Aggressive Angiomyxoma of the Pelvis: 35-Year Experience.

BACKGROUND: Aggressive angiomyxoma is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. Although many are estrogen and progesterone hormone receptor positive, the pathogenesis is unknown. Due to its rarity, there is a paucity of literature relating to this pathology. This article presents a case series on the management of aggressive angiomyxoma of the pelvis. OBJECTIVE: To present a 35-year experience managing aggressive angiomyxoma of the pelvis. DESIGN: This was a retrospective single-system analysis. SETTINGS: This study was conducted at a quaternary referral academic health care system. PATIENTS: All patients treated for aggressive angiomyxoma of the pelvis. INTERVENTIONS: All patients underwent surgical or medical management of their disease. MAIN OUTCOME MEASURES: The primary outcomes were disease recurrence and mortality. Secondary outcomes included risk factors for recurrence. RESULTS: A total of 32 patients (94% women) were identified with a median follow-up of 65 months. Thirty patients (94%) underwent operative resection and 2 patients were treated solely with medical management. Fifteen achieved an R0 resection (negative microscopic margins) at the index operation, of which 4 (27%) experienced tumor recurrence. There were no mortalities. No risk factors for disease recurrence were identified. LIMITATIONS: Limitations to our study include its nonrandomized retrospective nature, single health care system experience, and small patient sample size. CONCLUSIONS: Aggressive angiomyxoma is a rare, slow-growing tumor with locally invasive features and a high potential for recurrence even after resection with negative margins. Imaging modalities such as CT or MRI should be obtained to aid in diagnosis and surgical planning. Workup should be paired with preoperative biopsy and testing for hormone receptor status, which can increase diagnostic accuracy and guide medical treatment. Close posttreatment surveillance is imperative to detect recurrence. See Video Abstract . ANGIOMIXOMA AGRESIVO DE PELVIS EXPERIENCIA DE AOS: ANTECEDENTES:El angiomixoma agresivo es un tumor mesenquimal muy raro que se encuentra más comúnmente en las regiones pélvica y perineal. Aunque muchos son positivos para los receptores hormonales como el estrógeno y la progesterona, la patogénesis es aún desconocida. Debido a su rareza, existe escasa literatura relacionada con esta patología. Este artículo presenta una serie de casos sobre el tratamiento del angiomixoma agresivo de pelvis.OBJETIVO:Presentar una experiencia de 35 años en el manejo del angiomixoma agresivo de pelvis.DISEÑO:Este fue un análisis retrospectivo de sistema único.AJUSTES:Este estudio se llevó a cabo en un sistema de salud académico de referencia de nivel cuaternario.PACIENTES:Todos los pacientes tratados por angiomixoma agresivo de pelvis.INTERVENCIONES:Todos los pacientes se sometieron a tratamiento quirúrgico y/o médico de su enfermedad.PRINCIPALES MEDIDAS DE RESULTADO:Los resultados primarios fueron la recurrencia de la enfermedad y la mortalidad. Los resultados secundarios incluyeron factores de riesgo de recurrencia.RESULTADOS:Se identificaron un total de 32 pacientes (94% mujeres) con una mediana de seguimiento de 65 meses. Treinta (94%) fueron sometidos a resección quirúrgica y dos fueron tratados únicamente con tratamiento médico. Quince lograron una resección R0 (márgenes microscópicos negativos) en la operación inicial, de los cuales cuatro (27%) experimentaron recurrencia tumoral. No hubo mortalidades. No se identificaron factores de riesgo para la recurrencia de la enfermedad.LIMITACIONES:Las limitaciones de nuestro estudio incluyen su naturaleza retrospectiva no aleatoria, la experiencia de un solo sistema de atención médica y el tamaño pequeño de la muestra de pacientes.CONCLUSIONES:El angiomixoma agresivo es un tumor raro, de crecimiento lento, con características localmente invasivas y un alto potencial de recurrencia incluso después de una resección con márgenes negativos. Se deben obtener modalidades de imágenes como CT y/o MRI para la ayuda diagnóstica y la planificación quirúrgica. El estudio debe combinarse con una biopsia preoperatoria y pruebas del estado de los receptores hormonales, que pueden aumentar la precisión del diagnóstico y guiar el tratamiento médico. Es imperativa una estrecha vigilancia posterior al tratamiento para detectar recurrencia. (Traducción-Dr Osvaldo Gauto ).

Diagnosis and cardiac transplantation of a Carney syndrome-induced cardiac myxoma combined with dilated cardiomyopathy: a case report.

BACKGROUND: Carney syndrome is an uncommon autosomal disorder closely linked to mutations in the PRKAR1A gene. Skin lesions are the most pronounced feature of Carney syndrome, affecting over 80% of individuals with this condition. This syndrome is characterized by a triad of myxomas, skin pigmentation, and endocrine hyperfunction, featuring multiple endocrine neoplasms with skin and cardiac involvement. Dilated cardiomyopathy, a primary cardiomyopathy, is defined as the dilation and impaired systolic function of the left or both ventricles. Its clinical presentation varies from being asymptomatic to heart failure or sudden cardiac death, making it a leading global cause of heart failure. Currently, Dilated cardiomyopathy has an estimated prevalence of 1/2500-1/250 individuals, predominantly affecting those aged 30-40 years, with a male-to-female ratio of 3:1. This case report describes a heart failure patient with cardiac myxoma caused by Carney syndrome combined with dilated cardiomyopathy. The patient was successfully treated for heart failure by heart transplantation. CASE PRESENTATION: Herein, we report a case of heart failure due to Carney syndrome that resulted in cardiac myxoma combined with dilated cardiomyopathy. A 35-year-old male was admitted to the hospital three years ago because of sudden chest tightness and shortness of breath. Echocardiography indicated myxoma, and a combination of genetic screening and physical examination confirmed Carney syndrome with cardiac myxoma. Following symptomatic management, he was discharged. Surgical interventions were not considered at the time. However, the patient's chest tightness and shortness of breath symptoms worsened, and he returned to the hospital. A New York Heart Association grade IV heart function was confirmed, and echocardiography indicated the presence of dilated cardiomyopathy accompanied by cardiac myxoma. Ultimately, the patient's heart failure was successfully treated with heart transplantation. CONCLUSIONS: Cardiac myxoma caused by Carney syndrome combined with heart failure caused by dilated cardiomyopathy can be resolved by heart transplantation.

Cerebral Embolism and MINOCA Secondary to Left Atrial Myxoma after Occlusion of Atrial Septal Defect by Amplatzer Occluder: A Case Report.

Primary heart tumors are rare, with atrial myxomas being the most common type. Atrial myxomas can lead to embolisms, heart obstruction, and systemic symptoms. Herein, we report a case of 72-year-old woman who presented with a left atrial myxoma at the atrial septal defect occluder, a new acute cerebral infarction, and MINOCA (myocardial infarction with no obstructive coronary atherosclerosis). Left atrial myxoma is a common primary cardiac tumor; however, left atrial myxomas arising after percutaneous atrial septal defect occlusion are rare. Additionally, the patient presented with a new case of multiple systemic emboli. The patient underwent surgical resection of a left atrial myxoma, occluder, and left atrium, and atrial septal repair, and was discharged with good recovery for outpatient follow-up. The possibility of a cardiac tumor, especially an atrial myxoma, which can lead to a series of complications, should be considered at the closure site after percutaneous atrial septal closure. Therefore, active surgical treatment and long-term follow-up are warranted in such cases.

Incidental finding of atrial myxoma following curative treatment for paediatric neuroblastoma.

Cardiac tumours are uncommon in the general population and even more so in the paediatric population. Here we present a case of an asymptomatic 7-year-old male with history of high-risk neuroblastoma who underwent 1-year post-treatment surveillance scan with an incidental finding of intracardiac lesion found to be an atrial myxoma.

The Role of Methylation Analysis in Distinguishing Cellular Myxoma from Low-Grade Myxofibrosarcoma.

Cellular myxoma is a benign soft tissue tumor frequently associated with GNAS mutation that may morphologically resemble low-grade myxofibrosarcoma. This study aimed to identify the undescribed methylation profile of cellular myxoma and compare it to myxofibrosarcoma. We performed molecular analysis on twenty cellular myxomas and nine myxofibrosarcomas and analyzed the results using the methylation-based DKFZ sarcoma classifier. A total of 90% of the cellular myxomas had GNAS mutations (four loci had not been previously described). Copy number variations were found in all myxofibrosarcomas but in none of the cellular myxomas. In the classifier, none of the cellular myxomas reached the 0.9 threshold. Unsupervised t-SNE analysis demonstrated that cellular myxomas form their own clusters, distinct from myxofibrosarcomas. Our study shows the diagnostic potential and the limitations of molecular analysis in cases where morphology and immunohistochemistry are not sufficient to distinguish cellular myxoma from myxofibrosarcoma, particularly regarding GNAS wild-type tumors. The DKFZ sarcoma classifier only provided a valid prediction for one myxofibrosarcoma case; this limitation could be improved by training the tool with a more considerable number of cases. Additionally, the classifier should be introduced to a broader spectrum of mesenchymal neoplasms, including benign tumors like cellular myxoma, whose distinct methylation pattern we demonstrated.

Cardiac myxoma as a rare acute heart failure etiology in paediatrics: A case report.

Cardiac myxoma is extremely rare in children. However, if not treated immediately, it may cause varying symptoms until sudden death. A-9-years old male Javanese child was brought to the emergency department of Prof. Soekandar General Hospital, Mojokerto with progressive dyspnoea since one month which got worse in the left decubitus position. There was no significant past medical history. Physical examination revealed hypotension, mitral stenosis, tricuspid regurgitation, and pulmonary congestion. Transthoracic echocardiography revealed a round pedunculated 3x3.3 cm mass in the Left Atrium that swingingly moved to the Left Ventricle during diastole. This was diagnosed provisionally as Myxoma with a differential of thrombus. After stabilization, he was referred to a tertiary hospital for emergency excision. Histopathology confirmed the myxoma. There were no symptoms and activity limitations during the 6 months follow-up. To the best of our knowledge, this is the first paediatric cardiac myxoma with Acute Heart Failure symptoms reported in Indonesia. Echocardiography is imperative for diagnosing myxoma. Appropriate and timely management results in an excellent outcome.

Clinical characteristics, management, and outcomes of pulmonary valve myxoma: systematic review of published case reports.

BACKGROUND: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma. METHODS: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively. RESULTS: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively. CONCLUSIONS: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.

Diode laser on excision of giant peripheral odontogenic myxoma: a rare case report and literature review.

It was to report a rare case of peripheral odontogenic myxoma removed with high-power diode laser and to do an extensive review of studies of odontogenic cysts and tumors treated with high-power laser (HPL). This is a rare case of a 63-year-old male patient with a peripheral odontogenic myxoma measuring approximately 10 cm in the attached gingiva region of tooth 16 removed with a high-power diode laser (808 nm, 3 W, in continuous mode, under constant suction, with 400-µm optical fiber). A literature review was also carried out looking for articles that involved the use of HPL in the treatment of odontogenic cysts and tumors, without restriction of year or language. In the present case, there was no need for suturing, no postoperative discomfort, and minimal bleeding during the procedure. In a 12-month follow-up period, there were no signs of recurrence. Only two cases of intra-osseous odontogenic myxomas treated with HPL and 10 cases involving other odontogenic cysts and tumors were found. All studies showing HPL to be effective in treating these lesions. Despite the different types of lasers used and different parameters, it is observed that lasers are effective in the treatment of odontogenic lesions.

Clinical Characteristics of Acute Lower Extremity Ischemia Due to Left Atrial Myxoma: A Rare Case Report with Review of Literature.

Emboli caused by cardiac myxomas mostly occur in the cardiovascular or cerebrovascular systems and rarely in the lower extremity vasculature. We introduce the rare case of a patient with left atrial myxoma (LAM) whose right lower extremity (RLE) suffered from acute ischemia due to tumor fragments, along with a review of the relevant literature, and highlight the clinical characteristics of LAM. An 81-year-old female presented with acute ischemia of RLE. Color Doppler ultrasound showed no blood flow signal far from the RLE femoral artery. Computed tomography angiography showed an occlusion of the right common femoral artery. A transthoracic echocardiogram revealed a left atrial mass. Femoral artery embolectomy was performed under local anesthesia, followed by thoracotomy with tumor resection under general anesthesia on postoperative day seven. The tumor was pathologically confirmed as an atrial myxoma. A literature search of the PubMed database returned 58 cases of limb ischemia due to LAM, and the conclusions drawn from the statistical analysis were that emboli from LAM occurred most commonly in the aortoiliac and bilateral lower limb vasculature and were rarely associated with upper extremity and atrial fibrillation. Multisystem embolism is characteristic of cardiac myxoma. The removed embolus should be examined pathologically for signs of a cardiac myxoma. Lower-limb embolisms should be promptly diagnosed and treated to avoid osteofascial compartment syndrome.

Recurrent multi-cameral cardiac myxomas in a child with Carney complex.

Cardiac myxoma is a relatively rare tumour, usually solitary, that occurs primarily in the left atrium of adults, but comprises only 30% of cardiac tumours in children. We recently treated a 12-year-old girl with multiple recurrent myxomas in three cardiac chambers(following surgical resection 3 years earlier). Genomic analysis showed the PKAR1A mutation typical for Carney complex.

Characteristics and outcomes of patients with primary abdominopelvic aggressive angiomyxoma: a retrospective review of 12 consecutive cases from a sarcoma referral center.

BACKGROUND: Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor that mostly arises from the pelvic and perineal soft tissues. Few studies reported its characteristics and outcomes previously due to its rarity and challenges of treatments. This study aimed to investigate the clinical characteristics as well as surgical and short-term survival outcomes of primary abdominopelvic AAM. METHODS: Medical records of patients who were admitted to surgery with pathological confirmation of primary abdominopelvic AAM at Peking University International Hospital from January 2016 through December 2021 were retrospectively retrieved from our retroperitoneal tumor database. Demographics, operative outcomes and pathological findings were collected. Patients received followed-up routinely after the surgery. Survival probabilities were calculated and determined through Kaplan-Meier analysis. RESULTS: A total of 12 consecutive patients (male/female 4:8) were included in this study. The median age was 45 years old. The clinical presentation varied among individuals, consisting of 2 abdominal discomforts, 4 constipations, 1 lumbago, 1 prolonged menstruation, and 1 buttock swelling. R0/R1 resection was achieved in 100% of patients. Postoperatively, 50% of patients developed various complications including 3 fistulas and 3 wound infections. No operative mortality was observed. Histopathology of all patients was suggestive of AAM. Immunohistochemistry was done with a 91.7% positive rate for estrogen and progesterone receptors. The median recurrence-free survival time was 38 months. There were no cases of deceased or presented with distal metastasis during a median of 42 months' follow-up. CONCLUSIONS: The clinical manifestations of abdominopelvic AAM are mostly atypical. Surgical resection with curative intents remains the mainstay treatment of this disease, which was strongly suggested in experienced sarcoma centers due to the high probability of severe postoperative complications. In addition, long-term follow-up is necessary due to the high rate of local recurrences.

[Synchronous left atrial myxoma and gastric cancer: a case report]. / Sinkhronnye miksoma levogo predserdiya i rak zheludka. Klinicheskii sluchai.

The authors present a 57-year-old patient with synchronous left atrial myxoma and gastric cancer undergoing staged treatment. Distal gastrectomy with gastroduodenostomy at the first stage was followed by resection of the left atrial myxoma after 22 days. Postoperative period was uneventful after both interventions. The follow-up examination revealed favorable clinical status and no cancer progression.

Cardiac tumors-sex-related characteristics and outcomes after surgical resection.

OBJECTIVES: Cardiac tumors represent a rare and heterogeneous pathological entity, with a cumulative incidence of up to 0.02%. Gender was previously reported to influence outcomes after tumor surgery. This study aimed to investigate for gender-related differences in outcomes after cardiac surgery. METHODS: Between 2009 and 2021, 95 male and 88 female patients underwent surgery for tumor extirpation in our center. Preoperative baseline characteristics, intraoperative data, and long-term survival were analyzed. The diagnosis was confirmed postoperatively by (immune-)histopathological analysis. RESULTS: There were no significant differences in baseline characteristics and survival. Myxoma was the most common tumor type overall and was more diagnosed in women (n = 36 vs. n = 62, p ≤ 0.001). Sarcoma was the most common malignant tumor type (n = 5). Tumor location at the atrial septum was more likely in women (n = 26 vs. n = 16, p = 0.041), whereas ventricular localization was more common in male patients (n = 20 vs. n = 7, p = 0.001). Minimally invasive tumor extirpation was significantly more often performed in women, and in-hospital stay was shorter in female patients. CONCLUSION: The localization and dignity of cardiac tumors differ between genders, not affecting survival. Surgical tumor extirpation remains the gold standard of treatment for cardiac tumors in both genders as it is highly effective and associated with good long-term survivorship.

Prolapsing giant right atrial myxoma in a young male presenting as pulmonary embolism.

A 22-year male presented with complaints of dyspnea. Multimodality imaging revealed a polypoidal right atrial mass with submassive pulmonary embolism. The patient underwent urgent surgery. The pathological examination confirmed it as cardiac myxoma. Cardiac myxoma, a most common primary cardiac tumor, is commonly found in the left atrium. The right atrium is an uncommon site and the usual mode of presentation is the tumor or thrombus embolization to the pulmonary circulation.

Minimally Invasive Myxoma Resection: A Single-Center 5 Years' Experience.

BACKGROUND: There is an increasing demand for minimally invasive myxoma resection. This study aimed to investigate the safety and feasibility of minimally invasive myxoma resection. METHODS: In this retrospective study, we collected information from 95 patients who underwent myxoma resection between January 2016 and December 2020. Based on the operative approach, the patients were divided into the minimally invasive myxoma resection (Mini-MR) group (N = 30) and the sternotomy myxoma resection (SMR) group (N = 65). Intraoperative and postoperative data were compared between the two groups. RESULTS: The postoperative ventilator-assisted time, CSICU time, and postoperative hospital stay were shorter in the Mini-MR group than in the SMR (13.05 ± 4.98 vs. 17.07 ± 9.52 h; 1.73 ± 0.29 vs. 2.27 ± 1.53 d; 6.20 ± 1.50 vs. 9.48 ± 3.37 d, respectively), and the difference was statistically significant (P < 0.05). Mini-MR had lower postoperative drainage and blood transfusion rate in the first 24 h compared with SMR (38.93 ± 69.62 vs. 178.25 ± 153.06 ml; 26.6% vs. 63.1%), and the differences were statistically significant (P < 0.05). CONCLUSION: Mini-MR has the advantages of less CSICU stay time, less ventilator time, less postoperative drainage in the first 24h, less blood transfusion, fewer postoperative hospital stays, and faster recovery. Mini-MR is a safe and feasible surgical procedure for myxoma resection.

Right Ventricular Outflow Tract Myxoma with Absent Pulmonary Valve.

Myxoma is the most common benign cardiac tumor. Absent pulmonary valves, often treated surgically in childhood, are associated with 3-6% of tetralogy of Fallot. It is unusual for absent pulmonary valves without other congenital heart diseases to be asymptomatic until adulthood. Here, we report the unique case of an 80-year-old female with right ventricular outflow tract myxoma and absent pulmonary valve that has, to our knowledge, never been reported. We successfully performed a simple mass resection and pulmonary valve implantation.

Complete Thoracoscopic Resection of Left Ventricular Myxoma - A Case Report.

Cardiac myxoma is a common cardiac tumor. Most are found in the left cardiac system, of which 75% of myxomas are located in the left atrium [Pinede 2001], and the origin of the left ventricle is relatively rare. Surgical resection is the most effective method for the treatment of myxoma, but because of the complex anatomy of the left ventricle, most of the reported cases are performed through the traditional median thoracotomy through the ascending aorta and vena cava to establish cardiopulmonary bypass. It is rare to establish cardiopulmonary bypass through the femoral artery and femoral vein to remove left ventricular myxoma under complete video-assisted thoracoscopy. This paper reports the surgical process and perioperative echocardiographic, magnetic resonance, radiological and pathological features of a completely thoracoscopic resection of left ventricular myxoma.

Carney complex syndrome manifesting as cardioembolic stroke: a case report and review of the literature.

BACKGROUND: "Carney Complex (CNC) is a familial lentiginosis syndrome, caused by PRKAR1A mutations that lead to cyclic AMP-dependent protein kinase (PKA) signaling pathway abnormalities, predisposing to a variety of skin tumors, myxomas and endocrine tumors. METHODS/RESULTS: We describe a Greek family diagnosed with CNC after recurrent embolic strokes, secondary to left-sided atrial myxomas. There are limited cases in the literature describing this type of presentation for CNC; typically, most cases present with an endocrine syndrome. Our case serves as a reminder of this rare, underdiagnosed syndrome and its wide phenotypic spectrum. It is followed by a review of the current literature on cases with cerebrovascular disease as a manifestation of CNC. CONCLUSION: The co-occurrence of emboligenic cardiac myxomas and skin lesions should be an indication for screening for CNC.

Intramuscular Eyelid Myxoma.

Myxomas are a heterogeneous group of mesenchymal tumors. Soft tissue myxomas are divided into myocardial, intramuscular, juxta-articular, superficial, aggressive, and nerve sheath myxomas. Although benign, myxomas have site-specific biologic behavior and syndromic associations, which can influence prognosis and management. In addition, myxomas need to be distinguished from malignant neoplasms, such as myxofibrosarcomas, low-grade fibromyxoid sarcomas, myxoid liposarcomas, and peripheral nerve sheath tumors. While myxomas can occur throughout the body, these tumors arise more commonly in the thigh, shoulder, buttocks, and upper extremity, and less often in the head and neck. Rarely, myxomas can arise in a periocular location, typically in the conjunctiva and eyelid skin. In this case report, we present a patient with recurrent intramuscular myxoma of the eyelid and discuss the differential diagnosis and syndromic associations of this neoplasm. To our knowledge, there have been no prior reports of intramuscular myxoma of the eyelid and orbit.

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

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