A League of Its Own? Established and Emerging Therapies in Undifferentiated Pleomorphic Sarcoma

Graves, Laurie; Jeck, William R.; Grilley‐Olson, Juneko E.

doi:10.1007/s11864-023-01054-7

Cited by 4 publications

(2 citation statements)

References 62 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Soft tissue sarcomas (STS) are rare malignant tumours arising from mesenchymal tissues which make up around 2% of all adult malignancies [ 1 ]. Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH) or high-grade spindle cell sarcoma, is an aggressive form of soft tissue sarcoma which makes up 10–20% of STS [ 2 ]. It is commoner in males and frequently presents as a soft tissue tumour in the thigh and wall of the trunk.…”

Section: Introductionmentioning

confidence: 99%

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

Gatt,

Casingena,

Pisani

et al. 2023

surg case rep

View full text Add to dashboard Cite

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.

show abstract

Section: Introductionmentioning

confidence: 99%

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

Gatt,

Casingena,

Pisani

et al. 2023

surg case rep

View full text Add to dashboard Cite

show abstract

“…Por ejemplo, la trabectedina y el pazopanib demuestran actividad en varios subtipos de sarcomas de partes blandas, pero su estudio en pacientes con sarcoma pleomórfico indiferenciado es más limitado. El sarcoma pleomórfico indiferenciado tiene unas características que lo diferencian del resto de sarcomas de partes blandas, incluyendo una mayor expresión de PD-1/PD-L1, una mayor infiltración de células T, una mayor presentación de antígenos y una mayor carga tumoral mutacional (TMB); por ello los ensayos clínicos deberían centrarse en lo que lo hace diferente respecto a los otros sarcomas en lugar de extrapolar los resultados de otros sarcomas de partes blandas a todos los subtipos 240 .…”

Section: Algunos Autores Han Creado Protocolos Terapéuticos En Funció...unclassified

Utilidad de los estudios genómicos basados en paneles de NGS (Next Generation Sequencing) para el manejo clínico de los sarcomas en pacientes pediátricos

Gutiérrez

View full text Add to dashboard Cite

Clinical managem ent of sarcomas is complex because th ey are rare a.nd heterogeneous tumo rs. Management requires a coord inated multidiscip linary approach, especially in children. Genomic ch aracterization of this complex group of tumors con tributes to the identification of progn ostic biomarkers and to the continued exp ansion of therapeutic options. In this article, we p resen t the positive experience of two Spanish hospitals in the use of gen omic analysis in the overall clinical management of sarcomas in children and young adults. We describe on a case-by-case basis h ow gen omic analysis h as con tribu ted to both diagnosis an d treatm en t.

show abstract

Undifferentiated pleomorphic sarcoma of the adrenal gland: a case report and literature review

Xiaochuan,

Wei,

Chaoyong

et al. 2024

Front. Oncol.

View full text Add to dashboard Cite

Undifferentiated pleomorphic sarcoma (UPS) is a rare type of tumor, and UPS originating in the adrenal gland is even rarer. Up to now, there have been no reports in English literature of UPS originating from the adrenal gland. This case report presents a 44-year-old female patient with UPS of the adrenal gland, who has shown no signs of recurrence or metastasis half a year after undergoing resection of a left adrenal tumor. A retrospective analysis of the patient’s diagnosis and treatment process is conducted, with the aim of providing a reference for the diagnosis and treatment of adrenal UPS.

show abstract

A League of Its Own? Established and Emerging Therapies in Undifferentiated Pleomorphic Sarcoma

Cited by 4 publications

References 62 publications

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

Utilidad de los estudios genómicos basados en paneles de NGS (Next Generation Sequencing) para el manejo clínico de los sarcomas en pacientes pediátricos

Undifferentiated pleomorphic sarcoma of the adrenal gland: a case report and literature review

Contact Info

Product

Resources

About