Indian J Sex Transm Dis Indian J Sex Transm Dis
Official Publication of the Indian Association for the Study of Sexually Transmitted Diseases
Indian J Sex Transm Dis
The Journal | Search | Ahead Of Print | Current Issue | Archives | Instructions | Subscribe | Login    Users online: 79   Home Email this page Print this page Bookmark this page Decrease font size Default font size Increase font size


 
CASE REPORT
Year : 2007  |  Volume : 28  |  Issue : 2  |  Page : 103-105
 

Angiomyxoma of genitalia: A case report


Department of STD, Rangaraya Medical College, Government General Hospital, Kakinada, Andhra Pradesh, India

Correspondence Address:
T S Chandra Gupta
Department of STD, Government General Hospital, Kakinada - 533 001, AP
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0253-7184.39016

Rights and Permissions

 

   Abstract 

Angiomyxoma is a rare tumor developing from myxoid cells. There are two types of angiomyxomas, superficial - which grows near the surface and aggressive - which involves the deeper structures. A 15-year-old unmarried girl presented with an asymptomatic mass on the genitalia of 3 years duration. Her general and systemic examination was within normal limits. Patient was referred to Urology Department where the mass was excised and was sent for histopathological examination, which clinched the diagnosis of superficial angiomyxoma. The patient is kept under follow-up. This case is a typical example which highlights the role of STD department in diagnosing, treating, and reassuring the patient with a nonvenereal disease, causing apprehension.


Keywords: Aggressive angiomyxoma, Carney′s complex, superficial angiomyxoma


How to cite this article:
Chandra Gupta T S, Rani B S, Swarna Kumari G, Murthy V S. Angiomyxoma of genitalia: A case report. Indian J Sex Transm Dis 2007;28:103-5

How to cite this URL:
Chandra Gupta T S, Rani B S, Swarna Kumari G, Murthy V S. Angiomyxoma of genitalia: A case report. Indian J Sex Transm Dis [serial online] 2007 [cited 2020 Aug 13];28:103-5. Available from: http://www.ijstd.org/text.asp?2007/28/2/103/39016



   Introduction Top


Angiomyxoma is a rare tumor developing from myxoid cells which are a type of cells found in body's connective tissue. It is unique in being structurally benign and behaviorally locally malignant. It is of two types: (1) superficial which grows near the surface and (2) aggressive which involves the deeper structures. There is paucity in its complete description in most of the available literature since the time it was first reported in 1983. [1]

One such a rare case of angiomyxoma of genitalia without Carney's complex [1],[2],[3] (spotty pigmentation, cardiac myxoma, and endocrine overactivity) presented to the STD department of Government General Hospital, Kakinada, AP in March 2006.


   Case Report Top


A 15-year-old unmarried girl presented with an asymptomatic mass on the genitalia of 3 years duration. It has been slowly growing and attained the present size. There was no similar illness in the past. There was no history of fever or genital ulcer disease. Her menstrual history was normal. The patient denied any premarital exposure. Her general and systemic examination was within normal limits. On examination, there was a single skin colored pedunculated mass of size 10 cm 2.5 cm arising from left labium majus [Figure - 1]. It was lobulated in appearance with variable consistency (soft to firm). There were no visible pulsations or cough impulse. Skin over the swelling was not pinchable. It was a nonreducible and noncompressible swelling. Per vaginal and per speculum examinations were not done as she was not married. Rest of the external genitalia was normal.

A differential diagnosis of neurofibroma, fibrolipoma, filariasis, and Lymphogranuloma venereum were considered. Blood, urine analysis, X-ray chest, X-ray pelvis, and cardiac evaluation were normal. Patient was referred to Urology Department for opinion and necessary treatment. The mass was totally excised and was sent for histopathological examination [Figure - 2]. The section showed normal epidermis. Subepidermal region was nodular. Each nodule was hypocellular with spindle and stellate-shaped cells against myxoid background. There were plenty of capillary-sized blood vessels with perivascular infiltrate made up of lymphocytes, eosinophils, and a few neutrophils. Focally arborizing pattern of blood vessels were also seen. There was no mitotic activity. Features were suggestive of Cutaneous superficial angiomyxoma [Figure - 3],[Figure - 4].


   Discussion Top


Superficial angiomyxoma was first reported by Allen et al. in 1988. [1] It is a rare benign tumor developing from myxoid cells. Superficial angiomyxoma grows near the surface measuring 3-4 cm in size as a subcutaneous nodule [2] and mostly involves the genitalia, [4] trunk and head and neck. [1] It affects mostly middle-aged adults, i.e., between fourth to sixth decade with equal sex predilection. It is polypoid and is mostly associated with Carney's complex which is a triad of spotty pigmentation, cardiac myxomas, and endocrinal over activity. [1]

As seen in light microscope, the lesion has lobular or multinodular appearance at low magnification. It is poorly circumscribed with extension into subcutaneous tissue. A sparse proliferation of spindle and stellate-shaped cells is seen in extensive myxoid stroma. There is often a prominent vasculature that is focally arborizing. A mixed cellular infiltrate particularly of neutrophils is a feature. [1],[5]

Clinically it should be differentiated from cyst, skin tag, and neurofibromatosis. Complete surgical removal is the treatment of choice, but it has a recurrence rate of 30-40%. [1]

The other variety, i.e., aggressive angiomyxoma was described by Steeper and Rosai in 1983 for morphologically distinctive slowly growing locally infiltrative tumor. It occurs chiefly in genital, perineal, and pelvic regions in women of child-bearing age group. Male to female ratio is 1:6. Size ranges from a few centimeters to more than 20 cm. It is not associated with Carney's complex. [1]

Grossly they are soft and partly circumscribed. On cross section they have gelatinous appearance. Microscopically the tumor is composed of widely scattered stellate and spindle-shaped cells with variable-sized thick and thin-walled vascular channels in myxoid stroma. [1] Clinically it needs to be differentiated from bartholin cyst, periurethral cyst, and hernia. It needs complete surgical removal but has a local recurrence rate of 30-40%.

Contrary to the typical description, the patient here is an adolescent girl. The site of involvement, clinical features, and histopathology are consistent with standard description of superficial angiomyxoma except the size and prominent neutrophilic infiltration. It is more of a pathological diagnosis than a clinical one. Thus despite the lack of a few features, pathologist still opines that it is a case of superficial angiomyxoma.

The patient was kept under follow-up for 15 months. There have been no signs of recurrence.


   Conclusion Top


This is one such an example of nonvenereal disease occurring on genitalia causing apprehension to the patient. This also stands as an example where in STD Department has played a pivotal role in diagnosing, getting treatment, and reassuring the patient through follow-up, especially in the clinical setting of known recurrence.

 
   References Top

1.Benign soft tissue tumors and peudo tumor of miscellaneous type. In : Weiss SW, Goldblun JR, editors. Enzinger and Weiss's Soft Tissue Tumors. 4 th ed. Mosby: 2001. p. 1437-53.  Back to cited text no. 1    
2.Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with and without epithelial components: Report of 30 tumors in 28 patients. Am J Surg Pathol 1988;12:519-30.  Back to cited text no. 2    
3.Takahashi H, Hida T. Carney complex: Report of a Japanese case associated with cutaneous superficial angiomyxomas, labial lentigines and a pituitary adenoma. J Dermatol 2002;29:790-6.  Back to cited text no. 3    
4.Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutaneous myxoma): A clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol 1997;16:325-34.  Back to cited text no. 4    
5.Calonje E, Guerin D, McCormick D, Fletcher CD. Superficial angiomyxoma: Clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23:910-7.  Back to cited text no. 5    


    Figures

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



 

Top
Print this article  Email this article
Previous article Next article

    

 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (74 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed4617    
    Printed189    
    Emailed0    
    PDF Downloaded165    
    Comments [Add]    

Recommend this journal