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.

Aggressive Angiomyxoma of Vagina: A Rare Case Report

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 47 Year old female, with chief complaints of prolapsed uterus since 6 months. During clinical examination, posterior vaginal wall swelling was also identified. Total Abdominal Hysterectomy with Bilateral Salpingoophrectomy was done along with removal of posterior vaginal wall swelling. On histopathology, diagnosis of aggressive angiomyxoma was made. We report this case because of its rarity.

Angiomixoma agresivo: reporte de un caso y experiencia global con 168 casos / Aggressive angiomyxoma: Case report and global experience of 168 cases

Resumen ANTECEDENTES: el angiomixoma vulvar agresivo es una neoplasia poco frecuente, de origen mesenquimatoso, que suele afectar la región pélvica y perineal, con alto grado de infiltración y recurrencia local. El tratamiento de elección es la extirpación quirúrgica. CASO CLÍNICO: mujer de 39 años de edad, que inició con una tumoración vulvar derecha de 6 cm, de consistencia blanda, móvil, no dolorosa, sin ulceraciones, clasificada como quiste de la glándula de Bartholin; se efectuó resección quirúrgica de la lesión sin inconvenientes ni complicaciones. En el servicio de Cirugía Oncológica del Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, se solicitó una tomografía axial computada que evidenció una tumoración heterogénea, hipodensa, vascularizada, con componente quístico que desplazaba el conducto vaginal, útero y vejiga, con infiltración de tejidos blandos y piel, de 15 x 10 x 28 cm en el labio mayor derecho. Con la biopsia incisional se estableció el diagnóstico de angiomixoma agresivo profundo. En diciembre de 2016 se extirpó el tumor vulvoperineal. El reporte patológico fue de tumoración de 30 x 25 x 7 cm, con receptores positivos para estrógenos y progesterona, e intensidad +++ en más de 80%. La paciente recibió tratamiento coadyuvante con tamoxifeno; actualmente se encuentra asintomática y en vigilancia. CONCLUSIONES: la prescripción de agentes antiestrogénicos disminuye el tamaño tumoral. Este tratamiento supone abordajes quirúrgicos menos agresivos. Por la recurrencia del angiomixoma agresivo se sugiere la vigilancia a largo plazo e implementar un esquema de mantenimiento con fármacos antiestrogénicos.

Abstract BACKGROUND: Aggressive angiomyxoma of the vulva is a very rare mesenchymal neoplasm, which has a preference for the pelvic and perineal regions. It is defined to be aggressive because of its tendency to infiltrate and local recurrence. The treatment of choice is surgical excision. CLINICAL CASE: A 39-year-old female, who started with a 6 cm right vulvar tumor, soft, mobile, non-painful, without ulcerations, classified as a cyst of the Bartholin's gland; surgical resection of the lesion was performed without incidents or complications. In the Oncology Surgery service (Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán), a computerized axial tomography was requested, wich evidenced a heterogeneous, hypodense, vascularized tumor with cystic component that displaced the vaginal canal, uterus and bladder, with infiltration in soft tissue and skin, of 15 x 10 x 28 cm on the right upper lip. The incisional biopsy established of deep aggressive angiomyxoma diagnosis. In December 2016 the vulvoperineal tumor was extirpated. The Department of Pathological Anatomy reported a tumor of 30 x 25 x 7 cm, with estrogen and progesterone positive receptors, and intensity +++ in more than 80%. The patient received adjuvant treatment with tamoxifen. Currently the patient is asymptomatic and under follow-up. CONCLUSIONS: The use of antiestrogenic agents has been shown to decrease tumor size. This protocol requires less aggressive surgical approaches. Given the tendency of these tumors to recur, it is necessary to provide long-term follow-up considering the treatment with antiestrogens as a maintenance therapy.

Aggressive angiomyxoma of the vulva: A case report

@#Aggressive Angiomyxoma is a rare, slow growing, benign mesenchymal tumor arising from the pelvis and perineum which commonly affects women in the reproductive age group. Though benign, it is locally infiltrative with a marked tendency for local recurrence. We report a case of aggressive angiomyxoma of the vulva with translevator extension into the pelvic cavity in a 33 year old G2P2(2002). She presented with a left labial mass that recurred two years after excision was done. Debulking of the mass was carried out by abdominal and perineal approach. Histopathologic studies of the mass confirmed aggressive angiomyxoma. Long-term periodic follow-up with imaging studies was advised because of its high rate of recurrence in spite of negative tumor margins after wide excision

Perioperative nursing care to 8 patients with for pelvic cavity and genital tract aggressive angiomyxoma / 现代临床护理

Objective To summarize the experience in perioperatively nursing 8 patients with pelvic cavity and genital tract aggressive angiomyxoma (AAM). Method The perioperative nursing care was given to 8 AAM patients who were hospitalized in the department of obstetrics and gynecology from January 2010 to June 2015. Results The abdominal and perineal wounds were healed well without complications. All patients were recovered and discharged. One case had a relapse 2 years and another did 3 years after operation. Conclusion Such nursing measures as preoperative psychological nursing, close observation of the disease conditions, nursing cooperation during rescue for postoperative bleeding, treatment of postoperative complications and enhancing health education concerning the importance of long-term follow-up can be helpful for their recovery.

Aggressive Angiomyxoma of the Vulva: Libyan Female Case Report.

Aims and Background: Aggressive angiomyxoma is rare tumor of pelvic and perineal organs, occurring usually in women of reproductive age, and carrying a high tendency to local infiltration and relapse. Most literature about Aggressive angiomyxomaconsists of isolated case reports. Presentation of Case: We reported a case of 41-year-old Libyan woman complaining of a slow and progressive growth of a right vulvar labia major a pedunculated tumor with long a stalk was detected, measuring of 12.0x8.0 cm. Wide surgical resection of the tumor were performed. Histopathology diagnosed a large aggressive angiomyxoma with uninvolved resection margins. Patient remains without recurrence at 8.0-months followup. Discussion and Conclusion: Non Aggressive angiomyxomais rare but should be excluded in any large vulval mass. We expect that awareness accompanied with wide free safe margin excision has important role to prevent the recurrence of tumor.

Aggressive Angiomyxoma: an Unusual Presentation

Aggressive angiomyxoma is an uncommon mesenchymal myxoid tumor that is characterized by slow growth and frequent local recurrence. It is currently regarded as a nonmetastasizing tumor. We describe a case of recurrent aggressive angiomyxoma with invasion into the veins including the inferior vena cava and the right atrium and with pulmonary metastases. Our case, together with those unusual cases documented in previous reports, may lead to a reappraisal of the nature of aggressive angiomyxoma.

Angiomixoma agresivo en hombres: Reporte de caso clínico y revisión de la literatura / Aggressive angiomyxoma in men: Clinical case report and literature review

Se presenta el caso clínico de un angiomixoma agresivo, de presentación en un paciente de sexo masculino, con localización presacra, el cual fue diagnosticado e intervenido en el Hospital Militar Central de Bogotá. Se realizó un abordaje tipo Kraske con la escisión completa de la lesión y mínimo impacto funcional y estético para el paciente. Esta clase de tumores son extremadamente infrecuentes y predominan en mujeres, con una localización pélvica. La presentación en hombres es aún más exótica, con pocos casos reportados en la literatura. Su manejo obliga a la extirpación completa de la lesión y al seguimiento clínico e imagenológico periódico, dada su alta tasa de recidiva.

We report a case of a male patient with an aggressive angiomyxoma located in the presacral space which was diagnosed and clinically treated at the Hospital Militar Central in Bogotá, Colombia. Kraske’s posterior approach was used to completely excise the lesion with minimal functional and aesthetic impact on the patient. Aggressive angiomyxomas are extremely rare. They are most commonly found in pelvic locations in women. They are even more exotic in men, with very few cases reported in the literature. Management requires complete removal of the lesion and the clinical and imaging follow-up given their high rate of recurrence.

Retrospective of 3 cases of ureteral aggressive angiomyxoma / 国际外科学杂志

Objective To summarize the clinical and pathological features and the diagnosis and treatment of ureteral aggressive angiomyxoma (AAM),to deepen understanding in the disease.Method s Diagnosis and treatment of 3 cases of ureteral AAM from January 1991 to January 2011.Results Three cases of ureteral AAM were female,post-operative pathological diagnosis was made,no recurrence occurred during follow up.Conclusions Ureteral AAM is rare,occur frequently in women,with clinical symptoms hidden,not typical.Diagnosis is based on histopathological and immunohistochemical examinations.Attention should be paid to differential diagnosis from soft tissue and other mucinous tumors.Surgery is not easy and complete,with disease easy to relapse after surgery.

Angiomixoma agresivo en un hombre: reporte de un caso / Aggressive angiomyxoma in a man: a case report

El Angiomixoma agresivo es una neoplasia de diferenciación incierta descrita por primera vez por Steeper y Rosai (1983), que tiene predilección por la región pélvica y perineal de mujeres entre la 3ra y la 6ta década de la vida, afectando con menor frecuencia al sexo masculino, con casos descritos de localización escrotal. En Venezuela hasta la fecha, no hay casos informados de este tumor en pacientes masculinos. Presentamos un caso de localización pélvica, confirmado mediante estudios de inmuno-histoquímica en un varón de 27 años de edad con una recidiva luego de 3 años de la exéresis inicial.

Aggressive angiomyxoma is a neoplasm of uncerta in differentiation first described by Steeper and Rosai (1983), which has a predilection for the pelvic and perineal region of females between the 3rd and 6th decade of life, affecting males less frequently, with reported cases of scrotal location. In Venezuela, to date, no cases were reported in male patients. A case of pelvic location we presented, confirmed by immunohistochemistry studies in a male of 27 years-old with a relapse after three years of the initial excision.

Aggressive angiomyxoma of the vulva in an adolescent girl / 대한산부인과학회지

Aggressive angiomyxomas (AAMs) are rare, benign, but locally aggressive, hypocellular soft tissue neoplasms found in the vulvovaginal region. AAMs are most commonly found in women in their reproductive years. Only about 150 cases of this rare, soft tissue tumor have been reported thus far, most of which are reported from the gynecologic, obstetric, urologic, and pathologic fields. AAMs are considered to be a slow-growing mesenchymal tumor with a locally infiltrative growth pattern. There are only two reports of metastasic disease. We have managed a case of AAM which occurred during adolescence and was pathologically-confirmed.

Aggressive angiomyxoma of the vulva in an adolescent girl / 대한산부인과학회지

Aggressive angiomyxomas (AAMs) are rare, benign, but locally aggressive, hypocellular soft tissue neoplasms found in the vulvovaginal region. AAMs are most commonly found in women in their reproductive years. Only about 150 cases of this rare, soft tissue tumor have been reported thus far, most of which are reported from the gynecologic, obstetric, urologic, and pathologic fields. AAMs are considered to be a slow-growing mesenchymal tumor with a locally infiltrative growth pattern. There are only two reports of metastasic disease. We have managed a case of AAM which occurred during adolescence and was pathologically-confirmed.

A case of aggressive angiomyxoma of the vulva / 대한산부인과학회지

Aggressive angiomyxoma is an uncommon soft-tissue neoplasm which affects the pelvis and genital organs of the young female predominantly. This tumor has a high tendency of local recurrence, but it usually does not develop metastasis. Although surgical excision with wide tumor-free margins may be necessary to obtain a complete cure, some of patients experience local recurrences. Many of the tumors are estrogen receptor (ER) and progesterone receptor (PR) positive and medical therapy with gonadotropin-releasing hormone (GnRH) agonists showed to be effective in a few cases. We present a case of 29-years-old women who has aggressive angiomyxoma which was misdiagnosed as bartholin duct cyst. She was treated by wide local excision with postoperative adjuvant GnRH agonist therapy for 3 months. She is now free of disease during 9 months of follow up. We have described this case with the brief of literature.

A case of recurrent aggressive angiomyxoma of the vulva in the adolescence / 부인종양

Aggressive angiomyxoma is a rare, locally infiltrative soft tissue tumor that usually arises in the vulvoperitoneal region of young female. Frequent relapses are common. Clinicians should consider the diagnosis of aggressive angiomyxoma when a patient presents with an atypical vulvoperineal mass, because an incorrect diagnosis may lead to repeated surgical procedures. Treatment is wide surgical excision. Medical management with a GnRH agonist and radiation therapy with total dose of 60 Gy may be helpful adjuvant treatment in recurrent aggressive angiomyxoma. We experienced a case of recurrent aggressive angiomyxoma and report it with a brief review of literatures.

A Case of Recurrent Aggressive Angiomyxoma of the Vulva / 대한산부인과학회지

Aggressive angiomyxoma (AAM) is an extremely uncommon neoplasm of the female genitalia. This tumor arises from soft tissue of the perineum, and affects predominantly young women. Although surgical excision with wide tumor-free margins may be necessary to obtain a complete cure, some of patients experience local recurrences because of the infiltrative nature of this tumor. We experienced a case of recurrent huge AAM on the right vulva in 29-year-old woman and reported it with a brief review of literature.

Aggressive Angiomyxoma at Ischiorectal Fossa / 대한산부인과학회잡지

Aggressive angiomyxoma is a rare soft tissue tumor associated with high risk of local recurrence but lacks metastatic potential. This tumor arise from soft tissue of the perineum or lower pelvis, and affect predominantly yonug women. Aggressive angiomyxoma should be distinguished from more common benign and malignant tumor or tumor like conditions of the pelvic soft part. We report a case of an ususual angiomyxoma in ischiorectal fossa with a brief review of the literature.

Aggressive Angiomyxoma of the Vagina / 대한산부인과학회잡지

Aggressive angiomyxoma is a rare pathological condition which affects mainly the soft tissues of the pelvis and perineum in women. It is circumscribed totally or partially and has a infiltrative margin, so it has a tendency for local recurrence. We report a 33-year-old woman who presented with a mass arising within the vaginal wall which was painless, soft and movable like a cyst on palpation. The mass was surgically excised and pathologically proved to be an aggressive angiomyxoma.

Imaging Diagnosis of Aggressive Angiomyxoma / 实用放射学杂志

Objective To investigate the imaging features of aggressive angiomyxoma.Methods CT findings in 3 patients and MRI finding among one of them with pathologically proved aggressive angiomyxoma were retrospectively analyzed and the relevant literature were reviewed.Results In all the 3 cases,CT and MR imaging demonstrated a well defined mass arising from the pelvis,perineum or vulva.The tumours displaced but did not invade adjacent structures of the pelvic.In 2 cases,there was marked enhancement following injecting contrast materials or T_2WI with internal swirling pattern.Recurrent tumour in one case was of similar imaging features to the primary lesion.The small cystiform area could be seen inside the lesion in one case.Conclusion Aggressive angiomyxoma carries certain characteristics in CT and MRI manifestations.MRI is more excellent than CT in delineating the site,shape and the extent of these lesions.

Aggressive Angiomyxoma of the Scrotum: A case report

Aggressive angiomyxoma is a rare, locally aggressive tumor and occurs exclusively in the pelvic and perineal regions of women. But this tumor can occur in men, and the ratio of man to woman is about 1:6. We report a case of aggressive angiomyxoma of the scrotum in a 75-year-old man. The resected mass measured 23 21 10 cm and weighed 1,150 g, and was covered with thick but intact scrotal skin. The cut surface of the mass showed pale yellow to grayish white loose and edematous fibrous tissue with focal cystic degeneration. Microscopically, the mass was composed of loosely arranged bland-looking spindle or stellate cells and variable sized blood vessels with thickened and hyalinized wall. The spindle cells were strong positive for vimentin, focal, weak positive for desmin and smooth muscle actin but negative for S-100 protein. The spindle cells were also positive for estrogen and progesterone receptors.

A Case of Angiomyofibroblastoma of the Vulva / 대한산부인과학회잡지

Angiomyofibroblastoma is a recently described mesenchymal tumor characterized by unique morphologic features, a propensity to arise in the vulva, and a non-aggressive beni-gn biologic behavior. It is typically well circumscribed and superficially located tumor and its cellularity is variable with hypercellular and hypocellular areas composed of bland-looking, pump stromal cells showing the immunophenotype of vimentin-/desmin - positive, and muscle specific actin(MSA)-/alpha-smooth muscle actin(ASMA) - negative. Spindle or plasm-acytoid cells are the characteristic cell types of most tumors. It is similar to an aggressive pelvic angiomyxoma in histology. Because of histologic similarity and differences in the clinical course and treatment, distinction from aggressive angiomyxoma was needed. It rep-resents new type in the expanding spectrum of tumors of which myofibroblasts constitute an integral component. We experienced a case of angiomyofibroblastoma of the vulva occuring in a 42 years old woman. The lesion was relatively well encapsulated mass(3 X 1.5 X 0.9 cm in dimension), showing intimate admixture of hypercellular and hypocellular area. The tumor contained prominent, somewhat ectatic vessels surrounded by smooth muscle cells, some of which blend or fan out from the muscular walls of the vessels. The margin was relatively free from tumor cells. At immunohistochemical stain, vimentin and desmin was diffuse and weak positive, smooth muscle actin was very a few and weak positive in tumor cells.