Soft Tissue Leiomyosarcoma With Microsatellite Instability, High Tumor Mutational Burden, and Programmed Death Ligand-1 Expression Showing Pathologic Complete Response to Pembrolizumab: A Case Report

Tay, Timothy Kwang Yong; Yeong, Joe Poh Sheng; Chen, Eileen Xueqin; Sam, Xin Xiu; Lim, Jimmy; Chan, Jason Yongsheng

doi:10.1200/po.22.00068

Cited by 5 publications

(3 citation statements)

References 32 publications

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“…As such, there is significant interest in clinical trials evaluating the role of biomarkers for patients who have LMS and other sarcomas. While large-scale studies analysing the efficacy of immunotherapy in populations of patients including leiomyosarcoma have not shown dramatic responses for patients with LMS, our case report indicates there may be a role for its administration in select patients 20–22. Continuing to better understand the biochemical mechanisms behind LMS and disease response to immunotherapies remains key in revolutionising clinical management of rare and aggressive cancers such as LMS.…”

Section: Discussionmentioning

confidence: 78%

PD-1 Inhibition in metastatic high tumour mutational burden (TMB) leiomyosarcoma with clinicopathological correlates

Pandita,

Dave,

Schulte

2023

BMJ Case Rep

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Leiomyosarcoma (LMS) is a subtype of sarcoma derived from smooth muscle cells. Unfortunately, this malignancy has a high rate of metastatic disease. Palliative systemic therapy has historically relied on cytotoxic agents such as doxorubicin, which have low rates of response. Immunotherapy has not been shown to be effective for most patients with sarcoma, including those with LMS. However, this has not been well described for patients with LMS and high tumour mutational burden (TMB). Herein, we report the case of a woman in her late 50s with metastatic high TMB (>10) leiomyosarcoma treated with pembrolizumab.

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Section: Discussionmentioning

confidence: 78%

PD-1 Inhibition in metastatic high tumour mutational burden (TMB) leiomyosarcoma with clinicopathological correlates

Pandita,

Dave,

Schulte

2023

BMJ Case Rep

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“…Among them, 12 could be evaluated for tumor response, with one complete response, three partial responses, six stable diseases, and two progressive diseases. 27 Other small series or case reports have also reported heterogeneous responses to ICI in MSI-H/MMRD STSs 21 , 28 ; however, these series did not include patients with known Lynch syndrome. Whether germline predisposition or specific histological subtypes are associated with increased efficacy of ICI in patients with MSI-H/MMRD STSs will require further investigation and larger cohorts.…”

Section: Discussionmentioning

confidence: 99%

Metastatic Malignant Perivascular Epithelioid Cell Tumors With Microsatellite Instability Within Lynch Syndrome Successfully Treated With Anti-PD1 Pembrolizumab

Djerroudi

Masliah‐Planchon

Brisse

et al. 2023

JCO Precision Oncology

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“…The keynote 158 study was a major success, demonstrating the efficacy of pembrolizumab in non-colorectal high microsatellite instability [90], thus paving the way for microsatellite instability testing in sarcomas. Albeit rare [91], some soft tissue sarcomas may exhibit this biological propriety, with or without PD-L1 expression, prompting pembrolizumab as an effective treatment; recently, Yon Tay et al described a case of complete tumoral response in a soft tissue LMS with these features [92].…”

Section: Treatmentmentioning

confidence: 99%

When Vessels and Sarcomas Combine: A Review of the Inferior Vena Cava Leiomyosarcoma

Gama,

Almeida,

Oliveira

et al. 2024

JVD

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Leiomyosarcomas (LMSs) are malignant neoplasms of soft muscle differentiation that can be classified into five distinct groups according to site-related origin: intra-abdominal, subcutaneous or deep soft tissue of the limbs, cutaneous, external genitalia, and vascular. This distinction reflects different biological behaviors as well as molecular changes, thus reflecting different prognoses and therapeutic options. Vascular LMSs are the least frequent, arising from the walls of the blood vessels, most commonly from the inferior vena cava. Due to its deep location, symptoms are non-specific, and the disease presents at an advanced stage, sometimes with metastases. Surgery is the treatment of choice, associated with chemo- and radiotherapy. Due to its rarity, most departments have minimal experience handling this disease. This article reviews the current knowledge on vascular leiomyosarcomas, particularly the inferior vena cava leiomyosarcoma.

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Soft Tissue Leiomyosarcoma With Microsatellite Instability, High Tumor Mutational Burden, and Programmed Death Ligand-1 Expression Showing Pathologic Complete Response to Pembrolizumab: A Case Report

Cited by 5 publications

References 32 publications

PD-1 Inhibition in metastatic high tumour mutational burden (TMB) leiomyosarcoma with clinicopathological correlates

PD-1 Inhibition in metastatic high tumour mutational burden (TMB) leiomyosarcoma with clinicopathological correlates

Metastatic Malignant Perivascular Epithelioid Cell Tumors With Microsatellite Instability Within Lynch Syndrome Successfully Treated With Anti-PD1 Pembrolizumab

When Vessels and Sarcomas Combine: A Review of the Inferior Vena Cava Leiomyosarcoma

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