A case report of hepatic perivascular epithelioid cell tumours (pecomas) and literature review

MedPharmRes ◽  
2018 ◽  
Vol 2 (2) ◽  
pp. 39-42
Author(s):  
Duy Nguyen ◽  
Truong Nguyen ◽  
Thu Phan ◽  
Dat Ngo
Keyword(s):  
Normal Liver ◽  
Extramedullary Hematopoiesis ◽  
Epithelioid Cell ◽  
Thick Wall ◽  
Benign Tumors ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
Enhanced Ct ◽  
Hypervascular Lesion ◽  
The Family

Perivascular epithelioid cell tumours (PEComas) belongs to the family of mesenchymal neoplasms that can occur in many organs, but rarely found in liver. Preoperative diagnosis could be challenging due to unspecific and variable radiologic patterns, which can be clinically misdiagnosed with hepatocellular carcinoma and other benign tumors. This report aims to announce a case of hepatic PEComa with extramedullary hematopoiesis. A 44- year- old woman accidentally presented a nodular mass in the left hepatic segment on ultrasonography with normal liver function result. Abdominal enhanced CT Scan showed hypervascular lesion. Morphology exibits classical characteristics of PEComa with mature fat tissue predominance, thick-wall vessel; somewhat amount of extramedullary hematopoiesis was also recognized. Tumor cells are diffusely immunoreactive with HMB45. PEComa is a rare disease in liver, pathologists need to recognize three components of this tumor to avoid misdiagnosing with malignant conditions.

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.

scholarly journals PEComa: A Perivascular Epithelioid Cell Tumor in the Liver—A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Faseeh Khaja ◽  
Allison Carilli ◽  
Said Baidas ◽  
Aravindhan Sriharan ◽  
Shanedelle Norford
Keyword(s):  
Malignant Tumors ◽  
Mtor Inhibitors ◽  
Epithelioid Cell ◽  
Soft Tissue Tumors ◽  
Cell Tumor ◽  
The Body ◽  
Benign Tumors ◽  
Review Of The Literature ◽  
Perivascular Epithelioid Cell ◽  
Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. Usual treatment is surgery for benign tumors and chemotherapy including mTOR inhibitors for malignant tumors. Here we discuss the radiological and pathological diagnosis, evaluation, and management of a hepatic PEComa. We describe a 51-year-old patient who was diagnosed incidentally after unusual physical exam findings.

scholarly journals An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Zofia Tynski ◽  
Way Chiang ◽  
Albert Barrett
Keyword(s):  
Clear Cell ◽  
Middle Age ◽  
Epithelioid Cell ◽  
Ligamentum Teres ◽  
Cell Tumor ◽  
The Third ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
First Case ◽  
Sugar Tumor

Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.

scholarly journals Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Saime Unluoglu ◽  
Umit Bayol ◽  
Nilay Korkmaz ◽  
Bekir Ozenen ◽  
Fuat Ipekci ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Case Reports ◽  
Smooth Muscle Actin ◽  
Epithelioid Cell ◽  
Epithelioid Cells ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
Granular Cytoplasm ◽  
Diagnostic Difficulties ◽  
Gross Examination

Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed. Gross examination of the specimen revealed a2×1,5×1 cm secondarily ulcerated, solid, nodular, gray white tumor mass in the ileal wall. Histologically, tumor cells were composed of nests of round-polygonal epithelioid cells with abundant clear to slightly eosinophilic granular cytoplasm and round vesicular nuclei. The nests were separated by thin fibrovascular septa. Minimal necrosis and low mitotic activity were noticed in the tumor. Immunohistochemically, tumor cells were positive for SMA, HMB45, and Melan-A and negative for CD10, RCC, CD45, CD117, CD34, EMA, and Desmin. Diagnosis was PEComa of the ileum. We report the case of ileal PEComa to remind the unusual presentation (diverticulitis) of these tumors, besides rarity and diagnostic difficulties.

Gene of the month: TFE 3

2020 ◽  
Vol 73 (11) ◽  
pp. 691-694
Author(s):  
Karen Pinto ◽  
Runjan Chetty
Keyword(s):  
Soft Part ◽  
Granular Cell ◽  
Epithelioid Cell ◽  
Nuclear Staining ◽  
Granular Cell Tumour ◽  
Gene Fusions ◽  
Chondroid Syringoma ◽  
Perivascular Epithelioid Cell ◽  
The Family ◽  
A Cell

Transcription factor enhancer 3 (TFE3), on the short arm of chromosome Xp11.23 and its protein, belongs to the microphthalmia transcription family (MiTF) of transcription factors. It shares close homology with another member of the family, MiTF which is involved in melanocyte development. When a cell is stressed and/or starved, TFE3 protein translocates into the nucleus. TFE3 gene fusions with multiple different partner genes occur in several tumours with resultant nuclear expression of TFE3 protein. The main tumours associated with TFE3 gene fusions are: renal cell carcinoma, alveolar soft part sarcoma, a subset of epithelioid haemangioendotheliomas (EHE), some perivascular epithelioid cell tumours and rare examples of ossifying fibromyxoid tumour and malignant chondroid syringoma. TFE3 immunohistochemistry is of use in routine diagnostic practice with the aforementioned tumours harbouring TFE3 fusions leading to nuclear staining. In addition, there are tumours lacking TFE3 fusions but also display TFE3 nuclear immunolabeling, and these include: granular cell tumour, solid pseudopapillary neoplasm of the pancreas and ovarian sclerosing stromal tumour.

Malignant Perivascular Epithelioid Cell Tumor of the Gallbladder: A Case Report and Review of Literature

2014 ◽  
Vol 138 (9) ◽  
pp. 1238-1241 ◽  
Author(s):  
Liena Zhao ◽  
Karl H. Anders
Keyword(s):  
Clear Cell ◽  
Epithelioid Cell ◽  
Ligamentum Teres ◽  
Cell Tumor ◽  
Falciform Ligament ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
Sugar Tumor ◽  
Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors are rare mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The perivascular epithelioid cell tumor family includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomic sites. Perivascular epithelioid cell tumors have been reported previously in various sites, but to our knowledge not in the gallbladder. We report here, for the first time, a malignant perivascular epithelioid cell tumor arising in the gallbladder.

Perivascular Epithelioid Cell Tumor

2009 ◽  
Vol 133 (4) ◽  
pp. 648-654 ◽  
Author(s):  
Henry B. Armah ◽  
Anil V. Parwani
Keyword(s):  
Epithelioid Cell ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
Not Otherwise Specified ◽  
Granular Eosinophilic Cytoplasm ◽  
Female Predominance ◽  
Eosinophilic Cytoplasm ◽  
Sugar Tumor ◽  
Normal Tissue Counterpart ◽  
Perivascular Epithelioid Cell Tumors

Abstract Perivascular epithelioid cell tumors are mesenchymal neoplasms defined by the presence of histologically and immunohistochemically distinctive perivascular epithelioid cells. The perivascular epithelioid cell has no known normal tissue counterpart and coexpresses myoid and melanocytic markers. This tumor family shows marked female predominance and includes angiomyolipoma, clear cell sugar tumor, lymphangioleiomyomatosis, and a group of rare, morphologically and immunophenotypically similar tumors arising at a variety of visceral and soft tissue sites. This latter subset has been collectively termed perivascular epithelioid cell tumors–not otherwise specified. They are usually composed of epithelioid, but occasionally spindled, cells with clear to granular eosinophilic cytoplasm and focal perivascular accentuation. The mainstay of treatment is wide excision. Although most cases are benign, a subset behaves in a malignant fashion. Since few malignant cases have been reported, firm criteria for malignancy have yet to be established. This review focuses on the perivascular epithelioid cell tumors–not otherwise specified subset.

scholarly journals Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Idania Lubo ◽  
Ileana Fermín ◽  
Olindo Massarelli ◽  
Roberta Gobbi ◽  
Paolo Cossu Rocca
Keyword(s):  
Clinical Outcome ◽  
Clinical Course ◽  
Mitotic Rate ◽  
Epithelioid Cell ◽  
World Health ◽  
Review Of The Literature ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
The World ◽  
Health Organization

The Perivascular Epithelioid Cell tumours (PEComas) are rare mesenchymal neoplasms recognized as entity by the World Health Organization. The tumour cells have an uncertain origin and are characterized by distinctive histological and immunohistochemical features. We report a case of PEComa occurring as intraorbital lesion in a 47-year-old man. We found only two other cases described in the literature and we considered all three cases together in order of histology, immunohistochemistry, and clinical outcome. We found a strict histological overlapping and quite similar immunohistological results. All three cases showed a favourable clinical course probably related to small size of tumours (<5 cm), low mitotic rate (<2 mitoses in 50 HPF), and absence of necrosis.

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