scholarly journals Aggressive Angiomyxoma of the Vulva: A Bizarre Perineal Lesion

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Adamantia Zizi-Sermpetzoglou ◽  
Despoina Myoteri ◽  
Kalliroi Koulia ◽  
Vassilios Kontostolis ◽  
Hippokratis Moschouris ◽  
...  

Introduction. Aggressive angiomyxoma is a rare, slowly growing, and benign tumour of mesenchymal origin, which affects women of reproductive age and is associated with a high risk of local recurrence.Case Presentation. A case of a 47-year-old white female is presented herein, with a large polypoid, gelatinous mass on the right labia majora, measuring 26 × 21 × 6 cm. Histopathologically, the lesion was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. Another specific feature was the presence of variable-sized thin-walled capillaries and thick-walled vascular channels. The patient underwent wide local excision of the tumour with clear margins and developed local recurrence 18 months later.Discussion. Aggressive angiomyxoma of the vulva needs to be distinguished from benign myxoid tumors with a low risk of local recurrence as well as from malignant myxoid neoplasms. Usually wide local excision with tumour-free margins and occasionally hormonal manipulation is the treatment of choice.

2016 ◽  
Vol 6 (1) ◽  
pp. 8
Author(s):  
Gouthaman Shanmugasundaram ◽  
Sivasundari Maharajan

Aggressive Angiomyxoma (AA) is a rare locally aggressive soft tissue tumor with high chance of local recurrence occuring in young women of reproductive age group. These neoplasms are characterized by a mixture of spindle or stellate cells in a loosely myxoid stroma with prominent vascular component containing large, thickwalled blood vessels. A 35 year old multiparous lady presented with right vulval swelling which was rapidly progressing in size. Local examination revealed a soft fleshy mass occupying whole of the right labia majora. In view of the rapidly enlarging lesion over a short duration, we suspected a soft tissue neoplasm and preceded with wide local excision of the mass with adequate margins in all dimensions, eventhough we did not have a preoperative tissue diagnosis. To our surprise, final histopathology was Aggressive Angiomyxoma (AA). The treatment of choice for Aggressive Angiomyxoma is wide local excision with negative margins and the same was done in our case. Recurrence of the tumor may be avoided by wide local excision with adequate margins. It is possibly a hormonally responsive neoplasm. Treatment with Gonadotropin-releasing hormone is an emerging therapy.Overall prognosis of AA is good.


2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Byung Jun Kim ◽  
Hyeonwoo Kim ◽  
Ung Sik Jin ◽  
Kyung Won Minn ◽  
Hak Chang

Background. Dermatofibrosarcoma protuberans (DFSP), a rare low-grade sarcoma of fibroblast origin, tends to extend in a finger-like fashion beyond macroscopic tumor margins. Therefore, incomplete removal and subsequent recurrence are common. This study aimed to determine the efficacy of wide local excision (WLE) for controlling local recurrence of DFSP.Methods. The medical records of 90 DFSP patients who received WLE at our hospital between June 1992 and January 2015 were retrospectively reviewed. WLE was conducted including a 3 cm (range, 1 to 5 cm) safety margin according to tumor size, location, and recurrence status. Clinical and tumor characteristics and surgical methods were evaluated for risk factor analysis and local recurrence-free survival.Results. DFSP occurred most often in patients in their 30s (30%) and on the trunk (51.1%). Five patients (5.5%) experienced local recurrence during the 43.4-month follow-up period. Recurrence was found at a mean of 10.8 months after WLE. Although no factors were significantly associated with recurrence, recurrences were more frequent in head and neck. Recurrence-free survival was 87% in 6 years and 77% in 7 years.Conclusions. WLE with adequate lateral and deep margins can effectively control local recurrence rate and is a simple and effective method to treat DFSP.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Haider Alyousef ◽  
Elsawi M. Osman ◽  
Mohamed A. Gomha

Introduction. Liposarcoma is a rare pathological entity. By far it is the most common histological subtype of genitourinary sarcomas in adults. Approximately two hundred cases were reported in the literature. We are hereby presenting a case with a typical clinical scenario of paratesticular liposarcoma.Case report. A 75-year-old gentleman presented with a painless right hemiscrotal swelling that was progressively increasing in size over the last 6 years. Testicular tumour markers were negative. Imaging showed a heterogenous mass with fat component. Subsequently he underwent wide local excision that included the paratesticular mass along with the right testicle and all right inguinal canal contents up to the deep inguinal ring with the sparing of right illioinguinal nerve. Histopathological examination showed a well differentiated liposarcoma of the spermatic cord. He remained recurrence-free so far after 18 months of followup.Conclusion. Radical orchidectomy with wide local excision comprises the cornerstone of treatment of paratesticular liposarcoma. Due to the rarity of the disease there is no definite universal consensus of opinion as regards the role of radiotherapy and chemotherapy.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1984778
Author(s):  
Ann M John ◽  
Gina Francisco ◽  
Radhika Srivastava ◽  
Hamza Bhatti ◽  
Babar K Rao

Dermatofibrosarcoma protuberans is an uncommon low-grade malignant tumor that can invade locally and rarely metastasize. Dermatofibrosarcoma protuberans has a high rate of local recurrence due to incomplete excision, especially in deep tissues. Morbidity is often related to multiple local recurrences and removal of excessive tissue with large, complex repairs. We present a case of incompletely excised dermatofibrosarcoma protuberans after initial wide local excision. We subsequently employed a “deep” vertical Mohs micrographic surgical technique to remove the remaining tumor while creating a flap with the tumor-free superficial portion to preserve tissue and avoid a complicated repair. The patient is tumor-free for 7 years.


2010 ◽  
Vol 126 ◽  
pp. 17-18
Author(s):  
Vinay Rawlani ◽  
Donald W. Buck ◽  
Karl Y. Bilimoria ◽  
Jeffrey D. Wayne ◽  
John Y.S. Kim

Sign in / Sign up

Export Citation Format

Share Document