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Angiomyofibroblastoma (AMFB) is an exceedingly rare mesenchymal tumor of the lower genital tract. AMFB primarily affects the pelviperineal region, especially the vulvar in premenopausal women. Typically, AMFB is a benign disease and does not have the potential for metastasis or recurrence, requiring complete surgical excision. Its accurate differentiation from aggressive angiomyxoma is critical due to varying prognoses. A 51-year-old woman, diagnosed with mucinous carcinoma of the breast, presented with a 12 cm abdominopelvic mass identified during breast cancer staging. Imaging suggested an ovarian origin; however, surgical exploration revealed a stalk-attached cystic mass in the anterior body of the uterus. Histopathology confirmed AMFB. Immunohistochemical analysis showed positivity for estrogen and progesterone receptors and smooth muscle actin. The patient continued breast cancer treatment postoperatively without pelvic mass recurrence or complications for a postoperative follow-up period of one year. This case highlights AMFB's potential uterine body origin, expending known tumor sites and complicating diagnosis due to overlapping features with other mesenchymal tumors. Accurate diagnosis using immunohistochemical markers and pathological features is essential to avoid unnecessary aggressive treatments. The uterine location in this case suggests a possible shared pathogenesis with uterine myomas, warranting further research into their connection. Reporting the first case of AMFB originating in the uterine body enhances understanding of this rare condition and underscores the importance of clinical awareness and precise diagnostic strategies to guide management and improve outcomes. [ABSTRACT FROM AUTHOR]

Vulvo-vaginal stromal tumours are a rare and diverse group of mesenchymal neoplasms unique to hormone-responsive stroma of the vulva and vagina. These tumours are mostly benign, except for the locally aggressive deep angiomyxomas. Often these tumours pose diagnostic challenges, resembling certain malignant vulvo vaginal tumours.This case series highlights clinicopathological features of four angiomyxomas; a single angiomyofibroblastoma, and another superficial myofibroblastoma, including their clinical outcomes. All patients were in their 4th or 5th decade of life. Only 1/4 angiomyxomas was correctly diagnosed at the referring hospitals. Three out of four patients harbouring angiomyxomas achieved clinical remission post-surgery, while one patient was lost to follow-up. By immunohistochemistry, tumor cells showed variable positivity for desmin, SMA, ER, and PR, and negativity for S100P and CD34. The angiomyofibroblastoma was initially misdiagnosed as a liposarcoma, and the patient was lost to follow-up after diagnosis. Immunohistochemically, the tumor cells were diffusely positive for SMA and ER; weakly and focally positive for desmin, and negative for AE1/AE3, CD34, and S100P. The patient with superficial myofibroblastoma is in clinical remission post-excision with an 18-month follow-up. Immunohistochemically, the tumor cells showed CD34 positivity. Therapeutically, none of the patient received adjuvant treatment, except for a single patient with angiomyxoma, who underwent chemoradiation for a synchronous cancer cervix post-surgery.This case series provides valuable insights into the clinical heterogeneity, diagnostic intricacies, and outcomes of vulvo-vaginal stromal tumours from an oncology centre in India, further contributing to a better understanding of these rare tumours.

Angiomyofibroblastoma (AMFB) is an exceedingly rare mesenchymal tumor of the lower genital tract. AMFB primarily affects the pelviperineal region, especially the vulvar in premenopausal women. Typically, AMFB is a benign disease and does not have the potential for metastasis or recurrence, requiring complete surgical excision. Its accurate differentiation from aggressive angiomyxoma is critical due to varying prognoses. A 51-year-old woman, diagnosed with mucinous carcinoma of the breast, presented with a 12 cm abdominopelvic mass identified during breast cancer staging. Imaging suggested an ovarian origin; however, surgical exploration revealed a stalk-attached cystic mass in the anterior body of the uterus. Histopathology confirmed AMFB. Immunohistochemical analysis showed positivity for estrogen and progesterone receptors and smooth muscle actin. The patient continued breast cancer treatment postoperatively without pelvic mass recurrence or complications for a postoperative follow-up period of one year. This case highlights AMFB’s potential uterine body origin, expending known tumor sites and complicating diagnosis due to overlapping features with other mesenchymal tumors. Accurate diagnosis using immunohistochemical markers and pathological features is essential to avoid unnecessary aggressive treatments. The uterine location in this case suggests a possible shared pathogenesis with uterine myomas, warranting further research into their connection. Reporting the first case of AMFB originating in the uterine body enhances understanding of this rare condition and underscores the importance of clinical awareness and precise diagnostic strategies to guide management and improve outcomes.

Angiomyofibroblastoma (AMFB) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region. It is usually diagnosed in middle-aged women. Histopathology and immunohistochemical study remain the key to diagnosis. Like other benign mesenchymal vulval tumors, AMFB shows indolent behavior and rarely recurs after complete surgical excision. Herein, we present a case of vulvar AMFB in a 51-year-old woman to highlight the diagnostic difficulties when considering this rare entity.

Key Clinical Message: Angiomyofibroblastoma is a benign soft tissue tumor and a form of genital stromal mesenchymal tumor that primarily affects the vulva. It could possibly affect the reproductive‐aged women's lower genital tract (vagina). Angiomyofibroblastoma is a rare benign soft tissue tumor primarily affecting the vulva in reproductive‐aged women. We report a 67‐year‐old female complaining of a painless mass in her right vulva spreading to the right inguinal region over the past 2 years. The first clinical impression was a canal of Nuck hernia, diagnostic laparoscopy was planned to rule hernia out. The vulvar mass was excised, and a histopathology examination revealed Angiomyofibroblastoma. [ABSTRACT FROM AUTHOR]

Ling Chen,1 Qiuwei Sun,1 Junchao Zhang,1 Xizhong Xu,2 Xiaofang Fan1 1Department of Ultrasound Medicine, Affiliated Hospital of Jiangnan University, Wuxi City, Jiangsu Province, People’s Republic of China; 2Department of Gynecology, Affiliated Hospital of Jiangnan University, Wuxi City, Jiangsu Province, People’s Republic of ChinaCorrespondence: Xiaofang Fan, Department of Ultrasound Medicine, Affiliated Hospital of Jiangnan University, No. 200, Huihe Road, Binhu District, Wuxi City, Jiangsu Province, 214062, People’s Republic of China, Tel + 86-13861843829, Email fanxiaolll@126.comBackground: Angiomyofibroblastoma (AMFB) is an uncommon disease with few literature reports, leading to the poor understanding of its diagnosis, treatment, and postoperative follow-up plans among gynecologists.Objective: To study the clinical and pathological features of vulvar AMFB and discuss its treatment and prognosis.Case Summary: The 3 cases were characterized by a gradually increasing painless mass in the vulva. Preoperative diagnosis was difficult and mainly depended on ultrasonic examination. Immunohistochemistry confirmed clear boundaries of AMFB. This condition could be completely cured by surgery, and the prognosis was good.Conclusion: The vulvar AMFB is a rare tumor that is frequently misdiagnosed before surgery. Ultrasound is preferred in auxiliary diagnosis, and surgery remains the best treatment, and long-term follow-up is necessary to avoid recurrence or other complications.Keywords: angiomyofibroblastoma, diagnosis, surgery, follow-up

Abstract Background Cellular angiofibroma (CA) is a rare, benign mesenchymal tumor first described by Nucci et al. (Am J Surg Pathol 21:636–644, 1997. 10.1097/00000478-199706000-00002). It affects both men and women, although it is more common in middle-aged women. CA is well circumscribed and usually observed on the body surface, primarily in distal genital regions. Aggressive angiomyxoma and angiomyofibroblastoma are clinically and histologically similar; therefore, it may be necessary to distinguish between CA and these similar tumors. We present a rare case of CA, with atypical features, in the retroperitoneal space during pregnancy. Case presentation The presence of a 130 mm tumor was detected in a 19-year-old woman. The tumor, located in the retroperitoneal space, was found during first pregnancy examination. At 16 weeks of gestation, the woman developed nausea and fever, and it was diagnosed with acute pyelonephritis. After a few days, the amniotic membranes prematurely ruptured, leading to a miscarriage. The woman underwent a tumor resection, after miscarriage. This case presented with atypical features of CA. This included the young age of the patient, and presence of a tumor in the retroperitoneal space. Conclusion In this case, the diagnosis of CA was difficult due to the rarity of the disease and its atypical clinical features. From this experience, we recommend that the discussion on the efficacy of surgical treatment and pregnancy outcomes should be done based on individual case, and not generalized.

Key Clinical Message Angiomyofibroblastoma is a benign soft tissue tumor and a form of genital stromal mesenchymal tumor that primarily affects the vulva. It could possibly affect the reproductive‐aged women's lower genital tract (vagina). Abstract Angiomyofibroblastoma is a rare benign soft tissue tumor primarily affecting the vulva in reproductive‐aged women. We report a 67‐year‐old female complaining of a painless mass in her right vulva spreading to the right inguinal region over the past 2 years. The first clinical impression was a canal of Nuck hernia, diagnostic laparoscopy was planned to rule hernia out. The vulvar mass was excised, and a histopathology examination revealed Angiomyofibroblastoma.

Background: Cellular angiofibroma (CA) is a rare, benign mesenchymal tumor first described by Nucci et al. (Am J Surg Pathol 21:636–644, 1997. 10.1097/00000478-199706000-00002). It affects both men and women, although it is more common in middle-aged women. CA is well circumscribed and usually observed on the body surface, primarily in distal genital regions. Aggressive angiomyxoma and angiomyofibroblastoma are clinically and histologically similar; therefore, it may be necessary to distinguish between CA and these similar tumors. We present a rare case of CA, with atypical features, in the retroperitoneal space during pregnancy. Case presentation: The presence of a 130 mm tumor was detected in a 19-year-old woman. The tumor, located in the retroperitoneal space, was found during first pregnancy examination. At 16 weeks of gestation, the woman developed nausea and fever, and it was diagnosed with acute pyelonephritis. After a few days, the amniotic membranes prematurely ruptured, leading to a miscarriage. The woman underwent a tumor resection, after miscarriage. This case presented with atypical features of CA. This included the young age of the patient, and presence of a tumor in the retroperitoneal space. Conclusion: In this case, the diagnosis of CA was difficult due to the rarity of the disease and its atypical clinical features. From this experience, we recommend that the discussion on the efficacy of surgical treatment and pregnancy outcomes should be done based on individual case, and not generalized. [ABSTRACT FROM AUTHOR]

Objective： To investigate the clinical characteristics, diagnosis procedure and treatment method of the patient with recurrent vulvar angiomyofibroblastoma (AMFB)，and to improve the understanding of clinical doctors to this disease. Methods：The clinical data, imaging examination results, pathological examination results and immunohistochemical examination results of 1 patient with recurrent vulvar AMFB were collected； the above clinical data were analyzed, and the relevant literatures were reviewed. Results：A 50-year-old female patient underwent excision of vulvar mass 5 years ago in the local hospital for a vulvar mass，the postoperative pathology suggested AMFB. The patient was now admitted to the hospital due to a 6-month self-reported vaginal mass and a high degree of suspicion of recurrence of AMFB. The gynecological examination suggested a swelling under the labia major on the left side, with complete skin surface, deep protrusion towards the rectum and unclear boundary with the bowel；the size of tumor was about 5. 0 cm×4. 0 cm×4. 0 cm，with regular shape, clear boundary，and poor activity，and there was no obvious tenderness，ulceration and skin lesion. Superficial ultrasound showed a slightly low anechoic light mass with a range of 5. 0 cm×4. 0 cm×4. 0 cm at the left labia major, in which multiple mass hyperechoics were observed. Color Doppler flow imaging （CDFI) showed visible blood flow signals in and around the labia major. Conclusion：Although recurrence and malignancy of AMFB are rare, postoperative follow-up of AMFB should be given high priority to reduce the recurrence and increase the cure rate. [ABSTRACT FROM AUTHOR]

Angiomyofibroblastoma is a benign, usually small neoplasm typically constituted by spindle-shaped and epithelioid cells in a vascularized, myxoid-fibrous stromal background. It is most often seen in the superficial genitalia of female patients of reproductive age. However, various clinical and histologic features have been reported, including tumors in male patients, malignant transformation, extragenital sites, huge sizes, and a prominent lipomatous pattern. We report the clinical and pathologic features of one such tumor: a 23.5 cm lipomatous angiomyofibroblastoma of the vulva in a 40-year-old female patient. We also discuss important diagnostic considerations when approaching such large tumors, particularly in the setting of a biopsy specimen. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Angiomyofibroblastoma (AMF) is a rare benign soft-tissue tumor that most frequently affects the lower genital tract of young to middle-aged women. It mainly consists of two components: stromal cells and prominent vasculature. Clinically, it is usually asymptomatic and resembles Bartholin's cyst. Although it is a benign tumor, cases with recurrence and sarcomatous transformation have been reported. Due to the overlapping of histopathological picture, diagnostic perplexity often arises between AMF and aggressive angiomyxoma (AAM). AMF being benign in nature is treated by local excision, whereas AAM is a more infiltrative lesion that has a higher tendency for local recurrence.

Vulvo-vaginal stromal tumours are a rare and diverse group of mesenchymal neoplasms unique to hormone-responsive stroma of the vulva and vagina. These tumours are mostly benign, except for the locally aggressive deep angiomyxomas. Often these tumours pose diagnostic challenges, resembling certain malignant vulvo vaginal tumours. This case series highlights clinicopathological features of four angiomyxomas; a single angiomyofibroblastoma, and another superficial myofibroblastoma, including their clinical outcomes. All patients were in their 4th or 5th decade of life. Only 1/4 angiomyxomas was correctly diagnosed at the referring hospitals. Three out of four patients harbouring angiomyxomas achieved clinical remission post-surgery, while one patient was lost to follow-up. By immunohistochemistry, tumor cells showed variable positivity for desmin, SMA, ER, and PR, and negativity for S100P and CD34. The angiomyofibroblastoma was initially misdiagnosed as a liposarcoma, and the patient was lost to follow-up after diagnosis. Immunohistochemically, the tumor cells were diffusely positive for SMA and ER; weakly and focally positive for desmin, and negative for AE1/AE3, CD34, and S100P. The patient with superficial myofibroblastoma is in clinical remission post-excision with an 18-month follow-up. Immunohistochemically, the tumor cells showed CD34 positivity. Therapeutically, none of the patient received adjuvant treatment, except for a single patient with angiomyxoma, who underwent chemoradiation for a synchronous cancer cervix post-surgery. This case series provides valuable insights into the clinical heterogeneity, diagnostic intricacies, and outcomes of vulvo-vaginal stromal tumours from an oncology centre in India, further contributing to a better understanding of these rare tumours., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

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. [ABSTRACT FROM AUTHOR]

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Angiomyofibroblastoma (AMF) is a rare benign soft-tissue tumor that most frequently affects the lower genital tract of young to middle-aged women. It mainly consists of two components: stromal cells and prominent vasculature. Clinically, it is usually asymptomatic and resembles Bartholin's cyst. Although it is a benign tumor, cases with recurrence and sarcomatous transformation have been reported. Due to the overlapping of histopathological picture, diagnostic perplexity often arises between AMF and aggressive angiomyxoma (AAM). AMF being benign in nature is treated by local excision, whereas AAM is a more infiltrative lesion that has a higher tendency for local recurrence. [ABSTRACT FROM AUTHOR]

Mesenchymal tumours of the vulva are rare and consist of two types, difficult to distinguish but with different prognoses. Angiomyofibroblastoma (AMFB) is a benign tumour, whereas Aggressive Angiomyxoma (AA) is an infiltrating tumour. We describe a 22-year-old nulliparous patient with a vulvar mass sized 19 cm in diameter. After preoperative assessment by ultrasound, chest X-ray, and MRI, wide excision on the tumour was done and diagnosed as AMFB. Differentiation from AA is being discussed.

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 variety of extratesticular tumors and tumor-like conditions can occur in the scrotum. Angiomyofibroblastoma is a rare, slow-growing, well-circumscribed mesenchymal tumor, found predominantly in women, and is even a more unusual occurrence in the male genital tract. We describe a case of a 64-year-old male presenting with a scrotal painless mass. On sonography, a well-circumscribed extratesticular mass was present. As no specific imaging findings were present, it was a surprise to learn that the subsequently excised mass proved to be an angiomyofibroblastoma-like tumor. To the best of our knowledge, the sonographic appearance of this tumor in the scrotum has been reported previously in only one case.

Introduction: Angiomyofibroblastoma is a rare benign myofibroblastic neoplasm which mainly occurs in the soft tissues of the pelvi-perineal region of females. Aim: To present an unusual case of angiomyofibroblastoma mimicking an inguinal hernia in a 62-year-old male. Material and methods: The patient was hospitalized with an irreducible, painless inguinal mass and surgical intervention for inguinal hernia was decided. The well-defined nodular mass was sent for histological examination. Results: Under microscope, proliferation of spindle and oval cells around thin-walled vessels was observed, being intermingled with mature adipocytes. We did not identify necrosis, haemorrhage, cytologic atypia or mitotic figures. The tumour cells displayed positivity for desmin, vimentin, CD34, oestrogen and progesterone receptors, a low Ki67 index and unusual nuclear positivity for c-theta (PKCθ). They were negative for smooth muscle actin (SMA), S100, CD44, maspin, synaptophysin, DOG1 and CD117. The case was diagnosed as angiomyofibroblastoma, the main challenge being the differential diagnosis with aggressive angiomyxoma, which can present a similar histologic aspect and immunophenotype and recurs more frequently. No recurrences were observed 8 months after the surgery. Conclusions: Angiomyofibroblastoma should be included in the differential diagnosis of inguinal hernia. This is the fourteenth case of angiomyofibroblastoma diagnosed in males. [ABSTRACT FROM AUTHOR]

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 that primarily arises in the vulvar region. The tumor grows slowly but becomes quite large. Case: A 29-year-old nulliparous woman was diagnosed with having an AMF that was asymptomatic and huge. The AMF distorted both labia. This case was managed with a total vulvectomy and by addressing the serious skin defect caused by this surgery. A "double-barreled" colostomy was performed to protect this patient from any potential stool-induced infection. After these procedures, she received negative-pressure wound therapy (NPWT) dressings for 2 months, and reconstructive surgery with full-thickness skin grafts from both thighs was then performed. The colostomy was closed once the skin defect was closed. Results: The reconstructive surgery was successful with no adverse outcomes. Conclusions: It is important to confirm AMF histopathologically. When AMF is suspected for a vulvar lesion, complete excision of this tumor is the treatment of choice. NPWT is an appropriate way to facilitate wound healing. In a limited-resource country, NPWT can be useful for patient care. [ABSTRACT FROM AUTHOR]

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. [Cukurova Med J 2015; 40(4.000): 822-825]

Angimyofiblastomlar(AMFB), genital stromal tümörler içerisinde yer alan,nadir izlenen benign mezenkimal tümörlerdir. Genellikle orta-yaş bayanlarda görülüp, sıklıkla labium majörü daha az sıklıklada vajinayı etkileyebilmektedir. AMFB benzeri tümörler adı altında, nadiren erkekler de görülebilmektedir. Yerleşim yeri ve klinik prezentasyonu ile bartolin kistleri, alt genital tract lipomları veya nadiren de agresif anjiyomiksomalar ile karışabilmektedir. Vulvar kitle ile başvuran hastalarda, ön tanıda AMFB ler unutulmamalıdır. Tedavisi genellikle basit cerrahi eksizyondur.Bu olguda, bartolin kist yanlış ön tanısı ile medikal tedavi verilen AMFB olgusundan bahsedildi

Lipomatous angiomyofibroblastoma (LAMF) is a rare superficial muco-cutaneous tumor of the female genital tract in women of reproductive age and early menopause. We report the case of a 36-year-old woman who presented with an asymptomatic 1 cm round vulvar mass. The tumor showed both hypercellular and hypocellular areas composed of spindle and epithelioid cells set in a loose fibromyxoid background. Anastomosing networks of small to medium sized dilated vessels, surrounded by condensed spindle cells, were distributed equally throughout the tumor. Mature adipocytes comprised more than half of the tumor's volume. The neoplastic cells were reactive for both desmin and vimentin and did not express CD34, smooth muscle actin, S100 protein and muscle-specific actin. Making the right diagnosis may be challenging to medical professionals as LAMF may mimic both clinically and histologically several other benign and malignant gynecological lesions. LAMF has a benign course without evidence of local recurrence or metastatic potential.

Abstract: 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. [ABSTRACT FROM AUTHOR]

Objective：To investigate the clinicopathological features, diagnosis, treatment and prognosis of female genital angiomyofibroblastoma (AMF). Methods：Retrospective analysis of the clinical data of 10 female genital AMF patients treated during January 2012 to October 2017, combined with related literatures, summarized their clinical and pathological features, diagnosis, treatment and prognosis. Results：The average onset age was (49.90±7.62) years old in 10 patients with AMF. The patients had no obvious clinical symptoms, and no specificity in imaging examination. Under the microscope, the cell-rich area and cell-sparse area were alternately distributed, the tumor tissue was composed of significantly small lumen, thin tube wall and dilated blood vessels which were surrounded by eosinophilic circular or fusiform tumor cells, and the tumor matrix was loose, edema and mucus like, and nuclear fission was rare. All the 10 patients underwent partial complete resection. 8 cases were followed up and the median follow-up time was 37 months. No recurrence occurred. Conclusions：There is no specificity in the clinical manifestation and imaging of AMF which occurs in female genital organs. The diagnosis and differential diagnosis mainly depends on the pathological features and immunohistochemistry. The surgical treatment is the main treatment and the prognosis is good. [ABSTRACT FROM AUTHOR]

A 50-year-old woman (gravida 2, para 2) first noticed a small nodule in the right labium majus 2 years prior to the initial visit to the Hachinohe Red Cross Hospital (Hachinohe, Japan), which had gradually increased in size. On physical examination, a well-circumscribed, elastic, firm, goose egg-sized, subcutaneous mass protruding from the right labium majus was identified. On magnetic resonance imaging (MRI), the lesion was hypointense on T1-weighted images and was well-circumscribed, strongly enhanced and homogeneous on gadolinium-enhanced images, measuring 7.5x4 cm. The same tumor had measured 2.6 cm on an MRI performed 6 years earlier. Based on the clinical course and imaging findings, angiomyofibroblastoma was diagnosed and surgical resection of the tumor was performed. The tumor was well-circumscribed and highly vascular. The intraoperative blood loss was 70 ml. Histopathologically, the tumor cells were concentrated around blood vessels, were spindle-shaped to oval with mild atypia, and were positive for vimentin, desmin, neural cell adhesion molecule (N-CAM), CD-34, estrogen receptor and progesterone receptor, and negative for S-100. Based on these findings, the diagnosis of angiomyofibroblastoma was confirmed. Angiomyofibroblastoma is a benign mesenchymal tumor that occurs in the female external genitalia. Differentiation of this tumor from aggressive angiomyxoma, a fast-growing infiltrative malignancy that occurs in the same region, may be challenging. The diagnosis of angiomyofibroblastoma is usually based on the histopathological findings of the resected specimen. The present case is of value, as the angiomyofibroblastoma was successfully diagnosed preoperatively based on the clinical course and imaging findings. [ABSTRACT FROM AUTHOR]

Angiomyofibroblastoma is a rare benign soft tissue tumor that arises predominantly in the female genital tract. It occurs less commonly in the scrotum, spermatic cord, and the retroperitoneum. Its diagnosis is based on specific histological and immunopathological features. However, the condition has never been previously described in the extremities. We present the first case report of angiomyofibroblastoma presenting as a slow-growing tumor in the foot of a 48-year-old lady. [ABSTRACT FROM AUTHOR]

Lipomatous angiomyofibroblastoma is extremely rare, with only 16 cases reported to date. We herein describe an additional case arising in the left vulvar region of a 49-year-old woman. The resected 5.3-cm yellowish tumor predominantly consisted of fat cells (85% of the tumor), together with medium- and small-sized vessels, multifocal fibrotic areas and pseudoangiomatous spaces. Spindle, rounded, and/or epithelioid tumor cells proliferated in a nest and/or cord-like pattern, or singly within perivascular fibrous tissues and between fat cells. The tumor cells were positive for vimentin, estrogen receptor, progesterone receptor, B-cell lymphoma 2, and CD10, but were negative for desmin, cytokeratin, epithelial membrane antigen, S-100 protein, human melanoma black 45, C-kit and p40. Ultrastructural examination revealed that these tumor cells exhibited fibroblastic characteristics. Lipomatous angiomyofibroblastoma should be discriminated from other lipomatous tumors, including spindle cell lipoma, angiomyolipoma and cellular angiofibroma containing numerous fat cells. [ABSTRACT FROM AUTHOR]

A variety of extratesticular tumors and tumor-like conditions can occur in the scrotum. Angiomyofibroblastoma is a rare, slow-growing, well-circumscribed mesenchymal tumor, found predominantly in women, and is even a more unusual occurrence in the male genital tract. We describe a case of a 64-year-old male presenting with a scrotal painless mass. On sonography, a well-circumscribed extratesticular mass was present. As no specific imaging findings were present, it was a surprise to learn that the subsequently excised mass proved to be an angiomyofibroblastoma-like tumor. To the best of our knowledge, the sonographic appearance of this tumor in the scrotum has been reported previously in only one case. [ABSTRACT FROM AUTHOR]

Cellular angiofibroma represents a rare mesenchymal tumor. It is composed by spindle cells and prominent blood vessels. Most cases occur in middle-aged women and predominantly affects genital region with asymptomatic neoformations. The differential diagnosis includes a number of other mesenchymal lesions and it's performed by clinical and histological characteristics and immunohistochemical findings. We report a cellular angiofibroma case in the scrotal region, manifested as multiple pedunculated neoformations, treated with surgery. [ABSTRACT FROM AUTHOR]