Three cases of paraurethral angiofibroblastoma

Three case reports of angiofibroblastoma.

Case 1

A 32 year old lady presented with an asymptomatic, but gradually enlarging vaginal lump over 3 months. On examination, there was a small 3x2 cm rubbery tissue mass arising from the left paraurethral region. An examination under anesthesia and excisional biopsy was thus organized. However she defaulted on her surgery due to anxiety, and failed to attend her follow up appointment. Six months later she returned complaining od pain and bleeding on voinding. On examination, the solitary paraurethral lump had noe enlarged to a size 8x8x4 cm, amd had become ulcerated and infected ( figure 1). There was no associated inguinal lymphaadenopathy. Due to the pain and voiding difficulties, an examination under anesthesia, cystoscopy and excision of the mass was promptly carried out. Intraoperatively, the tumour was found to be localized to the paraurethral regionand had not invaded into the urethra or bladder. It was found that the tumour was well circumscribed and was able to be ‘shelled out’ relatively easily. The patient did not require any indwelling catheter post operatively and subsequently made an uneventful recovery ( figure 2). Histopathology confirmed the mass to be a benign angiofibroblastoma.

Case 2.

A 42 year old Para 2+1,  This patient initially presented on 9.10.2008 because she noticed a mass per vaginal which had been present for 2 years, it was reducible but protruded back out immediately. The mass was firm, nodular and mobile, measuring 4x3 cm. The mass progressive got bigger and causing voiding dysfunction.  An examination under anesthesia was carried out on 226.2009. An indwelling catheter was inserted to assist in the surgery. The mass was very close to the urethra and has distorted the anatomy of the urethra. The mass was easily enucleated.  The estimated blood loss was 100mls. There were no intraoperative complications. The catheter was kept for three days. The histology was consistent with suburethral angiofibroblastoma (4x3cm).

Case 3.

This a 65 year old lady Para 6, presented with elongated and firm mass near the urethral meatus. The mass was initially small but over six year its size has increased and causing pain and difficulty in walking. She also has difficulty in micturation. She has to move the elongated mass to one side. On examination there was a 7x4x4 cm elongated mass with the tip of the distal part of the mass appeared fungating and necrosing. The proximal part of the mass had a 4cm stock/ base. She underwent examination under anaesthesia and excision of the mass. The surgery was straight forward. Check cystoscopy was normal. The histology was consistent with    Angiomyofibroblasroma.

Discussion

Angiomyofibroblastoma is a rare mesenchymal tumour of the female genital tract that was only first described in 1992(1). This tumour is predominately found in the vulval region, bu can also arise from the vagina, clitoris, labia majora and perineum. Unusual cases involving the male scrotal and inguinal regions have been reported 9(1). They have been reported in women from the age of 23-86 (mean 45.8) years. They usually appears as a painless lump that may have been present for a few weeks or up to 13 years. Clincally, this tumour can be mistaken for a bartholin gland cyst, skene’s gland cyst, urethral diverticulum or Gardner duct cyst.

Angiomyofibroblastoma is a slow growing tumour that is usually well circumscribed, and has a soft rubbery consistency with a bulging, pink, section surface. Histologically, this tumour is composed of two components: the blood vessels and stromal cells. It shows alternating hypercellular and hypicellular oedematous ares, in which numerous thin walled, small to medium sized vessels are irregularly distributed throughout. The tumour cells show immune reaction for vimentin and desmin and , more recently, it was noted to be muscle specificactin-positive or Alfa-smooth muscle actin-positive (2,3,4). It is typically benign in nature. Only one case of a malignant transformation of an angiomyofibroblastoma ( ‘angiomyofibrosarcoma’) has been reported(3).

Angiomyofibroblasroma may have been reported as an aggressive angiomyxoma. Unlike Angiomyofibroblasroma, aggressive angiomyxoma affects deeper tissues with infiltrative margins, and tends to recur (4). The pathogenesis of Angiomyofibroblasromais still unclear, although it has been proposed that it may originated from an immature mesenchymal cell in the sub epithetial myxoid zone of the lower female genital tract, or in perivascular areas. The outcome in these patients were good, and is always favorable with simple excision of the tumour mass.

References

1.      Fetchers CDM, Tsang WY, Fisher C, lee KC & Chan JK. Angiomyofibroblasromaof the vulva. A benign neoplasm distinct from aggressive angiomyxoma. Am J Surg Patho 1992; 16;373-82.

1. 2.   Hiroshi K, Noriomi M, Yoshikazu S, Masanori M, Taiji T & Takashi s. Angiomyofibroblasroma of the female urethra. Int J Urol 1999; 6:268-270

1. 3.   Nielsen GP,Young RH, Dickersin GR & Rosenberg AE. Angiomyofibroblasromaof the vulva with sarcomatous transformation (‘Angiomyofibrosarcoma)’. Am J surg Pathol 1997;30:3-10

1. 4.     Steeper TA & Rosai J. Aggressive angiomyxoma of the female pelvis and perineium. Report of nine cases of a distinctive type of gynaecologis soft-tissue neoplasma. Am J Surg Pathol 1983; 7:463-465.

1. 5.  Fukunaga M, Nomura K, Matsumoto K, Doi K, Endo Y & Ushigome S. Vulval Angiomyofibroblasroma: Clinicopathological analyisi of six cases. Am J clin Pathol 1997; 6:45-51

VAGINAL DISCHARGE:NOTES FOR PMC STUDENTS

 Vaginal Discharge

Vaginal discharge is a common presenting symptom in any physician’s office. Vaginal discharge may be physiological or pathological. Although abnormal vaginal discharge often prompts women to seek screening for sexually transmitted infections (STIs), vaginal discharge is poorly predictive of the presence of an STI.¹Clinicians need to be aware of emerging epidemiological data, the different presentations of vaginal discharge, and how to approach their management so that the symptom can be treated according to its aetiology.²

Vaginal discharge is a common gynaecological condition among women of childbearing age that frequently requires care. It derives from physiological secretion of cervical and Bartholin’s glands and desquamation of vaginal epithelial cells resulting from bacterial action in the vagina.³The amount of mucus produced by the cervical glands varies throughout the menstrual cycle. Vaginal discharge that suddenly differs in colour, odour, or consistency, or significantly increases or decreases in amount, may indicate an underlying problem like an infection.⁴ Increased amount of vaginal discharge can be due to emotional stress, ovulation, pregnancy or sexual excitement.

Aetilogy

Physiological Discharge

Many women have what they perceive as an abnormal vaginal discharge at some point in their lives, but usually it is just a normal physiological discharge. This is a white or clear, non-offensive discharge that varies with the menstrual cycle. The quality and quantity of vaginal discharge may alter in the same woman in cycles and over time. Factors that can influence physiological discharge are:-

1)      Age

-          Prepubertal

-          Reproductive

-          Pregnancy

-          Hormonal contraceptions

-          Menopause

2)      Local facors

-          Semen

-          Personal hygiene and habits

-          Menstruation

Pathological Discharge

Pathological vaginal discharge can be further divided by specific age groups which are prepubertal group, reproductive group and menopause group.

Common causes of pathological vaginal discharge for each age group are:-

Prepubertal	Reproductive	Menopause
Nonspecific bacterial vaginitis	Foreign bodies (ex: IUCD, tampon, condom)	Cervical or endometrial carcinoma
Foreign bodies	Allergic to local irritant	Actropic vaginitis
Sexual abuse	Infections	FB (ex: vaginal pessary)

Common causes of infections:-

-          Candidiasis: Acute vulvovaginal candidiasis / recurrent vulvovaginal candidiasis

-          Bacterial vaginosis

-          Trichomoniasis

-          Chlamydia trachomatis

-          Neisseria gonorrhoea

-          Pelvic inflammatory disease

Principles of management

1)      History⁵

-          Characteristics of the discharge - Onset, duration, colour, odour, consistency.

-          Any associated symptoms - Itch, dyspareunia, abdominal pain, abnormal vaginal bleeding or pyrexia is more likely to indicate sexually transmitted infection.

-          Sexual history - Is patient at increased risk of sexually transmitted infection (age <25 years, new sexual partner or more than one sexual partner in past year, previous sexually transmitted infection)

-          Contraceptive use

-          Pregnancy

-          Concurrent medications and previous treatments

-          Medical conditions such as diabetes, immunocompromised state.

-          Non-infective causes of discharge such as allergic reaction, known cervical ectopy or polyps, genital tract malignancy, foreign body (such as tampons).

2)      Examination⁵

-          Abdominal palpation for tenderness or mass.

-          Inspect the vulva for discharge, erythema, ulcers, other lesions or skin changes.

-          Bimanual pelvic examination for adnexal or uterine tenderness or mass, and for cervical motion tenderness (this can indicate pelvic inflammatory disease).

-          Speculum examination to inspect vaginal walls, cervix, and characteristics of discharge.

-          Take endocervical swabs if there is risk of sexually transmitted.

-          High vaginal swabs are of limited diagnostic value except in pregnancy, post-instrumentation, failed treatment, recurrent symptoms, or to confirm candidiasis.

Bacterial Vaginosis

Bacterial vaginosis is the most common cause of infective vaginal discharge. It causes profuse and fishy smelling discharge without itch or soreness. This condition is characterised by an overgrowth of anaerobic bacteria and occurs and remits spontaneously. Asymptomatic bacterial vaginosis in non-pregnant women does not require treatment. The condition is associated with poor pregnancy outcomes, endometritis after miscarriage, and pelvic inflammatory disease. Antibiotics are the mainstay of therapy for bacterial vaginosis. Medications include metronidazole, clindamycin, and metronidazole vaginal gel.

Vulvovaginal Candidiasis

The prevalence of asymptomatic carriage of Candida in women is 10%. Symptoms are vulval itch and soreness and thick white non-offensive discharge. There is no evidence that combined oral contraceptives cause candidiasis. Asymptomatic vulvovaginal candidiasis does not need treatment. Vulvovaginal candidiasis can be acute or recurrent. Recurrent vulvovaginal candidiasis diagnosed when there are 4 or more episodes of VVC in 1 year.

Chlamydia Trachomatis

Chlamydia trachomatis is the most common sexually transmitted infection caused by a bacterium. Chlamydia can cause a purulent vaginal discharge, but it is asymptomatic in 80% of women. It was thought that 10-40% of untreated chlamydial infections will result in pelvic inflammatory disease. This has recently been challenged by a large observational study, which reported that only 5.6% of women developed this disease,⁶ and by a small prospective study that reported an even lower rate of 1%.⁷ Chlamydia is treated with either single dose of Azithromycin or twice daily dose of Doxycycline.

Neisseria gonorrhoea

Neisseria gonorrhoea may present with a purulent vaginal discharge but is asymptomatic in up to 50% of women. Major symptoms include vaginal discharge, dysuria, intermenstrual bleeding, dyspareunia and mild lower abdominal pain. The true prevalence and epidemiology in the general community is not known. Gonorrhoea may be complicated by pelvic inflammatory disease.Culture is the most common diagnostic test for gonorrhoea, followed by the deoxyribonucleic acid (DNA) probe, and then the polymerase chain reaction (PCR) assay and ligand chain reaction (LCR).

Trichomonasvaginalis

Trichomonasvaginalis can cause an offensive yellow vaginal discharge, which is often profuse and frothy, along with associated symptoms of vulval itch and soreness, dysuria, and superficial dyspareunia, but many patients are asymptomatic. The true prevalence and epidemiology in the general community is not known. Usually an oral antibiotic called metronidazole (Flagyl) is given to treat trichomoniasis.

Persistent Vaginal Discharge

It would be difficult to proceed further for women who complain of persistent vaginal discharge with repeated negative STI screen results. When minimal discharge is evident, it is worth discussing again personal hygiene practices and douching, the basis for physiological discharge, and inquiring whether there are psychosexual difficulties as a result of the patient's continued symptoms.

If use of spermicides and lubricants are contributing to symptoms, alternative contraception choices should be discussed. An extensive cervical ectropion can cause heavy mucoid discharge. After the menopause, atrophic vaginal changes may predispose women to infective vaginitis. Intravaginal oestrogen replacement, with pessaries or cream, gradually improves the condition of the vaginal epithelium and reduces the susceptibility to infection.

Underlying gynaecological disease must be considered in all women with unexplained persistent vaginal discharge. Gynaecological neoplasms, such as benign endocervical and endometrial polyps, can present with vaginal discharge, and malignancy needs to be excluded.

Conclusion

Many women self-diagnose and self-treat episodes of vaginal infection with over the counter treatments. Some women may subsequently present with history of recurrence and never having had this diagnosis confirmed by any microbiological tests. It is important to confirm the diagnosis and to ensure a full sexual health screen has been done to exclude concurrent infection. Management of vaginal discharge requires an empathic approach with reassurance and psychological support as necessary.

Reference

1.      Vaginal discharge—causes, diagnosis, and treatment

      BMJ 2004; 328 doi: http://dx.doi.org/10.1136/bmj.328.7451.1306 (Published 27 May 2004)

2.     Vaginal discharge

    BMJ. 2007 December 1; 335(7630): 1147–1151. doi:  10.1136/bmj.39378.633287.80 PMCID:        

    PMC2099568 Clinical Review

3.      Pathological Vaginal Discharge among Pregnant Women: Pattern of Occurrence and Association in a    
     Population-Based SurveyTânia Maria M. V. da Fonseca,1 Juraci A. Cesar,2 Raúl A. Mendoza-Sassi,2  
     and Elisabeth B. Schmidt3

4.      Source: Vaginal discharge | University of Maryland Medical Centerhttp://umm.edu/health/medical
      /ency/articles/vaginal-discharge#ixzz2foJJk45j

5.      Abnormal vaginal discharge

      BMJ 2013; 347 doi: http://dx.doi.org/10.1136/bmj.f4975 (Published 13 August 2013)

6.      Low N, Egger M, Sterne JA, Harbord R, Ibrahim F, Lindblom B, et al. Incidence of severe  
      reproductive tract complications associated with diagnosed genital chlamydial infection: the Uppsala 
      women's cohort study. Sex Transm Infect 2006;82:212-8.

7.      Morré SA, van den Brule AJC, Rozendaal L, Boeke AJ, Voorhorst FJ, de Blok S, et al. The natural 
      course of asymptomatic Chlamydia trachomatis infections: 45% clearance and no development of  
      clinical  PID after one-year follow up. Int J STD AIDS 2002;13(suppl 2):12-8.