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Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures
  1. Philip Cowie,
  2. Ben Eastwood,
  3. Sarah Smyth and
  4. Hooman Soleymani majd
  1. Department of Gynaecological Oncology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  1. Correspondence to Hooman Soleymani majd; hooman.soleymani{at}


Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

  • gynecological cancer
  • vascular surgery

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Intravascular (intravenous) leiomyomatosis (IVL) is a benign and rare smooth muscle tumour of the uterine myometrium, which grows within vascular channels but does not invade the surrounding tissue.1–3 IVL growth is often observed in the uterine veins and has been reported to extend into the pelvic veins, inferior vena cava and right-sided heart chambers.4–6 The aetiology of IVL remains unresolved.3 6 7 On one hand, IVL pathology has rarely been reported in the absence of uterine fibroids and is proposed to originate from fibroid invasion of vascular structures.8 9 Recent RNA sequencing data support a possible common origin for IVL and uterine fibroids, though distinct molecular features were present.10 Conversely, case reports exist in which IVL pathology is observed in the absence of uterine fibroids and with primary origins in vascular structures as opposed to the uterus, suggesting direct growth from the venous wall.11 The majority of cases to date (<400) have been described in premenopausal women with a history of fibroids4; age at presentation varies significantly12 as do symptoms that may include menstrual abnormalities, bloating or syncope, dyspnoea and oedema when cardiac extension is present.7 13 As IVL remains a rare diagnosis, no guidelines or consensus exists as to the key diagnostic features or surgical approach to this pathology.

This case widens the possible scope of IVL presentations and offers an insight to surgical techniques aimed at providing a curative outcome without significant postoperative morbidity in the presence of extensive pelvic growth. Our patient’s presentation was highly atypical and novel among published reports. Extension to the pelvic sidewall is more commonly seen with leiomyosarcoma, which was a reasonable preoperative diagnostic conclusion given the features of a smooth muscle tumour on hysteroscopic biopsy. However, it must now be noted that presentation of IVL may be more varied than previously thought, with the absence of intracaval and/or intracardiac extension not ruling out advanced IVL disease. Additionally, we demonstrate that surgical resection of pelvic masses extending to the pelvic sidewall can be achieved with clear margins and preservation of critical neurovascular structures. In this case, we undertook a modified laterally extended endopelvic resection (LEER)14 15 approach following radical hysterectomy and bilateral salpingo-oophorectomy. Careful identification and skeletonisation of key neural structures including the obturator, pudendal and lumbosacral nerves allowed for complete removal of the extensive pelvic mass along with the internal iliac vessels without postoperative neurological morbidity. This approach ultimately resulted in a curative operation for a patient who, prior to surgery, was undergoing a palliative procedure for intractable stage IV leiomyosarcoma.

Case presentation

We present the case of a 77-year-old gravida 4, para 3 female patient who attended her General Practioner with a 2–3-month history of vague lower abdominal pain, diarrhoea and urinary frequency. She had identified a pelvic mass but could not recall how long for. She reported no vaginal or rectal bleeding. Her medical history includes diverticular disease, hypercholesterolaemia and hypertension, currently treated with amlodipine 5 mg and atorvastatin 20 mg. Gynaecological history comprises 10 years of combined hormone replacement therapy following her menopause around 50 years of age. There have been no abnormal cervical screens. Her only previous surgical interventions were midline C-section and appendicectomy, both in her twenties. Family history could not be elucidated as the patient was adopted. She is a non-smoker with a body mass index of 28.

Investigations were requested through the 2-week wait referral pathway. Ultrasound identified uterine and right adnexal masses measuring 8 cm and 4.2 cm, respectively (figures 1 and 2). The uterine mass was noted to be heterogeneous, predominantly solid with ill-defined margins and diffuse vascularity via colour Doppler, while the right adnexal mass was solid and well defined (figure 3). CA-125 was low at 18 U/mL. Pelvic MRI showed no pelvic lymphadenopathy but confirmed the presence of a contiguous mass with aggressive spread into the right ovarian vein (figure 4). Chest CT imaging showed indeterminate pulmonary nodules (figure 5). Hysterosopic biopsy was conducted to aid diagnosis—the histology reported cellular smooth muscle cells staining positive for desmin, actin, oestrogen receptor (ER) and progesterone receptor (PR) and negative for B cell development marker (CD) 10, with a raised mindbomb E3 ubiquitin protein ligase 1. It was suggested that, although the criteria for leiomyosarcoma were not met, it could not be ruled out based on biopsy samples. Gynaecological oncology and sarcoma multidisciplinary team (MDT) discussions concluded that appearances were consistent with a stage IV leiomyosarcoma with recommendation for urgent palliative surgical resection for symptom management.

Figure 1

Transvaginal ultrasound scan demonstrating a predominantly solid heterogeneous area within the uterus with ill-defined margins measuring 8 cm in length.

Figure 2

Transvaginal ultrasound scan of the right adnexa demonstrating a solid well-defined 4.2 cm lesion.

Figure 3

Transvaginal ultrasound scan of the uterus with diffuse vascularity on application of colour Doppler.

Figure 4

MRI pelvis T2-weighted coronal section demonstrating a grossly abnormal uterus. A lobular mass replaces the normal myometrial tissue at the fundus, extending through the serosa into the right adnexa. Some cystic degeneration is noted. The right ovary is not seen separately from these masses, and the right adnexal mass extends superiorly along the right ovarian vein.

Figure 5

CT chest with contrast demonstrating a 6 mm apical segment right lower lobe subpleural nodule.

The patient underwent midline laparotomy with findings of a large uterine tumour extending into the right pelvic sidewall, engulfing both internal iliac vessels, abutting the external iliac artery and penetrating into the obturator fossa. The mass lay in close proximity to the pelvic nerves and was also engulfing the right gonadal vessels, extending to the insertion of the right ovarian vein. Tumour mobilisation was achieved with tunnelling of the ureters within the parametrium and anterior colpotomy. En bloc radical hysterectomy and bilateral salpingo-oophorectomy was completed with retrograde Hudson technique. Intraoperative findings were in keeping with an aggressive but resectable tumour, leading to the decision to proceed with LEER to achieve complete margins. The right internal iliac vessels were secured and divided both proximally and distal to the tumour to achieve full vascular control in anticipation of blood loss. The lumbosacral trunk (L4 and L5 and S2, S3 and S4), pudendal nerve, obturator nerve and hypogastric plexus were skeletonised and preserved from the level of the individualised sacral nerve roots, with development of dissection planes and tunnelling using cold scissors and avoiding the use of bipolar diathermy (figures 6 and 7). The total estimated blood loss was 4 litres.

Figure 6

Intraoperative images following modified laterally extended endopelvic resection demonstrating neurovascular structures of the pelvis.

Figure 7

Intraoperative images following modified laterally extended endopelvic resection demonstrating neurovascular structures of the pelvis.

Outcome and follow-up

The patient was admitted to the high-dependency unit (Churchill Overnight Recovery Unit) postoperatively and escalated to the intensive care unit due to hypotensive episodes requiring metaraminol infusion. She received patient-controlled analgesia and prophylactic low-molecular-weight heparin (LMWH) as per protocol. Oxygen requirements increased postoperatively but were gradually weaned subsequent to this. Postoperative inpatient complications included a urinary tract infection and small bilateral pulmonary emboli managed with antibiotics and treatment dose LMWH, respectively. Neurological examination of the lower limbs showed an impressive preservation of function as detailed in figure 8. By day 5, the patient was mobilising with physiotherapy support, with successful trial without catheter on day 7. The patient was discharged on day 10 with follow-up care between gynaecology clinic, thrombosis clinic and specialist lung nodule MDT in view of the primary pathology, postoperative emboli and CT findings, respectively.

Figure 8

A timeline of significant postoperative events, investigations and milestones. CTPA, computed tomography pulmonary angiogram; ITU, intensive care unit; TWOC, trial without catheter; UTI, urinary tract infection.

Due to the association between IVL and right atrial fibroids, a follow-up echocardiogram was requested and reported as normal. She will be under annual surveillance and review by the gynae-oncology team. Secondary prevention for thrombosis was based on local guidelines with haematology review at 3 months, when she was switched to apixaban. Consideration to stopping anticoagulation will be given by haematology after 12 months post-op. The lung MDT confirmed that the lung nodules were not in keeping with malignancy but were not amenable to biopsy. They have booked a follow-up CT scan at 12 months.

Over a month post discharge, the patient developed stress-predominant mixed urinary incontinence and back pain. Urology identified enterococcus growth that resolved with nitrofurantoin treatment, but the symptoms remained. Regular pelvic floor exercises did not improve the symptoms. Speculum examination showed completely healed wound and no evidence of fistulae or other abnormalities. Postvoid residual volume was 12 mL. Flexible cystoscopy and CT scan of the kidneys, ureters and bladder did not identify pathology. MRI spine at 7 months post-op demonstrated severe central canal stenosis compressing L5 nerve roots. A cause has not yet been established for the incontinence, but urogynaecology and spinal appointments are awaited.

Postoperative histology revealed an unexpected diagnosis of IVL. Macroscopically, the uterine mass was multinodular and dominating the myometrium, containing widespread haemorrhages and extending into the broad ligament as displayed in figure 9.

Figure 9

Macroscopic images of excised lesions. Left: the uterus with leiomyomatous nodules; right: leiomyomatous nodules within the broad ligament (direct extension).

Microscopically, the tumour was composed of multiple, well-circumscribed nodules of varying sizes made up of uniform elongated cells with fusiform nuclei (figure 10). Inclusions were noted in numerous nuclei. Nuclear pleomorphism was absent, and the mitotic activity was estimated at 5 per 10 high-power fields (4/1 mm2). Some focal degenerative changes were observed, but coagulative tumour cell necrosis was absent. Nodules composed of similar cellular material were found intravascularly and extending into the broad ligament (figure 11). The right pelvic lymph nodes did not display any tumorigenic changes.

Figure 10

Microscopic image of excised leiomyomatous nodule within the uterus. H&E staining shows uniform, elongated cell morphology and focal degenerative changes.

Figure 11

Microscopic image of excised leiomyomatous lesion within a blood vessel. H&E staining shows a highly similar composition to biopsy samples from the uterus, confirming the extension of the leiomyomatous pathology to local vascular sites.

Immunohistochemistry showed strong positivity for all smooth muscle markers (desmin, actin and caldesmon) and negative staining with CD10. ER staining was positive (7/8) and PR staining also positive (8/8). Staining for cyclin D1 was also positive. This immunohistological profile confirms the leiomyomatous nature of the lesion. No features of malignancy were identified.


This case extends the current understanding of the rare pathology IVL. It highlights the possibility of presentations mimicking leiomyosarcoma, in which the tumour mass may extend significantly within the pelvis in the absence of advancement to intracaval or intracardiac spaces. This creates a degree of diagnostic uncertainty preoperatively and suggests that additional diagnostic features are required for recognition of IVL pathology. Furthermore, surgical procedures for IVL are not standardised, and in cases of significant pelvic disease, we have outlined an approach that provides optimal functional outcomes with curative potential. This is also valuable in the context of other pelvic tumours, such as leiomyosarcoma, which may also extend to the pelvic sidewall and obturator fossa.

A number of the preoperative investigation findings in this case pointed towards a diagnosis of leiomyosarcoma. The histological analysis was supportive of leiomyosarcoma, although not all criteria were met. Features such as cellular atypia and coagulative tumour cell necrosis may distinguish IVL from leiomyosarcoma.7 16 However, it is worth noting that the absence of these characteristics does not rule out leiomyosarcoma; it merely indicates that the biopsy may not be fully representative of the mass. The presence of lung nodules and the extension of the primary mass to the pelvic sidewall were both highly suggestive of leiomyosarcoma. Furthermore, our patient’s history is not typical of IVL, having had no history of uterine fibroids and being postmenopausal—features present in most IVL case reports other than a previous notable exception.17 The importance of a distinction between leiomyosarcoma and IVL preoperatively should not be understated. The patient in this case was consented to undergo a palliative procedure with the knowledge that her disease was incurable; however, complete resection of IVL has a low recurrence rate and good long-term survival,6 12 18 which is a markedly different prognosis.

The most effective treatment for IVL is complete surgical resection, which can be greatly complicated by the extension of the mass into vascular sites such as pelvic vessels and more distally to the heart. Unfortunately, the insidious development of IVL often results in extensive disease being found at diagnosis.5 This has led to discussion and consideration of one-step and two-step surgeries often involving cardiothoracic input in published cases.19–21 However, in the case presented here, the highly unusual IVL extension to the pelvic sidewall and obturator fossa presented a unique challenge for complete resection of the mass while sparing vital neurovascular structures and avoiding significant postoperative morbidity.

In the presented case, our surgical approach was initially designed as a debulking procedure to provide symptomatic relief to the patient. Intraoperative findings revealed an aggressive tumour, and a modified LEER procedure was undertaken. By ensuring that neurovascular structures were identified prior to committing to excision, it was possible to ensure resectability of the tumour without significant morbid sequelae. LEER is a novel surgical salvage technique, which aims to completely resect tumours extending to, and fixing on, the pelvic sidewall.14 15 LEER requires at least two of total mesorectal excision, total mesometrial resection and total mesovesical resection. In addition, pelvic floor muscles and the internal iliac vessels may be resected for complete removal of the tumour mass. This approach allowed complete removal of the IVL with no residual pathology. Ultimately, this constituted a modified version of a LEER as no pelvic floor muscles were removed. Significant morbidity can be realised with resection of extensive pelvic masses as the anatomy of pelvic nerve structures is complex and damage to the pudendal, lumbosacral or obturator nerves risks a range of debilitating symptoms such as urinary and faecal incontinence, paraesthesia of the external genitalia and perineum and functional impairment of numerous muscles of the leg and foot. In order to limit damage to the pelvic nerve structures, a great deal of consideration and care is required including extensive knowledge of ontogenetic and pelvic neural anatomy. The nerve-sparing approach employed in this case offers an opportunity to reduce morbidity following LEER procedures and variants thereof. We highlight that the patient in this case remains not only disease-free but also retains function of her urogenital tract and lower limbs following the advanced surgical procedure performed.

Despite being a rare disease, it is clear that the presentation of IVL may vary significantly and in some cases mimic other more common gynaecological cancers. As the literature expands on this pathology, it is important to evaluate how clear diagnoses can be made in a timely manner. Once IVL pathology reaches advanced stages, surgery may become highly challenging both due to pelvic and distant (eg, cardiac) extension. It would therefore be valuable to further define diagnostic criteria with a focus on identifying discriminating presenting symptoms for earlier detection. Furthermore, there is lack of medical treatments for patients with incomplete or inoperable IVL pathology; the use of anti-oestrogenic therapy has been described previously,22 but evidence for the efficacy of these treatments is lacking.12 Such targeted therapeutics would also be highly valuable in cases such as the one presented here to limit progression of possible distant lung metastases. Beyond diagnosis and intervention, there is a lack of guidelines on the long-term follow-up of these patients. In particular, it will be necessary to outline the need for, and frequency of, cardiology and gynaecology follow-up for those patients in whom complete resection cannot be achieved, and recurrence is likely.

Patient’s perspective

I started feeling poorly roughly 2 years ago, having pains in my groin, went to see a doctor but just gave me pain relief. A year passed and I went to stay with our granddaughter during Covid lockdown. I felt the pain getting increasingly worse, so I made an appointment to see my GP, and she sent me to the hospital for an ultrasound, and it all started from there. I Had MRI scans to finally get a diagnosis, not personally to me but to my husband and daughter, and was told I had terminal cancer and would live until April 2021. Leading up to the operation, I was very anxious and had to take beta-blockers to get through the day. I was then introduced to my consultant (my saviour) and started to hope that I’d be ok as I was in excellent hands. He explained what he was going to do; I remember saying to him ‘do whatever you need to do I am in your hands which I trust’. I had excellent daily care, the staff were excellent and my consultant came every day with his team to see how I was. At my postoperative appointment with my consultant, he gave me the best news I’d ever had—it wasn’t cancer. The recovery has been slow, but day by day, I’m getting better. I would like to take this opportunity to say how grateful and blessed I am to have had the best surgeon and team and thank you for saving my life.

Learning points

  • Intravascular leiomyomatosis cases are rare, though it has been suggested it should be considered in women with a history of fibroids and intravascular filling defects. This case suggests that the presentation and patient history may be more varied than previously thought.

  • Careful consideration of the pelvic neural structures can facilitate surgical removal of pelvic masses, including those adherent to the pelvic sidewall, without the loss of critical nerve functions.

  • The distinction between IVL and leiomyosarcoma from limited biopsy samples may be challenging with huge implications for a patient’s prognosis.

Ethics statements

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  • PC and BE contributed equally.

  • Contributors PC and BE performed literature review and primary composition of manuscript. SS performed senior review and provided clinical patient care. HSM proposed the article, performed senior review and provided clinical patient care.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.