Journal of Cancer Research and Therapeutics Medknow Publications on behalf of the Association of Radiation Oncologists of India (AROI) ISSN: 0973-1482 EISSN: 1998-4138 Vol. 7, Num. 1, 2011, pp. 69-71

Journal of Cancer Research and Therapeutics, Vol. 7, No. 1, January-March, 2011, pp. 69-71

Brief Communication

Localized cavernous hemangioma of the uterus involving adenomyotic foci

Daryoush Saeed-Vafa¹, Erin Myers², Yajue Huang¹, J Stuart Ferriss², Varsha Manucha¹

¹ Department of Pathology and Laboratory Medicine, Temple University School of Medicine, Philadelphia, PA, USA
² Department of Obstetrics, Gynecology and Reproductive Sciences, Temple University School of Medicine, Philadelphia, PA, USA

Correspondence Address: Varsha Manucha, Department of Pathology and Laboratory Medicine, Temple University School of Medicine, B339, Outpatient Bldg., 3401 N Broad Street, Philadelphia, PA 19140, USA, varsha.manucha@tuhs.temple.edu

Code Number: cr11014

PMID: 21546746

DOI: 10.4103/0973-1482.80471

Abstract

Keywords: Cavernous hemangioma, uterus, localised

Introduction

A cavernous hemangioma of the uterus is an extremely uncommon lesion, mostly described in pregnant women presenting with uncontrolled bleeding. We present a rare case of a cavernous hemangioma, localized to the cornu of the uterus, in a non-pregnant woman with a history of heavy menstrual bleeding and unremitting pelvic pain.

Case Report

A 27 year-old Hispanic female presented with a four-year history of pelvic pain and menorrhagia. The patient reported that her symptoms worsened after the delivery of her first child three years ago. She described the pain as a constant, dull ache in the right lower quadrant of the abdomen that was worse at night and after long periods of standing. The pain radiated down her right leg and was unrelieved with over-the-counter analgesics such as non-steroidal anti-inflammatory drugs. Her past medical history was significant for anxiety and asthma. Four years prior to presentation, the patient had a pelvic ultrasound performed to investigate her pain. The ultrasound was essentially normal except for slight expansion of the junctional zone within the fundus and slight textural heterogeneity of the fundal myometrium suggestive of very early adenomyosis. Following this evaluation, the patient became pregnant with her first child and had an uneventful Cesarean delivery. Six months postoperatively, the patient noted an increase in her right lower quadrant pain. Computed tomography (CT) of the abdomen and pelvis was performed and documented an 8.6 x 9.3 x 8.4 cm, heterogeneous, hyper-vascular mass arising from the right uterine cornu [Figure - 1].

Over the next year, the patient was followed with serial ultrasounds that confirmed that the dimensions of the mass were stable and it was alternatively described as multiple varices within the posterior portion of the fundus of the uterus. A progesterone-containing intra-uterine device (IUD) was placed to control her menorrhagia which resulted in near complete cessation of her bleeding but did not relieve her pain. Within six months, the patient noted escalation of her abdominal pain and constant pain in her right lower extremity as well. A pelvic ultrasound and magnetic resonance imaging (MRI) demonstrated that the mass had increased in size to 11.0 x 6.5 x 7.0 cm and that the hyper-vascular pattern persisted. She was then referred to gynecologic oncology for evaluation.

With her increased symptoms and concern for an underlying malignancy a discussion of surgical management was made with the patient. Under general anesthesia an exploratory laparotomy was performed. During surgery an abnormal trabeculated tumor arising in the right uterine cornu was identified which was partially wedge-resected and sent for intraoperative consultation to the pathology department. A preliminary frozen diagnosis of vascular malformation was made based on which it was decided not to extend the procedure.

Pathological Results

Upon gross examination of the specimen, the segment of the uterus measured 3.5 x 3.0 x 1.5 cm and weighed 17 g. The serosal surface was pink-tan, shiny and rough. It was bi-valved to reveal a portion of endometrial cavity measuring 1.8 x 0.8 cm. It was covered by a scant red endometrium measuring 0.1 cm in thickness. The myometrium was pink-tan with a prominent trabeculated appearance and measured 1.6 cm in thickness [Figure - 2].

Histologically, the tumor was composed of large dilated and cavernous vascular spaces lined by flat and bland endothelial cells. It diffusely involved the myometrium and the endometrium [Figure - 3]. There were also foci of adenomyosis, most invaded by the vascular spaces [Figure - 4], [Figure - 5]. The tumor extended within 0.1 cm of the serosal surface. The endometrium was atrophic and showed few scattered inactive glands intervening with the vascular spaces.

Immunohistochemistry was performed on the paraffin-embedded section. The flattened lining cells stained strongly for CD31 [Figure - 4] and CD34 and were negative for AE1/AE4 and D2-40. A final diagnosis of cavernous hemangioma involving the endomyometrium in the uterine cornu and adenomyosis was rendered.

The patient was discharged home in good condition and was doing well at one-month follow-up.

Discussion

A cavernous hemangioma of the uterus may either occur as an isolated lesion of the uterus ^{[1],[2],[3],[4],[5],[6]} or may be associated with pelvic or extra-pelvic hemangiomatosis. ^[7] There have been eight case reports that describe isolated involvement of the uterus in pregnant women and highlight the importance of uterine cavernous hemangioma as a rare but important cause of refractory uterine bleeding at the time of delivery. ^[1],[2] Uncommonly, they may be localized to a portion of the uterus and present in a non-pregnant woman with either uncontrolled vaginal bleeding ^[3],[4],[5] or unremitting pelvic pain (as in our case). Salm R ^[6] surveyed the literature for diffuse cavernous hemangioma of the uterus in the early part of the 20 ^th century. He recorded two cases of isolated and localized cavernous hemangioma of the uterus in non-pregnant multiparous women, who presented with uncontrolled vaginal bleeding and underwent hysterectomy. Recent literature documents only three published cases of cavernous hemangioma in non-pregnant women [Table - 1]. In two cases, the women were perimenopausal. In the third case, a 14-year-old female had to undergo an emergent hysterectomy for life-threatening bleeding. ^[5]

Based on a review of the literature and our own findings, it can be suggested that a cavernous hemangioma has no predilection for age and that its relationship to pregnancy is coincidental. It can further be theorized that localized forms of a cavernous hemangioma preexist and mainly go undetected during pregnancy or delivery. It is unknown whether this lesion begins localized and later becomes diffuse through a particular molecular pathway. Current treatment options poorly relieve the progressive symptoms of uterine bleeding and/or unremitting pelvic pain associated with a localized cavernous hemangioma. In pregnant women, close follow-up is recommended as it can increase the rate of successful vaginal and Cesarean section deliveries with retention of the uterus. ^[1],[2] In non-pregnant women, uterine artery embolization or internal artery ligation is conservatively recommended; however, the exact choice remains debated. In any patient not responding to conservative treatment, the possibility of a hysterectomy should be discussed.

The present case highlights an additional clinical symptom of pelvic pain that a non-pregnant patient may present with and involvement of adenomyotic foci. Since these cases can present with an uneventful obstetric history the clinical suspicion needs to be high as an early diagnosis and intervention can decrease future complications.

Conclusion

A localized cavernous hemangioma of the uterus is a rare lesion that may be clinically silent during pregnancy and may present with menorrhagia or unrelenting pelvic pain.

References

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