A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa
Abstract Background PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same...
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| Format: | Article |
| Language: | English |
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BMC
2024-04-01
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| Series: | Diagnostic Pathology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13000-024-01485-2 |
| _version_ | 1797199660899631104 |
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| author | Hikaru Tsukita Kei Koyama Takahiro Ishinari Ayana Takahashi Ken Miyabe Michinobu Umakoshi Makoto Yoshida Yukitsugu Kudo-Asabe Akiko Nishida Naohiko Otsuka Ouki Yasui Ikuma Kato Noriyoshi Fukushima Akiteru Goto |
| author_facet | Hikaru Tsukita Kei Koyama Takahiro Ishinari Ayana Takahashi Ken Miyabe Michinobu Umakoshi Makoto Yoshida Yukitsugu Kudo-Asabe Akiko Nishida Naohiko Otsuka Ouki Yasui Ikuma Kato Noriyoshi Fukushima Akiteru Goto |
| author_sort | Hikaru Tsukita |
| collection | DOAJ |
| description | Abstract Background PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration. Case presentation A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa. Conclusions A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized. |
| first_indexed | 2024-04-24T07:19:18Z |
| format | Article |
| id | doaj.art-29ee95cc55e74563b4249ca1244e1726 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-04-24T07:19:18Z |
| publishDate | 2024-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-29ee95cc55e74563b4249ca1244e17262024-04-21T11:07:12ZengBMCDiagnostic Pathology1746-15962024-04-011911610.1186/s13000-024-01485-2A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComaHikaru Tsukita0Kei Koyama1Takahiro Ishinari2Ayana Takahashi3Ken Miyabe4Michinobu Umakoshi5Makoto Yoshida6Yukitsugu Kudo-Asabe7Akiko Nishida8Naohiko Otsuka9Ouki Yasui10Ikuma Kato11Noriyoshi Fukushima12Akiteru Goto13Department of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineDepartment of Pathology, Nihonkai General HospitalDepartment of Surgery, Noshiro Yamamoto Medical Association HospitalDepartment of Surgery, Noshiro Yamamoto Medical Association HospitalDepartment of Molecular Pathology, Yokohama City University Graduate School of MedicineDepartment of Pathology, Jichi Medical UniversityDepartment of Cellular and Organ Pathology, Akita University Graduate School of MedicineAbstract Background PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration. Case presentation A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa. Conclusions A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.https://doi.org/10.1186/s13000-024-01485-2PEComaInflammatory subtypePancreatic tumorαSMAMelan AHMB45 |
| spellingShingle | Hikaru Tsukita Kei Koyama Takahiro Ishinari Ayana Takahashi Ken Miyabe Michinobu Umakoshi Makoto Yoshida Yukitsugu Kudo-Asabe Akiko Nishida Naohiko Otsuka Ouki Yasui Ikuma Kato Noriyoshi Fukushima Akiteru Goto A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa Diagnostic Pathology PEComa Inflammatory subtype Pancreatic tumor αSMA Melan A HMB45 |
| title | A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa |
| title_full | A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa |
| title_fullStr | A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa |
| title_full_unstemmed | A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa |
| title_short | A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa |
| title_sort | case of pancreatic pecoma with prominent inflammatory cell infiltration the inflammatory subtype is a distinct histologic group of pecoma |
| topic | PEComa Inflammatory subtype Pancreatic tumor αSMA Melan A HMB45 |
| url | https://doi.org/10.1186/s13000-024-01485-2 |
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