Perivascular epithelioid cell tumor of the descending colon mimicking a gastrointestinal stromal tumor: a case report

Iwamoto, Ryuta; Kataoka, Tatsuki R.; Furuhata, Ayako; Ôno, Kazuo; Hirota, Seiichi; Kawada, Kenji; Sakai, Yoshiharu; Haga, Hironori

doi:10.1186/s12957-016-1046-7

Cited by 10 publications

(7 citation statements)

References 12 publications

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“…PEComa is rare mesenchymal tumor that has been reported in several organs such as the kidney, lung, and uterus [5] . PEComa rarely occurs in the gastrointestinal tract and commonly present with non-specific clinical signs [2] , [6] . Depending on the organ and the size of the tumor, clinical presentations include abdominal pain, melena, rectal bleeding, obstruction and sometimes a patient can also be asymptomatic [2] .…”

Section: Discussionmentioning

confidence: 99%

PEComa in the rectum: A case report and review of the literature on epithelioid angiomyolipoma

Yeon

Sung

Roh

et al. 2021

International Journal of Surgery Case Reports

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Introduction and importance Perivascular epithelioid cell tumor (PEComa), especially angiomyolipoma (AML) is a rare mesenchymal tumor in wide array of anatomic locations such as the kidney, lung, uterus, and gastrointestinal tract. AML is commonly found in the kidneys and classified as a classic AML or epithelioid AML. We report a case of epithelioid AML diagnosed in the rectum, treated by robot assisted low anterior resection. Presentation of case A 45-year-old woman was referred to our hospital because when an intramural rectal mass was detected on a colonoscopic examination performed during a regular health checkup. Colonoscopic examination revealed an intramural mass, 2 cm in diameter, bulging in the rectal wall, 6 cm from the anal verge. Based on abdominal and pelvic computed tomography images and magnetic resonance imaging findings, the patient was suspected of having gastrointestinal stromal tumor of the rectum. The patient was treated by robot assisted low anterior resection under the diagnosis of GIST. The patient improved without any postoperative complication and was diagnosed as epithelioid AML, a type of PEComa. Discussion AML diagnosed in gastrointestinal tract is very rare and among them, epithelioid AML has possibility of malignancy. However, confirmed diagnosis before surgical resection is difficult because PEComa shows nonspecific imaging characteristics. Treatment of choice of epithelioid angiomyolipoma is surgical resection. Conclusion Because epithelioid AML has the potential for malignancy, clinicians must be aware of the knowledge of the characteristics and natural history of epithelioid AML.

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Section: Discussionmentioning

confidence: 99%

PEComa in the rectum: A case report and review of the literature on epithelioid angiomyolipoma

Yeon

Sung

Roh

et al. 2021

International Journal of Surgery Case Reports

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“…[ 1 2 ] Gastrointestinal tract PEComas are uncommon with PEComas of the small bowel considered exceedingly rare. [ 3 4 5 ] Malignant PEComas must have two of the following criteria: size >5 cm, mitosis >1/50 high-power fields, high nuclear grade and cellularity, and necrosis or vascular invasion. [ 2 ] On imaging, malignant PEComas may mimic other mesenchymal tumors such as GIST and desmoid tumors.…”

Section: Discussionmentioning

confidence: 99%

“…[ 2 ] On imaging, malignant PEComas may mimic other mesenchymal tumors such as GIST and desmoid tumors. [ 4 ] On CT, malignant PEComas are usually well-circumscribed, hypo or iso-attenuating to muscle with necrotic components. [ 6 ] Whereas benign PEComas usually demonstrate minimal or no FDG uptake, malignant PEComas[ 7 8 ] are often intensely FDG avid, possibly related to upregulation of mammalian target of rapamycin pathway which controls glucose transporter 1 function.…”

Section: Discussionmentioning

confidence: 99%

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Malignant perivascular epithelioid cell tumor of the ileum on 18F-fluorodeoxyglucose positron emission tomography/computed tomography with pathological correlation

et al. 2021

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A 75-year-old woman presented with a 1-month history of abdominal pain. Contrast-enhanced computed tomography (CT) demonstrated a large solid mass in the left lower abdominal quadrant, suspicious for malignancy. Staging with 18 F-fluorodeoxyglucose (FDG) positron emission tomography/CT imaging demonstrated intense FDG uptake in the mass with no evidence of metastatic disease. Complete surgical resection was performed, and histopathological analysis confirmed a malignant perivascular epithelioid cell tumor of the ileum.

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“…We did a literature search using PubMed to investigate the clinicopathological features of colonic PEComa and found 21 primary cases, including that of our patient [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] . These are summarized in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Laparoscopically resected perivascular epithelioid cell tumor of the ascending colon demonstrating submucosal tumor morphology: a case report

Nagai

Kiyomatsu

Kume

et al. 2020

Preprint

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Background: The differential diagnosis for a colonic submucosal tumor (SMT) can include many types of cancer. We report a case of a perivascular epithelioid cell tumor (PEComa), a rare mesenchymal neoplasm that demonstrates SMT morphology, in the ascending colon.Case presentation: A mass was incidentally detected by computed tomography (CT) in a 53-year-old man. Colonoscopy revealed an SMT with central ulceration in the ascending colon, and 18F-fluoro-2-deoxy-D-glucose positron emission tomography CT demonstrated a high maximum standardized uptake value of 7.33. Preoperative diagnosis was inconclusive. Given the malignant potential of the tumor, we performed a laparoscopic right hemicolectomy with D3 lymph node dissection. The tumor was 5.5 cm × 4.1 cm and located in the submucosa and muscular propria. There was no lymph node metastasis (0/46). Based on the positive immunohistochemical stainings of both melanocytic (HMB45) and muscle (desmin) markers, we diagnosed the tumor as a PEComa. In the 17 months since surgical resection, the patient has not experienced recurrence, but careful observation should continue because of the malignant potential of the tumor.Conclusions: Although rare, a PEComa should be considered in the differential diagnosis of colonic SMT. Immunohistochemical staining of melanocytic and muscle markers is useful for the diagnosis.

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Perivascular epithelioid cell tumor of the descending colon mimicking a gastrointestinal stromal tumor: a case report

Cited by 10 publications

References 12 publications

PEComa in the rectum: A case report and review of the literature on epithelioid angiomyolipoma

PEComa in the rectum: A case report and review of the literature on epithelioid angiomyolipoma

Malignant perivascular epithelioid cell tumor of the ileum on 18F-fluorodeoxyglucose positron emission tomography/computed tomography with pathological correlation

Laparoscopically resected perivascular epithelioid cell tumor of the ascending colon demonstrating submucosal tumor morphology: a case report

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