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.     

Aggressive angiomyxoma of the pelvis and perineum: a case report and review of the literature

Aggressive angiomyxoma is a rare mesenchymal tumour arising from soft tissue of the pelvis and perineum. Other features of this tumour are non-specific clinical signs, local invasion, a high local recurrence rate, and recurrence long after initial excision. We provide a case report with a review of the literature. A 57-year-old woman with a large bulky mass in the perineum was admitted to our institution. She had previously undergone iterative surgery for excision of pelvic and perineal masses. The preoperative diagnostic procedure is described as well as the therapeutic surgical approach (a double simultaneous laparotomic and perineal approach was opted for). The surgical procedure was difficult, time-consuming and dangerous owing to an extensive area of hard retroperitoneal sclerosis involving the low urinary viscera, the vaginal stump and the mesorectum. Radical excision of the mass favoured the re-establishment of the normal pelvic anatomy, resolution of the preoperative symptoms and complete recovery of working capability. Surgical excision is the gold standard in the treatment of aggressive angiomyxoma. This benign neoplasm may sometimes present a malignant course owing to involvement of pelvic viscera. It is also associated with a high late recurrence rate due to local aggressiveness, and longterm follow-up is therefore necessary.     

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     

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.     

Aggressive angiomyxoma of the pelvis surgical management in a case with delayed diagnosis

Introduction and importanceAggressive angiomyxoma is characterized as a non-capsulated soft mass with the ability to progress to surrounding tissues but without metastasis to distant tissues. Slowing tumor extension leading delayed tumor diagnosis, expression of different types of hormonal receptors, therapeutic ineffectiveness of noninvasive treatment approaches and misdiagnosis have remained as the major challenges for managing this tumor.Case presentationHerein, we described a case of aggressive angiomyxoma located in the posterior of the uterus and vagina that as successfully managed surgically to remove tumor mass followed by gonadotropin-releasing hormone (GnRH) agonist to prevent tumor recurrence.Clinical discussionSurgical resection is the treatment of choice in aggressive angiomyxoma with complete success rate, however despite such successfulness, about two-thirds of patients experienced postoperative recurrence rate that could be prevented by hormone-based therapy especially GnRH agonist.ConclusionAggressive angiomyxoma is a rare tumor with locally invasive behavior. As misdiagnosis is common imaging like MRI with DWI should be considered. The best treatment is surgical resection by experienced surgeons in tertiary referral hospitals. Even with complete resection, the recurrence rate is high. So adjuvant medical treatment seems to be necessary.     

Local recurrent aggressive angiomyxoma presenting as a para urethral tumor 64 months after the first resection: a case report

We report a case of local recurrent aggressive angiomyxoma presenting as a para-urethral tumor. A 28-year-old woman visited our hospital with a complaint of a painless vulval mass. Magnetic resonance imaging (MRI) of the pelvis showed the para-urethral tumor to be 2.5 x 3.0 cm. The tumor was resected, and diagnosed histopathologically as aggressive angiomyxoma. The patient showed a painless vulval mass again at 64 months after the first resection. MRI of the pelvis showed the paraurethral tumor to be 2.5 x 3.0 cm. The tumor was resected, and diagnosed histopathologically as aggressive angiomyxoma. The patient showed no recurrence at 4 months after the second resection.     

Case of local recurrence of the periurethral proximal-type epithelioid sarcoma successfully treated by repeated regional excision

Proximal-type epithelioid sarcomas are rare soft tissue neoplasms occuring in the soma or thigh and often repeat recurrence and metastasis. We present a case of locally recurrenced proximal-type epithelioid sarcoma that could be treated by regional excision alone. A 62-year-old man visited our institute for a growing mass in the perineal region. Computed tomography (CT) showed a periurethral tumor 22 x 13 mm in diameter in the perineal region. The tumor was excised regionally, and the pathological examination with immunohistochemical staining revealed that the tumor was proximal-type epithelioid sarcoma. Local recurrence of the tumor occurred 2 years 7 months later without any metastatic lesion, and regional excision was performed again. Pathological diagnosis was proximal type epithelioid sarcoma and it was identical to the primary tumor. The patient is free of the disease 1 year after the second surgery of the tumor.     

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.     

Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma.

Aggressive angiomyxoma of pelvic soft parts is a rare lesion with a high risk of recurrence. We report 10 cases of angiomyofibroblastoma, a hitherto uncharacterized benign tumor of the vulva histologically mimicking aggressive angiomyxoma. All patients had a vulval mass, often clinically diagnosed as a Bartholin's cyst. There was no recurrence after excision. The tumors were well circumscribed, measuring 0.5-12 cm in maximum dimension. They were characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels (predominantly of the capillary type) were irregularly distributed. Spindled, plump spindled, and oval stromal cells were aggregated around the blood vessels, sometimes forming solid compact foci, or were loosely dispersed in the hypocellular areas. Their nuclei were bland, but rare ones were enlarged and hyperchromatic in four cases. Some cells had abundant eosinophilic hyaline cytoplasm and eccentrically placed nuclei. Mitotic figures were absent or very sparse. Scattered throughout were thin, wavy strands or thick bundles of collagen. Mast cells were readily seen in eight cases. Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not cytokeratin, muscle-specific actin, alpha-smooth muscle actin, or S-100 protein. Ultrastructural studies showed well-developed rough endoplasmic reticulum, Golgi apparatus, abundant intermediate filaments, and pinocytotic vesicles in the stromal cells. Angiomyofibroblastoma can be distinguished from aggressive angiomyxoma by its circumscribed borders, much higher cellularity, more numerous blood vessels (which lack prominent hyalinization), frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation.     

Aggressive angiomyxoma in the scrotum: a case report

A case of aggressive angiomyxoma occurring in the scrotum is reported. A 57-year-old Japanese man presented with a 2-year history of non-tender soft mass in the scrotum. Computed tomography and magnetic resonance imaging revealed a well-defined mass 8 cm in diameter extending from the medial aspect of the left ischium. Angiography revealed faint vascularity fed by the left internal pudendal artery. Excision was performed by a scrotal approach, and histological examination confirmed an aggressive angiomyxoma. The patient showed no recurrence at 3 months follow-up.     

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.     

精索旁侵袭性血管黏液瘤的临床特征(附2例报告及复习文献)

目的探讨精索旁侵袭性血管黏液瘤的临床特征。方法报告2例精索旁侵袭性血管黏液瘤患者的临床资料,结合文献复习进行讨论。结果2例患者的精索旁侵袭性血管黏液瘤均手术完整切除,经随访6~8个月,无肿瘤复发。病理诊断为精索旁侵袭性血管黏液瘤。结论侵袭性血管黏液瘤临床罕见,在男性好发于精索旁,确诊依赖病理学检查,治疗需手术完整切除肿瘤,术后应长期严密随访。     

Periurethral mass formations following bulking agent injection for the treatment of urinary incontinence

PURPOSE: Durasphere is gaining popularity as a bulking agent for treating women with stress urinary incontinence. We present a series of patients with periurethral mass formation following Durasphere injection. MATERIALS AND METHODS: The charts of 135 women with a mean age of 69.4 years (range 46 to 83) who underwent Durasphere periurethral injections were retrospectively reviewed. Patients who had a periurethral mass were identified and their clinical data were collected and analyzed. RESULTS: Four patients (2.9%) were diagnosed with periurethral mass formation 12 to 18 months (average 14.7) following a Durasphere injection. Clinical presentation varied, including irritative voiding symptoms, pelvic pain and urinary incontinence. All patients were found to have a tender and tense periurethral mass. A radiopaque mass was revealed during videourodynamic study in 1 patient. Incision, and transvaginal and endoscopic drainage or transvaginal excision were used to treat these masses. Intraoperative and pathological findings as well as operative outcomes are presented. CONCLUSIONS: Irritative or obstructing voiding symptoms, pelvic pain or a periurethral mass in patients with a history of Durasphere or other periurethral bulking agent injection should alert the physician to the possibility of periurethral mass formation. The true incidence of this late complication remains to be determined.     

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.     

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.     

Aggressive fibromatoses in orthopedics

Aggressive fibromatoses which may develop either in soft tissue or in the bone present considerable problems for the pathologist trying to establish a diagnosis as well as for the radiologist and surgeon. In radiographs, a destruction of the soft and osseous tissue is seen which suggests a malignant tumor. Histologically a monomorphic connective tissue prevails in the biopsy showing no essential signs of malignancy. Under pathoanatomical aspects often a benign proliferation of the connective tissue is assumed. Surgically the tumor may either be removed in a too radical and mutilating way, or the excision may remain incomplete. Two cases of desmoplastic bone fibroma (aggressive fibromatosis in the ulna and in the sacrum) are described in which the complete tumor removal led to healing, whereas the incomplete excision of the tumor resulted in recurrences. Aggressive fibromatosis represents a semimalignant tumor which has a locally destructive and invasive growth tendency but does not metastasize. The various fibromatoses are defined with regard to their biological growth tendency and the therapeutic consequences are discussed.     

腹壁韧带样瘤16例临床分析

目的 探讨腹壁韧带样瘤的临床特点和治疗方法.方法 回顾性分析1998年1月至2010年10月收治的16例腹壁韧带样瘤患者的临床资料,全组患者均经手术治疗.结果 本组16例患者均行广泛切除术,复发2例,复发率12.5%(2/16).结论 腹壁韧带样瘤呈侵袭性生长的特性,治疗应按低度恶性软组织肿瘤处理.治疗首选外科手术.     

Angiomyofibroblastoma of the Vulva: Report of a Case

A 45-year-old woman was first seen by us 2 years after becoming aware of a slightly painful subcutaneous mass in her left vulva. The mass was 7.5 × 3.0 cm in size, well circumscribed, mobile, and rubbery. It was at first clinically considered to be a benign tumor. Microscopically, the resected mass was composed of spindle or polygonal tumor cells which were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background. Immunohistochemically, myxoid tumor cells were positive for vimentin but not for α-smooth muscle actin, CD34, CD31, desmin, or S-100 protein. The tumor was diagnosed as an angiomyofibroblastoma (AMBF), based on the typical findings of histology and immunohistochemistry. There are many histological types of vulvar tumors, and establishing a preoperative diagnosis is difficult in many patients. Rapid intraoperative pathological diagnosis should be performed if possible, considering the possibility of diseases such as AMFB and aggressive angiomyxoma (AAM). When AAM is suspected, the peripheral tissues should also be resected to prevent recurrence. Received: May 22, 2000 / Accepted: November 20, 2000     

Surgical treatment of hemangiomas of soft tissue

Hemangioma is one of the most common soft tissue tumors comprising 7% of all benign tumors. The etiology is unclear. Many treatment modalities for the symptomatic deep subdermal or intramuscular hemangioma have been used, but surgical excision is the preferred treatment. During the past 20 years, 89 patients with soft tissue hemangiomas were treated by surgical excision at the authors' institution. This study was done to define the clinical characteristics of pathologically proven hemangiomas and to evaluate the outcome of the operative procedures. Intralesional or marginal excision for symptomatic hemangiomas yields satisfactory results for pain relief, functional recovery, and avoidance of recurrence. According to the data a hemangioma of the soft tissues is a benign lesion in which more aggressive surgery (wide or radical excision) or other modalities such as radiation usually are not warranted.     

Diagnosis and management of periurethral cysts

BACKGROUND/AIMS: Periurethral cysts are a rare entity that may be confused with urethral diverticula. The protocol for diagnosis and management of these lesions is still unclear. We present our experience with six patients presenting with periurethral cysts. METHODS: From 2001 to 2005 we evaluated six patients with a paraurethral mass. History, physical examination, laboratory and radiographic findings were analyzed to determine factors helpful in mass diagnosis. Cyst excision was performed via trans-vaginal approach in all patients and outcomes of this approach were assessed. RESULTS: Six female patients, average age of 29.7 years, presented with the complaint of a paraurethral mass. Transvaginal sonography was performed in two patients to confirm the presence of a periurethral cyst. Cystourethroscopy in all patients revealed no communication between the cyst and the urethra or presence of other lesions. Pathology revealed a benign cyst in all patients. No cyst recurrence has been seen in any patient. CONCLUSION: Most periurethral cysts can be diagnosed by physical examination. The diagnosis may be confirmed with transvaginal sonography. Cystourethroscopy should be performed to rule out other pathology, but may be done in the same setting as surgical excision. Complete surgical excision is effective and is associated with minimal risk of recurrence during short-term follow-up.