A Case Series of Intravenous Leiomyomatosis and Literature Review W. Catarina Ang, Karen Kong, Rebecca Szabo The Royal Women’s Hospital Melbourne, Australia

Intravenous leiomyomatosis (IVL)

•  histologically benign smooth muscle tumor which intravascularly extends into uterine and systemic veins, in the absence of, or beyond confines of a leiomyoma1

•  tumor growth into venous system of myometrium and parametrium- 80%,

•  cardiac involvement- 10% to 40%, •  haematogenous spread to the lung has been

described2 •  ~300 cases described in literature, more in recent

years

A case series at the RWH

•  Retrospective review of IVL diagnosed on postoperative histopathology with IVL in <12 month period at RWH (2015-2016)

•  A literature review of the current management of this rare disease

Clinical symptoms

•  reproductive age usu Caucasian and rarely post-menopausal1,3

•  clinical sx vary according to disease

•  usu. dx late/advanced/post-mortem

•  pelvic mass •  abnormal uterine bleeding, pelvic pain & abdominal distention3

•  intracardiac involvement •  sx of RHF: congestive heart failure, dyspnoea, syncope1,3

A diagnostic challenge

•  Rare •  IVL resembles uterine fibroids macroscopically &

microscopically •  Dx commonly made on histopathological

examination1 •  Requires high clinical suspicion •  Requires more than a pelvic US to diagnosis tumor

growth into systemic venous system •  CT scan and MRI- projections into the vasculature •  cardiac echography- intracardiac involvement

Patient demographics

Patient N= 9

Symptoms menorrhagia

Mean age (yrs) Range (yrs)

43 38- 50

Pre-op imaging One patient had ultrasound & MRI features suggestive of atypical cellular fibroid

Operation

TAH 4

VH 1

TLH 1

Laparoscopic myomectomy

2

Hysteroscopic resection of fibroid

1

Intra-op and histology

•  suspicion of IVL was raised in only ONE of the 9 cases

•  Histopathology revealed venous invasion and extension beyond the tumor mass from which IVL originates

MRI view of IVL

3.9 cm mass within the posterior wall of the myometrium demonstrates some atypical features (irregular margins, high T2 signal intensity) with overall imaging features suggestive of an atypical (? Cellular) fibroid.

Treatment

•  Pathogenesis unclear •  From uterine tumour into the venous vasculature à disruption of

endothelium? •  Or from smooth muscles within vasculature?

•  Hysterectomy is recommended treatment of choice with resection of extra-uterine tumor when feasible3

•  Case reports •  improvement in symptoms with use of medications that decrease

oestrogen levels eg. GnRH agonists, aromatase inhibitors Bodner-Adler B, Bartl M, Wagner G. Intravenous leiomyomatosis of the uterus with pulmonary metastases or a case with benign

metastasizing leiomyoma? Anticancer Res. 2009;29(2):495. Biri A, Korucuoglu U, Zumrutbas N, Tiras B, Guner H. Intravenous leiomyomatosis treated with aromatase inhibitor therapy. Int J

Gynaecol Obstet. 2008;101(3):299.

Patient counselling

•  Hysterectomy once family completed

•  +/- BSO? No evidence

•  Baseline CT chest/abdo/pelvis

•  Education of signs/symptoms of IVL

spread and purpose of surveillance

Follow up

•  IVL recurrence has been reported 7 m to 15 yrs after hysterectomy

•  Detachment of IVL & haematogenous spread to the lung

•  Interval CT scan of chest, abdomen & pelvis in 2 years & 5 years to detect any intravascular recurrence in the future

•  Potentially lifelong

Is IVL more common than we thought?

Conclusions

•  IVL is (still) a rare condition •  can be a diagnostic challenge •  a high clinical suspicion for recurrence •  long term follow up required

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References 1 Ip PP, Tse KY, Tam KF. Uterine smooth muscle tumors other than the ordinary leiomyomas and

leiomyosarcomas: a review of selected variants with emphasis on recent advances and unusual morphology that may cause concern for malignancy. Adv Anat Pathol. 2010;17(2):91.

2 Fasih N, Prasad Shanbhogue AK, Macdonald DB, Fraser-Hill MA, Papadatos D, Kielar AZ, Doherty GP, Walsh C, McInnes M, Atri M. Leiomyomas beyond the uterus: unusual locations, rare manifestations. Radiographics. 2008;28(7):1931.

3 Vaquero ME, Magrina JF, Leslie KO. Uterine smooth-muscle tumors with unusual growth patterns. J Minim Invasive Gynecol. 2009;16(3):263.

4 Bodner-Adler B, Bartl M, Wagner G. Intravenous leiomyomatosis of the uterus with pulmonary metastases or a case with benign metastasizing leiomyoma? Anticancer Res. 2009;29(2):495.

5 Biri A, Korucuoglu U, Zumrutbas N, Tiras B, Guner H. Intravenous leiomyomatosis treated with aromatase inhibitor therapy. Int J Gynaecol Obstet. 2008;101(3):299.

6 Jonathan Rassi, E. Rene Rodriguez, Carmela D. Tan, Shetal N. Shah .Radiological Case: Intravenous leiomyomatosis. Appl Radiol. October 09, 2014

7 Victoria Valde´ s Devesa, Christopher R. Conley, William M. Stone, Joseph M. Collins, Javier F Update on intravenous leiomyomatosis: report of five patients and literature review. European Journal of Obstetrics & Gynecology and Reproductive Biology journal 171 (2013) 209–213