Pediatric primary splenic angiosarcoma: an aggressive multidisciplinary approach to the oncologic management of a rare malignancy.

Oscar K Serrano,Jonathan Gill,Esther Knapp,Mindy Statter,Kevin Huang,Danielle Mcclain,Galina Baran

doi:10.1186/1477-7819-12-379

Abstract

Primary splenic angiosarcoma is an extremely rare and aggressive neoplasm of the vasculature. Uniformly, primary splenic angiosarcoma is a fatal disease despite early diagnosis and treatment. Only patients with localized disease amenable to surgical resection achieve long-term, disease-free survival. We present a review of the literature and report a case of a 3-year-old girl with metastatic primary splenic angiosarcoma who was offered aggressive surgical and medical treatment with curative intent despite her advanced presentation.

Highlights

Primary splenic angiosarcoma (PSA) is an extremely rare and aggressive neoplasm of the vasculature that occurs in adults and children
PSA is a fatal disease despite early diagnosis and treatment
A number of environmental agents or predisposing conditions have been associated with the development of PSA in adults, including exposure to ionizing radiation [10], prior chemotherapy for lymphoma [12], or the presence of benign tumors of the spleen - namely hemangiomas or hemangioendotheliomas [5]

Summary

Background

Primary splenic angiosarcoma (PSA) is an extremely rare and aggressive neoplasm of the vasculature that occurs in adults and children. Our patient was given a gemcitabinedocetaxel chemotherapeutic regimen, which has never been described before in children with PSA This treatment regimen achieved a partial response and was followed by liver metastasectomy. Case presentation We report on a previously healthy 3-year-old girl who presented with complaints of abdominal pain and distention, and decreased oral intake in the setting of high fevers On physical examination, she was found to have massive splenomegaly. An abdominal X-ray showed a markedly enlarged spleen with displacement of bowel and an abdominal ultrasound revealed several hyperechoic nodules in an enlarged spleen This prompted magnetic resonance imaging (MRI) of the abdomen which showed massive splenomegaly with central hemorrhage and multiple nodular lesions in the liver (Figure 1). After extensive discussion in our multidisciplinary tumor board, and given her favorable response to chemotherapy, the option of living-related liver transplantation was made

Findings

Discussion

Conclusion