Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 5  |  Page : 458-460  

Aggressive angiomyxoma of scrotum in a young male: A rare entity


1 Department of Pathology, SVS Medical College and Hospital, Mahabubnagar, Yenugonda, Telangana, India
2 Department of Pathology, SVS Medical College and Hospital, Mahabubnagar, Yenugonda, , India, Telangana

Date of Submission14-Dec-2016
Date of Acceptance28-Feb-2017
Date of Web Publication14-Nov-2017

Correspondence Address:
Krishna Tadepalli
Department of Pathology, SVS Medical College and Hospital, Mahabubnagar, Yenugonda
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJDRDYPU.MJDRDYPU_297_16

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  Abstract 


Aggressive angiomyxoma (AAM) is a rare mesenchymal benign myxoid tumor of the pelvis and perineum which occurs almost exclusively in adult females. It rarely occurs in males and involves scrotum. Here, we report a case of aggressive angiomyxoma in a 15-year-old male presented with a slow-growing scrotal swelling for 6 months. About 150 cases were reported worldwide (few cases were reported in males). AAM in the scrotal region may present as a scrotal mass, often mistaken for a hernia or hydrocele.

Keywords: Aggressive angiomyxoma, rare entity, scrotal mass


How to cite this article:
Tadepalli K, Gattu V. Aggressive angiomyxoma of scrotum in a young male: A rare entity. Med J DY Patil Univ 2017;10:458-60

How to cite this URL:
Tadepalli K, Gattu V. Aggressive angiomyxoma of scrotum in a young male: A rare entity. Med J DY Patil Univ [serial online] 2017 [cited 2019 Sep 20];10:458-60. Available from: http://www.mjdrdypu.org/text.asp?2017/10/5/458/218189



TAG:2]Introduction[/TAG:2]

Aggressive angiomyxoma (AAM) was first described by Steeper and Rosai in 1983. AAM must be differentiated from other benign myxoid neoplasms given its propensity for local recurrence [1] AAM is a rare benign mesenchymal myxoid tumor of the pelvis and perineum which occurs almost exclusively in adult females. Overall, its incidence is about 6-fold higher in females.[2] It usually arises from the soft tissues of the pelvic region, perineum, vulva, and buttock. The adjective “aggressive” emphasizes the neoplastic character of the blood vessels, its locally infiltrative nature, and the high risk of local recurrence after surgical excision.[3] It is called angiomyxoma because of its myxoid nature and prominent vascularity.[4] Rarely, this tumor appears in males simulating inguinal hernia, testicular neoplasm, spermatic cord neoplasm, hydrocele, or spermatocele.[2]


  Case Report Top


A 15-year-old male child presented with a slow-growing scrotal swelling for 6 months. Clinical examination revealed diffuse scrotal swelling measuring about 6 cm × 5 cm × 3 cm. Ultrasound scrotum showed a diffuse subcutaneous scrotal wall thickening with hydrocele. Testis and epididymis on both sides showed no abnormalities. A subtotal excision of the scrotal mass was done and sent for histopathological examination.

Gross appearance

  • Received skin-covered soft-tissue mass measuring 6 cm × 5 cm × 3 cm
  • Cut section showed partially circumscribed, lobulated mass with infiltrating margins
  • The mass was gray-white with slimy areas or myxoid areas. No testes were identified.


Microscopic appearance

Sections studied showed ill-defined lobules, composed of predominantly hypocellular, myxoid areas with spindle to stellate cells. There are numerous blood vessels of varying sizes. The focal area showed perivascular mononuclear inflammatory infiltrate [Figure 1], [Figure 2], [Figure 3], [Figure 4].
Figure 1: Sections showing hypocellular and myxoid areas (H and E, ×100)

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Figure 2: Section showing spindle cells in myxoid stroma (H and E, ×400)

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Figure 3: Sections showing thin- and thick-walled blood vessels and stellate cells in myxoid background (H and E, ×400)

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Figure 4: Sections showing characteristic thick-walled blood vessels and stellate cells in hypocellular and myxoid areas. (H and E, ×400)

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  Discussion Top


AAM was first described by Steeper and Rosai in 1983. Around 150 cases have been described in literature since then.[4] It is a morphologically distinctive, slowly growing vulvovaginal mesenchymal neoplasm that occurs chiefly in adult women with a marked tendency for local recurrence. It usually does not metastasize, but few cases have been reported in literature where metastasis has occurred.[5],[6] Due to its locally infiltrative course and frequent recurrences, it has been termed aggressive.

The occurrence of AAM in men is extremely rare, and in men, AAM is usually derived from the pelviperineal interstitial tissue involving the scrotum (38%), spermatic cord (33%), perineal region (13%), and intrapelvic organs (8%).[3] AAM in the scrotal region may present as a scrotal mass, often mistaken for a hernia or hydrocele.[4] Detailed radiological workup may be helpful in detection, but histology is the gold standard for establishing the diagnosis.[4]

In both males and females, the gross and microscopic appearance and clinical course of disease are similar. On gross examination, the tumors are lobulated, soft to rubbery, solid masses. The cut surface reveals a glistening, soft homogeneous appearance. Recurrent tumors show more prominent areas of hemorrhage and fibrosis.[7]

Microscopically, the tumor is composed of widely scattered spindled to stellate-shaped cells with ill-defined cytoplasm and variably sized, thin- or thick-walled vascular channels in a myxoid stroma that is rich in collagen fibers. Cellularity is usually low, but focal areas show increased cellularity, particularly around large vessels. Mitotic figures are rare or absent. The stroma is characterized by prominent myxoid change with fine collagen fibrils, often with areas of erythrocyte extravasation. A characteristic feature is the presence of variably sized vessels that range from small thin-walled capillaries to large vessels with secondary changes including perivascular hyalinization and medial hypertrophy.[8]

Differential diagnosis of this tumor includes angiomyofibroblastoma, cellular angiofibroma, cutaneous myxoma, myxoid neurofibroma, intramuscular myxoma, and myxoid lipoma.[9]

Immunohistochemically, there is positivity for desmin (more so in the female counterpart of this lesion), smooth muscle actin (about half of the cases), CD34 (about half), and estrogen and progesterone receptors (100%, irrespective of gender).[1]


  Conclusion Top


Being a rare lesion, AAM must be considered in the differential diagnosis of scrotal swelling. Careful preoperative workup should be carried out in suspicious cases. Rejection with clear margins is necessary to prevent recurrence.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  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
[PUBMED]    
2.
Morag R, Fridman E, Mor Y. Aggressive angiomyxoma of the scrotum mimicking huge hydrocele: Case report and literature review. Case Rep Med 2009;2009:157624.  Back to cited text no. 2
[PUBMED]    
3.
Kondo T. Aggressive angiomyxoma in the inguinal region: A case report. J Med Case Rep 2010 8;4:396.  Back to cited text no. 3
    
4.
RehmanS, Muqim RU, Gul T, Wazir MA, Zarin M. Superficial angiomyxoma of the floor of the mouth in an infant. Pak J Med Sci 2010;26:478-81.  Back to cited text no. 4
    
5.
Siassi RM, Papadopoulos T, Matzel KE. Metastasizing aggressive angiomyxoma. N Engl J Med 1999;341:1772.  Back to cited text no. 5
    
6.
Blandamura S, Cruz J, Faure Vergara L, Machado Puerto I, Ninfo V. Aggressive angiomyxoma: A second case of metastasis with patient's death. Hum Pathol 2003;34:1072-4.  Back to cited text no. 6
[PUBMED]    
7.
Kumar S, Agrawal N, Khanna R, Khanna A. Aggressive angiomyxoma presenting with huge abdominal lump: A case report. Cases J 2008 28;1:131.  Back to cited text no. 7
    
8.
Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. 5th ed. Mosby, Inc: Saunders, Philadelphia United States; 2008.  Back to cited text no. 8
    
9.
Andres LA, Mishra MJ. Angiomyxoma diagnosed in a man presenting for abdominal lipectomy. Can J Plast Surg 2007;15:163-4.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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