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

Djerroudi, Lounes; Masliah‐Planchon, Julien; Brisse, H.; Helfre, Sylvie; Tzanis, Dimitri; Hamzaoui, Nadim; Laurence, Valérie; Bonvalot, Sylvie; Watson, Sarah

doi:10.1200/po.22.00627

Cited by 3 publications

(1 citation statement)

References 27 publications

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“…Angiomyolipoma (AML) is a benign, solid, mesenchymal tumor and is one of the perivascular epithelioid cell neoplasms (PEComas) [ 1 - 2 ]. It is the most common benign renal tumor but rarely encountered at extrarenal sites, the liver being the second commonest site [ 3 - 4 ], and is usually an incidental imaging finding [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Angiomyolipoma of Uterine Cervix: Report of a Rare Case

Kiran¹,

Ramanarasimhaiah²,

Khan³

et al. 2023

Cureus

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Angiomyolipoma (AML) is classified as perivascular epithelioid cell neoplasm (PEComas) and is commonly seen in the kidney. AML is a solid mesenchymal neoplasm rarely encountered at the extrarenal site. Extrarenal AML is infrequently seen in the female genital tract. Four cases of AML of the cervix have been reported in the literature to our knowledge. We report a case of a 44-year-old female patient who presented with complaints of “lower abdominal pressure” and a history of post-coital bleeding and human papillomavirus (HPV) infection. A cyst in the uterine cervix was found incidentally on computerized tomography (CT) scan of the abdomen and pelvis. The patient underwent a loop electrosurgical excision procedure. The histologic and immunohistochemical features of the cervical biopsy favored the diagnosis of AML. The patient underwent a laparoscopic hysterectomy with bilateral salpingectomy. Grossly, a 4 cm white soft-to-firm mass was identified within the anterior lip of the cervix. Microscopy of the mass showed smooth muscle proliferation with prominent blood vessels, and scant mature adipose tissue trapped in between the smooth muscle bundles. Immunohistochemical stains showed smooth muscle actin (SMA) and desmin highlighting the smooth muscle component of AML. The histology and immunohistochemistry of the cervical mass in the surgical specimen were identical to the biopsy specimen and a diagnosis of AML was made.

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Section: Introductionmentioning

confidence: 99%

Angiomyolipoma of Uterine Cervix: Report of a Rare Case

Kiran¹,

Ramanarasimhaiah²,

Khan³

et al. 2023

Cureus

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Durable response to pembrolizumab in hepatic metastasis from colonic carcinoma with Lynch syndrome: a case report

Xue,

Zhu,

Wang

et al. 2024

Front. Immunol.

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Pembrolizumab and other immunotherapies have become central in treating metastatic colon cancer, particularly effective in patients with mismatch repair deficiencies. We report a case involving a man who initially underwent radical surgery for sigmoid colon cancer on April 27, 2011, followed by hepatic tumor resection on September 21, 2017. Post-surgery, he received eight cycles of adjuvant chemotherapy with the CAPEOX regimen and was regularly monitored through CT and MRI scans. On August 24, 2022, liver metastases were detected, and he was diagnosed with Lynch syndrome (LS) due to germline mutation in the MSH2 and EPCAM genes. He commenced treatment with 200mg of pembrolizumab intravenously every three weeks on September 2, 2022, and demonstrated a sustained response. However, after 17 cycles, he developed a treatment related adverse event (TRAE) of pancreatic endocrine dysfunction, leading to type 1 diabetes, managed with subcutaneous insulin injections. After 30 cycles of treatment, no evidence of disease was observed. This case underscores the significant clinical benefits of first-line pembrolizumab in managing hepatic metastasis in colonic carcinoma associated with LS, despite the occurrence of TRAEs. It raises critical questions regarding the optimal duration of immunotherapy following a complete or partial response and whether treatment should be discontinued upon the emergency of TRAEs. Continued research and forthcoming clinical trials with checkpoint inhibitors are expected to refine treatment protocols for LS-associated carcinoma.

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Prices and Trends in FDA-Approved Medications for Sarcomas

Hwang,

Agulnik,

Schulte

2024

Cancers

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Sarcomas represent a diverse set of both malignant and benign subtypes consisting of often rare and ultra-rare conditions. Over the course of the last decade, there have been numerous FDA approvals for agents treating various sarcoma subtypes. Given this burgeoning landscape of sarcoma treatments, we seek to review current FDA-approved agents with respect to their rates of incidence, approval rates, and financial costs. We gathered clinical trial data by searching FDA approval announcements from 2013 to 2023. We determined the 30 day and one year cost of therapy for patients of FDA-approved sarcoma treatments in the aforementioned timeframe. From 2013 to 2023, 14 medications have been FDA-approved for sarcoma subtypes. The 30-day dosing prices for these medications range from $11,162.86 to $46,926.00. Since 2013, the rates of approval for sarcoma medications have been higher than in prior decades. Nonetheless, there remains the potential for significant financial toxicity for patients living with sarcoma.

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

Cited by 3 publications

References 27 publications

Angiomyolipoma of Uterine Cervix: Report of a Rare Case

Angiomyolipoma of Uterine Cervix: Report of a Rare Case

Durable response to pembrolizumab in hepatic metastasis from colonic carcinoma with Lynch syndrome: a case report

Prices and Trends in FDA-Approved Medications for Sarcomas

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