A solitary encapsulated pelvic aggressive angiomyxoma

Aggressive angiomyxoma (AAM) was first reported in 1983 as a distinct, slow growing, benign but locally infiltrative, soft tissue tumour. It usually arises in the pelvic and perineal organs, mostly in women. A 47-year-old woman was found to have a large encapsulated retroperitoneal aggressive angiomyxoma. The mass was completely excised via abdomino-perineal approach, and no recurrence noted on MRI at 19 months' follow-up. The encapsulation of this tumour together with other reported rare presentations, suggest an isolated mesenchymal cell origin. A review of the literature is provided.     

Deep (aggressive) angiomyxoma of the vagina misdiagnosed as Gartner cyst: A case report

Introduction and importanceAggressive angiomyxoma is a rare soft tissue tumor. Aggressive angiomyxoma is a slow-growing vulvovaginal mesenchymal neoplasm with a marked tendency for local recurrence, but with a low tendency to metastasize. As it has a predilection for the pelvic and perineal regions, Aggressive angiomyxoma is often misdiagnosed.This case report documented rare case of misdiagnosed Aggressive Angiomyxoma as Gartner duct cyst.Presentation of caseThis article report a case of 31 year old women who complained mass came out from vagina without any urinary symptom and trauma. Physical examination and ultrasound finding suggested that the mass was Gartner Duct cyst. Management in this case was excision of the vaginal cyst. Histopathology examination revealed Deep (aggressive) angiomyxoma.DiscussionThe rarity of Deep (Aggressive) Angiomyxoma makes the preoperative diagnosis fairly difficult. Aggressive angiomyxoma is often misdiagnosed as it may have similar clinical presentation to common lesions such as Bartholin cyst or prolapse vaginal wall, Gartner cyst or levator hernia. Aggressive Angiomyxoma should be considered as differential diagnosis in patient with vaginal cyst.ConclusionAggressive Angiomyxoma is rare condition. Preoperative diagnosis and management are challenging. Long term follow op and evaluation should be done due to high rate of recurrence.     

Pedicle Myocutaneous Flaps for Reconstruction Following Total Pelvic Exenteration of Intrapelvic Recurrent Rectal Cancer: Report of a Case

A vast metastatic tumor mass of recurrent rectal carcinoma in the intrapelvic organs is commonly considered unsuitable for total pelvic exenteration (TPE); first, because it is unlikely that it would improve the prognosis and health-related quality of life of the patient, and second, because of the difficulties involved in this surgical technique. However, by using a plastic surgery technique involving reconstruction by filling the pelvic dead space with pedicle myocutaneous flaps (PMF), primary wound closure and extensive resection of the perineum can be achieved, whereby postoperative metastasis may be prevented. We report herein the case of a 71-year-old man found to have local recurrence in the perineum with extensive invasion of the soft tissue as well as adjacent organs, 20 months after abdominal perineal resection for rectal carcinoma. TPE with extensive resection of the perineal soft tissue was performed, followed by perineal reconstruction and packing of the pelvic dead space with PMF, mainly constructed from the gracilis and sartorius muscles of both femurs. His postoperative course was uneventful and he has remained free of local recurrence and symptomatic perineal complaints for 1 year. In this report, we examine the effectiveness of using the gracilis muscle for PMF in intrapelvic and perineal reconstruction after TPE. Received: March 8, 2000 / Accepted: September 26, 2000     

Aggressive angiomyxoma of the scrotum

Aggressive angiomyxoma is a rare and nonmetastasizing soft tissue tumor of the pelvis and perineum and occurs almost exclusively in adult females. It infiltrates locally and has a high risk of local recurrence. Recommended treatment of the symptomatic patient is wide excision with tumor-free margins and close postoperative monitoring. Herein, a case of aggressive angiomyxoma in an adult male is described, which arose in the scrotum over 12 months. The tumor showed an intermediate signal on T1-weighted MRI images. Contrast-enhanced T1-weighted images showed good enhancement. Wide excision of the tumor was performed. The surgical specimen measured 7 x 5 x 5 cm in size and weighed 80 g. The tumor's surface was smooth and had a gelatinous cut surface. Grossly, it was encapsulated with a pleura-like membrane and had a finger-like projection. Microscopically, sections showed many walled vessels of various sizes, collagen fibrils, a loose myxoid background. and spindle stroma cells. MRI and CT showed the angiomatous and myxomatous nature of the tumor wall. To the authors' knowledge, this is the first report to describe MRI findings in scrotal angiomyxoma.     

女性生殖道和盆腔侵袭性血管粘液瘤的诊治进展

侵袭性血管粘液瘤是一种罕见的来源于间叶组织的肿物,好发于生育年龄女性,侵袭性和复发性是其重要特点。常见部位为外阴、阴道和盆腔。因其罕见,极易误诊。病理是诊断的金标准。手术切除是一线的治疗方法,但包括激素治疗、血管栓塞等在内的辅助治疗方法逐渐受到重视。AAM虽极易复发,但远处转移罕见。     

低位性直肠癌保肛术后局部复发的原因分析

目的:探讨低位直肠癌保肛术后局部复发的原因及有效预防措施.方法:回顾性分析1996年9月至2006年12月期间,术后局部复发10例的临床资料.结果:吻合口肠腔內复发4例,盆腔复发4例,会阴部复发1例,腹壁切口复发1例.结论:预防术后局部复发的措施是遵循全直肠系膜切除(TME)的手术原则,正确合理地选择手术方式,注意术中无瘤操作以及术前辅助放疗化疗,可明显降低术后局部复发率.     

Pelvic leiomyoma presenting as perineal hernia

Primary perineal hernias are rare and can be a diagnostic challenge. We report the case of a 45-year-old female patient who presented with painless perineal swelling suggestive of perineal hernia. Computed tomography (CT) scanning revealed a pelvic mass herniating through the pelvic floor into the perineum. The lesion was completely excised by an abdominoperineal approach. Histopathological examination of the lesion revealed a leiomyoma. This case report suggests that the possibility of perineal herniation of a pelvic leiomyoma should be considered in a female patient with suspected primary perineal hernia. We recommend an abdominoperineal approach for the surgical management of such a lesion.     

Aggressive angiomyxoma in the scrotum expressing androgen and progesterone receptors

Aggressive angiomyxoma is a rare benign mesenchymal myxoid tumor that arises from the pelvic soft tissues and perineum in relatively young females. This tumor has the ability to infiltrate locally and has a high risk of local recurrence after extirpation, but no potential to metastasize. We report here a rare case of aggressive angiomyxoma that developed in the scrotum of a 47-year-old male. Immunostaining of the resected specimen revealed that the tumor cell nuclei stained strongly and diffusely for androgen receptors (80% of the tumor cells), and moderately and partly for progesterone receptors (20% of the tumor cells). However, staining was negative for estrogen receptors. It is highly suggested that the growth of aggressive angiomyxoma in males may depend on androgen manipulation, contrary to its frequent and close association with estrogen receptor expression, which has been reported in females.     

Angiomyofibroblastoma-like tumors (cellular angiofibroma)

Angiomyofibroblastoma-like tumor (cellular angiofibroma) is a rare, circumscribed, slow-growing mesenchymal tumor that occurs predominantly in the vulva, perineum, and pelvis of women. We report two cases of this tumor in men arising as paratesticular masses of the scrotum, summarize the history of this tumor, and discuss why efforts should be made to differentiate it from aggressive angiomyxoma. Recommended treatment is complete surgical excision with long-term follow up exams, as local recurrence may occur many years after resection of the lesion.     

Aggressive Angiomyxoma of Inguinoscrotal Region Mimicking Inguinal Hernia: a Case Report

Aggressive angiomyxoma (AAM) is a rare mesenchymal tumour of pelvis and perineum, almost exclusively occurring in females. We are reporting a case of a 53-year-old gentleman who presented with a long standing inguinoscrotal swelling misdiagnosed as inguinal hernia, for which he underwent incomplete excision at local hospital and then was referred to our centre for completion surgery.     

Extended abdominoperineal resection with gluteus maximus flap reconstruction of the pelvic floor for rectal cancer

BACKGROUND: Intraoperative tumour perforation, positive tumour margins, wound complications and local recurrence are frequent difficulties with conventional abdominoperineal resection (APR) for rectal cancer. An alternative technique is the extended posterior perineal approach with gluteus maximus flap reconstruction of the pelvic floor. The aim of this study was to report the technique and early experience of extended APR in a select cohort of patients. METHODS: The principles of operation are that the mesorectum is not dissected off the levator muscles, the perineal dissection is done in the prone position and the levator muscles are resected en bloc with the anus and lower rectum. The perineal defect is reconstructed with a gluteus maximus flap. Between 2001 and 2005, 28 patients with low rectal cancer were treated accordingly at the Karolinska Hospital. RESULTS: Two patients had ypT0 tumours, 20 ypT3 and six ypT4 tumours. Bowel perforation occurred in one, the circumferential resection margin (CRM) was positive in two, and four patients had local perineal wound complications. Two patients developed local recurrence after a median follow-up of 16 months. CONCLUSION: The extended posterior perineal approach with gluteus maximus flap reconstruction in APR has a low risk of bowel perforation, CRM involvement and local perineal wound complications. The rate of local recurrence may be lower than with conventional APR.     

Laparoscopic and perineal excision of an infected "dumb-bell" shaped retrorectal epidermoid cyst

INTRODUCTION: Extraperitoneal cysts in the pelvis adjacent to the rectum are rare entities. They are of vague etiology, and the pathologic types of the acquired variety are dermoid, epidermoid, and teratoma. As far as we know, laparoscopic excision of a retrorectal epidermoid cyst has never before been reported. CASE REPORT: The patient was an elderly lady with a large pelvic cyst and another large cyst in the perineum, which displaced the vagina and the anus. Laparoscopy was performed; the pelvic cyst was punctured and then dissected out. The perineal extension of the cyst was excised via a perineal approach. The patient had an uneventful recovery and was discharged on the 4th postoperative day. Histopathologically, the cyst was of the epidermoid type. Follow-up after 26 months revealed no recurrence. DISCUSSION: Our case presented a diagnostic dilemma. Laparoscopic excision was tedious, though we completed the procedure successfully. Precaution was taken to avoid spillage of the contents and also to avoid injury to vital structures nearby, such as the iliac vessels and ureter. It is feasible to laparoscopically excise retrorectal cysts with a careful and meticulous dissection. CONCLUSIONS: The major advantages are rapid recovery with minimal morbidity and excellent cosmesis.     

Liposarcoma of the perineum and scrotum

A case of liposarcoma of the perineum and scrotum, which presented as a huge haematoma and was left in place for three years prior to excision, is presented. The patient was treated by wide excision of the tumour, orchiopexy to the inguinal region and radiotherapy. To date, 30 months following surgery, the patient is completely asymptomatic and no tumour recurrence is evident either on physical and rectal examinations or on chest X-rays, ultrasonography and abdominal and pelvic computerized tomography.     

Angiomyxoma diagnosed in a man presenting for abdominal lipectomy

Aggressive angiomyxoma is a rare tumour that is locally infiltrative but nonmetastasizing. It occurs nearly exclusively in adult women of childbearing age and almost always arises in the perineum and pelvic area. A case of angiomyxoma occurring in a middle-aged, morbidly obese man is reported. The clinical presentation was one of progressing scrotal edema and enlargement with subsequent development of scrotal abscesses, requiring several incisions and drainages as well as oral and intravenous antibiotics. His symptoms and body habitus left him in a significantly debilitated state, prompting him to seek treatment for his conditions. He initially presented for abdominal lipectomy. However, due to the chronic scrotal infections and enlargement, scrotectomy was recommended before any other surgical procedures. A scrotectomy was performed by the urology service. Pathological diagnosis of the excised tumour was an aggressive angiomyxoma. Given the rarity of this tumour, especially in males, the proper diagnosis and treatment of angiomyxoma is still being investigated. With this in mind, the physical signs and symptoms that characterize this condition are presented and treatment options are reviewed.     

Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist

Patients with aggressive angiomyxoma may experience local recurrences. We report a case of recurrent aggressive angiomyxoma medically treated successfully with a gonadotropin-releasing hormone agonist. A 34-year-old woman with a huge perineal tumor underwent an extensive resection of the abdominoperineal tumor combined with total pelvic exenteration. Histology showed aggressive angiomyxoma and the tumor cells were immunoreactive for estrogen and progesterone receptors. Although the patient had experienced no local recurrence for 12 months under adjuvant therapy with a gonadotropin-releasing hormone agonist, a recurrence occurred 3 months after the completion of adjuvant therapy. The patient underwent medical treatment with a gonadotropin-releasing hormone agonist and had a complete resolution of the recurrent tumor again. Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors.     

Management of colovesical fistulae associated with pelvic malignancy.

Thirteen patients with malignant colovesical fistulae are presented. The underlying pathology was cancer of the colon (seven cases), bladder (four cases) and cervix (two cases). The series demonstrates that wide surgical excision may be needed to achieve tumour clearance and that this may necessitate pelvic exenteration. Three patients who underwent inadequate tumour excision developed recurrence in the bladder, two with a fatal outcome. Wide excision of the bladder may be performed without urinary diversion by subtotal cystectomy and a reconstructive procedure. Substitution cystoplasty was performed on two patients at the time of tumour excision. Urinary tract involvement by such tumours is often extensive and optimal results may be achieved with a multidisciplinary approach.     

Leiomyosarcomas of the rectum. Amputation of the rectum or local resection?]

Two cases of high grade leiomyosarcoma of the rectum treated by local excision are reported. The first patient presented a local recurrence associated with liver and pulmonary metastases and eventually died, respectively 11 and 22 months after resection. The second patient's tumor recurred 3 months after excision and was treated with pelvic exenteration and CYVADIC regimen as chemotherapy and died 3 years later from local recurrence. A review of the literature indicates that local surgical excision in comparison to a more radical surgical approach, such as abdominoperineal resection, is as effective in improving survival. Lesions less than 2.5 cm in diameter, confined to the bowel can be treated by local surgical excision. To date no adjuvant therapy is effective in terms of survival or local control.     

泌尿生殖系侵袭性血管黏液瘤(附二例报告及文献复习)

目的　探讨泌尿生殖系侵袭性血管黏液瘤的临床特点及治疗。　方法　分析 2例泌尿生殖系侵袭性血管黏液瘤的临床资料。例 1,男 ,5 5岁 ,表现为腹股沟区无痛性肿物 ,B超示低回声肿物 ,行肿物完整切除术。例 2 ,女 ,14岁 ,主要症状有尿频、血尿、排尿困难和尿道口肿物 ,IVU、CT及三维血管成像等明确肿瘤范围及血供 ,行膀胱大部分切除及输尿管再植术。结合文献复习讨论。　结果　 2例患者随访 2～ 6个月 ,未见肿瘤复发和转移。例 1病理诊断为精索旁侵袭性血管黏液瘤 ,免疫组化 :Vimentin( )、Desmin( - )、SMA( )、S 10 0 ( - )。例 2病理诊断为膀胱侵袭性血管黏液瘤 ,免疫组化 :CD3 4 ( ) ,Desmin( - ) ,Vimentin( )Caldesmon ( - ) ,ER( - ) ,PR ( - ) ,SMA( ) ,S 10 0 ( - )。　结论　侵袭性血管黏液瘤是一种罕见的、有局部侵袭特性的良性肿瘤 ,确诊依赖病理学及免疫组织化学检查。治疗需行肿瘤完整切除 ,对于切除困难及复发性肿瘤可选择放疗及内分泌治疗。长期随访和影像学检查对确定肿瘤复发和手术治疗非常重要     

Aggressive angiomyxoma of the vulva: intra-operative pathological diagnosis is useful in deciding the scope of surgery and reducing recurrence

Aggressive angiomyxoma (AA) is a rare, slow-growing myxoid neoplasm of the pelvi-perineum. Because of its rarity, it is often initially misdiagnosed, frequently as a gynaecological malignancy. Surgical resection is the main treatment modality of AA, but, local recurrence rates are high. In order to reduce recurrence, the scope of surgical resection should be as broad as possible. Intra-operative pathological diagnosis is useful in deciding the scope of surgery, and wide excision may reduce recurrence.     

A case of primary extragonadal seminoma arising in the perineum

We report the first case of primary extragonadal seminoma arising in the perineum. A 34-year-old-man presented with a 2-cm painless mass in the perineum. He underwent surgical resection, and histology showed poorly differentiated adenocarcinoma. Three months later, local recurrence occurred. Whole evaluation including ultrasound and MRI of the testes were normal, so he underwent surgical wide excision, and pathology showed the tumor was pure seminoma. He received two courses of bleomycin, etoposide and cisplatin (BEP) therapy, and there has been no evidence of recurrence for 1 month.