HEPATIC EMBRYO SARCOMA: CASE REPORT

Case Presentation: V.G.O, 8 years old, female, brown, from Manaus-Am. She sought urgency with diffuse abdominal pain, colic, intermittent, continuous fever and malaise. She presented pain on palpation of the right hypochondrium and presence of hardened mass without peritoneal irritation. The first diagnosis was pyogenic liver abscess. The patient then underwent laparostomy to drain the lesion and performed a biopsy. The pathology showed mesenchymal neoplasia related to hemangioendothelioma type II. Immunohistochemistry reported high-grade sarcoma with muscle differentiation. Magnetic resonance imaging found heterogeneous expansive lesion measuring 5.2× 7.0× 5.8cm. She presented alpha-fetoprotein within normal limits. Surgery was performed for tumor resection, right hepatic trisegmentectomy, selective vascular exclusion through the approach of the glissonian pedicle, associated with partial resection of the monobloc anterior abdominal wall (reconstruction of the abdominal wall was performed with a marlex mesh). The biopsy confirmed the diagnosis of Undifferentiated Hepatic Embryonic Sarcoma. The child underwent adjuvant chemotherapy treatment according to the protocol without distant disease or local recurrence. Discussion: Undifferentiated hepatic sarcoma or Undifferentiated embryonal sarcoma of the liver (UESL) is a neoplasm of embryonic origin. Primary liver neoplasms account for about 2% of pediatric solid malignant tumors. The UESL represents 9 to 15% of liver tumors and affects children aged 5 to 10 years, more males. Embryonic liver sarcoma has a prognosis of about one year of survival. In the literature it presents just over 150 described cases. The clinical presentation is very diverse. Fever may accompany, as noted in the patient. UESL is not associated with high levels of standard alpha-fetoprotein confirmed in the reported case. Diagnosis is based on age, alpha-fetoprotein levels, and imaging exams depending on histopathological and immunohistochemical examination. In this case, they were positive confirming the diagnosis of embryonic sarcoma. The combination of radical surgery followed by chemotherapy has better survival outcomes. Final comments: UESL is a rare entity. Resection is the best therapeutic option, followed by adjuvant chemotherapy.

Publikationsverlauf

Artikel online veröffentlicht:
23. Oktober 2019