Key result
Splenic undifferentiated pleomorphic sarcoma is a rare and aggressive tumor with a poor prognosis, with the presented patient dying 34 days after splenectomy and liver metastasectomy.
Why the study?
Undifferentiated pleomorphic sarcoma of the spleen is extremely rare with only a handful of reported cases, leaving little consensus over ideal management.
Case Report (n=42)
Splenic undifferentiated pleomorphic sarcoma is an extremely rare and aggressive malignancy that poses significant diagnostic challenges and carries a poor prognosis despite radical surgical resection.
Answers are grounded in this paper and cite their sources.
Alerts clinicians to rare splenic sarcomas in nonspecific pain; leaves open evidence-based management.
BACKGROUND: Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. CASE PRESENTATION: This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. CONCLUSION: Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.
No takes yet. Share an insight, caveat, or question.
Gatt et al. (2023) conducted a case report in Undifferentiated pleomorphic sarcoma of the spleen (n=42). Splenectomy and tumor resection was evaluated on Survival. Splenic undifferentiated pleomorphic sarcoma is a rare and aggressive tumor with a poor prognosis, with the presented patient dying 34 days after splenectomy and liver metastasectomy.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: