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.

Aggressive angiomyxoma of the vagina: Case report and review of the literature / Angiomixoma agresivo de vagina: reporte de caso y revisión de la literatura

RESUMEN Objetivos: Presentar el caso de una paciente con diagnóstico de angiomixoma agresivo de vagina, y hacer una revisión de la literatura del diagnóstico, tratamiento y pronóstico. Materiales y métodos: Mujer de 46 años de edad, quien consulta por disuria y sensación de masa en vagina. Tras la exploración física y los estudios imagenológicos se observa tumoración en vagina con extensión hacia cavidad pélvica, la cual es extraída por cirugía vaginal. La patología quirúrgica mostró angiomixoma agresivo de vagina. La lesión presentó recurrencia al tercer mes posoperatorio requiriendo una nueva cirugía por vía retrorrectal. Se realizó una búsqueda en las bases de datos Medline vía PubMed, LILACS, SciELO y Google Scholar, con los términos: "Angiomixoma", "Agresivo" y "Vagina",incluyendo artículos de revisión, reportes y series de caso en inglés y español publicados desde 1995. Se extrajo información sobre el diagnóstico referente a síntomas, signos, pruebas de inmunohistoquímica e imágenes utilizadas, el tipo de tratamiento -quirúrgico o de otro tipo- y el pronóstico. Se hace descripción narrativa de los hallazgos Resultados: Se identificaron 23 títulos, de los cua- les 14 reportes de caso, 2 series de casos clínicos y un artículo de revisión cumplieron los criterios de inclusión. El 65 % de las pacientes tenía entre 30 y 50 años. El diagnóstico se hizo con estudios de inmunohistoquímica en 8 de los casos y se utilizaron imágenes diagnósticas en 12 casos; la tomografía mostró mejor delimitación de las lesiones. El trata- miento quirúrgico se aplicó en todos los reportes y se complementó con terapia hormonal en 4 casos. Se hizo seguimiento a 14 de los 17 casos reportados. Conclusión: El angiomixoma agresivo de vagina es una entidad poco frecuente. Es necesario evaluar la utilidad de las diferentes pruebas de inmunohistoquímica en los casos identificados como angiomixoma agresivo por histopatología. Se requiere evaluación del tratamiento hormonal como coadyuvante de la cirugía. El pronóstico es bueno.

ABSTRACT Objectives: To present the case of a patient diagnosed with aggressive angiomyxoma of the vagina and to conduct a review of the diagnosis, treatment and prognosis of this disease condition. Materials and Methods: A 46-year old female patient complaining of dysuria and vaginal mass sensation. Physical exploration and imaging studies revealed a tumor extending into the pelvic cavity. The mass was resected through a vaginal approach and pathology of the surgical specimen showed an aggressive angiomyxoma of the vagina. New retrorectar surgery was performed three months after the initial resection because of recurrence. A search was conducted in the Medline via PubMed, Lilacs, Scielo and Google Scholar databases using the terms "Angiomyxoma," "Aggressive" and "Vagina." The search included review articles, case reports and case series published in English and Spanish since 1995. The information extracted included diagnosis, symptoms, signs, immunohistochemistry and imaging studies used, type of treatment surgical or other - and prognosis. Findings are described in narrative form. Results: Overall, 23 titles were identified, of which 14 case reports, 2 clinical case series and 1 review article met the inclusion criteria. Sixty-five per cent of the patients were between 30 and 50 years of age. Diagnosis was made by immunohistochemistry in 8 cases, and diagnostic imaging was used in 12 cases. Computed tomography showed sharper contours of the lesions. Surgical treatment was applied in all reports, supplemented by hormonal therapy in 4 cases. There was follow-up in 14 of the 17 cases reported. Conclusion: Aggressive angiomyxoma of the vagina is rare. Assessment of the role of various immunohistochemical tests is needed in cases identified as aggressive angiomyxoma on histopathology. Evaluation of hormonal treatment as an adjunct to surgery is required. Prognosis is good.

Aggressive angiomyxoma of the vulva - a rare entity: case report and review of literature

Aggressive angiomyxoma (AA) is an extremely rare locally invasive mesenchymal tumor with a high risk of recurrence. Till date, only about 350 cases reported worldwide. Because of the rarity it should be considered as differential diagnosis whenever patient present with vulvovaginal growth. The diagnosis is clinched on histopathology. These are hormone-dependent and have estrogen and progesterone receptors. Hence sometimes GnRH agonists are used for ovarian estrogen secretion suppression but long-term use is not advocated due to side effects. A 45-year-old P4 L4 perimenopausal female presented to the GOPD with a 4×4×3 cms pedunculated painless globular mass on right labia majora. On palpation, the globular mass was firm, non-tender and with a smooth surface. Mass was excised and on gross histopathology, cut sections showed white myxoid areas. On microscopy epidermal lined tissue with stellate and spindle-shaped mesenchymal cells was found, embedded in a loose myxoid stroma with few collagen fibers. The cells were small and bland and lacked nuclear atypia. Small to medium-sized blood vessels were present with the thickened wall. Entrapped nerves and adipocytes were also present. No necrosis or mitosis was identified. All these features were suggestive of an aggressive angiomyxoma. Immunohistochemistry markers ER, PR, CD34, desmin, SMA were all positive. Imaging was done to rule out metastatic lesions and wide local excision was done around the stump with laparoscopic bilateral oophorectomy. Aggressive angiomyxoma is a rare disease. In women with asymptomatic growth in the vulvovaginal region, perineum or pelvis, aggressive angiomyxoma should be considered as a differential diagnosis. Ideal treatment is a wide local excision to prevent local recurrences, which are common and a hypoestrogenic milieu is created by either GnRH Agonists or by bilateral oophorectomy due to their hormone-sensitive nature.

Superficial Angiomyxoma of the Eyelid. A Case Report and Review of the Literature

@#Superficial angiomyxoma (SA) is an angiomyxoid cutaneous tumour. It has predilection for the head and neck, torso, limbs and genital tract. Our case is a 27-year-old female, presented with painless right medial canthal mass for two years. It was associated with tearing when the lesion grew larger. We received a nodular brownish tissue measuring 25x20x15mm with homogenous brownish cut surface. Microscopically, the tumour was partially circumscribed, exhibiting bland stellate to spindle cells of moderate cellularity with pale eosinophilic cytoplasm, indistinct border, in a loose collagenous myxoid matrix with numerous blood vessels of different calibre. The lesional cells were present at the resected margin and were nonreactive towards CD34, SMA and S100. SA of the eyelid is sometimes mistaken clinically as dermoid cyst or lipoma. Reports have shown increased risks of local recurrence following incomplete excision. Close association with Carney’s complex is an important feature. Careful clinicopathologic correlation and proper investigations are needed for optimal patient care.

Aggressive angiomyxoma of broad ligament: an uncommon mesenchymal tumor

Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelviperineal region. Authors present a case of a 70 years female presented with history of distention of lower abdomen with recurrent pain since 6 months. On clinical examination there was suspicion of fibroid. On USG it was reported as huge fleshy, soft tissue mass measuring 20x10x9 cm in lower abdominal flanks and pelvis with vascular pedicle attached to right parametrium. Pan-hysterectomy was performed. Right broad ligament showed mass measuring 18.5x10x6 cm. On histopathological examination it was diagnosed as a case of Aggressive angiomyxoma of broad ligament. We are presenting this case for its extreme rarity, clinicohisto-pathological and radiological findings.

Angiomixoma pélvico asociado a endometriosis infiltrativa profunda: reporte de un caso / Pelvic angiomyxoma associated to deep infiltrative endometriosis: case report

El angiomixoma de la pelvis es un tumor mesenquimal inusual; que dado su alto potencial de recidiva es relevante que la exéresis quirúrgica sea lo más radical posible. Por su parte, la endometriosis infiltrativa profunda, es otra entidad poco frecuente que requiere de tratamientos complejos. La co-existencia de ambas patologías representa una situación absolutamente infrecuente; de los que hay muy pocos casos reportado en la literatura occidental. Se presenta el caso de una mujer de 41 años de edad que desarrolló ambas entidades nosológicas de forma concomitante y que fue tratada quirúrgicamente con buenos resultados.

Angiomyxoma of the pelvis is an unusual mesenchymal tumor; that given its high potential for recurrence, it is relevant that the surgical resection be as radical as possible. For its part, to deep infiltrative endometriosis is another rare entity that requires complex treatments. The co-existence of both pathologies represents an absolutely infrequent situation; of which there are very few cases reported in western literature. We present the case of a 41-year-old woman who developed both clinical entities concomitantly and who was treated surgically with good results.

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

Angiomiofibroblastoma-like, un tumor excepcional: presentación de un caso clínico y diagnóstico diferencial / Angiomyofibroblastoma-like, an exceptional tumor: presentation of a clinical case and differential diagnosis

Presentamos un caso de Angiomiofibroblastoma-like (AML), en el que la ecografía fue importante para determinar la detección, localización y extensión local. Hallazgos: La ecografía demostró una lesión paratesticular sólida hipoecogénica de bordes bien definidos lo que sugirió lesión benigna extratesticular. La histología evidenció una neoplasia mesenquimal benigna tipo mixoide en cuyo diagnóstico diferencial se incluyen el Angiomixoma superficial (AMS), Angiomixoma agresivo (AMA), Angiomiofibroblastoma (AMF) y el AML. En base a estos hallazgos se realizó una revisión de tumores de similares características en la literatura, llegando finalmente al diagnóstico de AML cuya localización paratesticular sólo ha sido descrita en muy pocos casos hasta la fecha. Conclusión: La ecografía es útil para localizar los tumores paratesticulares y determinar su extensión local. No obstante, para llegar al diagnóstico definitivo es necesario realizar un estudio histológico e inmunohistoquímico de la tumoración.

We present a case of a paratesticular Angiomiofibroblastoma-like (AML) tumor in which ultrasound was important to give the specific location and local extension of the lesion. Findings: Ultrasound revealed a hypoechoic paratesticular lesion with well-defined borders, suggesting an extratesticular benign lesion. The histology showed a benign mesenchymal myxoid-type neoplasm. The differential diagnosis included superficial Angiomyxoma (AMS), Aggressive angiomyxoma (AMA), Angiomyofibroblastoma (AMF) and AML. Based on these findings, a review of similar tumors was carried out and ultimately led to the diagnosis of paratesticular AML. This location has been described only in a few cases in the literature. Conclusion: Ultrasound is useful to locate paratesticular tumors and determine their local extension. However, a definitive diagnosis still requires a histological and immunohistochemical study.

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.

Robotic extralevator excision of a retrorectal giant aggressive angiomyxoma

Aggressive angiomyxoma (AA) is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. For the complete excision of retrorectal tumors, with extension through the levator muscle into the ischioanal space, open anterior and posterior approaches are typically required. Herein, we report our experience with robotic excision of a giant presacral AA with extralevator extension into the ischioanal space and extraction via Pfannenstiel incision, which we found to be technically feasible, efficacious, and safe to perform. Mayo Clinic Institutional Review Board exemption status was obtained for this study.

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.

Alternative surgical approaches for aggressive angiomyxoma at different sites in the pelvic cavity

Aggressive angiomyxoma, a rare soft tissue benign neoplasm, predominantly occurs in the female pelvic peritoneum and perineum region during reproductive age. It is slow growing, locally infiltrative, and has a high risk of local recurrence and the neoplastic character of blood vessels. The standard treatment is surgery. We report three unusual aggressive angiomyxoma cases. The first case was a pedunculated mass of the left labium major; the second, a left perineal mass that infiltrated into the paravesical area via the obturator foramen; and the third, a big mass in the retroperitoneal cavity, found that growing aggressive angiomyxoma looked like lava expulsion in the pelvic area. After a thorough examination and full radiologic workup, we performed surgical excision in each patient via different approaches. Histopathologic findings were consistent with diagnosis of aggressive angiomyxoma. To date, no relapse has been observed.

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.

Recurrent aggressive angiomyxoma / Angiomixoma agressivo recidivante

INTRODUCTION: aggressive angiomyxoma is a highly aggressive, rare neoplasm of the mesenchymal tissue with a high recurrence rate. It represents an important differential diagnosis of pelvic tumors in women of reproductive age. This study aims to describe a case of aggressive angiomyxoma. CASE REPORT: woman, 37 years old, complained about a bulge on the right perianal region, and anal itching and burning, bleeding, tenesmus and incontinence. The proctologic examination confirmed the perianal bulge and extrinsic compression of the posterior wall of the rectum. Computed tomography (CT) of the pelvis showed a well-defined pelvic mass extending to the right rectal area. Exploratory laparotomy showed a mass of fibro elastic consistency adjacent to the pelvic organs and closely attached to the distal rectum, and performed a resection of the pelvic tumor afterward. Anatomopathological analysis revealed an aggressive angiomyxoma. Magnetic resonance imaging (MRI) of the pelvis showed signs of recurrence in the pelvic cavity on the right side of the rectum. A surgical procedure was performed to resect the lesion. After an asymptomatic period, the MRI showed solid growths located in the right ischiorectal fossa. A new surgical procedure identified only retention cysts in the pelvis and right ischiorectal fossa, only lysis of adhesions was performed. The patient is currently undergoing follow-up without disease recurrence. (AU)

INTRODUÇÃO: o angiomixoma agressivo é uma rara neoplasia do tecido mesenquimal de grande agressividade e alta taxa de recorrência. Representa um importante diagnóstico diferencial de tumorações pélvicas de mulheres em idade reprodutiva. Este estudo objetiva relatar um caso de angiomixoma agressivo. RELATO DE CASO: mulher, 37 anos, com queixa de abaulamento em região perianal direita, além de prurido e ardor anal, sangramento, tenesmo e incontinência anal. Exame proctológico confirmou o abaulamento perianal e compressão extrínseca da parede posterior do reto. Tomografia computadorizada (TC) de pelve evidenciou massa pélvica bem delimitada estendendo-se à região para-retal direita. Laparotomia exploradora demonstrou massa de consistência fibro-elástica adjacente aos órgãos pélvicos e intimamente aderida ao reto distal, sendo realizada ressecção do tumor pélvico. Anatomopatológico revelou angiomixoma agressivo. Ressonância nuclear magnética (RNM) de pelve demonstrou sinais de recidiva na escavação pélvica à direita do reto. Foi realizado procedimento cirúrgico para ressecção da lesão. Após período assintomática, RNM evidenciou processos expansivos sólidos localizados na fossa ísquio-retal direita. Novo procedimento cirúrgico identificou apenas cistos de retenção na pelve e fossa ísquio-retal direita sendo feita apenas lise de aderências. A paciente encontra-se em seguimento clínico sem recidiva da doença. (AU)

Aggressive angiomyxoma of the vulva in a patient with systemic lupus erythematosus.

Aggressive angiomyxoma is a rare, slow-growing mesenchymal neoplasm with a tendency to recur. It mainly involves the pelvis, vulva, perineum, vagina, and urinary bladder in adult women of reproductive age group. We describe a 26-year-old female with large swellings of both labia majora which was histologically diagnosed as aggressive angiomyxoma. She also had systemic lupus erythematosus. The swelling was surgically removed and she had no recurrence at 1-year follow-up. Although it is a rare tumor, it must be considered as a differential diagnosis for any mass in the perineum or soft tissue of the pelvis. Long-term follow-up is necessary for early diagnosis of local recurrence.

Novel Mutation in PRKAR1A in Carney Complex

A case of Carney complex in a Korean patient is presented. The patient had the characteristics of Carney complex including skin lesions, positive family history, and multiple myxomas including a superficial angiomyxoma in the perianal area. An extensive genetic analysis revealed a novel mutation in the protein kinase A type I-a regulatory subunit (PRKAR1A) gene, but not in the phosphodiesterase type 11A (PDE11A) gene. This is the first case wherein extensive genetic studies were performed in a patient with Carney complex in Korea.