Perivascular epithelioid tumors: Utility of the positron emission tomography with 18F-fluorodesoxyglucose (PET-TAC FDG) in their staging and follow-up

• Published in: Revista española de medicina nuclear Vol. 29; no. 5; pp. 258 - 262
• Main Authors: Navarro-Pelayo Lainez, M.M., Ramos-Font, C., Rebollo Aguirre, A.C., Rodríguez-Fernández, A., Llamas-Elvira, J.M.
• Format: Journal Article
• Language: English; Spanish
• Published: Spain Elsevier España 2010
• Subjects: 18F-fluordesoxiglucosa; 18F-fluorodesoxyglucose; Adult; Angiofibroma - diagnostic imaging; Angiofibroma - genetics; Chemotherapy, Adjuvant; Combined Modality Therapy; Endometrial Neoplasms - diagnostic imaging; Endometrial Neoplasms - pathology; Endometrial Neoplasms - surgery; Estadificación; Estrogens; Facial Neoplasms - genetics; Female; Fluorine Radioisotopes; Fluorodeoxyglucose F18; Humans; Hysterectomy; Incidental Findings; Kidney Neoplasms - diagnostic imaging; Lung Neoplasms - diagnostic imaging; Lung Neoplasms - drug therapy; Lung Neoplasms - secondary; Neoplasms, Hormone-Dependent - diagnostic imaging; Neoplasms, Hormone-Dependent - drug therapy; Neoplasms, Hormone-Dependent - secondary; Neoplasms, Hormone-Dependent - surgery; Neoplasms, Multiple Primary - diagnostic imaging; Perivascular Epithelioid Cell Neoplasms - diagnostic imaging; Perivascular Epithelioid Cell Neoplasms - drug therapy; Perivascular Epithelioid Cell Neoplasms - secondary; Perivascular Epithelioid Cell Neoplasms - surgery; Perivascular epithelioid neoplasms; Positron emission tomography; Radiopharmaceuticals; Response assessment; Respuesta terapéutica; Staging; Tomografía por emisión de positrones; Tomography, X-Ray Computed; Tuberous Sclerosis - diagnostic imaging; Tuberous Sclerosis - genetics; Tumor epitelioide perivascular; Tumor uterino; Uterine neoplasms; Respuesta terapéutica; Perivascular epithelioid neoplasms; Staging; Estadificación; Tumor uterino; 18F-fluorodesoxyglucose; 18F-fluordesoxiglucosa; Tumor epitelioide perivascular; Response assessment; Positron emission tomography; Uterine neoplasms; Tomografía por emisión de positrones
• ISSN: 1578-200X; 0212-6982; 1578-200X
• DOI: 10.1016/S1578-200X(10)70032-2

Perivascular epithelioid tumors (PEComas) are a rare group of mesenchymal neoplasms with an unpredictable natural history and uncertain malignant potential. Uterine involvement and their association with tuberous sclerosis are typical for these tumors. We present a case of a 40-year old patient who was incidentally diagnosed of a uterine PEComa and serial studies of PET-CT with FDG were performed for staging and therapeutic response assessment. FDG PET-CT proved to be a valuable tool for detecting unsuspected pulmonary metastases and defining the reassessment of the patient after chemotherapy. The findings suggest that since this is a rare tumor, which does not always have benign behavior, PET-CT may be a useful diagnostic imaging procedure for staging and clinical monitoring of patients who suffer this type of tumors. Los tumores epitelioides perivasculares son un raro grupo de neoplasias mesenquimales con una historia natural impredecible y un incierto potencial de malignidad. Es característica la localización uterina y su asociación a esclerosis tuberosa. Presentamos el caso de una paciente de 40 años de edad, en la que se diagnosticó de forma incidental un tumor epitelioide perivascular uterino y se le realizaron estudios seriados de tomografía por emisión de positrones (PET-TAC) con 18F-fluordesoxiglucosa para su estadificación y valoración de la respuesta terapéutica. La PET-TAC resultó ser una herramienta muy valiosa en la detección de metástasis pulmonares tardías y en la revaluación de la paciente tras la administración de quimioterapia. Los hallazgos sugieren que, dado que estamos ante un tumor infrecuente, que no siempre tiene un comportamiento benigno, la PET-TAC puede ser un procedimiento de imagen diagnóstica útil para realizar un estudio de extensión y un seguimiento clínico de las pacientes que sufran este tipo de neoplasias.