Hepatic and perihepatic PEComas: A study describing a series of five rare cases
Background: Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, with dual melanocytic and muscular differentiation. Hepatic PEComas are rare and difficult to diagnose, and their behavior is still unclear. Materials and Methods: Herein, we report a total of...
Saved in:
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2024-04-01
|
| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | https://journals.lww.com/10.4103/ijpm.ijpm_587_23 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1825205436614180864 |
|---|---|
| author | P Vijayanirmala Rajni Yadav Surbhi Goyal Adarsh Barwad Subham Bhowmik Rohan Malik Sujoy Pal Raju Sharma Puja Sakhuja Prasenjit Das |
| author_facet | P Vijayanirmala Rajni Yadav Surbhi Goyal Adarsh Barwad Subham Bhowmik Rohan Malik Sujoy Pal Raju Sharma Puja Sakhuja Prasenjit Das |
| author_sort | P Vijayanirmala |
| collection | DOAJ |
| description | Background:
Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, with dual melanocytic and muscular differentiation. Hepatic PEComas are rare and difficult to diagnose, and their behavior is still unclear.
Materials and Methods:
Herein, we report a total of five cases of hepatic and perihepatic PEComas over a period of the last 5 years from our and collaborating center’s archive. A detailed histological evaluation was done. A comprehensive panel of immunohistochemical stains was used and fluorescence in-situ hybridization analysis was performed for the TFE3 gene using break-apart probes.
Result:
All these patients were women, with an average age of presentation of 44 years. The lesions were in the right hepatic lobe: three cases, the left hepatic lobe: one case, and gastrohepatic ligament: one case. The preoperative clinicoradiological diagnoses were hepatocellular carcinoma (HCC), focal nodular hyperplasia, hemangioma, metastasis, and gastrointestinal stromal tumor, respectively. Surgical excision was performed in four cases with no further adjuvant therapy. Histopathological examination and subsequent immunophenotyping revealed a diagnosis of PEComa. Fluorescence in-situ hybridization analysis was performed for TFE3 gene rearrangement in four cases.
Conclusions:
This series highlights the fact that accurate histological diagnosis of hepatic or perihepatic PEComas is important to prevent unnecessary aggressive treatment, unlike primary hepatocellular carcinomas or hepatoid/epithelioid metastatic tumors. |
| format | Article |
| id | doaj-art-7c506af85a2c40278281b5a55eb4f94f |
| institution | Kabale University |
| issn | 0377-4929 0974-5130 |
| language | English |
| publishDate | 2024-04-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj-art-7c506af85a2c40278281b5a55eb4f94f2025-02-07T13:57:45ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49290974-51302024-04-0167235536110.4103/ijpm.ijpm_587_23Hepatic and perihepatic PEComas: A study describing a series of five rare casesP VijayanirmalaRajni YadavSurbhi GoyalAdarsh BarwadSubham BhowmikRohan MalikSujoy PalRaju SharmaPuja SakhujaPrasenjit DasBackground: Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, with dual melanocytic and muscular differentiation. Hepatic PEComas are rare and difficult to diagnose, and their behavior is still unclear. Materials and Methods: Herein, we report a total of five cases of hepatic and perihepatic PEComas over a period of the last 5 years from our and collaborating center’s archive. A detailed histological evaluation was done. A comprehensive panel of immunohistochemical stains was used and fluorescence in-situ hybridization analysis was performed for the TFE3 gene using break-apart probes. Result: All these patients were women, with an average age of presentation of 44 years. The lesions were in the right hepatic lobe: three cases, the left hepatic lobe: one case, and gastrohepatic ligament: one case. The preoperative clinicoradiological diagnoses were hepatocellular carcinoma (HCC), focal nodular hyperplasia, hemangioma, metastasis, and gastrointestinal stromal tumor, respectively. Surgical excision was performed in four cases with no further adjuvant therapy. Histopathological examination and subsequent immunophenotyping revealed a diagnosis of PEComa. Fluorescence in-situ hybridization analysis was performed for TFE3 gene rearrangement in four cases. Conclusions: This series highlights the fact that accurate histological diagnosis of hepatic or perihepatic PEComas is important to prevent unnecessary aggressive treatment, unlike primary hepatocellular carcinomas or hepatoid/epithelioid metastatic tumors.https://journals.lww.com/10.4103/ijpm.ijpm_587_23hepatic tumorshepatocellular carcinomaimmunohistochemistrypecomastfe3 |
| spellingShingle | P Vijayanirmala Rajni Yadav Surbhi Goyal Adarsh Barwad Subham Bhowmik Rohan Malik Sujoy Pal Raju Sharma Puja Sakhuja Prasenjit Das Hepatic and perihepatic PEComas: A study describing a series of five rare cases Indian Journal of Pathology and Microbiology hepatic tumors hepatocellular carcinoma immunohistochemistry pecomas tfe3 |
| title | Hepatic and perihepatic PEComas: A study describing a series of five rare cases |
| title_full | Hepatic and perihepatic PEComas: A study describing a series of five rare cases |
| title_fullStr | Hepatic and perihepatic PEComas: A study describing a series of five rare cases |
| title_full_unstemmed | Hepatic and perihepatic PEComas: A study describing a series of five rare cases |
| title_short | Hepatic and perihepatic PEComas: A study describing a series of five rare cases |
| title_sort | hepatic and perihepatic pecomas a study describing a series of five rare cases |
| topic | hepatic tumors hepatocellular carcinoma immunohistochemistry pecomas tfe3 |
| url | https://journals.lww.com/10.4103/ijpm.ijpm_587_23 |
| work_keys_str_mv | AT pvijayanirmala hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT rajniyadav hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT surbhigoyal hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT adarshbarwad hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT subhambhowmik hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT rohanmalik hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT sujoypal hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT rajusharma hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT pujasakhuja hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases AT prasenjitdas hepaticandperihepaticpecomasastudydescribingaseriesoffiverarecases |