Sarcoma indiferenciado (embrionario) de hígado en el adulto

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Abstract

Introducción: Los sarcomas indiferenciados (embrionario) del hígado (SIEH) son neoplasias infrecuentes. Se presentan principalmente en edad pediátrica y son considerados de mal pronóstico. En adultos, existen 71 pacientes publicados en el mundo, y en nuestro país no hay casos descritos en este grupo etario. Objetivos: Presentar el caso de una paciente adulta portadora de un SIEH, que fue sometida a cirugía resectiva y realizar una revisión del tema. Caso clínico: Mujer de 48 años de edad, con historia de dolor abdominal, masa palpable en epigastrio y hepatomegalia. Marcadores tumorales negativos, tomografía abdominal revela lesión focal de 14 centímetros en segmento 4. Se aborda quirúrgicamente, biopsia rápida revela tumor sólido maligno indiferenciado, y se realiza hepatectomía izquierda. Evolución postoperatoria favorable. Mediante el análisis histopatológico, histoquímico e inmunohistoquímico se diagnostica un SIEH y se corrobora una resección R0. Al noveno mes de evolución se pesquisa foco de recidiva hepática, inicia progresivo compromiso del estado general, falleciendo 22 meses después de la cirugía. Discusión: Actualmente se recomienda resección completa del tumor seguido de quimioterapia coadyuvante, con lo cual se han logrado sobrevidas libres de enfermedad mayores a 5 años. El dar a conocer las experiencias de casos aislados en esta patología tan infrecuente, permitiría aumentar la casuística mundial, mejorar las técnicas de enfrentamiento, y evaluar el impacto de la quimioterapia en el pronóstico.

Translated abstract

We report a 48 years old women presenting with a painful abdominal mass and hepatomegaly. An abdominal CAT scan showed a focal lesion of 14 cm diameter in liver segment 4. The patient was subjected to a left hepatectomy with a normal postoperative evolution. The pathological diagnosis of the surgical piece was an undifferentiated sarcoma. Nine months later, a local relapse was detected and the patient died 22 months after the operation.

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Management of primary liver sarcomas.

Murray F. Brennan, Leslie H Blumgart, Hedda J. Weitz … (2007)

Primary hepatic sarcoma is a rare entity. The objectives of the study were to define treatment and long-term outcome and to identify prognostic factors. Between January 1981 and December 2004, 30 patients with primary sarcoma of the liver and 5 patients with primary carcinosarcoma of the liver were treated. Patient demographics, tumor characteristics, treatment, and actuarial survival were analyzed. Of the 30 patients with primary hepatic sarcoma (10 epithelioid hemangioendothelioma, 5 embryonal sarcoma, 5 angiosarcoma, 3 solitary fibrous tumor, and 7 other types), 11 underwent R0-resection and had a 5-year disease-specific survival of 64%. Of these 11 patients, 4 had low-grade sarcoma and have not developed tumor recurrence. In the group of 7 patients with high-grade sarcomas who underwent R0-resection, both patients with angiosarcoma died within 11 months, whereas the 5 patients with embryonal sarcoma had a 5-year disease-free and disease-specific survival of 80%. Six of the 10 patients with an epithelioid hemangioendothelioma were managed without surgery, as they had diffuse, slowly progressing, or stable lesions; these patients had a 5-year disease-specific survival of 67%. Of the remaining 13 patients in whom R0-resection was not performed, there were no 3-year survivors. The prognosis for patients with primary carcinosarcoma of the liver was poor, with all but 1 patient dying within a year and no 3-year survivors. The outcome of patients with primary hepatic sarcoma depends primarily on tumor histology and the ability to achieve complete tumor resection. Improvements in outcome will require the development of more effective systemic therapies. (c) 2007 American Cancer Society.

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Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.

Ahmed Alharbi, Sivan Lieberman, Gidon Almogy … (2004)

Primary hepatic sarcoma is a rare tumour with a poor prognosis. From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution. The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed. There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS). The most common presenting symptoms were right upper quadrant pain and fever. Typical imaging findings were a heterogenous mass with poorly defined margins, pseudocapsule and aberrant vasculature. Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2). Five right hepatectomies and three trisegmentectomies were performed. An R (0) resection was possible in three cases. Two patients developed complications and there was one death. Adjuvant chemoradiotherapy was administered to 5/7 patients. Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection. The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass. Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy. Patients with resectable tumours should be referred for surgery. Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.

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Undifferentiated embryonal sarcoma of the liver in adults.

T Birkfellner, Hans Kreipe, Frank Lenze … (2008)

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. The authors analyzed demographics, treatment, and actuarial survival of all reported cases of UESL in patients aged >or=15 years (n = 67 patients). In addition, 1 case is presented of a patient with UESL who was treated successfully at the authors' institution. 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. 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. (c) 2008 American Cancer Society.

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Journal

Journal ID (publisher-id): rchcir

Title: Revista chilena de cirugía

Abbreviated Title: Rev Chil Cir

Publisher: Sociedad de Cirujanos de Chile (Santiago, , Chile )

ISSN (Electronic): 0718-4026

Publication date (Print and electronic): December 2010

Volume: 62

Issue: 6

Pages: 618-622

Affiliations

[03] Coquimbo orgnameHospital de Coquimbo orgdiv1Servicio de Anatomía Patológica Chile

[01] orgnameHospital de Coquimbo orgdiv1Servicio de Cirugía Chile

[02] orgnameUniversidad Católica del Norte Chile orgdiv1Instituto de Cirugía Chile

Article

Publisher ID: S0718-40262010000600013 Publisher ID: S0718-4026(10)06200600013

DOI: 10.4067/S0718-40262010000600013

SO-VID: e384798a-2749-4d6d-9ecc-44ed381247d5

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This work is licensed under a Creative Commons Attribution 4.0 International License.

History

Date accepted : 27 April 2010

Date received : 03 April 2010

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Figures: 0, Tables: 0, Equations: 0, References: 16, Pages: 5

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Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma
Authors: Gonzalo Araneda, Rafael Poniachik, Alejandro Freundlich …

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Sarcoma indiferenciado (embrionario) de hígado en el adulto Translated title: Undifferentiated (Embryonal) sarcoma of the liver in adults

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Most cited references 27

Management of primary liver sarcomas.

Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.

Undifferentiated embryonal sarcoma of the liver in adults.

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