Article Data

  • Views 1062
  • Dowloads 181

Original Research

Open Access Special Issue

Systemic therapy is effective in the management of leiomyomatosis

  • Alannah Smrke1,2,*,
  • Alexandra Ostler1
  • Siraj Yusuf1
  • Aisha B Miah1
  • Khin Thway1,3
  • Cyril Fisher4
  • Desmond Barton1
  • Alexander Lyon5
  • Ian Judson3
  • Paul H Huang3
  • Robin L Jones1,3
  • Charlotte Benson1,*,

1Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, SW3 6JJ London, UK

2BC Cancer Vancouver Centre, Vancouver, BC V5Z4E6, Canada

3The Institute of Cancer Research, SM2 5NG London, UK

4Department of Pathology, University Hospitals Birmingham NHS Foundation Trust, B15 2GW Birmingham, UK

5The Royal Brompton Hospital, SW3 6NP London, UK

DOI: 10.22514/ejgo.2022.013 Vol.43,Issue 3,June 2022 pp.83-90

Submitted: 07 December 2021 Accepted: 29 January 2022

Published: 15 June 2022

(This article belongs to the Special Issue Rare Gynecological Malignancies)

*Corresponding Author(s): Alannah Smrke E-mail:
*Corresponding Author(s): Charlotte Benson E-mail:


Objective: Leiomyomatosis is an intermediate neoplasm of uterine origin. There is limited understanding of its natural history and response to systemic therapy. The objective of this retrospective review is to describe the management and response to systemic therapy for leiomyomatosis. Methods: A retrospective chart review was performed from 1 January 2000 – 1 February 2021 for patients 18 years of age or older with leiomyomatosis treated at The Royal Marsden Hospital. Descriptive statistics were performed. Results: Fifteen female patients with a median age of 45 years (range 34–49) were identified. Patients were ethnically diverse (Black n = 4 [26%], Asian n = 1 [7%], Caucasian n = 6 [40%], other n = 3 [20%], Unknown n = 1 [7%]). Most patients presented with advanced disease (n = 12/15, 80%); common sites being abdomen (n = 9/12, 75%), abdominal veins (n = 6/12, 50%), including the IVC 4/12 (30%). At presentation, two patients (17%) had cardiac extension from the IVC. Eleven patients (73%) underwent surgery and 4 (27%)active surveillance as first treatment. All patients undergoing surgery had residual disease (n = 9/9 for those with available data). Ten patients received systemic therapy for advanced disease (median 3 treatments, range 1–5) and most patients (n = 9/10, 90%) were treated with endocrine therapy. Response rate (RR) and clinical benefit rate (CBR) was higher for GNRH agonist (RR 43%, CBR 86%) and GNRH agonist + aromatase inhibitor (AI) (RR/CBR 100%), than AI alone (RR 0%, CBR 66%). Patients were only treated with non-endocrine systemic therapy after failure of endocrine therapy, and no responses were seen. No patients had confirmed malignant transformation; two patients had clinical suspicion of transformation but no correlative features on repeat biopsy. Conclusions: Leiomyomatosis is a rare, but morbid condition. The mainstay of treatment for advanced disease is endocrine therapy, the choice of initial approach requires careful consideration within a multi-disciplinary team.


leiomyomatosis; intra-vascular leiomyomatosis; soft tissue neoplasm; systemic therapy; aromatase inhibitor

Cite and Share

Alannah Smrke,Alexandra Ostler,Siraj Yusuf,Aisha B Miah,Khin Thway,Cyril Fisher,Desmond Barton,Alexander Lyon,Ian Judson,Paul H Huang,Robin L Jones,Charlotte Benson. Systemic therapy is effective in the management of leiomyomatosis. European Journal of Gynaecological Oncology. 2022. 43(3);83-90.


[1] Board WCoTE. Female Genital Tumours: WHO Classification of Tumours, 5th Edition, Volume 4. 5th edn. WHO: Lyon, France. 2020.

[2] Li B, Chen X, Chu Y, Li R, Li W, Ni Y. Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases. Interactive Cardiovascular and Thoracic Surgery. 2013; 17: 132–138.

[3] Ordulu Z, Chai H, Peng G, McDonald AG, De Nictolis M, Garcia-Fernandez E, et al. Molecular and clinicopathologic characterization of intravenous leiomyomatosis. Modern Pathology. 2020; 33: 1844–1860.

[4] Buza N, Xu F, Wu W, Carr RJ, Li P, Hui P. Recurrent chromosomal aberrations in intravenous leiomyomatosis of the uterus: high-resolution array comparative genomic hybridization study. Human Pathology. 2014; 45: 1885–1892.

[5] Clement PB, Young RH, Scully RE. Intravenous leiomyomatosis of the uterus. a clinicopathological analysis of 16 cases with unusual histologic features. The American Journal of Surgical Pathology. 1988; 12: 932–945.

[6] Nogales FF, Matilla A, Carrascal E. Leiomyomatosis peritonealis disseminata. An ultrastructural study. American Journal of Clinical Pathology. 1978; 69: 452–457.

[7] Williams LJ, Pavlick FJ. Leiomyomatosis peritonealis disseminata: two case reports and a review of the medical literature. Cancer. 1980; 45: 1726–1733.

[8] Yu X, Zhang G, Lang J, Liu B, Zhao D. Factors Associated with Recurrence after Surgical Resection in Women with Intravenous Leiomy-omatosis. Obstetrics and Gynecology. 2016; 128: 1018–1024.

[9] Wang J, Yang J, Huang H, Li Y, Miao Q, Lu X, et al. Management of intravenous leiomyomatosis with intracaval and intracardiac extension. Obstetrics and Gynecology. 2012; 120: 1400–1406.

[10] Liu J, Liang M, Ma G, Liu X, Cheng N, Cao D, et al. Surgical treatment for intravenous-cardiac leiomyomatosis. European Journal of Cardio-Thoracic Surgery. 2018; 54: 483–490.

[11] Takeda T, Masuhara K, Kamiura S. Successful management of a leiomyomatosis peritonealis disseminata with an aromatase inhibitor. Obstetrics and Gynecology. 2008; 112: 491–493.

[12] Judson I, Messiou C. Vitamin D deficiency in the pathogenesis of leiomyoma and intravascular leiomyomatosis: a case report and review of the literature. Gynecologic Oncology Reports. 2020; 35: 100681.

[13] Mitsuhashi A, Nagai Y, Sugita M, Nakajima N, Sekiya S. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report. The Journal of Reproductive Medicine. 1999; 44: 883–886.

[14] Nagashima M, Komiyama S, Yoshida T, Kimura Y, Sadamoto S, Saito A, et al. Intravenous leiomyomatosis successfully treated by multidisciplinary treatment including GnRH antagonist Relugolix: A case report. Medicine: Case Reports and Study Protocols. 2021; 2: e0034.

[15] Lin Y, Wei L, Shun C, Cheng A, Hsu C. Disseminated peritoneal leiomyomatosis responds to systemic chemotherapy. Oncology. 2009; 76: 55–58.

[16] Mizoguchi C, Matsumoto H, Nasu K, Arakane M, Kai K, Narahara H. Intravenous leiomyomatosis treated with radical hysterectomy and adjuvant aromatase inhibitor therapy. Journal of Obstetrics and Gynaecology Research. 2016; 42: 1405–1408.

[17] Eisenhauer EA, Therasse P, Bogaerts J, Schwartz LH, Sargent D, Ford R, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). European Journal of Cancer. 2009; 45: 228–247.

[18] Valdés Devesa V, Conley CR, Stone WM, Collins JM, Magrina JF. Update on intravenous leiomyomatosis: report of five patients and literature review. European Journal of Obstetrics, Gynecology, and Reproductive Biology. 2013; 171: 209–213.

[19] Rock CL, Pande C, Flatt SW, Ying C, Pakiz B, Parker BA, et al. Favorable changes in serum estrogens and other biologic factors after weight loss in breast cancer survivors who are overweight or obese. Clinical Breast Cancer. 2013; 13: 188–195.

[20] Campbell KL, Foster-Schubert KE, Alfano CM, Wang C, Wang C, Duggan CR, et al. Reduced-calorie dietary weight loss, exercise, and sex hormones in postmenopausal women: randomized controlled trial. Journal of Clinical Oncology. 2012; 30: 2314–2326.

[21] Bekkers RL, Willemsen WN, Schijf CP, Massuger LF, Bulten J, Merkus JM. Leiomyomatosis peritonealis disseminata: does malignant transformation occur? A literature review. Gynecologic Oncology. 1999; 75: 158–163.

[22] Barjot PJ, Refahi N, Berthet P, Delautre VD. Intravenous leiomyomatosis of the uterus: a GnRH agonist utilisation before surgery. Journal of Obstetrics and Gynaecology. 1998; 18: 492–493.

[23] Sparano JA, Gray RJ, Makower DF, Pritchard KI, Albain KS, Hayes DF, et al. Adjuvant Chemotherapy Guided by a 21-Gene Expression Assay in Breast Cancer. New England Journal of Medicine. 2018; 379: 111–121.

[24] Faubion SS, Kuhle CL, Shuster LT, Rocca WA. Long-term health consequences of premature or early menopause and considerations for management. Climacteric. 2015; 18: 483–491.

Abstracted / indexed in

Science Citation Index Expanded (SciSearch) Created as SCI in 1964, Science Citation Index Expanded now indexes over 9,500 of the world’s most impactful journals across 178 scientific disciplines. More than 53 million records and 1.18 billion cited references date back from 1900 to present.

Biological Abstracts Easily discover critical journal coverage of the life sciences with Biological Abstracts, produced by the Web of Science Group, with topics ranging from botany to microbiology to pharmacology. Including BIOSIS indexing and MeSH terms, specialized indexing in Biological Abstracts helps you to discover more accurate, context-sensitive results.

Google Scholar Google Scholar is a freely accessible web search engine that indexes the full text or metadata of scholarly literature across an array of publishing formats and disciplines.

JournalSeek Genamics JournalSeek is the largest completely categorized database of freely available journal information available on the internet. The database presently contains 39226 titles. Journal information includes the description (aims and scope), journal abbreviation, journal homepage link, subject category and ISSN.

Current Contents - Clinical Medicine Current Contents - Clinical Medicine provides easy access to complete tables of contents, abstracts, bibliographic information and all other significant items in recently published issues from over 1,000 leading journals in clinical medicine.

BIOSIS Previews BIOSIS Previews is an English-language, bibliographic database service, with abstracts and citation indexing. It is part of Clarivate Analytics Web of Science suite. BIOSIS Previews indexes data from 1926 to the present.

Journal Citation Reports/Science Edition Journal Citation Reports/Science Edition aims to evaluate a journal’s value from multiple perspectives including the journal impact factor, descriptive data about a journal’s open access content as well as contributing authors, and provide readers a transparent and publisher-neutral data & statistics information about the journal.

Submission Turnaround Time