Angiomyxoma Research Articles

Rare Incidental Finding and Laparoscopic Excision of Broad Ligament Aggressive Angiomyxoma (AA) during a Planned Laparoscopic Uro-Gynecological Procedure

Objective: This Video Case report depicts the occurrence of a rare benign appearing broad ligament mass which was excised and later histologically diagnosed to be aggressive angiomyxoma.

Aggressive angiomyxoma of the vulva: report of three cases and literature review

Aggressive angiomyxoma of the vulva: report of three cases and literature review

En Bloc Resection of an Aggressive Angiomyxoma by a Novel Combination Laparoscopic and Open Perineal Approach

Study ObjectiveTo show a novel combination laparoscopic and open perineal approach to complete resection of aggressive angiomyxoma. DesignStep-by-step video demonstration of the combination approach (Canadian Task Force classification III). SettingCombined laparoscopic and open perineal approach was performed in the tertiary center. PatientA 46-year-old woman presented with an 8-cm vulvar mass, diagnosed as an aggressive angiomyxoma. The patient, who strongly desired to preserve her uterus and ovaries, provided informed consent for resection of the tumor by our combination approach, also approved by our Institutional Review Board. InterventionCombined laparoscopic and open perineal approach. Measurements and Main ResultsAggressive angiomyxoma is a rare mesenchymal neoplasm that occurs most often in the female pelviperineal region [1]. Aggressive angiomyxoma is locally infiltrative, and high postoperative local recurrence rates (36%–72%) due to incomplete resection have been reported [2]. Therefore, until recently, wide surgical excision with tumor-free margins have been the most commonly accepted treatment. However, aggressive angiomyxoma is a benign, slow-growing tumor, and because extensive surgical resection, which is associated with high operative morbidity rates, has not been shown to have a significant effect on prognosis, a more conservative procedure may be preferable [3]. The mass was located mainly at the left ischiorectal fossa, but it extended above the pelvic diaphragm and was attached to internal obturator muscle, vagina, bladder, urethra, and rectum. We excised the tumor completely and without complications by a combined laparoscopic and open perineal approach. Twelve months have passed since the surgery, and there has been no adjuvant treatment and no sign of recurrence. ConclusionOur combination approach to aggressive angiomyxoma in the pelviperineal region is technically feasible, and the good visualization and meticulous dissection provided during the laparoscopic portion of the surgery contribute to complete resection.

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.

Huge abdominal and perineal aggressive angiomyxoma: A misdiagnosed case report and literature review

Aggressive angiomyxoma (AA) is a distinctive soft tissue tumor with a high risk of local recurrence. Clinicians must be aware of this rare tumor pre-operatively. Excision is the preferred method of AA treatment. The case report presents a case of a 36-year-old woman who was difficulty in walking due to a non-painful tumor in the abdomen and perineum. She was misdiagnosed as abdomen neurofibroma for more than 10 years, and an operation was performed in 1997. However, the tumor was incompletely resected because its huge volume accompanies with extensive infiltration and bleeding. The tumors in her abdomen and perineum were growing gradually, and the latter became a large lump which impeded her daily life. In 2008, the perineal tumor was incompletely resected, which weighed 10725 g. The severe hemorrhage had been ceased by Gonadotropin-Releasing Hormone treatment. She is alive till now. Details of the history and operative procedures are presented. An AA diagnosis was made by microscopy immunohistochemically. Long-time misdiagnosis and improper treatment are the important reasons for making it impossible to be radically resected. Pathological and immunohistochemical examination are important for avoiding misdiagnosis. For this case, there is a remaining tumor in her abdomen. A special project including further follow-up and treatment will be taken out.

Pattern of skin cancer in north western region of Yemen

The pattern of skin cancer differs in different countries. The aim of this study is to identify the pattern of skin cancer in northwestern region in Yemen and to compare it to similar studies done in southern Yemeni governorates and in neighborhood. This is a descriptive prospective study of patients data with histologically diagnosed skin cancers carried out in the Saudi hospital in Sa'adah and Hajjah governorates in Yemen between Jan.2007 and Dec.2014. The data were collected and recorded on proforma and was analyzed according to the type of tumor, age and gender of the patients and anatomical site involved. Two histopathologists investigated the specimens. There were 324 Yemeni patients with skin malignancy, 204 were males (63%) and 120 were females (37%), the M: F ratio was 1.7:1. Most of the patients were over the age of 60 years and the mean age of all cases was 66.4 years. Squamous cell carcinoma was the most frequent (50%), followed by basal cell carcinoma (29%), Non-Hodgkin's lymphoma (4.9%), malignant melanoma (4%), and Kaposi's sarcoma (3.7%). Other rare primary skin cancers such as basosquamous carcinoma, adnexal carcinoma, dermatofibrosarcoma protuberans, neuroendocrine carcinoma, extramammary Paget's disease and aggressive angiomyxoma were recorded. Metastatic skin lesions were seen in six patients. The patterns of skin cancer in northwestern governorates of Yemen are nearly similar to international studies with higher incidence of squamous cell carcinoma. Head and neck was the most common affected site, followed by lower extremities. Community education about the risk of long exposure to sunlight is important, including coverage of the head and neck when working outdoor under sunny weather.

Bicyclist's vulva: Diagnostic and therapeutic aspects

The authors report a case of a rare vulvar condition in a young cyclist, known as bicyclist's vulva. The diagnosis was based on the clinical context (intensive cycling training with repeated saddle sores, responsible for painless unilateral swelling of the labium majus to a significant size and functional discomfort). Imaging examinations were used to characterize the lesion and its relationship with the pelvic organs. The lesion was composed of connective tissue, poorly demarcated and heavily collagenised without necrotic or hemorrhagic areas. Large cicatricial collagen bundles were observed. The fibroblasts revealed no atypia and just a few scattered undilated vessels ere noted. Special staining did not reveal any amyloid deposits. A complementary immunohistochemical analysis with anti-CD 34 antibody showed vascularization that was homogeneous and morphologically normal. The treatment was surgery, and in our case the cosmetic and functional impact of wide surgical excision of the lesion was improved by in situ injection of autologous fat. The pathology findings enabled us to rule out other perineal tumors such as aggressive angiomyxoma of the vulva and to describe the lesion microscopically.

Impact of Pathologist Involvement in Sarcoma and Rare Tumor Patient Support Groups on Facebook: A Survey of 542 Patients and Family Members.

- Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma). - A total of 542 people responded (403 were patients): 264 from groups with a pathologist, and 278 from groups without active pathologist involvement. Of groups with an active pathologist, respondents agreed the pathologist's posts helped them better understand their disease (107 of 119; 90%) and relieved some of their disease-related anxiety (92 of 119; 77%). And for these groups 98% (117 of 119) of respondents agreed that having a pathologist in their group was a good thing; 83% (192 of 232) wanted more pathologists involved. More respondents from groups with an active pathologist (219 of 236; 93%) than without one (215 of 252; 85%) agreed: "pathologists are an important part of the patient care team for patients with cancer and other rare tumors" ( P = .008). - This study is the first to evaluate the impact of pathologist interaction with Facebook patient support groups and to assess perceptions about the specialty of pathology from a large group of patients with rare tumors. Pathologist involvement in Facebook patient groups appears to positively influence patient perception of the importance of pathologists. We hope these data will encourage more pathologists to participate in Facebook patient support groups.

Aggressive Angiomyxoma of the Perineum in a 50-Year-Old Woman: Case Report and Current Level of Education

Background: Aggressive angiomyxoma is a rare soft tissue mesenchymal tumor, locally infiltrative with a tendency to repeated local recurrence. The first description was in 1983. Normally it occurs in the vulvovaginal region, perineum and pelvis of females in reproductive age. Local wide excision of the tumor is the primary management. The local recurrence rate is high. The expression of estrogen and progesterone receptors in aggressive angiomyxoma suggests a hormone dependency of the tumor Case report: A 50-year-old woman reported first in 2013 with a painless swelling at the perineum. She underwent surgical excision. Pathologic findings reported the tumor as an aggressive angiomyxoma. The patient did not appear to the follow-up examinations. In 2016 she reported again with a painless swelling at the perineum and underwent a local wide resection. The perineal body had to be reconstructed because of the deep infiltration of the tumor. Conclusion: Aggressive angiomyxoma is a rare disease, but when treating women with a painless swelling in the vulvovaginal region, perineum or pelvis aggressive angiomyxoma should be considered as a differential diagnosis. There is no standardised therapy described, but complete resection seems to be important. Descriptions of pharmacologic treatment exist with gonadotropinreleasing hormone agonists.

Aggressive angiomyxoma of scrotum in a man: A case report and literature review of 73 cases of aggressive angiomyxoma in men

Aggressive angiomyxoma is a mesenchymal tumor arising from the pelvis and perineum, classically seen in women and rarely found in men. Due to its rarity, there is a low index of suspicion for diagnosis. We describe an unusual case of aggressive angiomyxoma in a male with intellectual disability. A thorough review of existing 73 cases was also conducted. This report affirms vimentin as the most sensitive marker and S-100 is the most specific marker. Desmin seems to stain positively and negatively in a sporadic fashion, making it less reliable as an immunohistochemical marker. Wide excision is commonly regarded as the treatment for aggressive angiomyxoma in men. To date, there are no established clinical guidelines on the optimal duration of follow-up. Long-term follow-up may be required even up to 10 years to detect local recurrences.

Aggressive Angiomyxoma of the Paratestis: Case Report with a Comprehensive Review of Literature

Aggressive angiomyxoma (AA) is a rare mesenchymal tumor of the pelvis and perineum that occurs almost exclusively in adult women. Its incidence is approximately six fold higher in women. Rarely, this tumor appears in men, simulating inguinal hernia, testicular neoplasm, spermatic cord neoplasm, hydrocele, or spermatocele. Here we report a case of aggressive angiomyxoma in a 20yrs old male.

Myxoid and pseudocystic degeneration of the umbilical cord

Cystic, edematous, and myxoid lesions of the umbilical cord are rare conditions which have been sporadically reported in literature, most often detected at prenatal ultrasonography. Most reports of cystic cord conditions are ultrasonographic studies with a few featuring pathological descriptions. This is a report of a rare case of umbilical pseudocystic lesion with full pathological description. The possibility of this extremely myxoid lesion confounding the diagnosis of tumors such as angiomyxoma is highlighted.

Aggressive angiomyxoma of the uterine corpus: A rare presentation

Aggressive angiomyxoma (AA) is a rare benign soft tissue tumor usually affecting the female pelvis and perineum. A 16‑year‑old girl presented on June 23, 2016, with vaginal protrusion of 1‑year duration. Examination revealed protrusion through the introitus; it was hyperemic with necrosis. Investigation revealed urinalysis that revealed blood (+), leucocytes (+), and others normal; Swab (taken from purulent discharge on the protruded mass) microscopy revealed numerous pus cells; and culture yielded no growth. Abdominopelvic ultrasound (USS) showed left‑sided pelvic soft tissue mass with no features of metastasis. Other investigations were normal. She had polypectomy with subtotal abdominal hysterectomy. Histology revealed benign AA. A rare tumor of the genital tract had been presented with a diagnostic dilemma. However, confirmation of diagnosis was easy with histology. Keywords: Angiomyxoma; uterine corpus; vaginal protrusion

Aggressive Angiomyxoma Presenting as a Recurrent Cervical Polyp – A Case Report

Aggressive Angiomyxoma Presenting as a Recurrent Cervical Polyp – A Case Report

Perineal aggressive angiomyxoma in a menopausal woman

Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumour that is principally located in the soft tissues of the pelvis and perineum of young women. The primary features of this benign...

18F-FDG PET/CT in aggressive angiomyxoma of the pelvis

18F-FDG PET/CT in aggressive angiomyxoma of the pelvis

Aggressive angiomyxoma of the liver: a case report and literature review

BackgroundAggressive angiomyxoma (AAM) is a rare mesenchymal tumor that occurs almost exclusively in the soft tissue of the pelvis and perineum. AAM has both locally infiltrative and recurrent characteristics. Very few cases of AAM occurring outside of the pelvis and perineum have been reported. Here, we report a case of AAM originating in the liver of a 33-year-old female patient.Case presentationA 33-year-old woman underwent S8 subsegmentectomy after clinical diagnosis of a mucinous cystic neoplasm of the liver. Histological analysis revealed a tumor composed of spindle-shaped cells with vascular proliferation in a myxoid stroma. Immunohistochemically, the tumor cells stained positively for CD34, estrogen receptor (ER), and progesterone receptor (PgR) and negatively for S-100, EMA, CK19, CD99, HMB45, and α-smooth muscle actin. The tumor was diagnosed as AAM originating from the liver. The patient received no adjuvant chemotherapy. No sign of recurrence or distant metastasis has been noted for 10 months after the surgery.ConclusionsWe here report a second case of AAM originating from the liver, which is an uncommon location for this particular tumor.

Angiomyofibroblastoma of the vulva diagnosed preoperatively: A case report.

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 7.5×4 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.

Surgical Resection of Retroperitoneal Aggressive Angiomyxoma: Case Report and Review of the Literature.

Aggressive angiomyxoma is a benign stromal tumor with a higher prevalence in middle-aged women. The objective of this case report is to illustrate the aggressive clinical behavior of this benign tumor. We present the case of a 45-year-old female patient, with tumor recurrence after multiple surgical resections of a pelvis-subperitoneal angiomyxoma. Surgical excision of the tumor, with extensive pelvic dissection and organ resection, was performed. The 12-month follow-up showed no tumor recurrence. Based on this case, and the published literature we may conclude that surgical resection represents the main treatment of aggressive angiomyxoma, even though it is associated with significant morbidity and a poor local control of the tumor.