scholarly journals Rare Case of Aggressive Angiomyxoma of the Vulva: A Case Report

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. 1-4 ◽  
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.


2021 ◽  
Vol 11 (4) ◽  
pp. 100-102
Author(s):  
Jayakar Thomas

Aggressive Angiomyxoma is a very rare, distinctive, locally invasive soft tissue tumour, that occurs most commonly in the perineum, pelvis, vulva and vagina. It is seen mostly in women of reproductive age group. It has a tendency for recurrence locally, treatment being surgical excision. We hereby describe a case of 38-year-old patient with a pedunculated growth on the right labium majora.


Author(s):  
Shradha Prajapati ◽  
Meerabai V. ◽  
Jayalakshmi M. ◽  
Sredharan M.

AA is a rare locally invasive mesenchymal tumor predominantly presenting in women of reproductive age group, having a moderate to high risk for local relapse. Hence, it needs to be differentiated from another mesenchymal tumor occuring in this region. We present a case of a 50 year old female with a large, polypoidal, spongy mass on the right labia majora.


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.


2014 ◽  
Vol 244 (2) ◽  
pp. 187-194 ◽  
Author(s):  
Cassandra Y. Prpich ◽  
Alessandra C. Santamaria ◽  
James O. Simcock ◽  
Hoong Kien Wong ◽  
Judith S. Nimmo ◽  
...  

2015 ◽  
Vol 52 (4) ◽  
pp. 589
Author(s):  
H Malhotra ◽  
A Mathur ◽  
A Yadav ◽  
S Jasuja ◽  
A Jindal

2019 ◽  
Vol 6 (10) ◽  
pp. 3841
Author(s):  
Sreeramulu P. N. ◽  
Nipun A. C. ◽  
Anup . ◽  
Vikranth N. Suresh

Malignant soft tissue neoplasm exhibiting smooth muscle differentiation is called leiomyosarcoma (LMS). LMS arising from the smooth muscles of tunica media of the major blood vessels like inferior vena cava (IVC) is a rare type of neoplasm, accounting for less than 0.5% of adult soft tissue sarcoma, affecting <1/1,00,000 of all adult malignancies. The prognosis of this tumor is poor, as patients often present with intra or extra-luminal growth often with invasion of adjacent structures. Only 400 cases of IVC LMS have been reported. Here we report a case of 40 year old female with large asymptomatic mass per abdomen of 3 months duration occupying the central and right part of abdomen. Investigations revealed a large retroperitoneal mass of 20.4*22.8*22.5 cms arising from the right kidney, likely Renal cell carcinoma. On exploration a huge tumor of size 25*25*20 cms was seen arising from retroperitoneum on right side, right kidney its vessels and ureter could not be seen separately. IVC could not be delineated. Histopathology report revealed it to be LMS of vascular origin. We report one such case because although incidence of such cases is low but should be considered in the differential diagnosis of mass per abdomen which grows unusually fast.


Author(s):  
Siti Sarah Mohd Ramli ◽  
Asma Abdullah ◽  
Suria Hayati Md Pauzi ◽  
Masturah Ramli

Ceruminous adenoma is described as a glandular neoplasm of ceruminous glands. It is seen for less than one percent of all external ear tumours. Ceruminous adenoma cases were reported to have recurrence and residual tumour, however there was no malignant transformation known in ceruminous adenoma up to this date. Here, we report a young adult woman with two years history of progressive reduced hearing and tinnitus of the right ear. She was proven to have right moderate conductive hearing loss with pure tone audiometry. There was a cystic mass with serous content arising from the posterior wall of the right ear canal. We proceeded with wide local excision of the mass via trans-canal approach. Ceruminous adenoma was confirmed with histopathology and immunohistochemistry of CK7 staining. The hearing impairment was resolved completely post excision and there was no recurrence of the tumour on one year follow-up. We concluded wide local excision with appropriate margin of the mass is adequate to prevent recurrence in ceruminous adenoma cases.


2021 ◽  
Vol 14 (8) ◽  
pp. e240687
Author(s):  
Vivek Srivastava ◽  
Pratik K Jha ◽  
Awgesh Kumar Verma ◽  
Mumtaz Ahmad Ansari

Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed to the infiltrative nature and frequent local recurrences. They arise commonly from the vulvovaginal region, perineum or pelvis and are usually misdiagnosed as other common entities in these regions. Radiological investigations aid in the diagnosis and planning of surgery. However, the final diagnosis in most of the cases is established by histopathological examination. We herein report a case of a middle-aged woman presenting with recurrent large right vulvar mass highlighting the surgical challenges posed by its intrapelvic extension.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Wu-Yang Ji ◽  
Bin Luo ◽  
Xue-Wei Wang ◽  
Ying Xiao ◽  
Jin-Yi Tian

Abstract Background Extramammary Paget disease (EMPD) is an uncommon malignancy affecting apocrine gland–bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. The standard management strategy for EMPD without distant metastasis is a complete surgical removal, sometimes called wide local excision. However, there is no consensus regarding surgical margin width to decrease the high recurrence rate. Here we describe the first macroscopically visible satellite of axillary EMPD and wide local excision of both main lesion and the satellite lesion with only 0.5 cm margin, succeeded by a short review of the literature. Case presentation A 48-year-old female with a red macule in the right axilla was presented to our clinic. A well-demarcated 4 cm × 3 cm erythematous plaque was observed in the right axilla, and a similar lesion measuring 0.5 cm × 0.3 cm was found 3.5 cm away from the primary site. Breast and axillary node examination was unremarkable. Biopsy of the large plaque revealed Paget disease, then we performed a local extended excision of both lesions with a 0.5 cm margin, all margins negative indicated, by frozen pathology. Pathology revealed the nature of the satellite beside the main lesion also as Paget disease. The patient is currently followed-up for 20 months and has shown no signs of recurrence, with normal shoulder motor function. Conclusion We have report the first visible satellite of extramammary Paget disease, indicating the necessity of an extended local resection of both the main leision and the satellite lesion. Considering the anatomical structure of axillary Paget disease, a 0.5 cm negative surgical margin indicated by frozen pathology might be sufficient to sustain the shoulder motor function.


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