Undifferentiated Embryonal Sarcoma of the Liver in Adults

Abstract

Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignancy that most commonly affects the pediatric age group. This tumor very rarely occurs in adults and, in such instances, can pose a considerable diagnostic challenge for the clinicians, radiologists, and pathologists involved. The clinical presentation is most often non-specific, and the radiology shows a solid and cystic liver mass which has a considerable differential on imaging. Especially in adult patients, UESL is a diagnosis of exclusion meaning that all other diagnostic possibilities must be excluded before the diagnosis of UESL can be confidently made. From a pathology perspective, this means careful examination of the histology along with a comprehensive panel of immunohistochemistry for almost all cases of newly diagnosed UESL. The prognosis of UESL used to be dismal, but with advances in treatment and the introduction of a multimodality approach, there has been considerable progress in improving outcomes and survival for patients with this aggressive tumor. This chapter outlines the clinical, radiological, and pathological features of UESL. An in-depth discussion is undertaken to describe the diagnostic approach and the differential diagnosis for this rare and challenging tumor.

Hepatic undifferentiated embryonal sarcoma as a cause of acute abdomen

“…At the immunohistochemical level, most cases are positive for vimentin, desmin, alpha-1-antitrypsin, CD56, CD68, Bcell lymphoma and CD10. [5][6][7] Spontaneous tumor-associated hepatic hemorrhage is a rare surgical emergency and accounts for 1% of referrals to liver clinics. The most common cause of spontaneous hepatic hemorrhage is hepatocellular carcinoma.…”

“…The chemotherapy regimen used varies between institutions and oncologists since a standard treatment is not yet established, the regimens may include doxorubicin, ifosfamide, cyclophosphamide, vincristine and etoposide. 6,7 In pediatric population survival at 5 years is 86%. 6 Until 1990 a survival rate of 37% was reported, however, the prognosis has slowly improved with multimodal management that includes not only surgery but radiotherapy and chemotherapy, so survival has increase to 70-100% currently.…”

“…6,7 In pediatric population survival at 5 years is 86%. 6 Until 1990 a survival rate of 37% was reported, however, the prognosis has slowly improved with multimodal management that includes not only surgery but radiotherapy and chemotherapy, so survival has increase to 70-100% currently. 6,7 Single photon emission computed tomography (SPECT)/CT with 99 m Tc sulfide colloidal generates images of the reticuloendothelial (Kupffer) cells of the liver.…”

“…From 1978 to 2008 there was a record of 80 cases in the literature. 5,6 It is an aggressive mesenchymal tumor that occurs predominantly in the pediatric population between 6 and 10 years of age without gender predominance. In the adult population, it has a predisposition in the female gender.…”

Undifferentiated embryonal sarcoma of the liver with epithelioid features in an adult patient

“…Undifferentiated embryonal sarcoma of the liver (UESL) is a rare hepatic mesenchymal tumor with a high potential for invasion and poor prognosis. [1] Most UESL cases have been reported in the pediatric population at the ages of 6 to 10, and the disease is extremely uncommon in adults (2). Since UESL was first described in 1978, [2] approximately 89 adult UESL cases have been reported across the world.…”