Clinical characteristics and prognostic features of intravenous leiomyomatosis with inferior vena cava or intracardiac extension

Zhang, Guorui; Yu, Xin; Shi, Honghui; Fan, Qingbo; Lang, Jinghe; Liu, Bao

doi:10.1016/j.jvsv.2016.12.015

Cited by 26 publications

(17 citation statements)

References 22 publications

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“…Intravenous leiomyomatosis, a subtype of uterine leiomyoma, typically originates from the uterus and spreads along blood vessels, and may involve the heart in severe cases. 1 The condition is rare and is thought to occur in 0.4-1.97% of uterine leiomyomas. 3 Clinical manifestations of IVL are similar to uterine leiomyoma and right ventricular insufficiency.…”

Section: Discussionmentioning

confidence: 99%

“…Complete surgical excision is the standard therapy but recurrence can occur despite the use of adjuvant anti-estrogen therapy. 1 Currently there is no effective medical therapy that can be used in isolation or in combination with anti-estrogen therapy to prevent relapse or control recurrent disease after surgery. Here, we present a case of rapamycin, a mammalian target of rapamycin (mTOR) inhibitor, in treating recurrent IVL.…”

Section: Introductionmentioning

confidence: 99%

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Rapamycin (sirolimus) in treatment of recurrent intravenous leiomyomatosis: a case report

Zhang

Feng

Wang

et al. 2020

BJOG

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Rapamycin (sirolimus) in treatment of recurrent intravenous leiomyomatosis: a case report

Zhang

Feng

Wang

et al. 2020

BJOG

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“…[5] The knowledge of the lesion proliferation through the ovarian vessels is justified by the fact of a smaller number of metastases recorded after removal of the adnexa and gives the same hints on the appropriateness of removing adnexa to prevent relapse. Only Zhang et al [23] suggest that the scope of surgery (hysterectomy + bilateral salpingoophorectomy) is debatable and the issue remains to be clarified.…”

Section: Discussionmentioning

confidence: 99%

Natural history of leiomyomas beyond the uterus

et al. 2019

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Rationale: Most leiomyomas are located in the uterus. Leiomyomas are rarely found outside the uterus and classified as leiomyoma beyond the uterus (LBU). This group consists of disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis, parasite leiomyoma located in the broad ligament and retroperitoneal space. The descriptions of the patients who suffer from these types of leiomyomas are presented mainly in case reports. Patient concerns: A 34-year-old multiparous woman was operated on multiple recurrent uterine leiomyoma in parametrium. At one time, 32 leiomyomas were removed. Thirteen months following it, in next laparotomy, 132 leiomyomas were excised. Histologically, both were intravenous leiomyomas (IVLs). Diagnosis and Interventions: In follow-up, computed tomography (CT) and magnetic resonance imaging scans were performed to look for next recurrent leiomyoma. Accidentally, the mass was found in inferior vena cava which was diagnosed as intravenous vena cava leiomyoma. The mass was removed and the final diagnosis of intravenous myoma was confirmed in histopathology. Outcomes: CT scan performed 3 months after the surgery for leiomyoma in vena cava revealed no pathology. Next 10 months’ follow-up was uneventful. Lessons: The recurrent multiple uterine leiomyoma precede LBU. The uterine leiomyoma spreads intravenously route to parametria as parasite leiomyoma, then to vena cava. It has to be taken into account in follow-up.

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“…Treatment for ICLM varied because of its rarity and complexity, which included surgeries [12][13][14], anti-estrogen hormone therapies [9,12,15], and even a wait-andsee strategy [1,16]. Complete tumor resection was the most recommended [17,18], but sometimes it cannot be achieved due to the high risks of surgery [5,7,13,15,16,[19][20][21][22], patient's poor conditions [4,12,23,24], lack of medical resources [25], financial constraints [2,26], or patients' refusal [27,28].…”

Section: Introductionmentioning

confidence: 99%

The role of estrogen deprivation therapy in premenopausal women with primary unresectable intracardiac leiomyomatosis: a systematic review and meta-analysis

Liang

Lei

Xie

et al. 2021

Orphanet J Rare Dis

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Background Intracardiac leiomyomatosis (ICLM) is a rare life-threatening form of intravenous leiomyomatosis (IVLM). The incomplete resection and recurrence are associated with high morbidity and mortality. The objective of this study is to identify that whether estrogen deprivation therapies, including bilateral salpingo-oophorectomy (BSO)-based surgery and gonadotrophin releasing hormone agonists (GnRHa) administration, could bring benefits to patients with primary unresectable ICLM. Methods PubMed/MEDLINE (Ovid) was searched (up to May 2021) for studies reporting individual patient data on demographics, clinicopathological features, treatment, and follow-up information. Exclusion criteria were patients who may have been included in two or more publications. This study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results A total of 114 patients from 70 papers were included. Several reports showed that the tumor in the right atrium and inferior vena cava shrank dramatically after BSO-based surgery, or GnRHa administrated preoperatively in premenopausal women. The rate of complete resection was 64.04% in patients with ICLM, which was 85.25% in no/slight adhesion and no pulmonary nodules group, while 22.22% in firm/extensive adhesion and/or pulmonary nodules group (p < 0.0001). Meanwhile, the recurrence rates in patients with complete resection and incomplete resection were 4.29% and 37.84% respectively (p < 0.0001). Furthermore, complete resection with BSO had the lowest recurrence rate of 3.13%, incomplete resection with BSO had a progression rate of 45.45%, while incomplete resection with ovarian preservation had the highest progression rate of 75.00%. Conclusions The recurrence rate of ICLM was closely related to firm/extensive adhesion in IVC or above, and/or pulmonary nodules. BSO-based surgery might reduce the recurrence rate no matter ICLM could be completely resected or not. In addition, estrogen deprivation therapies could decrease tumor burden as a primary treatment, and further make a secondary complete resection feasible in premenopausal women with initially unresectable ICLM.

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Clinical characteristics and prognostic features of intravenous leiomyomatosis with inferior vena cava or intracardiac extension

Cited by 26 publications

References 22 publications

Rapamycin (sirolimus) in treatment of recurrent intravenous leiomyomatosis: a case report

Rapamycin (sirolimus) in treatment of recurrent intravenous leiomyomatosis: a case report

Natural history of leiomyomas beyond the uterus

The role of estrogen deprivation therapy in premenopausal women with primary unresectable intracardiac leiomyomatosis: a systematic review and meta-analysis

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