Primary fibrosarcoma of the liverCase report and review of the literature

Alrenga, Dharam P.

doi:10.1002/1097-0142(197508)36:2<446::aid-cncr2820360220>3.0.co;2-7

Cited by 30 publications

(9 citation statements)

References 8 publications

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“…Primary sarcomas of the liver are extremely rare tumors and represent approximately 0.2% of all primary liver tumors 1. They include a heterogeneous group of histologic types, such as angiosarcoma, leiomyosarcoma, epitheloid hemangioendothelioma, fibrosarcoma, malignant fibrous histiocytoma, and undifferentiated embryonal sarcoma of the liver (UESL) 2–5. UESL normally occurs in the pediatric age group, particularly between ages 6 years and 10 years.…”

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confidence: 99%

Undifferentiated embryonal sarcoma of the liver in adults

Lenze

Birkfellner

Lenz

et al. 2008

Cancer

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BACKGROUND Undifferentiated embryonal sarcoma of the liver (UESL), a rare tumor that predominantly affects children, generally has been considered an aggressive neoplasm with an unfavorable prognosis. More recent reports have indicated that modern multimodal treatment and supportive care improve the survival of children with UESL. Data regarding the treatment and survival of adults have not been reviewed comprehensively, and only a few adult patients with UESL have been reported in the literature. METHODS The authors analyzed demographics, treatment, and actuarial survival of all reported cases of UESL in patients aged ≥15 years (n = 67 patients). In addition, 1 case is presented of a patient with UESL who was treated successfully at the authors' institution. RESULTS The median survival of all patients with UESL who were analyzed was 29 months. Patients who underwent complete tumor resection followed by adjuvant chemotherapy survived over a median follow‐up of 28.5 months and had significantly better survival compared with patients who underwent surgical treatment alone. Patients who underwent an incomplete tumor resection had a tendency toward poorer outcomes. CONCLUSIONS To the authors' knowledge, this is the first report to demonstrate a significant effect on survival for adjuvant chemotherapy after complete surgical resection of UESL in adults. The role of neoadjuvant chemotherapy was not evaluated in this study. In the case study presented herein, combined therapy with surgery and chemotherapy led to a complete, sustained remission that has lasted for >6 years to date. Cancer 2008. © 2008 American Cancer Society.

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Undifferentiated embryonal sarcoma of the liver in adults

Lenze

Birkfellner

Lenz

et al. 2008

Cancer

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“…The various types of sarcomas include angiosarcomas, liomyosarcomas, liposarcomas and fibrosarcomas. The purpose of this case report is to share information on a rare tumor of the liver, a primary fibrosarcoma, as up to the late seventies the literature about this entity was restricted to autopsy diagnosis [1]. To our knowledge there are case reports from various parts of the world [2, 3], but there are no series of cases of primary fibrosarcoma of the liver.…”

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confidence: 99%

“…Though few reports recommend chemotherapy [9] in liver metastasis of retroperitoneal fibrosarcoma consisting of cyclophosphamide, vincristine, farmorubicin, and dacarbazine, there is no definite role as treatment for primary lesion of liver. Overall the prognosis reported by most of the authors is dismal [1, 2, 3, 10]. …”

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confidence: 99%

Primary Fibrosarcoma of the Liver: We Don’t Know Much

Ali

Shah

et al. 2008

Case Rep Gastroenterol

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A 60-year-old lady presented to us with a right upper abdominal mass. With a clinical diagnosis of liver tumor, she was evaluated with abdominal CT, MRI, nuclear scan, tumor markers, USG guided FNAC and other baseline investigations. On evaluation she had a massive right lobe tumor crossing the midline. In view of the ambiguous diagnosis she was subjected to laparotomy where the lesion was judged unresectable and a biopsy was taken. Histopathological examination showed the rare pathology of primary fibrosarcoma of the liver with features of homogeneous, spindle-shaped cells with abundant collagen fibers showing a classic herringbone pattern. Tissue samples were then sent to another referral cancer hospital for immunohistochemistry and immunoreactive vimentin was found in the tumor cells. Electron microscopically, the tumor cells were rich in rough endoplasmic reticulum without a basement membrane, and were surrounded by large amounts of collagen fibers. The fibroblastic character of the tumor cells was suggested by light and electron microscopy.

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“…Primary fibrosarcoma of the liver is an extremely rare neoplasm [1][2][3][4][5]. In this case the first histological examination showed a large right atrial mass attached to the lateral wall to be a fibroma with thrombus mass, but without signs of malignity.…”

Section: Discussionmentioning

confidence: 99%

“…Sarcomas constitute rare primary tumors of the liver and usually manifest unspecific symptoms [1]. In the present case the patient suffered mainly from cardiopulmonary symptoms.…”

Section: Introductionmentioning

confidence: 99%

Primary Fibrosarcoma of the Liver Infiltrating the Right Atrium of the Heart

Kelle¹,

Paetsch²,

Neuß³

et al. 2005

Int J Cardiovasc Imaging

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A rare case of a primary fibrosarcoma of the liver infiltrating the right heart is described in a 72-year-old woman. The patient presented with a history of progressive dyspnea and ascites and her general condition was poor. Preoperative cardiac magnetic resonance (CMR) imaging revealed a large mass, which originated from the liver and had infiltrated the right atrium via the inferior vena cava. The patient underwent tumor resection yet died shortly afterwards. Histologically the mass was classified as a fibrosarcoma with positive immunostaining for vimentin. We report the CMR imaging characteristics in this uncommon case. Preoperative CMR proved to be useful for clinical decision making and the planning of surgery.

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Primary fibrosarcoma of the liverCase report and review of the literature

Cited by 30 publications

References 8 publications

Undifferentiated embryonal sarcoma of the liver in adults

Undifferentiated embryonal sarcoma of the liver in adults

Primary Fibrosarcoma of the Liver: We Don’t Know Much

Primary Fibrosarcoma of the Liver Infiltrating the Right Atrium of the Heart

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