Treatment of Advanced Malignant Uterine Perivascular Epithelioid Cell Tumor with mTOR Inhibitors: Single-institution Experience and Review of the Literature

Starbuck, Kristen; Drake, Richard D.; Budd, G. Thomas; Rose, Peter G.

doi:10.21873/anticanres.11208

Cited by 45 publications

(38 citation statements)

References 14 publications

(12 reference statements)

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“…erefore, drugs such as sirolimus or everolimus that suppress mTOR signaling might be potential therapeutic options. Prolonged responses can be achieved with these medications, but costs and side effects are limiting factors [7,[14][15][16][17]. Most recently, encouraging results have been published with nanoparticle albumin-bound sirolimus (nab-sirolimus) for treatment of patients with advanced PEComas.…”

Section: Discussionmentioning

confidence: 99%

A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature

Luna

Mosquera

Comba

et al. 2020

Case Reports in Medicine

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Epithelioid angiomyolipomas (EAMLs) are mesenchymal tumors that are part of the family of the perivascular epithelioid cell neoplasms (PEComas). These tumors portray a potential aggressive behavior with metastatic lesions found in around 30% of reported cases. EAMLs might present sporadically or in association with the tuberous sclerosis complex (TSC). They typically involve the kidneys, liver, and lungs. It is extremely rare for these tumors to arise from other organs. The present report describes an unusual case of an adult patient with a history of TSC who developed EAML of the adrenal gland. Moreover, he presented with metastatic disease to the liver, a feature rarely described. The diagnosis of EAMLs can be challenging as they are hard to distinguish from other adrenal or renal tumors without a thorough histopathologic and immunohistochemical evaluation. Due to the potential aggressive behavior of these malignancies, timely diagnosis is extremely important and has significant therapeutic and prognostic implications.

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Section: Discussionmentioning

confidence: 99%

A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature

Luna

Mosquera

Comba

et al. 2020

Case Reports in Medicine

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“…All of the pediatric patients [14,[16][17][18] were treated with surgery as an initial management strategy. However, the preferred adjuvant therapy, including doxorubicin, paclitaxel, gemcitabine, and oxaliplatin alone or in combinations, is a matter of contention [5,9,13,14,27,28]. For benign PEComa, no standardized regimen has been provided to avoid recurrence after surgery [3,4].…”

Section: Folpe Et Al[11] Established a Series Of Criteria To Distingmentioning

confidence: 99%

“…PEComas NOS are typically diagnosed in women aged 38.9-56 years and are focused on the uterus and gastrointestinal (GI) tract, with the uterine corpus lesions accounting for 41% of the reported cases [1,[4][5][6][7][8]. Immunohistochemically, PEComas NOS typically exhibit positive melanocytic and myogenic markers, such as human melanoma black 45 (HMB45), melanoma antigen recognized by T cells (melan-A), and smooth muscle actin, and negative expressions of protein S100, cytokeratin, and desmin [9][10][11]. However, the histogenesis and corresponding normal cellular counterpart of PEComa remain unknown [1,3], and a standardized treatment strategy remains unestablished [2].…”

Section: Introductionmentioning

confidence: 99%

“…Because of their unpredictable histopathological manifestation, patients with aggressive PEComas have poor prognosis and do not respond well to chemotherapy [5,12,13]. The mammalian target of rapamycin (mTOR) inhibitor treatment remains controversial in patients lacking the tuberous sclerosis complex (TSC) gene mutation [5,9]. To date, radical surgical resection is an effective treatment for primary tumor and local recurrence prevention [2].…”

Section: Introductionmentioning

confidence: 99%

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Unusual Pediatric Sigmoid Perivascular Epithelioid Cell Tumor with Regional Lymph Node Metastasis Treated Using Gemcitabine and Docetaxel: A Case Report and Literature Review

Cheng

Kuo

Huang

et al. 2020

Preprint

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Background Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, and few pediatric cases have been reported. However, standardized treatment for the tumor type remains unestablished. Herein, we report a case of a pediatric patient with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. The patient was successfully treated with complete tumor resection and gemcitabine/docetaxel combination chemotherapy without local or distant recurrence after 12 months postsurgery.Case presentation A 17-year-old female adolescent presented with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. She was treated with surgical resection of the tumor and cytotoxic chemotherapy with gemcitabine (900 mg/m2) and docetaxel (100 mg/m2) every 3 weeks. There was no recurrence of hematochezia stool, and complete response was achieved, with a progression-free survival of 12 months.Conclusion Surgical resection with adjuvant conventional cytotoxic chemotherapy can be considered as the first-line treatment for early-stage gastrointestinal PEComa.

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“…Since mTOR signaling can be regarded as the tumor driver in these cases, its inhibition represents a promising target for pharmacologic therapy. Although some reports have shown beneficial effects of drugs directed against mTOR, others have not and some authors have doubted the general importance of this therapy [9,24,25,27]. It appears that the combination of genetic findings in the TSC1/TSC2 complex and staining of the mTOR signaling pathway may predict response to mTOR inhibitors.…”

Section: Clinical Managementmentioning

confidence: 99%

Complexity of PEComas

et al. 2019

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Treatment of Advanced Malignant Uterine Perivascular Epithelioid Cell Tumor with mTOR Inhibitors: Single-institution Experience and Review of the Literature

Cited by 45 publications

References 14 publications

A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature

A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature

Unusual Pediatric Sigmoid Perivascular Epithelioid Cell Tumor with Regional Lymph Node Metastasis Treated Using Gemcitabine and Docetaxel: A Case Report and Literature Review

Complexity of PEComas

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