

The purpose of the present study was to increase the knowledge of angiomyofibroblastoma (AMF)-like tumors in males by describing the second case of this rare lesion in the Chinese population with a long period of follow-up and by reviewing the literature. AMF-like tumor is a rare, circumscribed, slow-growing mesenchymal tumor that occurs predominantly in the vulva, perineum and pelvis of females. The present report presents a case of left scrotal AMF-like tumor in a 37-year-old male. Complete surgical excision was performed. The tumor was composed of spindle-shaped cells and small vessels proliferating in the edematous stroma. Immunohistologically, the tumor cells stained positive for smooth muscle actin and negative for S-100, CD34 and actin. Following seven years of follow-up the patient was asymptomatic and no evidence of tumor was found. In addition, the current literature was reviewed and the characteristics of this tumor were summarized. AMF-like tumors must also be distinguished from spindle cell lipoma, solitary fibrous tumor and aggressive angiomyxoma.