Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics* ; Biomarkers, Tumor ; Humans ; Perivascular Epithelioid Cell Neoplasms/surgery* ; Stomach

Objective: To investigate hepatic perivascular epithelioid cell tumor (PEComa) diagnosis and treatment plan. Methods: 24 cases diagnosed with PEComa clinical manifestations, serum alpha fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen 199 (CA199), imaging findings, surgical methods, postoperative hospital stay, pathological results and prognosis were analyzed retrospectively from September 2015 to September 2020. Results: Majority of patients were females (79.2%), aged 41.5±11.4 years. Tumors were predominantly located in the right liver (50.0%). 76.7% of the cases were mostly clinically asymptomatic. AFP, CEA and CA199 indices were all negative. CT mostly showed low density tumor in the plain scan phase, enhanced in the enhancement phase, and enhanced and weakened in portal venous and equilibrium phase (66.7%). MRI manifestations of most tumors were hypointense on T₁WI and hyperintense on T₂WI (72.7%). B-ultrasound mostly showed hyperechoic mass in the tumor area with punctate vascular shadow (52.9%). Postoperative hospital stay was 9.0±2.4 days for laparoscopic surgery patients (n=13), 13.4±6.3 days for traditional laparotomy (hereinafter referred to as laparotomy, n=10), and 3 days for 1 patient with microwave ablation. All postoperative pathological results were positive for HMB45 and Melan-A. Follow-up results: 21 cases survived normally, with no tumor recurrence in the recent physical examination; two cases had tumor recurrence and they died two and three years after surgery, and one case was lost to follow-up. Conclusion: Hepatic PEComa more commonly occurs in middle-aged women, with no specific features for tumor markers and clinical manifestations. Some imaging findings are specific, so its features can be combined as a basis for diagnosis. Postoperative pathological examination results can confirm the diagnosis. Therefore, surgery remains the initial treatment plan. Microwave ablation and laparoscopic surgery are recommended as the preferred option because of shorter hospital stays and less trauma than open surgery.
Adult ; Biomarkers, Tumor/analysis* ; Carbohydrates ; Carcinoembryonic Antigen ; Female ; Humans ; Liver/pathology* ; MART-1 Antigen ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Perivascular Epithelioid Cell Neoplasms/surgery* ; Retrospective Studies ; alpha-Fetoproteins

Carcinoma, Papillary/surgery* ; Humans ; Parapharyngeal Space ; Perivascular Epithelioid Cell Neoplasms ; Thyroid Cancer, Papillary ; Thyroid Neoplasms/surgery*

Adolescent ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leg ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery

A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant pulmonary PEC tumor with lung to lung metastases was established. Herein, the difficulties of diagnosis and management we confronted are described.
Diagnosis, Differential ; Follow-Up Studies ; Humans ; Lung Neoplasms/*diagnosis/surgery ; Male ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms/*diagnosis/surgery ; Pneumonectomy ; Positron-Emission Tomography/*methods ; Thoracic Surgery, Video-Assisted/methods ; Tomography, X-Ray Computed/*methods

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Endometrial Neoplasms ; metabolism ; pathology ; surgery ; Endometrial Stromal Tumors ; metabolism ; pathology ; surgery ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery

Actins ; metabolism ; Angiomyoma ; metabolism ; pathology ; surgery ; Cranial Fossa, Middle ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; immunology ; S100 Proteins ; metabolism ; Skull Base Neoplasms ; metabolism ; pathology ; surgery

Perivascular epithelioid cell tumor (PEComa) is extremely rare, which originated from mesenchymal cells in the intestine, and composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. We report here on a case of PEComa in the sigmoid colon. A 62-year-old woman presented with hematochzia 10 days ago. Her abdominal computed tomography scan showed a 5 cm sized intraluminal fungating heterogeneously enhanced, high density mass, which infiltrated pericolic tissue surrounding the sigmoid colon. Colonoscopy showed a purple colored polypoid mass with lobulating contour in the sigmoid colon. She underwent laparoscopic anterior resection. On the histologic examination, the tumor consisted of polygonal epithelioid cells with sheet-like growth of nests, which looked like alveolar tissues in lung. The tumor cells were strongly positive stained with human melanoma black-45 (HMB-45). Pathologic examination was compatible with PEComa. Sixteen months after surgery, she did well without tumor recurrence after surgery. We review the literatures concerning PEComa of the intestine focusing on endoscopic findings.
Colonic Neoplasms/diagnosis ; Colonoscopy ; Female ; Gastrointestinal Hemorrhage ; Humans ; Melanoma-Specific Antigens/metabolism ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed

Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery

Actins ; metabolism ; Adenocarcinoma, Clear Cell ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Endometrial Stromal Tumors ; metabolism ; pathology ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism