Leimyosarcoma of the inferior vena cava (IVC) is a rare tumor, with about 200 cases reported in the world literature. There were 5 cases reported in Korea. We report a case of primary leiomyosarcoma of the inferior vena cava in a 50 year-old woman who presented with a heterogenous enhancing mass within IVC on CT scan and MRI scan. The tumor expanded the IVC with displacement of the adjacent structures. The patient underwent ultrasound guided percutaneous biopsy which was consistent with leiomyosarcoma. The patient was treated by surgical resection and synthetic graft replacement of the inferior vena cava and postoperative external beam radiation therapy. We report the case of leiomyosarcoma of the inferior vena cava with a review of relevant literatures.
*Blood Vessel Prosthesis Implantation ; Female ; Humans ; Leiomyosarcoma/diagnosis/*surgery ; Middle Aged ; Vascular Neoplasms/diagnosis/*surgery ; Vena Cava, Inferior/*surgery

Giant Cell Tumors ; diagnosis ; diagnostic imaging ; surgery ; Humans ; Leiomyosarcoma ; diagnosis ; diagnostic imaging ; surgery ; Male ; Middle Aged ; Radiography

BackgroundThe diagnosis and treatment of small-bowel diseases is clinically difficult. The purpose of this study was to evaluate the diagnostic and therapeutic value of double-balloon enteroscopy in small-bowel diseases.

MethodsThe history and outcomes of 2806 patients who underwent double-balloon enteroscopy from July 2004 to April 2017 were reviewed, which included 562 patients with obscure digestive tract bleeding, 457 patients with obscure diarrhea, 930 patients with obscure abdominal pain, 795 patients with obscure weight loss, and 62 patients with obscure intestinal obstruction. Examinations were performed through the mouth and/or anus according to the clinical symptoms and abdominal images. If a lesion was not detected through one direction, examination through the other direction was performed as necessary. Eighty-four patients with small-bowel polyps, 26 with intestinal obstruction caused by enterolith, and 18 with bleeding from Dieulafoy's lesions in the small intestine were treated endoscopically.

ResultsA total of 2806 patients underwent double-balloon enteroscopy, and no serious complications occurred. An endoscopic approach through both the mouth and anus was used in 212 patients. Lesions were detected in 1696 patients, with a detection rate of 60.4%; the rates for obscure digestive tract bleeding, diarrhea, abdominal pain, weight loss, and intestinal obstruction were 85.9% (483/562), 73.5% (336/457), 48.2% (448/930), 49.1% (390/795), and 62.9% (39/62), respectively. For patients with small-bowel polyps who underwent endoscopic therapy, no complications such as digestive tract bleeding and perforation occurred. Intestinal obstruction with enteroliths was relieved with endoscopic lithotripsy. Among the 18 patients with bleeding from small-bowel Dieulafoy's lesions, 14 patients were controlled with endoscopic hemostasis.

ConclusionDouble-balloon enteroscopy is useful for diagnosing and treating some small-bowel disease.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Double-Balloon Enteroscopy ; methods ; Female ; Gastrointestinal Hemorrhage ; diagnosis ; surgery ; Humans ; Intestinal Diseases ; diagnosis ; surgery ; Intestinal Obstruction ; Intestine, Small ; diagnostic imaging ; Leiomyosarcoma ; diagnosis ; surgery ; Lymphoma ; diagnosis ; surgery ; Male ; Middle Aged ; Polyps ; diagnosis ; surgery ; Young Adult

OBJECTIVETo study the treatment strategy and survival of patients with primary leiomyosarcoma of inferior vena cava (PIVCLMS).

METHODSClinical data of 12 cases with PIVCLMS admitted in Peking University People's Hospital from January 2006 to September 2014 were reviewed retrospectively. All cases were confirmed by pathology examination. Among them, there were 4 male and 8 female patients with a mean age of (54 ± 9) years old. Tumors arose from the inferior vena cava (IVC) upper segment in 5 patients, from the middle in other 7 patients. Cardiac extension was observed in 4 cases. Tumor resection was undertaken in 8 patients, the other 4 patients were inoperable. The series was analyzed to identify clinical outcome of surgical strategy and protective factors for patient survival.

RESULTSIn tumor resection group, 6 patients had radical resection and 2 underwent palliative resection. As for IVC reconstruction, caval wall resection with a direct suture was carried out in 6 patients or with prosthetic patch in 1 patient. The other 1 patient underwent a segment caval resection and prosthetic graft replacement in situ. In 4 cases of suprahepatic PIVCLMS cardiopulmonary bypass or perfusion by right atrial intubation was performed to assist bleeding control and maintain circulation stabilization, among them 1 patient survived for more than 101 months with no tumor recurrence or metastasis. Among the patients submitted to tumor resection 2 early postoperative deaths occurred, and another 2 patients had complications. All 4 patients submitted to non-resective operation (only neoplasm biopsy) died of PIVCLMS within 8 months. Except for 2 cases of early death, mean survival after tumor resection was (54 ± 40) months. Two patients presented local recurrence and hepatic metastasis at follow-up of 16 months and 68 months.

CONCLUSIONSTumor resection is the only therapy for PIVCLMS with an expectation for long-term survival. The applicant of cardiopulmonary bypass makes some inoperable indicated to tumor resection.

Adult ; Blood Vessel Prosthesis Implantation ; Cardiopulmonary Bypass ; Female ; Humans ; Leiomyosarcoma ; diagnosis ; surgery ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Vascular Neoplasms ; diagnosis ; surgery ; Vena Cava, Inferior ; pathology

Adult ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Leiomyoma ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; pathology ; Spinal Canal ; Spinal Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

Actins ; metabolism ; Bone Neoplasms ; metabolism ; pathology ; surgery ; Desmin ; metabolism ; Diagnosis, Differential ; Fibrosarcoma ; metabolism ; pathology ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neurilemmoma ; metabolism ; pathology ; Tibia ; Vimentin ; metabolism

Actins ; metabolism ; Adult ; Desmin ; metabolism ; Diagnosis, Differential ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Immunohistochemistry ; Leiomyoma ; pathology ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Male ; Mediastinal Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Abdominal Neoplasms ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Liposarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Actins ; metabolism ; Adult ; Amputation ; Arthroplasty, Replacement, Knee ; Bone Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Calmodulin-Binding Proteins ; metabolism ; Humans ; Leiomyosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Radiography ; Tibia ; diagnostic imaging ; surgery

OBJECTIVETo investigate whether the border-line uterine smooth-muscle tumor is different from leiomyoma or leiomyosarcoma in history and clinical manifestations.

METHODSThe medical records of 131 surgically treated patients suffering from the so-called cellular leiomyoma or mitotically active leiomyoma of the uterus treated from 1984 to 2002 were retrospectively reviewed. All pathological sections of these patients were reviewed by a senior pathologist. Chi-square test and Kaplan-Meier life table were used for statistical analysis.

RESULTSThe overall 5-year survival rate of patients with the border-line uterine smooth-muscle tumors was 100%, however, 19.1% (24/131) of whom developed a late recurrence and 8 patients had repeated recurrences with a more shortened course and aggressive potential changes of mitosis and cellular atypia. The overall 5-year survival rate the patients with recurrence was 91.7%, but only 75.0% for those with repeated recurrences.

CONCLUSIONPatients with border-line uterine smooth-muscle tumor do possess some difference in nature from the leiomyoma or leimyosarcoma. Long-term follow-up is very important for these patients after surgery.

Adult ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Leiomyoma ; pathology ; surgery ; Leiomyosarcoma ; pathology ; surgery ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Smooth Muscle Tumor ; mortality ; pathology ; surgery ; Survival Rate ; Uterine Neoplasms ; mortality ; pathology ; surgery