|Year : 2007 | Volume
| Issue : 2 | Page : 103-105
Angiomyxoma of genitalia: A case report
TS Chandra Gupta, B Sudha Rani, G Swarna Kumari, V Srinivasa Murthy
Department of STD, Rangaraya Medical College, Government General Hospital, Kakinada, Andhra Pradesh, India
T S Chandra Gupta
Department of STD, Government General Hospital, Kakinada - 533 001, AP
Source of Support: None, Conflict of Interest: None
| 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|| |
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. 
One such a rare case of angiomyxoma of genitalia without Carney's complex ,, (spotty pigmentation, cardiac myxoma, and endocrine overactivity) presented to the STD department of Government General Hospital, Kakinada, AP in March 2006.
| Case Report|| |
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|| |
Superficial angiomyxoma was first reported by Allen et al. in 1988.  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  and mostly involves the genitalia,  trunk and head and neck.  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. 
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. ,
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%. 
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. 
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.  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|| |
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|| |
|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. |
|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. |
|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. |
|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. |
|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. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]