Rare Case Of Aggressive Angiomyxoma Of Vault

Author information

Chaudhari HK*,Parulekar SV**.

( * Associate Professor, ** Professor and Head, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Aggressive angiomyxoma  is a slow-growing a rare mesenchymal  soft tissue tumor which arises in the pelvis and perineal region. It is not a common  type of tumor in this location.  Surgical removal  is the main treatment of aggressive angiomyxoma. Here, we present a case of a vaginal aggressive angiomyxoma in a 70 year old woman in which the diagnosis was only made after histological examination. The appearance,, presentation, diagnosis and treatment of this rare tumor are outlined.

Introduction

Aggressive angiomyxoma is a unusual deep soft tissue tumor occurring in the pelviperineal regions of young female patients.[1] As the name suggests, it may have a locally aggressive course and may exert pressure on adjacent organs. This slowly growing painless neoplasm was first described by Steeper and Rosa in 1983.[2] It is slow growing but problematic due to frequent local recurrence (30-72%). However metastasis are very uncommon. Misdiagnosis is very frequent problem. Diagnosis is mostly made on histopathology following surgical removal . We report a case of aggressive angiomyxoma in 70 years female presenting with a lesion resembling granulation tissue on vault of vagina following a vaginal hysterectomy done 11.5 years ago.

Case Report

A 70 year old woman, married for 50 years, para 2 living 2, presented with a complaint of something coming out per vaginum for 5-6 months. The symptom was aggravated by coughing, sneezing and lifting heavy weights. She had undergone a vaginal hysterectomy 11.5 years ago for a similar complaint. Operative notes of hysterectomy  were  not available. There was no history of any major medical or surgical illness in the past. There was no history of any bowel related symptoms. Her general and systemic examination revealed no abnormality. There was trunkal obesity. Speculum examination showed central transverse type moderate cystocele, mild central transverse type rectocele , lax perineum, and a granuloma of approximately 0.5 x .2 x .2  cm at right angle of vault. The Granuloma was soft and did not bleed to touch. The uterus was absent. There were no pelvic masses. Her Pap showed atrophic inflammatory with atypical epithelial cells. Results of tests for fitness for anesthesia were normal.

Figure 1. Gross appearance of the tumor.

An anterior colporrhaphy and a posterior colpoperineorraphy were done after excision of the Granuloma at the vault of vagina. She made an uneventful recovery. Her speculum and vaginal examination done agter 5 days revealed a healed vault and no pelvic abnormality.

Histopathological examination of the granulation tissue from the vaginal vault  showed a polypoidal tumor composed of bland spindled  and stellate cells  embedded in loose myxoid stroma. Numerous vessels were seen within the tumor which were dilated and showed extravasation of red blood cells. The stroma also showed numerous mixed inflammatory infiltrate. There was no evidence of necrosis, atypia or increased mitosis. A diagnosis of aggressive angiomyxoma of the vagina was made. At three monthly follow up examinations, the patient was found to be well, without any local recurrence or distant metastases.

Figure 2. Polypoidal tumour. (10X)

Figure 3. Tumor shows numerous dilated blood vessels ,inflammatory cells in myxoid stroma. (45X)

Figure 4. Dilated vessels ,neutrophils ,lymphocytes , few eosinophils in myxoid stroma. (100X)

Figure 5. Bland spindle cells are seen in myxoid stroma. (100X)

Discussion

Aggressive angiomyxoma of the genital region is a unusual condition that has been reported to show a peak incidence in young women in the third and fourth decades of life.[3-11] The tumor particularly involves the pelvis, often in the vagina, vulva, perineum or buttocks.[4] The tumor typically grows slowly and insidiously. It usually takes 2 months to 17 years for the patients to go to the hospital with a mass after the initial discovery. It may become larger and occupy the whole pelvic region and invade paravaginal and pararectal spaces displacing pelvic structures.[5,15] Gross examination shows it as a soft, bulky mass whose external surface is smooth and usually without a capsule. Some have finger-like projections into neighboring tissues. It has a gelatinous, homogeneous consistency. Focal areas of hemorrhage and congestion are seen on cut surface. It generally displaces rather than invades adjacent viscera, and is rarely damaging. Although invasions are rare, it can invade the bladder, bowel and pelvic bone.[3,6,7,8,11] Histologically, the tumor consists of a hypocellular population of small, to some extent stellate and spindle-shaped neoplastic cells with thin cytoplasmic processes.[3,5,10,15] The cells are scattered in a loose myxoid matrix composed of delicate curling collagen fibrils and hyaluronic acid, which gives the tumor a pale-pink color by eosin staining.[6,7,10] At the same time there is also an accompanying particularly vascular component, ranging from minute  capillary-like vessels to larger and thick-walled vessels with a distinct smooth muscle cells but with no evidence of anastomosis.[3,7,9-11] It takes long time to grow, but local recurrences of tumor are often seen (30-72% of the cases), sometimes even years later. Preoperative identification  is difficult due to rarity of the condition and an absence of gross characteristic features. It should be considered in every mass in the genital, perianal and pelvic region. Awareness  of the condition is essential to avoid inappropriate surgical treatment and wrong prognostication. Surgery is the approved treatment and it consists of a wide excision, but the importance of total destruction does not seem to be of crucial significance. The appropriateness of radiation therapy, embolization and/or hormonal treatment  has not been clearly proven yet but may become future another possibility. The term "aggressive" was chosen to give special importance to the neoplastic quality of the blood vessels, its locally infiltrative quality and the high danger of local recurrence, not to indicate its malignant nature.[12,13,8,10] However, there are reports of metastasized disorder  in the literature.[14,15] Hence meticulous follow up needs to be done over many years after initial surgical treatment, and local recurrence as well as distant metastases should be looked for.

References

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Citation

Chaudhari HK, Parulekar SV. Unruptured Pregnancy In Rudimentary Horn Of Unicornuate Uterus. JPGO 2017. Volume 4 No. 5. Available from: http://www.jpgo.org/2017/05/rare-case-of-aggressive-angiomyxoma-of.html