Rhabdomyosarcoma in the Inferior Vena Cava with Secondary Budd-Chiari Syndrome.

Fujita, Hideo; Kawata, Koichi; Sawada, Takahisa; Mizutani, Tetsu; Iwasaki, Yasunobu; Shirono, K; Kounosu, Hiroshi; Shirakata, S

doi:10.2169/internalmedicine.32.67

Cited by 9 publications

(3 citation statements)

References 10 publications

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“…9 Nonmyogenic sarcomas, which are derived from the intima, are even more infrequent and are typically seen in the arterial system, particularly the pulmonary artery. 10 Intimal sarcomas have also been reported in the Superior vena cava, Inferior vena cava and brachiocephalic vein. 11,12 Typically leiomyosarcoma and angiosarcoma carry a better prognosis than undifferentiated intimal sarcoma with a mean 5-year survival of 33-53%.…”

Section: Discussionmentioning

confidence: 99%

Leiomyosarcoma of Superior Vena Cava and Inferior Vena Cava - Two Rare Case Reports

Khanna¹,

Bhattacharya²

2018

IJCRR

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Objective: This article attempts to describe two cases of vascular leiomyosarcoma, one arising from the superior vena cava in a 22 year old male patient, and the other arising from the inferior vena cava in a 53 year old female. The objective was to investigate and diagnose the two cases. Material and Methods:Imaging studies (CT scan) were done in both the cases subsequent to initial clinical examination. After surgical resection, the specimens of the tumors were sent to the Pathology Department for histopathological examination. The sections were stained using Haematoxylin and Eosin (H & E). Immunohistochemistry (IHC) was also done.Results: On gross examination, the first case showed a segment of a large vessel with a mass attached to the inner wall and protruding outside through the lumen. Tumor on cut section was solid, firm, homogenous grayish white with focal necrotic areas.In the second case, the vessel wall with an attached firm to hard lesion was received. On histological examination, both the cases were diagnosed to be malignant spindle cell tumor. Immunohistochemical staining revealed that the tumor cells were diffusely positive for SMA and Desmin and negative for S100 and CK. The two cases were reported as vascular leiomyosarcoma, one of the Superior vena cava and the other case of Inferior vena cava. Conclusion:Vascular leiomyosarcoma is a very rare tumor and the Inferior vena cava is the most common site (50%). The Superior vena cava (SVC) is exceptionally involved and only a dozen cases have been previously reported. An accurate imaging and histopathological diagnosis is essential as they can affect the prognosis and treatment approach. So such cases should be thoroughly examined and followed.

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Section: Discussionmentioning

confidence: 99%

Leiomyosarcoma of Superior Vena Cava and Inferior Vena Cava - Two Rare Case Reports

Khanna¹,

Bhattacharya²

2018

IJCRR

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“…These tumors are most often derived from the medial smooth muscle and are usually leiomyosarcomas, but intimal sarcomas, leiomyomas, synovial sarcoma, angiosarcoma, and rhabdomyosarcoma have been reported [ 1 – 4 ]. Nonmyogenic sarcomas, which are derived from the intima, are even more infrequent and are typically seen in the arterial system, particularly the PA [ 5 , 6 ]. Intimal sarcomas have also been reported in the superior vena cava (SVC), IVC, and brachiocephalic vein [ 7 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated Intimal Sarcoma of the Inferior Vena Cava with Extension to the Right Atrium and Renal Vasculature

Afzal

Alsahhar

Podduturi

et al. 2015

Case Reports in Cardiology

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Primary sarcomas of the great vessels (aorta, pulmonary artery, and inferior vena cava (IVC)) are exceedingly rare. We report a rare case of an undifferentiated intimal sarcoma of the IVC with extension to the right atrium, adrenal, and renal veins. The patient underwent extensive resection, reconstruction of the IVC, and subsequent adjuvant chemotherapy. Patient has tolerated chemotherapy and, at 17 months after resection, the patient remains free of tumor recurrence. Undifferentiated intimal sarcomas remain a rare entity with only five cases of venous undifferentiated intimal sarcomas reported in the literature, two of which occurred in the IVC. Intimal sarcomas tend to carry a poor prognosis with the limited literature available on treatment approaches. Our objective is to highlight this rare entity and possible treatment approach which we utilized. Primary sarcomas of IVC need to be included as part of a complete differential diagnosis in patients with atrial masses or recurrent pulmonary emboli.

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“…The tumor was associated with Budd-Chiari syndrome. It was difficult to decide whether the tumor took its origin in the vena cava or the liver (Fujita et al 1993).…”

Section: Rhabdomyosarcoma In/of the Inferior Vena Cavamentioning

confidence: 99%

Tumors of the Striated Muscle Cell Lineage: Hepatobiliary Rhabdomyosarcoma and Rhabdomyoma

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Rhabdomyosarcomas (RMS) are a group of aggressive malignancies derived from a skeletal muscle cell lineage. These neoplasms occur in several distinct body regions in children and adults. One subset of RMA develops in the hepatobiliary tract, mainly in the pediatric age group, associated with a distinct clinical and radiological syndrome. Hepatobiliary RMS is defined as a mucosal-type RMS arising from the wall of intrahepatic or extrahepatic bile ducts. This neoplasm is the most common biliary tract tumor in children, but is overall rare, accounting for only 0.8 % of all RMS and 1.3 % of malignant liver tumors in childhood. The neoplasm can form polypoid masses that protrude into the bile ducts. Histologically, most hepatobiliary RMS are embryonal RMA, whereas alveolar RMS is a very rare hepatic malignancy. Histologically, embryonal RMA of the liver shows a loose neoplastic tissue with desmin-positive stellate cells and cells resembling rhabdomyoblasts. Frequently, a cellular layer is found underneath the biliary tract epithelium, the so-called cambium. RMS of the hepatobiliary tract can rarely also occur in adults.

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Rhabdomyosarcoma in the Inferior Vena Cava with Secondary Budd-Chiari Syndrome.

Cited by 9 publications

References 10 publications

Leiomyosarcoma of Superior Vena Cava and Inferior Vena Cava - Two Rare Case Reports

Leiomyosarcoma of Superior Vena Cava and Inferior Vena Cava - Two Rare Case Reports

Undifferentiated Intimal Sarcoma of the Inferior Vena Cava with Extension to the Right Atrium and Renal Vasculature

Tumors of the Striated Muscle Cell Lineage: Hepatobiliary Rhabdomyosarcoma and Rhabdomyoma

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