scholarly journals Intravascular leiomyomatosis with cardiac extension: a case report

Author(s):  
Einat Shaked ◽  
Ram Sharoni ◽  
Debra Gershov West ◽  
Eli I Lev

Abstract Background Intravascular leiomyomatosis with intracardiac extension is a rare benign tumor seen exclusively in women, characterized by proliferation of uterine smooth muscle cells through the venous circulation into the inferior vena cava and the right heart chambers. Case summary A 47 years old women with history of previous hysterectomy due to myomatosis, presented with nausea, anorexia and bilateral lower limb swelling over the preceding two months. An outpatient abdominal ultrasound discovered a mass in the Inferior vena cava. Echocardiogram and Computed tomography demonstrated a large intravascular mass extending from the pelvis to the right heart chambers. The tumor was completely removed in a concomitant open-heart surgery and laparotomy. Post operative course was uncomplicated. A month later the patient was feeling well and in good clinical condition. The histological analysis consisted with intravascular leiomyomatosis. Discussion Intracardiac leiomyomatosis is a rare clinical condition which requires high index of suspicion. Multimodality imaging is usually required to establish the preoperative diagnosis, although the final diagnosis is achieved with tissue investigation. Complete surgical resection of the tumor is curative and associated with good long-term prognosis.

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Dimitrios Siamkouris ◽  
Marc Schloesser ◽  
Amr Yousef ◽  
Elmar Offers

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract. The major cause of GIST is the presence of an abnormal form of tyrosine protein kinase (KIT) protein also known as CD117, which causes uncontrollable growth of the gastrointestinal cells. Most studies report incidences between 10 and 15 cases of GISTs per million. Metastases to the liver and peritoneum are the most frequent. We report a case of advanced GIST with a liver metastasis infiltrating the inferior vena cava (IVC) and extending to the right atrium in the form of a large, floating, isolated intracardiac liver metastasis with diastolic prolapsing through the tricuspid valve. This is a very rare manifestation. One week after heart surgery and removal of a 5×6 cm tumor mass from the right atrium and the IVC, echocardiography depicted an early recurrence.


2019 ◽  
Vol 47 (7) ◽  
pp. 3465-3474
Author(s):  
Guangze Luo ◽  
Hongrui Pan ◽  
Jiaxue Bi ◽  
Yudong Luo ◽  
Jiechang Zhu ◽  
...  

Objective This study was performed to investigate the surgical treatment of intravenous leiomyomatosis involving the right heart. Methods The clinical data of five patients with intracardiac leiomyomatosis treated from April 2002 to October 2017 at a single center were retrospectively analyzed. Results All five patients underwent successful intravenous and right atrial tumor removal via abdominal and inferior vena cava incisions. In three patients, these incisions were combined with thoracotomy and a right atrial incision, and in two patients, they were combined with uterine and bilateral fallopian tube and ovarian resection. One patient with advanced disease underwent a one-stage procedure and died thereafter. Of the remaining four patients who underwent follow-up for 1.5 to 12.0 years, one developed recurrence at 1 year postoperatively. The recurrent tumor, which was pathologically confirmed to be an intravenous leiomyoma, was removed via inferior vena cava and internal iliac vein incisions without subsequent recurrence. Conclusions The main treatment goal for inferior vena cava leiomyomas involving the right heart is to first address the severe obstruction of cardiac blood flow and then pursue second-stage surgery. Concurrent thoracotomy appears unnecessary because moderately sized right heart tumors can be gently removed via the inferior vena cava.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Hirofumi Sonoda ◽  
Keisuke Minamimura ◽  
Yuhei Endo ◽  
Shoichi Irie ◽  
Toru Hirata ◽  
...  

A 76-year-old Japanese man was referred to our hospital with chief complaint of right hypochondoralgia. Abdominal ultrasound showed a retroperitoneal tumor in the suprarenal region of the right kidney. Computed tomography revealed an enhanced lobular tumor with irregular, circumscribed, and indistinct border. Ultrasound-guided biopsy was performed. The tumor consisted of spindle-shaped cells with a giant nucleus and multinuclear cells. The diagnosis was leiomyosarcoma by immunohistochemical staining. The patient underwent surgery accessed by a right eighth intercostal thoracoabdominal incision. The tumor was completely resected, accompanied by removal of the posterosuperior segment of the right hepatic lobe, right adrenal gland, and a portion of the inferior vena cava (IVC). The histopathologic diagnosis was leiomyosarcoma arising from the IVC. We present a rare case of a successfully managed leiomyosarcoma of the IVC. This case suggests the importance of curative surgical resection of the tumor due to low efficacy of adjuvant chemotherapy for leiomyosarcoma.


2002 ◽  
Vol 17 (2) ◽  
pp. 80-82 ◽  
Author(s):  
M. Gaudino ◽  
Paola Spatuzza ◽  
Francesco Snider ◽  
Nicola Luciani ◽  
Gregorio Cina ◽  
...  

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