RESUMO
Clear cell tumors of the lung (CCTL), or "sugar tumors" of lung, are very uncommon lesions and are mostly benign perivascular epithelioid cell (PEC) tumors with no specific morphologic features. Fewer than 100 cases have been reported; the aggressive nature demonstrated in sporadic reports has rarely been described in the literature. Although the course is generally described as benign, eight reported cases showed malignant behavior. We report a case of a PEC with a malignant presentation in a young man, correlating the main characteristics of the tumor with other cases reported in the literature to better elucidate this rare presentation. We also performed a literature review of reports on benign and malignant CCTL cases, with a focus on clinical, imaging, and immunohistochemical differentiation. CCTLs are rare tumors that require histopathological and immunohistochemical confirmation; to date, criteria that can predict malignant evolution are lacking.
RESUMO
Clear cell "sugar" tumor of the lung is a rare benign tumor arising from perivascular epithelioid cells (PECs). They belong to a group of mesenchymal neoplasms called PEComas. Although widely presumed as benign, due to their rich vascular stroma they are usually avid for the different types of contrast agents used in imaging studies, mimicking a malignant lesion. We report the case of a 66-year-old man in whom a solitary pulmonary nodule was discovered during oncological staging for an adenocarcinoma of the prostate who underwent an anatomical pulmonary segmentectomy. The final pathology result was a perivascular epithelioid cell tumor (pulmonary PEComa or clear "sugar" cell tumor).
Los tumores de células claras "de azúcar" (CCTL) son lesiones benignas muy infrecuentes. Forman parte de un grupo de neoplasias mesenquimales denominadas PEComas que se originan de las células epiteloides perivasculares. Por su rico estroma vascular, suelen tener avidez por los distintos tipos de contrastes utilizados en los estudios de diagnóstico por imágenes, simulando lesiones de estirpe maligna. Presentamos el caso de un paciente de 66 años con hallazgo de un nódulo pulmonar durante la estadificación oncológica por adenocarcinoma de próstata al que se le realizó una segmentectomía pulmonar anatómica. El resultado definitivo de anatomía patológica fue tumor de células epiteloides perivasculares (PEComa pulmonar o tumor de células claras "de azúcar").
Assuntos
Adenocarcinoma , Neoplasias Pulmonares , Neoplasias de Células Epitelioides Perivasculares , Masculino , Humanos , Idoso , Neoplasias de Células Epitelioides Perivasculares/diagnóstico por imagem , Neoplasias de Células Epitelioides Perivasculares/cirurgia , Pulmão/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/cirurgiaRESUMO
Resumen Los tumores de células claras "de azúcar" (CCTL) son lesiones benignas muy infrecuentes. Forman parte de un grupo de neoplasias mesenquimales denominadas PEComas que se originan de las células epiteloides perivasculares. Por su rico estroma vascular, suelen tener avidez por los distintos tipos de contrastes utilizados en los estudios de diagnóstico por imágenes, simulando lesiones de estirpe maligna. Presentamos el caso de un paciente de 66 años con hallazgo de un nódulo pulmonar durante la estadificación oncológica por adenocarcinoma de próstata al que se le realizó una segmentectomía pulmonar anatómica. El resultado definitivo de anatomía patológica fue tumor de células epiteloides perivasculares (PEComa pulmonar o tumor de células claras "de azúcar").
Abstract Clear cell ''sugar'' tumor of the lung is a rare benign tumor arising from perivascular epithelioid cells (PECs). They belong to a group of mesenchymal neoplasms called PEComas. Although widely presumed as benign, due to their rich vascular stroma they are usually avid for the different types of contrast agents used in imaging studies, mimicking a malignant lesion. We report the case of a 66-year-old man in whom a solitary pul monary nodule was discovered during oncological staging for an adenocarcinoma of the prostate who underwent an anatomical pulmonary segmentectomy. The final pathology result was a perivascular epithelioid cell tumor (pulmonary PEComa or clear "sugar" cell tumor).
RESUMO
We present the case of a young male patient with an initial diagnosis of a rhabdomyoma that was surgically treated at a different hospital when he was 17. After a 2-year disease-free period, the patient presented another intra-cardiac mass. He refused surgical treatment and died 5 years later. Post-mortem immunochemistry studies of both tumors led to the diagnosis of a primary malignant cardiac PEComa with histopathologic characteristics that resembled a rhabdomyoma with abundant "spider cells."
RESUMO
Perivascular epithelioid cell neoplasms (PEComas) are a tumor family defined as such just a couple of decades ago. They make an unusual group of neoplasms, which can appear in different locations of the organism. PEComas are usually considered to be benign tumors, but there are some histological features that make some subgroups suspicious of malignancy. The treatment of these tumors consist in their surgical resection, with no current effective complementary oncological treatment known. We present the clinical case of a woman that underwent surgery for a resection of a hepatic lesion labeled afterwards as a PEComa with malignant features.
Los tumores de células neoplásicas perivasculares epitelioides (PEComas) son una familia de tumoraciones caracterizada apenas un par de décadas antes. Componen un grupo inusual de neoplasias, que puede aparecer en distintas localizaciones del organismo. Por lo general, los PEComas se consideran tumores benignos, pero hay ciertas características histológicas que hacen de algunos subgrupos lesiones sospechosas de una malformación maligna. El tratamiento de estos tumores consiste en la resección quirúrgica, pero no existe tratamiento oncológico por completo eficaz. Se presenta el caso clínico de una mujer sometida a la resección de una lesión hepática con diagnóstico posterior de PEComa con rasgos de proceso maligno.