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.

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.

Perivascular Epithelioid Cell Tumor of the Oral Mucosa

2005 ◽  
Vol 129 (5) ◽  
pp. 690-693 ◽  
Author(s):  
Ioannis G. Koutlas ◽  
Stefan E. Pambuccian ◽  
Jose Jessurun ◽  
J. Carlos Manivel ◽  
Rajaram Gopalakrishnan
Keyword(s):  
Soft Tissues ◽  
Smooth Muscle Actin ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Muscle Actin ◽  
Epithelioid Cells ◽  
Ultrastructural Examination ◽  
Thin Filaments ◽  
Perivascular Epithelioid Cell ◽  
Granular Cytoplasm

Abstract Perivascular epithelioid cell tumors (PEComas) are a family of tumors defined by the coexpression of melanocytic and muscle markers. Examples have been reported in many organs, soft tissues, and bone. Further expanding the list of locations, we report a case arising in the hard palate. Histologically, the tumor was composed of large elongated or epithelioid cells with granular cytoplasm. Immunohistochemically, tumor cells were positive for HMB-45, Melan A/MART-1, CD10, smooth muscle actin, desmin, and calponin. Ultrastructural examination revealed stage I melanosomes, thin filaments, and dense plaques. Recurrence has not been reported after 20 months. To our knowledge, this is the first detailed description of an intraoral PEComa.

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.

ABI-009 (nab-sirolimus) in advanced malignant perivascular epithelioid cell tumors (PEComa): Preliminary efficacy, safety, and mutational status from AMPECT, an open label phase II registration trial.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 11005-11005 ◽  
Author(s):  
Andrew J. Wagner ◽  
Vinod Ravi ◽  
Kristen N. Ganjoo ◽  
Brian Andrew Van Tine ◽  
Richard F. Riedel ◽  
...  
Keyword(s):  
Case Reports ◽  
Mtor Inhibitors ◽  
Epithelioid Cell ◽  
Fisher Exact Test ◽  
Primary Analysis ◽  
Open Label ◽  
Exact Test ◽  
Perivascular Epithelioid Cell ◽  
Mutational Status ◽  
Malignant Pecoma

11005 Background: Malignant PEComa is a rare, aggressive sarcoma, with no approved treatment or prior clinical trials. Case reports suggest mTOR activation through mutations or deletions of TSC1 or TSC2 and activity of mTOR inhibitors in this disease. ABI-009 is an albumin-bound mTOR inhibitor with increased tumor uptake. The AMPECT trial is the first prospective study in malignant PEComa. Methods: Eligible patients (pts) with centrally confirmed PEComa receive ABI-009 (100 mg/m2 IV, wkly, 2/3 wks) until progression or unacceptable toxicity. Primary endpoint: ORR by independent review (IR), assessed every 6 wks (RECIST v1.1). Secondary endpoints: duration of response (DOR), PFS6, PFS, and safety. Exploratory endpoints (EE): investigator-assessed (IA) outcomes and mutational status. Sample size: 30 efficacy-evaluable pts based on target ORR of 30% (primary analysis planned when all pts treated ≥6 mo). Results: EE and safety are reported (IR pending). As of Feb 12, 2019, enrollment is complete; 34 pts treated, 31 evaluable for efficacy, 42% (13/31) pts ongoing Rx. IA ORR is 42% PR (13/31, 95% CI: 24.5, 60.9), 35% SD (11/31), and 23% PD (7/31); 69% of PRs were reached at 1st restaging (wk 6); 69% PRs are ongoing, with 5 pts >1yr and 2 pt >2 yrs on Rx (all ongoing). Other IA outcomes: median DOR is not reached; PFS6 is 66%; median PFS is 8.9 mo (95% CI: 5.5, -). The most common (>30%) nonhematologic treatment-related AEs (TRAEs) of any grade: mucositis (65%), fatigue (53%), nausea/weight loss (35% each), diarrhea (32%); the most common (>15%) hematologic TRAEs: anemia (44%) and thrombocytopenia (18%). Pneumonitis (15%) was G1/G2. The most common (>10%) G3 TRAEs: mucositis (18%), anemia (12%); No grade ≥4 TRAEs. TSC1 or TSC2 mutations occurred in 5 and 9 (no overlap) of 25 pts with known mutational status, respectively. PR was seen in 100% (9/9) pts with TSC2 mutation, 20% (1/5) pts with TSC1 mutation, and 9% (1/11) pts without mutation in TSC1 or TSC2, P < 0.0001 (2x3 Fisher exact test). PR was significantly higher in pts with TSC2 mutations vs pts without mutation in TSC1 or TSC2, P = 0.0001 (Fisher exact test). Disease control (PR+SD) was seen in 93% (13/14) pts with TSC1 or TSC2 mutations vs 55% (6/11) pts without mutation in TSC1 or TSC2, P = NS. Conclusions: Preliminary IA outcomes showed that ABI-009 treatment of PEComa resulted in substantial and durable responses with manageable toxicities. TSC2 mutations were associated with IA response. Clinical trial information: NCT02494570.

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.

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.

scholarly journals Malignant Subcutaneous Perivascular Epithelioid Cell Tumor of Anterior Abdominal Wall

2021 ◽  
Vol 36 (2) ◽  
pp. e239-e239
Author(s):  
David Eng Yeow Gan ◽  
Rebecca Xin Yi Choy ◽  
Harivinthan Sellappan ◽  
Firdaus Hayati ◽  
Nornazirah Azizan
Keyword(s):  
Anterior Abdominal Wall ◽  
Rectus Sheath ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Resection Margins ◽  
Epithelioid Cells ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell ◽  
Spindle Cells ◽  
Subcutaneous Lesion

Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal tumors with discrete histological and immunohistochemical characteristics. Even rarer among them are cutaneous and subcutaneous PEComas. We describe a 34-year-old woman who presented with a large anterior abdominal subcutaneous lesion showing intact overlying skin and no obvious invasion of the abdominal musculature. A wide local excision was performed. Histopathology revealed a solitary tumor measuring 75 × 55 × 90 mm with epithelioid cells in nests with thin fibrovascular septa and spindle cells. Resection margins were clear with no invasion to the skin or rectus sheath. Tumor cells were positive for HMB-45 but negative for other markers. This is the largest subcutaneous PEComa reported to date.

Pigmented Perivascular Epithelioid Cell Tumor of the Kidney

2009 ◽  
Vol 133 (12) ◽  
pp. 1981-1984 ◽  
Author(s):  
Masaharu Fukunaga ◽  
Tohru Harada
Keyword(s):  
Tumor Cells ◽  
Spindle Cell ◽  
S100 Protein ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Cell Renal Cell Carcinoma ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Spindle Cell Proliferation ◽  
Clear Cytoplasm

Abstract A case of pigmented perivascular epithelioid cell tumor of the kidney in a 57-year-old woman with a clinically indicated tuberous sclerosis is presented. The lesion was a 3.0-cm, well-demarcated, black-colored mass. The tumor was histologically characterized by an epithelioid arrangement of round to polygonal cells with round nuclei and clear cytoplasm containing abundant melanin. Tumor cells showed mild atypia and low mitotic activity. A spindle cell proliferation was focally observed. There were no adipose elements or thick-walled vascular vessels. The stroma demonstrated intervening, thin, fibrovascular septa. Immunohistochemically, the tumor cells were strongly positive for HMB-45 but negative for epithelial and muscle markers, vimentin, and S100 protein. The patient had no evidence of disease 3 months after surgery. Pathologists and clinicians should know about the existence of pigmented perivascular epithelioid cell tumor of the kidney. This type of tumor should be differentiated from clear cell renal cell carcinoma or malignant melanoma.

scholarly journals Hepatic perivascular epithelioid cell tumor: Five case reports and literature review

2015 ◽  
Vol 38 (1) ◽  
pp. 58-63 ◽  
Author(s):  
Zhen Liu ◽  
Yafei Qi ◽  
Chuanzhuo Wang ◽  
Xiaobo Zhang ◽  
Baosheng Wang
Keyword(s):  
Literature Review ◽  
Case Reports ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell

Malignant Perivascular Epithelioid Cell Tumor Mimicking Renal Cell Carcinoma: A Diagnostic Pitfall

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S62-S62
Author(s):  
Natalya Hakim ◽  
Yevgen Chornenkyy ◽  
Shadi Qasem
Keyword(s):  
Clear Cell ◽  
Metastatic Potential ◽  
Epithelioid Cell ◽  
Initial Diagnosis ◽  
World Health ◽  
Epithelioid Cells ◽  
Mesenchymal Tumors ◽  
Fuhrman Grade ◽  
Perivascular Epithelioid Cell ◽  
Eosinophilic Cytoplasm

Abstract Introduction Perivascular epithelioid cell tumors (PEComas) are defined by the World Health Organization (WHO) as mesenchymal tumors arising from perivascular epithelioid cells with myomelanocytic immunophenotype. In the kidney, they are often referred to as epithelioid angimyolipoma (AML). Most are benign but some can be malignant. Their rarity and wide differential render diagnosis challenging. Case Presentation A 56-year-old male presented with a 3-month history of flank pain. Computed tomography (CT) identified bilateral necrotic heterogeneous enhancing kidney masses, with right significantly larger than left. Initially, the patient underwent a right radical nephrectomy. On gross examination, no tumor infiltration was observed. Histology demonstrated sheets of highly atypical cells with clear cytoplasm and easily identifiable nucleoli. Areas of spindle and rhabdoid differentiation were present. The initial diagnosis was renal clear cell (RCC) carcinoma, Fuhrman grade 4, with extensive rhabdoid and focal sarcomatoid features. A follow-up CT demonstrated a hypodense area within the right hepatic lobe. Status post liver resection and histomorphological examination, the lesion was identical to the renal mass except for focal areas of prominent epithelioid cells with eosinophilic cytoplasm. Immunohistochemical (IHC) stains of both masses were positive for Melan-A, HMB-45, and EMA but negative for PAX8, SOX10, S100, SMA, desmin, and cytokeratin. The initial diagnosis was amended to malignant AML with hepatic metastasis. Discussion and Conclusion Renal PEComa (epithelioid AML) is a rare tumor with a prevalence of 0.44%. While most are benign, some are malignant with metastatic potential. The differential diagnosis for PEComa is broad and includes more common tumors like RCC, melanoma, clear cell sarcoma, and liposarcoma. The presence of spindle and epithelioid histology with eosinophilic cytoplasm in RCC should prompt additional IHC workup (HMB45, Melan-A, SMA, and EMA) in order to exclude PEComa/AML.

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