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Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

ABSTRACT: Budd-Chiari syndrome (BCS) is a rare intrahepatic vascular disease that is characterized by a hepatic venous outflow obstruction. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma. Here, we report a case of BCS that was caused by intracaval ILs. A woman presented to the emergency department (ED) with abdominal distension that had gradually progressed over a period of 3 years. Bedside ultrasonography and contrast-enhanced computed tomography (CECT) showed a large ascites and pelvic mass. The mass continued to the inferior vena cava and the right atrium. The intracaval mass was obstructing the left and middle hepatic veins. We established a tentative diagnosis of BCS caused by intracaval ILs and attempted surgical resection. Complete resection of the intracaval mass failed because of adhesion; however, she was discharged from the hospital without any postoperative complications. After 3 months, a pelvic ultrasonography showed a recurrence of a 4 × 3 cm pelvic mass. The mass size increased to 6 cm after 30 months. ILs can cause secondary BCS and can lead to life-threatening conditions. Owing to its extreme rarity, early detection in the ED is challenging. Bedside ultrasonography and CECT can enable the early recognition of BCS by ILs.

SUBMITTER: Park SY

PROVIDER: S-EPMC7764919 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

Park Sin-Youl SY Yeo In Hwan IH Kim Yun Jeong YJ Kim Jong Kun JK

Medicina (Kaunas, Lithuania) 20201214 12

Budd-Chiari syndrome (BCS) is a rare intrahepatic vascular disease that is characterized by a hepatic venous outflow obstruction. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma. Here, we report a case of BCS that was caused by intracaval ILs. A woman presented to the emergency department (ED) with abdominal distension that had gradually progressed over a period of 3 years. Bedside ultrasonography and contrast-enhanced computed tomography (CECT) showed a large ascites and pelv ...[more]

PMID: 33327445

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Project description:Intravenous leiomyomatosis (IVL) is an unusual uterine smooth muscle proliferation that can be associated with aggressive clinical behavior despite a histologically benign appearance. It has some overlapping molecular characteristics with both uterine leiomyoma and leiomyosarcoma based on limited genetic data. In this study, we assessed the clinical and morphological characteristics of 28 IVL and their correlation with molecular features and protein expression, using array comparative genomic hybridization (aCGH) and Cyclin D1, p16, phosphorylated-Rb, SMARCB1, SOX10, CAIX, SDHB and FH immunohistochemistry. The most common morphologies were cellular (n = 15), usual (n = 11), and vascular (n = 5; including 3 cellular IVL showing both vascular and cellular features). Among the immunohistochemical findings, the most striking was that all IVL showed differential expression of either p16 or Cyclin D1 in comparison to surrounding nonneoplastic tissue. Cytoplasmic phosphorylated-Rb was present in all but one IVL with hyalinization. SMARCB1, FH, and SDHB were retained; S0X10 and CAIX were not expressed. The most common genetic alterations involved 1p (39%), 22q (36%), 2q (29%), 1q (25%), 13q (21%), and 14q (21%). Hierarchical clustering analysis of recurrent aberrations revealed three molecular groups: Groups 1 (29%) and 2 (18%) with associated del(22q), and Group 3 (18%) with del(10q). The remaining IVL had nonspecific or no alterations by aCGH. Genomic index scores were calculated for all cases and showed no significant difference between the 14 IVL associated with aggressive clinical behavior (extrauterine extension or recurrence) and those without (median scores 5.15 vs 3.5). Among the 5 IVL associated with recurrence, 4 had a vascular morphology and 3 had alterations of 8q. Recurrent chromosome alterations detected herein overlap with those observed in the spectrum of uterine smooth muscle tumors and involve genes implicated in mesenchymal tumors at different sites with distinct morphological features.

Project description:BackgroundExtra-pelvic intravenous leiomyomatosis (IVL) extending into inferior vena cava (IVC) or heart (i.e. intracardiac leiomyomatosis, ICL) is an extremely rare benign disease. No consensus has been reached on the optimal surgical strategy. The aim of this study is to introduce four types of one-stage surgical strategies including less invasive options and a guideline to select patient-specific strategy for this disease.MethodsTwenty-four patients of extra-pelvic IVLs receiving one-stage resections at the Zhongshan Hospital from July 2011 to November 2019 were reviewed retrospectively. Base on the initial experiences of the indiscriminate choices of tumor thrombectomies through sterno-laparotomy under cardiopulmonary bypass (CPB) in 6 ICLs, an anatomy-based guideline for four types of surgical strategies was developed and applied for the next 18 patients.ResultsUnder the direction of guideline, tumor thrombectomies through single laparotomy were applied without CPB in 2 ICLs and 4 IVLs confined in IVC, or with CPB in 7 ICLs. Guideline-directed double-incisions with CPB were applied in only 5 ICLs, including 1 receiving mini-thoracotomy and 4 receiving sternotomy because of tumor adherences with right atriums in 2 and with pulmonary arteries in 2. All 24 patients accomplished one-stage panhysterectomy, bilateral adnexectomy and complete resections of intracaval and intracardiac tumors. For residual pelvic intravenous tumors in 19 patients, 17 received macroscopically complete resections while the other 2 failed because of high risk of hemorrhage. Intraoperative blood losses, operation time and hospitalization expense in the single-laparotomy non-CPB group were significantly lesser than the other groups. In CPB groups, inpatient stay and hospitalization expense in the single-incision group were significantly lesser than the double-incisions group. All patients were alive and free of recurrences during a mean follow-up of 35.4 ± 27.2 months (range, 1-100 months). The pelvic tumor residues in 2 patients remained unchanged for 51 and 52 months since operation, respectively.ConclusionsFor various extra-pelvic IVLs, the 4 types of surgical strategies including less invasive options are feasible, providing these are selected by a guideline base on the tumor extension and morphology. The proposed guideline is believed to accommodate more patients receiving less invasive surgery without compromising the curative effect.

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Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

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Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

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