A giant retroperitoneal liposarcoma with hemoperitoneum: A rare tumor with unusual presentation

Abstract

Retroperitoneal liposarcoma (RPLS) is a rare mesenchymal tumor which accounts for 0.02–0.7% of all malignancy. The presentations of these tumors are non-specific and early diagnosis is often missed. We are reporting a rare case of 17-year-old girl who came to us with complaints of abdominal distension and other non-specific symptoms but after few hours of admission, she had acute abdomen with hemodynamic instability. She underwent emergency laparotomy and found to have a giant 25 × 20 × 10 cm ruptured retroperitoneum tumor causing hemoperitoneum. The tumor involved left ovary and fallopian tube as well. Complete resection of tumor with left salpingooophorectomy was done. The patient was kept in intensive care unit and discharged in good health after 15 days. This presentation of RPLS has not been reported yet and may demand early diagnosis and management of these tumors. The histopathology revealed dedifferentiated liposarcoma with rhabdomyosarcomatous differentiation. The patient is completely fine at 16 months of follow-up while writing this report. Again, heterologous dedifferentiation of liposarcoma with spindle cell component is a rare morphologic spectrum. Liposarcomas can have various histologic types. In dedifferentiated types, the line of differentiation needs to be identified to decide further line of treatment. Therefore, thorough histopathological analysis and immunostaining is needed. Treatment includes complete surgical resection. Role of radiotherapy and chemotherapy is debatable and is still under trial. Dedifferentiated RPLS with rhabdomyosarcomatous differentiation is a rare tumor and its presentation as acute abdomen and hemoperitoneum has never been reported previously.