Primary Bone Marrow Epithelioid Hemangioendothelioma Treated With Gemcitabine and Docetaxel

Sabile, Jean; Stump, Michael; Fitzpatrick, Fielding C; Skelton, Matthew R.

doi:10.1200/op.20.00360

Cited by 2 publications

(3 citation statements)

References 4 publications

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“…Notably, gemcitabine is particularly active in sarcomas of vascular origin, representing a therapeutic option for the histology-driven treatment of angiosarcoma, epithelioid hemangioendothelioma, and intimal sarcoma. 22 – 25 , 37 Furthermore, we knew that gemcitabine does not have dose-limiting toxicity. Thus, in a neoplasm with an indolent course, with a long life expectancy, this element represents a great advantage.…”

Section: Discussionmentioning

confidence: 99%

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Safety and effectiveness of gemcitabine for the treatment of classic Kaposi’s sarcoma without visceral involvement

et al. 2022

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Background: Classic Kaposi’s sarcoma (CKS) is a rare, multifocal, endothelial cell neoplasm that typically occurs in elderly people with previous infection by human herpes virus-8. Prospective trials are rare, and the choice of drugs relies on prospective trials performed on HIV-associated Kaposi’s sarcoma (KS). Pegylated liposomal anthracyclines and taxanes are considered the standard first- and second-line chemotherapy, respectively. Despite the indolent biologic behavior, the natural history is characterized by recurrent disease. This condition of chronic administration of cytotoxic drugs is often associated with immediate/long-term adverse events. Methods: This was an observational, retrospective study to evaluate the effectiveness and safety of gemcitabine in patients with CKS. From January 2016 to September 2021, the patients were treated with gemcitabine 1000 mg/m2 on days 1 and 8, with cycles repeated every 21 days. The treatment was administered as first or second line. Results: Twenty-seven (27) patients were included in the study. Twenty-one (21) out 27 patients (77.8%) achieved a partial response (PR), including 8 patients with major response (MR) (29.6%) and 13 patients with minor response (mR) (48.2%); 2 (7.4%) showed a complete response (CR), 3 (11.1%) a stable disease (SD), and 1 (3.7%) a progressive disease (PD). Tumor responses were generally rapid, with a median time to first response of 4 weeks (range, 3–12 weeks). Patients who responded had disease improvement with flattening of the skin lesions, decrease in the number of lesions, and substantial reduction in tumor-associated complications. Median duration of response was 19.2 months. Common adverse events were grades 1/2 thrombocytopenia, and grade 1 noninfectious fever. No patient discontinued treatment as a result of adverse events. Conclusion: Our study showed that gemcitabine is effective and well tolerated, acts rapidly on cutaneous lesions, and allows substantial symptom palliation, without dose-limiting toxicity. Gemcitabine represents a safe and effective option for the treatment of CKS.

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Section: Discussionmentioning

confidence: 99%

“…21 Interestingly, in the context of sarcomas, gemcitabine showed activity in the treatment of advanced vascular sarcomas, including angiosarcoma, epithelioid hemangioendothelioma, and intimal sarcoma. [22][23][24][25] Based on this background, gemcitabine could represent a rational therapeutic approach in classic Kaposi sarcoma.…”

Section: Introductionmentioning

confidence: 99%

Safety and effectiveness of gemcitabine for the treatment of classic Kaposi’s sarcoma without visceral involvement

et al. 2022

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“…Gemcitabine as an anti-metabolite mediates its cytotoxic effects via integration into the DNA of tumor cells leading to an abortion of DNA synthesis (Plunkett et al 1995 ). To our knowledge, only case series of patients with advanced EHE treated with gemcitabine, mostly in combination with docetaxel, are reported (Frezza et al 2021 ; Sabile et al 2021 ; Zhou et al 2020 ). According to these publications, systemic treatment of EHE with gemcitabine, at least in combination with docetaxel, could lead to a stabilization of the disease in some patients.…”

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confidence: 99%

NOTCH3 missense mutations as predictor of long-term response to gemcitabine in a patient with epithelioid hemangioendothelioma

Schmidt

Mattern²,

Singer³

et al. 2023

J Cancer Res Clin Oncol

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Purpose Epithelioid hemangioendothelioma (EHE) as a very rare malignant vascular tumor belongs to the heterogenous group of soft-tissue sarcomas. Depending on the clinical course of the disease, interdisciplinary treatment concepts are required, including surgery, radiotherapy and systemic cancer therapy. However, due to its uncommonness, standard treatment options are lacking so far, especially in advanced disease with distant metastases. Methods and results Here we report on an unusual case of a patient with metastasized EHE showing long-term response to second line treatment with gemcitabine over almost 2 decades. Cancer genome sequencing of the patient’s tumor tissue detected a NOTCH3 missense mutation which could provide an explanation for these clinical findings. NOTCH3 is known to be a mediator of resistance towards gemcitabine-based cancer treatment, at least in pancreatic cancer and non-small cell lung cancer. Conclusion The observation that this missense mutation of NOTCH3 is associated with an increased response to treatment with gemcitabine in EHE can be used prospectively to assess NOTCH3 as potential biomarker for predicting therapy response to gemcitabine.

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Primary Bone Marrow Epithelioid Hemangioendothelioma Treated With Gemcitabine and Docetaxel

Cited by 2 publications

References 4 publications

Safety and effectiveness of gemcitabine for the treatment of classic Kaposi’s sarcoma without visceral involvement

Safety and effectiveness of gemcitabine for the treatment of classic Kaposi’s sarcoma without visceral involvement

NOTCH3 missense mutations as predictor of long-term response to gemcitabine in a patient with epithelioid hemangioendothelioma

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