Different surgical strategies of patients with intravenous leiomyomatosis

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Abstract

Intravenous leiomyomatosis (IVL) is a rare benign tumor. The study aimed to assess outcomes of patients treated surgically for IVL.

Between November 2002 and January 2015, 76 patients were treated for IVL. The stage of IVL was evaluated preoperatively by echocardiography and enhanced computerized tomography (CT) scan, and graded into 4 stages according to intravascular tumor progression. We recorded age, lower limb edema before surgery, surgical parameters, and hospitalization expenses. Patients were followed up every 6 months and tumor recurrence was assessed by CT and ultrasound. Patients were followed up for a mean of 4.5 ± 2.5 years (range 1–13 years) and there was no operative, hospital, or long-term mortality or were lost to follow-up.

The rate of lower extremity edema, amount of blood loss, postoperative transfusion, length of intensive care unit (ICU) stay, postoperative hospitalization, and hospitalization expenses differed significantly between patients at different presurgery stages. Tumors recurred in 4 of 7 patients with stage I IVL that opted for surgery that preserved the ovaries and uterus. No recurrence was observed in patients graded stage II or more, in all of which the uterus and ovaries were removed. Recurrence was observed in only 4 of 76 cases of IVL, all of whom opted for surgery that spared the ovaries and uterus.

Different surgical strategies should be decided based on the staging to completely remove the tumor and ensure the safety of patients. Removal of both ovaries is necessary for inhibiting tumor growth and avoiding recurrence.

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Most cited references 26

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Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases.

Bin Li, Xin-Xin Chen, Ya-Dong Chu … (2013)

Intracardiac leiomyomatosis is rare but has been increasingly reported in recent years. Owing to its rarity, intracardiac leiomyomatosis has been reported only as isolated case reports and case series. This disorder is thought to be underestimated and easily overlooked in the clinic, while it is dangerous owing to the risk of sudden death caused by total outflow tract obstruction. We performed an electronic literature search for intracardiac leiomyomatosis and identified 194 cases that were reported in English from 1974 (the first reported case) to September 2012. Our aim is to provide a detailed and comprehensive review of the clinical presentation, diagnosis, histopathological characterization, treatment and prognosis of this disorder. According to our analysis, intracardiac leiomyomatosis is most common in the fifth decade, and the mean age of detection is ~50 years. Most patients had undergone previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma when admitted. The most common clinical presentations were dyspnoea, syncope, oedema of the lower extremities and palpitation. Transoesophageal echocardiography, computed tomography and magnetic resonance imaging are helpful in the preoperative diagnosis and to guide the surgical management. Complete removal guarantees an excellent outcome, with no recurrence or postoperative death, while incomplete removal leads to recurrence in one-third of patients. Anti-oestrogen therapy is not imperative after incomplete removal owing to its inability to prevent recurrence.

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Intravenous leiomyomatosis of the uterus: a clinicopathologic study of 18 cases, with emphasis on early diagnosis and appropriate treatment strategies.

Jing Du, Donghui Guo, Huidong Li … (2011)

Intravenous leiomyomatosis is a rare variant of leiomyoma that could result in death. Early and accurate diagnosis and appropriate treatment strategies play a dominant role in good prognosis. Eighteen cases of Intravenous leiomyomatosis , along with clinicopathologic data, were retrieved from our database. Most of the patients who ranged in age from 33 to 54 years (median, 44 years) presented with a pelvic mass or abnormal uterine bleeding. The diagnosis was confirmed by a immunohistochemical staining for smooth muscle actin, CD34, and Ki67. Surgical exploration confirmed the presence of a uterine mass (mean size, 5.08 cm). Wormlike plugs were identified within the broad ligament in 5 cases. The tumor penetrated to the inferior vena cava in 1 case. Histologic variants were noted in 33.33% (6/18) of our cases, which were classified as cellular intravenous leiomyomatosis (3 cases) and intravenous leiomyomatosis with papillary-like contour (1 case) and with fat metaplasia (2 cases). The 18 cases are made up 0.097% of all genital smooth muscle tumor cases of the hospital. The ratios of intravenous leiomyomatosis with uterine leiomyoma, with adenomyosis, with uterine leiomyoma and adenomyosis were 38.89% (7/18), 11.11% (2/18), and 27.78% (5/18), respectively. Follow-up information was available for 16 patients, with a follow-up duration of 26 to 104 months (mean, 55 months). Three cases (16.67%) recurred in patients younger than 40 years (33, 34, and 37 years). We propose that young patients undertake hysterectomy and unilateral salpingo-oophorectomy if they do not have any birthing requests. The cases of intravenous leiomyomatosis were underestimated because early diagnosis was easily missed. It is important to adequately sample all uterine leiomyomas and carefully examine the soft tissue on either side of the lower uterine segment below the peritoneal reflection to identify early-stage intravenous leiomyomatosis. Copyright © 2011 Elsevier Inc. All rights reserved.

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Intravenous Leiomyomatosis: An Unusual Intermediate between Benign and Malignant Uterine Smooth Muscle Tumors

Zehra Ordulu, Marisa R Nucci, Paola Dal Cin … (2016)

Intravenous leiomyomatosis is an unusual smooth muscle neoplasm with quasi-malignant intravascular growth but a histologically banal appearance. Herein, we report expression and molecular cytogenetic analyses of a series of 12 intravenous leiomyomatosis cases to understand better the pathogenesis of intravenous leiomyomatosis. All cases were analyzed for expression of HMGA2, MDM2 and CDK4 proteins by immunohistochemistry based on our previous finding of der(14)t(12;14)(q14.3;q24) in intravenous leiomyomatosis. Seven of 12 (58%) intravenous leiomyomatosis cases expressed HMGA2, and none expressed MDM2 or CDK4. Co-localization of hybridization signals for probes from the HMGA2 locus (12q14.3) and from 14q24 by interphase fluorescence in situ hybridization (FISH) was detected in a mean of 89.2% of nuclei in HMGA2-positive cases by immunohistochemistry, but in only 12.4% of nuclei in negative cases, indicating an association of HMGA2 expression and this chromosomal rearrangement (p=8.24×10−10). Four HMGA2-positive cases had greater than two HMGA2 hybridization signals per cell. No cases showed loss of a hybridization signal by interphase FISH for the frequently deleted region of 7q22 in uterine leiomyomata. One intravenous leiomyomatosis case analyzed by array comparative genomic hybridization revealed complex copy number variations. Finally, expression profiling was performed on three intravenous leiomyomatosis cases. Interestingly, hierarchical cluster analysis of the expression profiles revealed segregation of the intravenous leiomyomatosis cases with leiomyosarcoma rather than with myometrium, uterine leiomyoma of the usual histological type, or plexiform leiomyoma. These findings suggest that intravenous leiomyomatosis cases share some molecular cytogenetic characteristics with uterine leiomyoma, and expression profiles similar to that of leiomyosarcoma cases, further supporting their intermediate, quasi-malignant behavior.

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Author and article information

Journal

Journal ID (nlm-ta): Medicine (Baltimore)

Journal ID (iso-abbrev): Medicine (Baltimore)

Journal ID (publisher-id): MEDI

Title: Medicine

Publisher: Wolters Kluwer Health

ISSN (Print): 0025-7974

ISSN (Electronic): 1536-5964

Publication date Collection: September 2016

Publication date (Electronic): 16 September 2016

Volume: 95

Issue: 37

Electronic Location Identifier: e4902

Affiliations

[a ]Department of Cardiac Surgery

[b ]Department of Vascular Surgery

[c ]Department of Gynecology

[d ]Department of Liver Surgery

[e ]Department of Surgery, Peking Union Medical College Hospital

[f ]School of Medicine, Tsinghua University, Beijing, China.

Author notes

[∗ ]Correspondence: Guotao Ma, Department of Cardiac Surgery, Peking Union Medical College Hospital, Beijing 100730, China (e-mail: maguotao2000@ 123456163.com ).

Article

Accession ID: 04902

DOI: 10.1097/MD.0000000000004902

PMC ID: 5402609

PubMed ID: 27631266

SO-VID: 78586dc1-10f9-43dc-b16c-cdbf58b30ab5

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially. http://creativecommons.org/licenses/by-nc/4.0

History

Date received : 22 June 2016

Date revision received : 24 August 2016

Date accepted : 25 August 2016

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Keywords: intravenous leiomyomatosis,surgical strategies,uterine fibroids

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Keywords: intravenous leiomyomatosis, surgical strategies, uterine fibroids

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