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Weekly Chest CasesArchive of Old Cases

Case No : 669 Date 2010-08-22

  • Courtesy of Woon Jung Kwon / Ulsan University Hospital, Ulsan University School of Medicine
  • Age/Sex 50 / F
  • Chief ComplaintChest discomfort, syncope for 2 months
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Intravascular leiomyomatosis
Radiologic Findings
Chest radiograph shows several small pulmonary nodules in both lungs. Axial CT images in lung windows demonstrate well-demarcated randomly distributed, rounded nodules.

Axial contrast enhanced chest CT images show extensive low attenuated filling defects within the right atrium and inferior vena cava. Enhanced CT scans through the lower abdomen demonstrates relative homogenous enhancing huge mass with the left ovarian vein thrombus (arrow).

Total abdominal hysterectomy and biltateral salphingo-oopherectomy for uterine mass and surgical resection of the intravascular and right atrial tumor were performed.

Gross photography demonstrates the serpent-shaped tumor in the right atrium and inferior vena cava showing a yellowish-white, fibrotic and trabeculated cut surface.

High power field microphotographs of tumor (x100, x400, H-E stain) show spindle tumor cells with oval to elongated cigar shaped nuclei and abundant fibrillar eosinophilic cytoplasm.
Brief Review
The spectrum of smooth-muscle tumors arising from the uterus ranges from benign leiomyoma to malignant leiomyosarcoma but also includes a variety of lesions with growth patterns that extend outside the uterus, including parasitic leiomyoma, intravenous/intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma.
Intravascular leiomyomatosis (IVL) is defined by a proliferation of benign smooth muscle within veins outside the confines of a leiomyoma or even when no leiomyoma is present. There are two main theories regarding the pathogenesis of IVL. One states that the tumor arises from within the uterine veins, and the other states that it results from intravascular invasion by a leiomyoma. Rarely, these can extend through the pelvic and ovarian veins to the inferior vena cava and reach the right atrium of the heart. Due to their slow-growing nature, these tumors tend not to respond to conventional chemotherapy. Treatment options include total abdominal hysterectomy and bilateral salphingo-oopherectomy for uterine lesions, with resection of distant and intravenous lesions. The specimens should be measured for estrogen and progesterone receptors as estrogen is thought to play an important role in the growth and development of the disease.
Benign metastasizing leiomyoma is a rare disease first described by Steiner in 1939. The disease is characterized by uterine leiomyoma in young adulthood, with pulmonary metastases occurring in the pre-menopausal period. Benign metastasizing leiomyoma can have a benign indolent clinical course with long-term stability. Radiographically, benign metastasizing leiomyoma presents typically as solitary or multiple pulmonary nodules, and on CT these nodules enhance homogenously. Although the multiplicity of lesions raises the question of metastatic disease, in the clinical setting of a hormonally active woman with a history of uterine leiomyomas and no known primary malignancy, the radiologist can add benign metastasizing leiomyoma to the diagnostic considerations even before tissue sampling.
References
1. Cohen DT, Oliva E, Hahn PF, Fuller AF Jr, Lee SI. Uterine smooth-muscle tumors with unusual growth pattern: imaging with pathologic correlation. AJR 2007;188:246-255
2. Koh DM, Burn PR, King DM. Benign metastasizing leiomyoma with intracaval leiomyomatosis. Br J Radiol 2000;73:435-437
3. Arif S, Ganesan R, Spooner D. Intravascular leiomyomatosis and benign metastasizing leiomyoma: an unusual case Int J of Gynecol Cancer 2006;16:1439-1478
Keywords
Vascular, Lung, Benign tumor,

No. of Applicants : 93

▶ Correct Answer : 22/93,  23.7%
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Goa Medical College , India Paresh Desai
  • - SMG-SNU boramae medical center , Korea (South) Kwang Nam Jin
  • - Catholic medical center , Korea (South) hyuna kim
  • - CHUV , Switzerland ANNE-SOPHIE KNOEPFLI
  • - Osaka University , Japan Osamu Honda
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Dong-A University Hospital , Korea (South) Bo Sung Kim
  • - Meenakshi Medical College & RI , India Jaiger C
  • - Shinchon Severance hospital , Korea (South) Hye Mi Kim
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - SMC , Korea (South) JIHYE MIN
  • - attikon hospital , Greece konstantinos kagouridis
  • - Onomichi municipal hospital , Japan Junya Itakura
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - second hospital xian jiaotong university , China li ming
  • - HOSPITAL MOINHOS DE VENTO porto alegre , Brazil ana zanardo
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - IRSA , France jean-luc BIGOT
  • - NDMVP Nashik , India Imran Jindani
▶ Correct Answer as Differential Diagnosis : 18/93,  19.4%
  • - Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
  • - radiologist, aditya imaging centre , India vivek patel
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Department of Diagnostic Radiology, Chonbuk National University Hospital , Korea (South) Eun Jung Choi
  • - Chonnam university hospital , Korea (South) Minyoung Jung
  • - CNUH , Korea (South) Seoin Jeong
  • - CNUH , Korea (South) Hyedoo Jung
  • - Chonnam university hospital , Korea (South) LEE HYUN
  • - Chonnam National University Hospital , Korea (South) Daun Lee
  • - aarthi scans , India abirami mahadevan
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Saga University , Japan Ryoko Egashira
  • - jobless , France jean-baptiste Noel
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - chonnam university hospital , Korea (South) shin hyo hyun
  • - Pneumology GHSR , Reunion fabrice paganin
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