Unexpected Liver Embryonal Sarcoma in the Adult: Diagnosis and Treatment

Abstract

Undifferentiated embryonal sarcoma of the liver is a malignancy with poor prognosis observed more frequently in children between 6 and 10 years old and very rarely found in adults. We present a case of embryonal sarcoma of the liver in a 60-year-old woman without significant medical history who presented to our attention with constitutional symptoms. Preoperative assessments did not show alterations in blood chemistry or tumor markers. Imaging studies showed a huge mass lying in the right abdominal quadrants, strictly adherent to the liver. The tumor was partially cystic with a thickened wall, sporadic contrast enhancement, and solid component. The patient underwent excision of the mass with associated liver bisegmentectomy S5-S6. Postoperative course was uneventful. The definitive histological diagnosis revealed the presence of embryonal sarcoma of the liver. We describe the clinical, histopathological, and therapeutic options adopted in the multimodal treatment of this disease.

Undifferentiated Embryonal Sarcoma of the Liver in Adults

“…UESL is the third most common primary liver malignancy in children after hepatoblastoma and hepatocellular carcinoma (1,7). This tumor has been reported in adults, but these cases are exceptionally rare (3). UESL most commonly occurs in the right lobe of the liver with many patients being asymptomatic for considerable periods of time (3).…”

“…This tumor has been reported in adults, but these cases are exceptionally rare (3). UESL most commonly occurs in the right lobe of the liver with many patients being asymptomatic for considerable periods of time (3). Those patients who are symptomatic may present with non-specific features such as nausea, vomiting, weight loss, fever, and abdominal pain and distention (1,2).…”

“…Despite the presence of a pseudocapsule, cords of normal hepatocytes and bile ducts are commonly entrapped along the periphery of this tumor (Figure 3D) (2). Periodic acid-Schiff positive diastase (PASD)-resistant eosinophilic globules are often seen in the tumor stroma or within the cytoplasm of some of the tumor cells (Figures 3E and F) (2,3,6). Around half of UESLs will have areas of extramedullary hematopoiesis (14) and an extremely rare case with osteoid formation has been reported (13).…”

“…Around 60 cases have been reported in adults since this pediatric tumor was first described. The average age for these adult cases was 24 years and the oldest was 84 years of age (3). Because of the limited tissue encountered on biopsy specimens of adult patients, undifferentiated embryonal sarcomas can pose a considerable diagnostic conundrum.…”

“…Timely detection and surgical resection along with neoadjuvant therapy are essential in achieving favorable outcomes (1,2). In pediatric patients, the 5-year survival is around 86%, with surgical resection being the most important aspect of treatment (1,3).…”