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UROLOGICAL IMAGES
Year : 2019  |  Volume : 35  |  Issue : 1  |  Page : 81-82
 

Prominent swelling on erection: Perineal angiomyxoma as a rare entity


Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission29-Jul-2018
Date of Acceptance13-Oct-2018
Date of Web Publication02-Jan-2019

Correspondence Address:
Aditya Prakash Sharma
Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/iju.IJU_221_18

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   Abstract 

Perineal angiomyxoma is a rare entity, more commonly seen in females. We report a case of a 44 year old male who presented with a perineal swelling which became prominent with penile erections. Magnetic Resonance Imaging (MRI) revealed a T1 hypointense and T2 hyperintense midline lesion (4.6 × 2.5 × 5 cm) in relation to corpus spongiosum, with ill defined fat planes with the bulbospongiosus muscle and progressive enhancement on dynamic contrast sequence. A differential diagnosis of soft tissue sarcoma or hemangioma was made and the mass was completely excised via a midline perineal incision. The histopathology revealed features consistent with angiomyxoma.



How to cite this article:
Tyagi S, Mavuduru RS, Bora GS, Devana SK, Sharma AP. Prominent swelling on erection: Perineal angiomyxoma as a rare entity. Indian J Urol 2019;35:81-2

How to cite this URL:
Tyagi S, Mavuduru RS, Bora GS, Devana SK, Sharma AP. Prominent swelling on erection: Perineal angiomyxoma as a rare entity. Indian J Urol [serial online] 2019 [cited 2019 Jul 17];35:81-2. Available from: http://www.indianjurol.com/text.asp?2019/35/1/81/249247



   Introduction Top


Perineal angiomyxoma is a rarity.[1],[2] They usually occur within the perineum, vulva and inguinal areas, more commonly in females (6:1). Here, we report a case of angiomyxoma in a male who presented with a perineal swelling which became prominent with penile erections.


   Case Report Top


A 44-year-old male presented with a painless swelling in the perineum which became more prominent with penile erections. On physical examination, there was a firm, nontender, nonpulsatile, irreducible, noncompressible mass in the anterior perineal area, on the left of the median raphe. Sonography revealed a well-defined lobulated lesion in relation to the root of the penis abutting corpus spongiosum and left corpus cavernosum without any obvious communication. Contrast-enhanced magnetic resonance imaging (CEMRI) revealed a T1 hypointense and T2 hyperintense midline lesion (4.6 cm × 2.5 cm × 5 cm), with ill-defined fat planes with bulbospongiosus muscle and progressive enhancement on dynamic contrast sequence, suggesting a suspicion of soft tissue sarcoma or hemangioma [Figure 1]. After discussion with the radiologist, a contrast-enhanced ultrasound was performed for a multiphasic and real-time delineation of the vascular nature of the mass and it did not reveal any feeding vessel and a possible diagnosis of benign nerve sheath tumor was also suspected besides soft tissue sarcoma.
Figure 1: Contrast-enhanced magnetic resonance imaging of the patient showing hyperintense lesion in the perineum on T2-weighted image (a) Axial; (b) Sagittal)

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The patient underwent excision of the perineal mass through a midline perineal incision. The mass was encapsulated lying deep to left bulbospongiosus muscle and was completely excised [Figure 2]. Gross specimen showed well-encapsulated soft spongy mass.[Figure 3]a Histopathology showed encapsulated tumor with spindle-shaped and stellate cells embedded in myxoid background suggestive of angiomyxoma [Figure 3]b. At 3-month follow-up, the patient is asymptomatic. First described by Steeper and Rosai,[1] perineal angiomyxomas are extremely rare with around 150 reported cases till date. Incidence in male to female is 1:6.[2] MRI is the current imaging of choice and the lesions appear hypointense on T1-weighted, hyperintense on T2-weighted sequences, and usually have a characteristic whorled appearance after contrast administration.[3] Aggressiveness of angiomyxoma is decided by the vascularity dispersed irregularly throughout the parenchyma and mitotic figures.[4] We report a rare case of angiomyxoma of the anterior perineal triangle in a male, presenting with the prominence of perineal swelling on penile erection.
Figure 2: Intra-operative photograph showing (a) Perineal mass and (b) its relation to bulbospongiosus muscle

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Figure 3: (a) Gross specimen showing well-encapsulated soft spongy mass. (b) Photomicrograph showing encapsulated tumor with spindle-shaped and stellate cells embedded in myxoid background. Tumor cells are elongated and have vesicular nuclei and moderate cytoplasm with intervening vessels consistent with angiomyxoma

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

 
   References Top

1.
Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7:463-75.  Back to cited text no. 1
    
2.
Micci F, Brandal P. Soft tissue tumors: Aggressive angiomyxoma. Atlas Genet Cytogenet Oncol Haematol 2007;11:340-3.  Back to cited text no. 2
    
3.
Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: Findings on CT and MR imaging. AJR Am J Roentgenol 1999;172:435-8.  Back to cited text no. 3
    
4.
van Roggen JF, van Unnik JA, Briaire-de Bruijn IH, Hogendoorn PC. Aggressive angiomyxoma: A clinicopathological and immunohistochemical study of 11 cases with long-term follow-up. Virchows Arch 2005;446:157-63.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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