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The role of (18)F-FDG PET/CT imaging in patient with malignant PEComa treated with mTOR inhibitor.

Sun L, Sun X, Li Y, Xing L - Onco Targets Ther (2015)

Bottom Line: Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established.Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activation of mTOR pathway and may guide selectively inhibiting mTOR pathway treatment.The tumor had shown significant avidity on PET/CT as well as an evident response to sirolimus (rapamycin, Rapamune™) that supports the utility of mTOR inhibitors as an effective treatment for malignant PEComa.

View Article: PubMed Central - PubMed

Affiliation: School of Medicine and Life Sciences, University of Jinan-Shandong Academy of Medical Sciences, Jinan, Shandong, People's Republic of China.

ABSTRACT
Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established. Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activation of mTOR pathway and may guide selectively inhibiting mTOR pathway treatment. We report a malignant PEComa patient who presented for (18)F-FDG positron emission tomography/computed tomography (PET/CT) restaging. The tumor had shown significant avidity on PET/CT as well as an evident response to sirolimus (rapamycin, Rapamune™) that supports the utility of mTOR inhibitors as an effective treatment for malignant PEComa. Therefore, (18)F-FDG PET/CT is helpful in restaging and guiding treatment for malignant PEComa with mTOR inhibitors.

No MeSH data available.


Related in: MedlinePlus

Pathological findings of the pulmonary metastases from malignant uterine PEComa.Notes: (A) Hematoxylin and eosin stain, magnification ×100. (B and C) HMB-45 and SMA immunohistochemical stain, magnification ×200. (D) Ki-67 immunohistochemical stain, magnification ×200. The average Ki-67 labeling index is 40% in this tumor. Background staining was identified by negative controls in which the sections were performed by substitution of primary antibodies with phosphate buffer solution.Abbreviations: HMB-45, human melanoma black 45; PEComa, perivascular epithelioid cell tumor; SMA, smooth muscle actin.
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f1-ott-8-1967: Pathological findings of the pulmonary metastases from malignant uterine PEComa.Notes: (A) Hematoxylin and eosin stain, magnification ×100. (B and C) HMB-45 and SMA immunohistochemical stain, magnification ×200. (D) Ki-67 immunohistochemical stain, magnification ×200. The average Ki-67 labeling index is 40% in this tumor. Background staining was identified by negative controls in which the sections were performed by substitution of primary antibodies with phosphate buffer solution.Abbreviations: HMB-45, human melanoma black 45; PEComa, perivascular epithelioid cell tumor; SMA, smooth muscle actin.

Mentions: A 46-year-old female presented to our institution with solitary renal and pulmonary lesions 2 years after the initial diagnosis of uterine leiomyosarcoma. Left nephrectomy and right lower lung lobe wedge resection were performed. Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the initial uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases. Pulmonary metastases from malignant uterine PEComa display nests of epithelioid cells with small round nuclei surrounding thin-walled capillary vessels (Figure 1A). Tumor shows diffusely positive staining for melanocytic marker and smooth muscle marker (Figure 1B and C). A high Ki-67 labeling index of 40% indicates the aggressive behavior of the tumor (Figure 1D).


The role of (18)F-FDG PET/CT imaging in patient with malignant PEComa treated with mTOR inhibitor.

Sun L, Sun X, Li Y, Xing L - Onco Targets Ther (2015)

Pathological findings of the pulmonary metastases from malignant uterine PEComa.Notes: (A) Hematoxylin and eosin stain, magnification ×100. (B and C) HMB-45 and SMA immunohistochemical stain, magnification ×200. (D) Ki-67 immunohistochemical stain, magnification ×200. The average Ki-67 labeling index is 40% in this tumor. Background staining was identified by negative controls in which the sections were performed by substitution of primary antibodies with phosphate buffer solution.Abbreviations: HMB-45, human melanoma black 45; PEComa, perivascular epithelioid cell tumor; SMA, smooth muscle actin.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4525788&req=5

f1-ott-8-1967: Pathological findings of the pulmonary metastases from malignant uterine PEComa.Notes: (A) Hematoxylin and eosin stain, magnification ×100. (B and C) HMB-45 and SMA immunohistochemical stain, magnification ×200. (D) Ki-67 immunohistochemical stain, magnification ×200. The average Ki-67 labeling index is 40% in this tumor. Background staining was identified by negative controls in which the sections were performed by substitution of primary antibodies with phosphate buffer solution.Abbreviations: HMB-45, human melanoma black 45; PEComa, perivascular epithelioid cell tumor; SMA, smooth muscle actin.
Mentions: A 46-year-old female presented to our institution with solitary renal and pulmonary lesions 2 years after the initial diagnosis of uterine leiomyosarcoma. Left nephrectomy and right lower lung lobe wedge resection were performed. Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the initial uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases. Pulmonary metastases from malignant uterine PEComa display nests of epithelioid cells with small round nuclei surrounding thin-walled capillary vessels (Figure 1A). Tumor shows diffusely positive staining for melanocytic marker and smooth muscle marker (Figure 1B and C). A high Ki-67 labeling index of 40% indicates the aggressive behavior of the tumor (Figure 1D).

Bottom Line: Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established.Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activation of mTOR pathway and may guide selectively inhibiting mTOR pathway treatment.The tumor had shown significant avidity on PET/CT as well as an evident response to sirolimus (rapamycin, Rapamune™) that supports the utility of mTOR inhibitors as an effective treatment for malignant PEComa.

View Article: PubMed Central - PubMed

Affiliation: School of Medicine and Life Sciences, University of Jinan-Shandong Academy of Medical Sciences, Jinan, Shandong, People's Republic of China.

ABSTRACT
Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established. Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activation of mTOR pathway and may guide selectively inhibiting mTOR pathway treatment. We report a malignant PEComa patient who presented for (18)F-FDG positron emission tomography/computed tomography (PET/CT) restaging. The tumor had shown significant avidity on PET/CT as well as an evident response to sirolimus (rapamycin, Rapamune™) that supports the utility of mTOR inhibitors as an effective treatment for malignant PEComa. Therefore, (18)F-FDG PET/CT is helpful in restaging and guiding treatment for malignant PEComa with mTOR inhibitors.

No MeSH data available.


Related in: MedlinePlus