scholarly journals Hepatic Perivascular Epithelioid Cell Neoplasms: An Analysis of 16 Cases

2021 ◽  
Author(s):  
Yubin Fu ◽  
Jiahao Xue ◽  
Penglei Ge ◽  
Rufei Guo ◽  
Lin Li ◽  
...  
Keyword(s):  
Radiofrequency Ablation ◽  
Partial Hepatectomy ◽  
Liver Tumors ◽  
Clinical Manifestations ◽  
Epithelioid Cell ◽  
Diagnosis And Treatment ◽  
Imaging Features ◽  
Perivascular Epithelioid Cell ◽  
S 100 ◽  
Hmb 45

Abstract Background: Hepatic perivascular epithelioid cell neoplasm (Hepatic PEComa) is a rare type of mesenchymal tumor and its diagnosis and treatment are controversial. To investigate its clinical features and to guide the diagnosis and treatment of this tumor.Methods: Retrospectively analysing the clinical data of 16 patients with pathologically confirmed hepatic PEComa, who were admitted to the First Affiliated Hospital of Zhengzhou University from April 2014 to April 2020, and summarizing its clinical symptoms, Imaging features, histological and immunohistochemical characteristics, therapy and prognosis. Results: All 16 patients were female, aged from 23 to 65 years (mean, 44±12 years). Clinical manifestations, preoperative routine laboratory tests and imaging examinations were lack of specificity. Immunohistochemical results showed that the positive rates of HMB-45, Melan-A and SMA were 100% (16/16), 100% (16/16) and 81.3% (13/16) respectively. The negative rates of Hepatocytes and S-100 were 100% (16/16) and 75% (12/16), respectively. With regard to treatment, eleven patients had undergone “laparotomy for partial hepatectomy” or “laparoscopic partial hepatectomy”. Three patients had undergone “radiofrequency ablation of liver tumors”. One patient had undergone chemotherapy with the " gemcitabine + nedaplatin + apatinib " regimen. Twelve of sixteen patients were followed up for 6- 55 months (mean, 26±18 months), one case of an intrahepatic recurrent lesion was found 14 months after radiofrequency ablation of liver tumors, and the remaining 11 cases showed no enlargement of the primary lesion, and no new lesions, metastasis or death. Conclusions: Hepatic PEComa lacks specific clinical manifestations and imaging features. The diagnosis depends on histological and immunohistochemical characteristics. The positive of HMB-45, Melan-A, and SMA and the negative of S-100 are helpful in the diagnosis of hepatic PEComa. Most hepatic PEComas are benign lesions, and radical resection is the best treatment with a favorable prognosis.

Atypical Angiomyolipoma of Kidney in a Patient With Tuberous Sclerosis: A Case Report With p53 Gene Mutation Analysis

2005 ◽  
Vol 129 (5) ◽  
pp. 676-679 ◽  
Author(s):  
Linglei Ma ◽  
Diane Kowalski ◽  
Khurram Javed ◽  
Pei Hui
Keyword(s):  
Tuberous Sclerosis ◽  
P53 Gene ◽  
Epithelioid Cell ◽  
P53 Mutation ◽  
P53 Gene Mutation ◽  
Perivascular Epithelioid Cell ◽  
The Family ◽  
P53 Immunoreactivity ◽  
Gene Mutation Analysis ◽  
Hmb 45

Abstract Angiomyolipoma (AML) is the most common benign mesenchymal tumor of the kidney. It belongs to the family of perivascular epithelioid cell tumors and is typically composed of blood vessels, adipose tissue, and smooth muscle– like cells, which are characteristically positive for HMB-45. Results of recent studies suggest that p53 mutation may play an important role in AML progression. Here, we describe a locally destructive renal AML in a patient with tuberous sclerosis. The tumor consisted of mostly epithelioid cells with marked nuclear pleomorphism and frequent mitoses and was positive for HMB-45. The diagnosis of atypical epithelioid AML was made. Codon alteration in the p53 gene was not detected, despite focal p53 immunoreactivity and single nucleotide polymorphism at exon 6. Our finding indicates no definite link between p53 abnormalities and the atypical appearance of AML. To the best of our knowledge, this is the second renal AML case investigated for p53 mutations.

Malignant Perivascular Epithelioid Cell Tumor Involving the Prostate

2003 ◽  
Vol 127 (2) ◽  
pp. e96-e98 ◽  
Author(s):  
Chin-Chen Pan ◽  
An-Hang Yang ◽  
Hung Chiang
Keyword(s):  
Seminal Vesicle ◽  
Tumor Cells ◽  
S100 Protein ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Epithelioid Cells ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Granular Cytoplasm ◽  
Hmb 45

Abstract Perivascular epithelioid cell tumor (PEComa) is a neoplasm chiefly composed of HMB-45–positive epithelioid cells with clear-to-granular cytoplasm and a perivascular distribution. We describe such a tumor involving the prostate and seminal vesicle in a 46-year-old man. The tumor had characteristic histologic features of PEComa. Immunohistochemically, the tumor cells were positive for HMB-45 but negative for epithelial markers, Melan-A, and S100 protein. The tumor behaved in a malignant fashion, and the patient died of the disease 4 years after diagnosis.

scholarly journals Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 553
Author(s):  
Luca Giannella ◽  
Giovanni Delli Carpini ◽  
Nina Montik ◽  
Valeria Verdecchia ◽  
Francesca Puccio ◽  
...  
Keyword(s):  
Color Doppler ◽  
Laparoscopic Hysterectomy ◽  
Smooth Muscle Tumor ◽  
Epithelioid Cell ◽  
Uterine Wall ◽  
Imaging Features ◽  
Rare Occurrence ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell ◽  
Ultrasound Evaluation

Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presence of nonspecific imaging features. We report a case of a uterine PEComa with particular reference to ultrasound’s role in characterizing this rare occurrence. Case presentation: a 45-year-old White woman came to our observation for cyclic abdominopelvic pain and chronic constipation. The pre-surgical ultrasound examination showed a heterogeneous tumor that was 4 cm in size, localized on the right anterolateral uterine wall. The mass had well-delimited borders and a central hypoechoic portion. The use of color Doppler showed a rich, irregular vasculature in the center with low impedance. The preoperative diagnostic hypothesis was of a smooth muscle tumor of uncertain malignant potential. After careful counseling, a surgical approach was decided upon, including a total laparoscopic hysterectomy with bilateral salpingectomy. The histological and phenotypical features were consistent with a uterine PEComa. At the last follow-up, two years after surgery, the patient is alive and well. Conclusions: Uterine PEComa is a rare occurrence that should be included in the differential diagnosis of uterine wall tumors. It can appear as a small uterine mass with heterogeneous echogenicity and a rich vascular pattern during an ultrasound evaluation. This diagnostic suspicion may assist in better surgical planning.

scholarly journals Hepatic perivascular epithelioid cell neoplasm: A clinical and pathological experience in diagnosis and treatment

2017 ◽  
Vol 6 (4) ◽  
pp. 487-493 ◽  
Author(s):  
Wenying Chen ◽  
Yeqing Liu ◽  
Yanyan Zhuang ◽  
Juanfei Peng ◽  
Fengting Huang ◽  
...  
Keyword(s):  
Epithelioid Cell ◽  
Diagnosis And Treatment ◽  
Perivascular Epithelioid Cell ◽  
Cell Neoplasm

Angioleiomyoma of Uterus: A Clinicopathologic Study of 6 Cases

2017 ◽  
Vol 26 (1) ◽  
pp. 18-23 ◽  
Author(s):  
Mayank Gupta ◽  
Mayur Suryawanshi ◽  
Ramani Kumar ◽  
Abraham Peedicayil
Keyword(s):  
Subcutaneous Tissue ◽  
Female Genital Tract ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Stromal Tumor ◽  
World Health ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Hmb 45

Background and Objectives. Angioleiomyoma is a benign perivascular neoplasm commonly involving subcutaneous tissue of extremities, head, and trunk region. They rarely involve the female genital tract. This study analyses clinicopathological features of 6 cases of uterine angioleiomyoma. Methods. Routine sections of 6 cases were reviewed and immunohistochemical markers namely muscle-specific actin, h-caldesmon, desmin, CD10, WT-1, HMB-45, and melan-A were done. Results. Of the 6 cases, 4 cases had tumor involving the corpus and 2 cases had tumor in the cervix. Grossly, all tumors had a whorled and congested cut surface. Microscopic examination of all the cases revealed circumscribed neoplasms composed of interlacing fascicles of benign perivascular smooth muscle cells with evenly distributed slit-like blood vessels (solid variant) along with vessels exhibiting thick muscular walls with swirling pattern (venous variant). In only 2 cases many dilated vessels were seen (cavernous variant). Immunohistochemically, all cases were positive for muscle-specific actin, h-caldesmon, and desmin. All cases were negative for CD10 and WT-1 ruling out endometrial stromal tumor and were negative for HMB-45 and melan-A ruling out perivascular epithelioid cell tumor (both endometrial stromal tumor and perivascular epithelioid cell tumor have prominent vessels but have different histomorphology). In all cases, surgical excision was curative and there were no intraoperative or postoperative complications. Follow-up of all the cases has been unremarkable. Conclusion. As the World Health Organization has not included angioleiomyoma in the classification of mesenchymal tumors of uterine corpus and cervix, we recommend that it should be included in the classification.

scholarly journals New recognition of hepatic perivascular epithelioid cell tumours (PEComa): a retrospective analysis of 35 cases

2020 ◽  
Author(s):  
Xiao Yang ◽  
Qing Yuan Wang ◽  
Xiao Zhou ◽  
Haoming Zhou ◽  
Wen Bo Jia ◽  
...  
Keyword(s):  
Smooth Muscle Actin ◽  
Clinical Manifestations ◽  
Epithelioid Cell ◽  
Tumour Recurrence ◽  
Preoperative Imaging ◽  
Perivascular Epithelioid Cell ◽  
Imaging Examination ◽  
Right Lobe ◽  
The Right

Abstract Purpose Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumours (PEComa).Patients and Methods Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of Hepatobiliary Center of the First Affiliated Hospital of Nanjing Medical University from January 2008 to February 2019 were retrospectively analysed, and the literature was reviewed.Results Twenty-nine females and 6 males were included in this study. The mean age of these patients was 48.0 years (range, 21-75 years). Thirteen patients complained of upper abdominal pain and discomfort, but the tumours in the other patients were identified by performing an imaging examination. Hepatic PEComas tended to occur in the right lobe of the liver (20 cases in the right lobe, 13 in the left lobe and 2 in the caudate lobe). Two cases were characterized by multiple tumours, and the remaining cases were single lesions (range, 1.2-12 cm). Only 8 cases were correctly diagnosed by the preoperative imaging examination, and the correct diagnosis rate was only 22.9%. The postoperative immunohistochemistry analysis showed that hepatic PEComas are positive for human melanoma black 45 (HMB-45), Melan-A and smooth muscle actin (SMA), with the exception of 1 case that was negative for Melan-A. All patients undergoing an operation accepted regular follow-up, and the average time was 66.5 months (range, 3-132 months). Two patients who experienced tumour recurrence and 1 patient who died due to cardiovascular disease, but the remaining patients showed no evidence of tumour recurrence or metastasis during the follow-up period.Conclusions Hepatic PEComa is a rare type of tumour that mainly occurs in young and middle-aged women. The lack of clinical manifestations and imaging findings increases the difficulty of determining a preoperative diagnosis, which mainly depends on the pathological examinations. Surgery is currently the only effective treatment, and long-term clinical follow-up is necessary due to the aggressive behaviour and relapse of hepatic PEComa in some cases.

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