MFH and high-grade undifferentiated pleomorphic sarcoma-what's in a name?

Delisca, Gadini O.; Mesko, Nathan W.; Alamanda, Vignesh K.; Archer, Kristin R.; Song, Yanna; Halpern, Jennifer L.; Schwartz, Herbert S.; Holt, Ginger E.

doi:10.1002/jso.23787

Cited by 27 publications

(21 citation statements)

References 32 publications

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“…21,22 This agrees with a prior SEER report of all patients with RPS, irrespective of tumor grade, which showed that adjuvant RT was associated with an increase in OS only in patients with malignant fibrous histiocytoma (although it should be noted that, in 2002, the World Health Organization declassified malignant fibrous histiocytoma and replaced it with high-grade undifferentiated pleomorphic sarcoma). 9,34 Of note, in our analysis of OS (Fig. 1), survival between the adjuvant RT and no RT groups is clearly divergent at the median OS but the difference between the groups seems to narrow significantly by approximately 80 months of follow-up.…”

Section: Discussionmentioning

confidence: 65%

The Benefit of Adjuvant Radiotherapy in High-grade Nonmetastatic Retroperitoneal Soft Tissue Sarcoma

Bates

Dhakal

Mazloom

et al. 2018

American Journal of Clinical Oncology

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Section: Discussionmentioning

confidence: 65%

The Benefit of Adjuvant Radiotherapy in High-grade Nonmetastatic Retroperitoneal Soft Tissue Sarcoma

Bates

Dhakal

Mazloom

et al. 2018

American Journal of Clinical Oncology

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“…One of the most common of these was malignant fibrous histiocytoma (MFH), a classification that has been rendered obsolete over the past decade. The majority of MFH in the retroperitoneum are now recognized and reclassified as undifferentiated pleomorphic sarcomas (UPS) or dedifferentiated liposarcomas (DDLPS), and correctly reclassifying an MFH/UPS into a DDLPS may require molecular testing . Additionally, the 7th edition of the American Joint Committee on Cancer changed the grading criteria for sarcomas from a four to a three‐grade system, classifying sarcomas as low, intermediate, and high grade .…”

Section: Introductionmentioning

confidence: 99%

Long‐term outcomes in treatment of retroperitoneal sarcomas: A 15 year single‐institution evaluation of prognostic features

Abdelfatah

Guzzetta

Nagarajan

et al. 2016

Journal of Surgical Oncology

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Background Retroperitoneal sarcomas are connective tissue tumors arising in the retroperitoneum. Surgical resection is the mainstay of treatment. Debate has arisen over extent of resection, changes in histological classification/grading, and interest in incorporating radiotherapy. Therefore, we reviewed our institution’s experience to evaluate prognostic factors. Methods Retrospective chart review of all primary RPS patients at Johns Hopkins Hospital from 1994 – 2010. Histologic diagnosis and grading were re-evaluated with current criteria. Prognostic factors for survival, and recurrence were assessed. Results 131 primary RPS patients met inclusion criteria. Median survival for patients who undergo en-bloc resection to negative margins (R0/R1) is 81.7 months. Surgical margins and grade were the most important factors for survival along with age, gender, presence of metastases and resection of ≥5 organs. 5 year survival for R0/R1 resection was 60%, similar to compartmental resection. Radiotherapy significantly decreased local recurrence (p=0.026) on multivariate analysis. Grade in leiomyosarcomas and dedifferentiation in liposarcomas dictated patterns of local vs distal recurrence. Conclusions En bloc surgical resection to R0/R1 margins remains the cornerstone of therapy and provides comparable outcomes to compartmental resections. Grade remains important for prognosis, and histology dictates recurrence patterns. Radiotherapy appears promising for local control and warrants further investigation.

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“…Despite aggressive surgery, local recurrences occur in 13–42% of patients and nearly 31–35% will develop metastatic disease. 2–4 Although the addition of chemotherapy has shown some benefit in meta-analyses, these responses are marginal at best, highlighting the need for improved therapeutic options for UPS patients. 5,6 The use of radiation therapy is associated with reduced local recurrences in high grade sarcomas; however, approximately 3–5% of UPS arise in a prior site of therapeutic radiation for an unrelated malignancy and are termed radiation-associated UPS (RA-UPS), making radiation therapy controversial in this subset of the patients due to the comorbidities of re-irradiation 7,8 .…”

Section: Introductionmentioning

confidence: 99%

Analysis of Clinical and Molecular Factors Impacting Oncologic Outcomes in Undifferentiated Pleomorphic Sarcoma

et al. 2016

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Background Undifferentiated pleomorphic sarcomas (UPS) present a diagnostic and therapeutic challenge. Identification of prognostic molecular markers is required for the discovery of novel treatment approaches. The aim of this study was to correlate clinicopathologic variables, expression of tyrosine kinase receptors, and markers of cell cycle progression and survival with oncologic outcomes. Methods A tissue microarray containing 208 primary UPS samples was analyzed by immunohistochemistry for protein markers and in situ hybridization for microRNA. Staining results were correlated with clinicopathologic features and oncologic outcomes. Univariate and multivariate analyses were conducted to assess associations between expression of protein markers, mi-RNA and outcome. Results At a median follow-up of 3.9 years (9 years for survivors), 5-year disease-specific survival (DSS) was 63%. Clinical variables associated with improved DSS included age < 61 years, tumor size < 10 cm, margin-negative resection and sporadic-tumor status. At the protein level, loss of cyclin D1 (p=0.06), pEGFR (p=0.023), pIGF-1R (p=0.022), and PTEN (p<0.001) and overexpression of AXL (p=0.015) were associated with reduced DSS on univariate analysis. Ki67, PCNA and pEGFR were more highly expressed in sporadic UPS than radiation-associated (RA-UPS) while RA-UPS samples expressed higher levels of both phosphorylated and total IGF-1R. Discussion Cyclin D1, AXL and PTEN are associated with cancer-specific outcomes and warrant further investigation in UPS. The differences in protein expression in sporadic versus RA-UPS may indicate that the activated molecular signaling nodes may be different for each specific histology and could also explain the aggressive phenotype seen in RA-UPS when compared to the sporadic lesions.

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MFH and high-grade undifferentiated pleomorphic sarcoma-what's in a name?

Abstract: Despite new classification nomenclature, there appears to be no identifiable prognostic implications for sarcomas that remain in the unclassifiable HGUPS group, as compared to the previously accepted MFH group.

Cited by 27 publications

References 32 publications

The Benefit of Adjuvant Radiotherapy in High-grade Nonmetastatic Retroperitoneal Soft Tissue Sarcoma

The Benefit of Adjuvant Radiotherapy in High-grade Nonmetastatic Retroperitoneal Soft Tissue Sarcoma

Long‐term outcomes in treatment of retroperitoneal sarcomas: A 15 year single‐institution evaluation of prognostic features

Analysis of Clinical and Molecular Factors Impacting Oncologic Outcomes in Undifferentiated Pleomorphic Sarcoma

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