Uterine Angioleiomyoma

Author Information

Parulekar SV.

(Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

Abstract

Uterine angioleiomyoma is a very rare tumor, usually seen in 40-60 years old women. It is made of smooth muscle cells and thick-walled blood vessels. An unusual case of uterine angioleiomyoma is presented.

Introduction

Angioleiomyoma is an extremely rare variant of uterine leiomyoma.[1] Its etiology is unclear. It is estrogen dependent. Chronic venous insuffficiency and minor injury play a role in its genesis. Its clinical presentation is usually like that of a leiomyoma and the diagnosis is made only after histopathological examination. There are only 42 cases of an angioleiomyoma of the uterus in the world literature. We present 43rd case of uerine angioleiomyoma (UAL) in the world literature.

Case Report

A 21 year old woman, married for 2 years, presented with a complaint of heavy menstrual bleeding for 6 months,  severe secondary dysmenorrhea for 1.5 years and primary infertility. She had menarche at the age of 12 years. Her menstrual cycles were regular, every 28-30 days, with blood flow for 3 to 4 days. Her past cycles had been painless. Her past medical and surgical history was not contributory. Her general and systemic examination showed no abnormality. Findings of abdominal examination were normal. Speculum examination showed a normal vagina and cervix. Bimanual pelvic examination showed uterus of 8 weeks’ size, with a posterior wall leiomyoma measuring about 3 cm in diameter. There was no tenderness. There was no other abnormality. Abdominopelvic ultrasonography (USG) showed an intramural leiomyoma in the posterior wall of the uterus measuring 3.5X2.4X1.4 cm. Counseling was done about her treatment options. In view of her severe dysmenorrhea not responding to treatment, she opted for myomectomy rather than management of her infertility and postponing myomectomy until after her childbirth. Her investigations for fitness for anesthesia showed normal results. Myomectomy was done. A leiomyoma measuring about 3 cm in diameter was removed and the uterus was reconstructed. The patient withstood the procedure well and made an uneventful recovery.

Gross examination of the leiomyoma showed it to be of size 3.5X2.5X1.5 cm. Its external surface was whitish and rough. Its cut surface was whitish and whorled. Microscopic examination showed intersecting bundles of smooth muscle cells. Thick walled blood vessels were found amidst and around the smooth muscle cells. There were swirling spindle cells (well-differentiated smooth muscle cells) arising from vascular walls. Some of the vessels were hyalinized. A diagnosis of angioleiomyoma of the uterus was made.

Figure 1. Angioleiomyoma is seen being enucleated (arrows).

Figure 2. Histopathological appearance of the angioleiomyoma. (100X)

Figure 3. Histopathological appearance of the angioleiomyoma. (400X)

Discussion

Angioleiomyoma is a very rare variant of uterine leiomyoma. It accounts for 0.34-0.40% cases of UAL.[2] It is usually seen in 40-60 years old women. Handler et al. Reported 11 cases between 1966 and 2007.[3].  Sharma et al. reported 16 cases in 2014[4]. Garg et al. Reported one more case in 2014.[5] Diwaker et al. Reported another series of 15 cases in 2015.[6] Most of the cases are those subjected to hysterectomy, and very few are of myomectomy. Our case was unusual in that she was only 21 years old. The variant develops as an estrogen dependent tumor, just like simple leiomyoma.[7] Angiogenesis is involved, which may be due to hypoxia, development of shearing forces which activate endothelial cells, growth factor coming from surrounding macrophages.[8,9] The genetic basis of UAL is different from that of a leiomyoma as cytogenetic clonal changes in the karyotype in UAL are not seen in conventional leiomyoma.[10]

Clinical features of UAL include abnormal uterine bleeding (menorrhagia) and dysmenorrhea. The enlarged uterus feels just like one with a conventional leiomyoma does. Thus it is not possible to distinguish between the two clinically. Investigations like USG, computerized tomography and magnetic resonance imaging are not very useful in making this distinction.[5] The diagnosis is most often made only on histopathological examination. Histopathologically UAL is of capillary type, venous type or cavernous type.[11] The capillary type shows a dense structure of narrow vessels and thick bundles of smooth muscles. The venous type shows thick vessels and bundles of smooth muscles. The cavernous type shows wide vessels and less of smooth muscle. Our case was of the venous type. UAL does not show any atypia, pleomorphism, mitosis, or necrosis. When in doubt, immunohistochemical studies are used. Tumors like fibroma, angiolipoma, angiofibroma, and angiomyofibroblastoma are positive for  desmin and vimentin but negative for smooth muscle actin.[12] 

It is sufficient to remove the tumor by total hysterectomy. No recurrence has been reported after that. But if the patient is young, a myomectomy is required. Since the tumor is benign, this should be sufficient. It is desirable to keep a long follow up in such cases.[13]  We are treating our patient for infertility and have counseled her to have annual follow up examinations subsequently for many years.

Acknowledgement

I thank Dr Gwndolyn Fernandes for providing the photographs of histopathology slides.

References

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Citation

Parulekar SV. Uterine Angioleiomyoma. JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/uterine-angioleiomyoma.html