Umbilical Cord Hemangiomas: A Multi-Institutional Case Series With Literature Review.

Umbilical cord hemangiomas are rare lesions, for which data on pregnancy outcome is lacking. This study combines a multi-institution 4-case series with a systematic literature search (n = 52) to determine possible pathologic lesion parameters which may have an effect on pregnancy outcome. Of all 56 pregnancies, lesion size ranged from 0.2 to 23.0 cm with pregnancy outcomes ranging from healthy liveborns (58.9%), liveborns with severe complications largely due to prematurity and/or fluid overload (12.5%), intrauterine/neonatal demise (25.0%), and pregnancy termination (3.6%). Of the 52 cases included for statistical analysis, there was no significant association between fetal outcome and vascular lesion location (P = .12) or fetal outcome and single umbilical artery involvement versus involvement of other vasculature (P = .29). The mean length of vascular lesions that resulted in healthy liveborns did not significantly differ from those resulting in severe fetal complications and/or demise (P = .72). Cases resulting in severe complications and/or demise were significantly earlier at delivery than those resulting in healthy liveborns (P < .001). Combined findings suggest that functional lesion characteristics, such as the degree of turbulent flow generated, have more significance than size, especially in early gestation losses. Moving forward, standardized reporting of pathologic lesion characteristics is paramount to better predict pregnancy prognosis.

Prenatal Diagnosis of Umbilical Cord Angiomyxoma: Case Studies and Literature Review of 45 Cases.

We describe two cases of umbilical cord (UC) angiomyxoma diagnosed prenatally by sonography in the second trimester of pregnancy. In both cases, a complex mass was detected at the placental insertion site, characterized by an echoic nodule surrounding the umbilical vessels and distal edematous Wharton's jelly. Follow-up scans showed that the mass grew mainly at the expense of its edematous component, with normal uteroplacental Dopplers throughout the remaining of the pregnancy. However, late-onset fetal growth restriction complicated the progress of pregnancy, requiring delivery by Cesarean section at 37 weeks' gestation in both cases. Neonatal courses were unremarkable. An extensive review of the English literature was also performed, collecting 45 similar cases including ours. Our experience as well as the review of the literature confirms that UC angiomyxoma is an uncommon, sporadic condition that is usually detected incidentally during prenatal sonography and presents as an isolated finding. Nevertheless, it represents a high-risk condition for pregnancy complications including prematurity, fetal growth restriction, and fetal demise.

Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group.

Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30-40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.

Varied Clinical Presentations of Aggressive Angiomyxoma of the Vulva: A Rare Entity.

Objective: Aggressive Angiomyxoma (AA) of the vulva is a slow-growing mesenchymal tumour with a tendency to local invasion and recurrence. Case report: We report two cases of vulvoperineal masses that were diagnosed to be Aggressive Angiomyxomas after surgical excision. Both patients presented to the Gynaecology OPD of All India Institute of Medical Sciences, Bathinda, Punjab, India, in 2020 and 2022 with complaints of a mass coming out of introitus of three years duration and 14 years duration, respectively. The first patient was managed by surgical excision of the mass via abdominoperineal approach, while the second patient underwent vaginal hysterectomy along with the removal of the mass. Both patients were given GnRH analogues after the surgery to avoid any further recurrences and have been in remission on follow-ups so far. Conclusion: Due to its rare occurrence, clinicians should consider the possibility of AA while encountering patients with vulvovaginal masses to avoid misdiagnosis and delayed management.

Angiomyxoma coexisting with focal nodular hyperplasia: A case report.

Angiomyxoma (AM) occurs almost exclusively in the soft tissues of the pelvic and perineal regions. AM is a highly uncommon condition that can be easily misdiagnosed when it is present in other regions of the body. The current study presents a case in which AM of the liver coexisted with focal nodular hyperplasia (FNH). A 56-year-old woman presented with two space-occupying lesions of the liver without any other clinical symptoms, and it was not easy to definitively diagnose the two intrahepatic lesions by imaging examinations. Due to the low incidence of AM in the liver, precise and clear clinical information on the condition is still unavailable, and the lesion was initially misdiagnosed as other hepatic tumors preoperatively. Once a tumor resection had been performed, a histopathological examination revealed that the microscopic features of the lesions were consistent with those of AM and FNH. The patient was followed up for 1 year, and no recurrence or metastasis was found. Surgical excision is an effective treatment for AM, and long-term follow-up is essential due to the risk of recurrence. The joint presentation of AM and FNH is rare in clinical practice, and although FNH of the liver is commonly reported, the difficulty of diagnosis increases when both conditions occur at the same time. Therefore, it is necessary to assist clinicians in making informed decisions regarding diagnosis and treatment.

Imaging features of a rare giant intra-abdominal aggressive angiomyxoma.

Aggressive angiomyxoma (AAM) is a rare benign tumor that arises from connective tissue, prominently located in the vulva, vagina, perineum, and pelvis and is mainly found in women aged about 20-40 years old. Giant intraabdominal tumors have rarely been described. These tumors develop slowly over time and are often difficult to diagnose due to various clinical findings, especially in the early stages. Even though surgery is the primary treatment method, the possibility of complete resection is sometimes limited because the tumor tends to infiltrate nearby structures, leading to local recurrence. Only about 10% of AAM cases can be accurately diagnosed before treatment, which causes ineffective outcomes. This article demonstrates a case of giant intra-abdominal AAM precisely diagnosed by suspicious signs on CT and MRI scans before starting treatment.

Aggressive angiomyxoma of transplanted kidney in male:A case report and brief review of literature.

This study presents the case of a 40-year-old male patient after renal transplantation. The CT scan revealed a large mass in the lower abdomen and pelvis, with a branch of the right external iliac artery intersecting the growth. After a comprehensive examination, it was shown that the mass originated from the transplanted kidney, and a radical nephrectomy (including the mass) was performed. We document a case of atypical angiomyolipoma (AAM) occurring in a transplanted kidney. This article reports the case study and a brief literature review of the clinical presentation, diagnosis and treatment of AAM.

Aggressive angiomyxoma of female pelvis and perineum: Retrospective study of 17 cases.

OBJECTIVE: Aggressive angiomyxoma is an uncommon mesenchymal neoplasm characterized by a high recurrence rate, usually observed in the lower genital tract of women during their reproductive age. STUDY DESIGN: Seventeen cases of aggressive angiomyxoma confirmed by pathology from January 2007 to December 2021 in Beijing Chao-yang Hospital were included. We collected clinical data and summarized the clinical and immunohistochemical features. RESULTS: All seventeen included patients were females, aged between 23 and 57 years (mean, 37.7 years; median, 42 years). Fourteen patients were newly diagnosed and three were recurrent. The tumors were located in vulva (58.8 %), vagina (23.5 %), buttock (11.8 %), and cervix (5.9 %). The tumors size were 2 to 15 cm in greatest dimension (mean 8 ± 4.4 cm, median 6 cm). Follow-up data was available for nine patients, which ranged from 25 to 124 months (mean, 82 months; median, 80 months). At the end of follow-up, no other recurrence or metastasis was reported. Immunohistochemical analysis showed immunoreactive for estrogen (10/11) and progesterone (8/11) receptor, desmin (6/8), smooth muscle actin (4/10), and vimentin (4/4), S-100 (1/8) and CD34 (1/7). The Ki67 level was less than 5 % in five cases. CONCLUSIONS: AAM is a hormone-sensitive, distinct rare mesenchymal neoplasm with high incidence of local recurrence. Surgery is the preferred treatment, with complete resection being an essential prerequisite for minimizing the risk of recurrence.

Incidental finding of an angiomyxoma of the urinary bladder: A case report of a rare but aggressive tumour.

Angiomyxoma is a rare mesenchymal tumor arising in the pelvic and perineal regions. Diagnosis of urinary bladder angiomyxoma is difficult, as it lacks typical signs and symptoms, and relies on immunochemistry. We present the case of a 53 year old patient presenting with an incidental finding of bladder tumor during an ultrasound. After a complete transurethral resection was performed, the pathology report led to angiomyxoma diagnosis. After 6 weeks a secondary TURB, along with a CT urogram showed no evidence of residual volume. Transurethral resection seems to be a safe and effective treatment of urinary bladder angiomyxoma.

[Superficial angiomyxoma on the sole of the foot]. / Superfizielles Angiomyxom der Fußsohle.

Superficial angiomyxoma is a rare, benign neoplasm consisting of spindle-shaped fibroblasts in a myxoid, vascularized stroma. Diagnostic clarification is usually based on histology and immunohistochemistry and is of particular importance depending on the clinical presentation, as malignant tumors may also be considered in the differential diagnosis. Herewith, we present a patient with a solitary, superficial angiomyxoma of the sole of the foot. Although it often occurs as an independent entity, it is recommended to exclude syndromes such as Carney complex.

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

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.

Deep Angiomyxoma of the Knee: a Rare Case Report.

Background: Deep angiomyxoma (DAM) is a slow-growing benign tumor with high risk of local recurrence after surgical resection. Although DAM in a lower extremity is extremely rare, clinicians must be aware of its possible occurrence. Suspicion can be made based on clinical examination and radiological imaging but final diagnosis is confirmed on histopathological examination and immunohistochemistry. We intend to present an extremely rare case of DAM in the knee, managed successfully with marginal excision. Case Presentation: A 4-year-old male child presented with the complains of a painless, progressively increasing, soft, non-tender, and fluctuant swelling in his right knee. The plain radiograph showed a non-calcified soft-tissue swelling and MRI revealed a multi-loculated cystic lesion with multiple septations. A pre-operative diagnosis of a benign cystic lesion was made. It was managed by marginal excision of the tumor and a histological diagnosis of DAM was made. IHC staining showed positivity for SMA, CD34 and vimentin were focally positive, while desmin and calponin were negative. At 12 months of follow-up, the patient had a normal painless gait and full knee ROM, without any local recurrence. Conclusion: DAM is a rare tumor which is often misdiagnosed. In this report, we present a rare case of benign cystic lesion which turned out to be DAM on HPE of resected specimen. Marginal excision of this lesion revealed good outcomes with no recurrence until 12 months of final follow-up. With this, we conclude that surgical excision should be the gold standard in cases of DAM.

Misleading clinical and imaging features in atypical aggressive angiomyxoma of the female vulvovaginal or perianal region: report of three cases and review of the literature.

Objectives: Aggressive (deep) angiomyxoma (AAM) is a rare mesenchymal tumor that typically originates from the vulvovaginal region, perineum, and pelvis in adult women. The objective of this case report and literature review is to comprehensively analyze the clinical, imaging, and pathological characteristics of atypical AAM in the female lower genital tract and pelvic floor in order to minimize preoperative misdiagnosis or missed diagnosis and ultimately optimize the clinical management strategy. Methods: The data of three cases with atypical AAM, which demonstrate similarities with other lesions observed in the female lower genital tract over the past 1.5 years, were retrospectively described. This description included clinical management, images and reports of ultrasonography (US) and magnetic resonance imaging (MRI), clinicopathological features, follow-up, and outcomes. In the Discussion section, a review of the literature on MEDLINE (PubMed) and Web of Science from the past 50 years was conducted. Results: The three cases all underwent preoperative ultrasonography, and two of them also underwent preoperative MRI examination. Complete resection of the lesions was performed in all three cases, followed by postoperative pathological examination. The histopathology of these three cases revealed invasive angiomyxoma, as confirmed by immunohistochemical staining, which demonstrated positive expression of desmin, vimentin, estrogen, and progesterone receptors. The patients experienced a smooth postoperative recovery. Ultrasound had a diagnostic accuracy rate of 100% (3/3) for locating and determining the extent of the lesions; however, its specific diagnostic accuracy rate for identifying the pathological type was only 33% (1/3). In contrast, MRI had a diagnostic accuracy rate of 100% (2/2) for locating and determining the extent of lesions but did not show any specific diagnostic accuracy for identifying the pathological types. Conclusions: Our findings indicate that even if a vulvovaginal lesion presents with a superficial location, small size, limited scope, and regular shape, suspicion of atypical AAM should arise when palpation reveals toughness, tensility, and deformability under pressure. US reveals a well-defined hypoechoic to anechoic mass with uniformly distributed coarse dot echoes, with or without detectable intratumoral blood flow signal. MRI shows prolonged T1 and T2 signals with inhomogeneous enhancement and evident diffusion restriction on diffusion-weighted imaging (DWI).

Corrigendum: Misleading clinical and imaging features in atypical aggressive angiomyxoma of the female vulvovaginal or perianal region: report of three cases and review of the literature.

[This corrects the article DOI: 10.3389/fonc.2024.1373607.].

Superficial Angiomyxoma in an Uncommon Area: A Case Report.

Superficial angiomyxomas, also known as cutaneous myxomas, are rare, benign soft tissue tumors that present as papulonodular or polypoid, asymptomatic, slow-growing lesions. They typically occur in the head, neck, trunk, and extremities of adults and may be isolated tumors or part of the Carney Complex. We present a case of SA with an uncommon area of presentation and a brief discussion of the importance of ruling out the presence of systemic syndromes such as the Carney Complex.

Long-term disease stability with bicalutamide in a man with aggressive angiomyxoma: case report and state of art.

Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm, which is commonly diagnosed in females and located in the perineal and pelvic region. Tissue specimens of AA patients often show positivity for estrogen (ER) and progesterone receptors (PgR), while some cases of androgen receptor (AR) positivity have been reported in males. When feasible, surgical excision represent the most effective treatment of AA; however, when experiencing advanced or recurrent disease, local disease control could be achieved with systemic hormonal treatment. To date, evidence regarding AA management in male patients is scarce, and only a few cases have been reported in literature. Hereby, we describe the case of a 59-year-old-man suffering from perineal AA with positivity for androgen receptors (AR) showing a long-lasting disease stability during the treatment with an AR-blocking drug (bicalutamide). A literature review regarding the state of art of AA management with a particular look to male patients is also provided.

Angiomixoma agresivo pélvico: reporte de caso y revisión / Aggressive pelvic angiomyxoma: case report and revision / Angiomixoma agressivo: relato de caso e revisão

El angiomixoma agresivo pélvico es un tumor de partes blandas extremadamente raro, del cual se han reportado alrededor de 350 casos hasta la fecha. A continuación se reporta el caso clínico de una paciente que presentó dicho tumor y se describe el proceso diagnóstico y su tratamiento quirúrgico. Por otro lado, se realiza una breve revisión de la literatura disponible hasta el momento sobre el tema.

Aggressive pelvic angiomyxoma is an extremely rare soft tissue tumor, of which around 350 cases have been reported to date. This article aims at reporting the case of a patient presenting said tumor, as well as describing its diagnostic approach and its surgical treatment. On the other hand, it aims at briefly reviewing the available literature on the subject.

O angiomixoma pélvico agressivo é um tumor extremamente raro dos tecidos moles, tendo sido descritos até à data cerca de 350 casos. Relatamos o caso de uma doente que apresentava este tumor e descrevemos o processo de diagnóstico e tratamento cirúrgico. É também feita uma breve revisão da literatura disponível até à data sobre o assunto.

Angiomixoma pélvico agresivo. Reporte de un caso / Aggressive pelvic angiomyxoma. A case report

El Angiomixoma Agresivo (AA) del suelo pélvico es una rara neoplasia mesenquimal de histología benigna, pero con un comportamiento característico localmente agresivo, que frecuentemente se presenta en mujeres en edad reproductiva. Suele presentar un crecimiento insidioso desde su origen en la musculatura perineal, lo que conlleva una clínica inespecífica, sutil y generalmente de larga evolución. El diagnóstico por la imagen, principalmente a expensas de la Tomografía Computarizada (TC) y la Resonancia Magnética (RM) con contraste, es de notable importancia, tanto por permitir detectar y caracterizar fiablemente una entidad poco frecuente, como por facilitar una planificación quirúrgica adecuada que permita obtener márgenes de resección libres de enfermedad, incluso en aquellos con infiltración de las estructuras vecinas. Presentamos el caso de una paciente adolescente con antecedente de tumoración en región vulvar derecha, en relación con extensión de un gran tumor retroperitoneal cuyo estudio histológico confirmó un Angiomixoma Agresivo.

Aggressive angiomyxoma of the pelvic floor is a rare mesenchymal neoplasm of benign histology, but with a characteristic locally aggressive behavior, which mostly occurs in women of reproductive age. It usually presents an insidious growth from its origin in the perineal musculature, which leads to a non-specific, subtle and generally long-lasting clinical course. Diagnostic imaging, mainly at the expense of CT and MRI with contrast, is of notable importance, both for allowing detection and reliable characterization of a rare entity, and for facilitating adequate surgical planning to obtain disease-free resection margins, even in those with infiltration of neighboring structures. We present the case of an adolescent patient with a history of tumor in the right vulvar region, which imaging studies confirmed to be the extension of a large retroperitoneal tumor whose pathological anatomy describes as an aggressive pelvic an angiomyxoma.