A systematic review

Chen, Zehong; Han, Siqi; Wu, Jialin; Xiong, Minmin; Huang, Yanqiao; Chen, Jianhui; Yuan, Yujie; Peng, Jianjun; Wu, Song

doi:10.1097/md.0000000000003890

Cited by 44 publications

(46 citation statements)

References 51 publications

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“…[ 4 , 8 , 9 ] This rare neoplasm seems to arise most commonly at retroperitoneal, visceral, abdominal, and pelvic sites but has been reported at almost every body site, and the growing list of reported sites include gastrointestinal, gynecologic, and genitourinary sites, the extremities, somatic soft tissue, and the skin; among these sites are the falciform ligament, uterus, uterine cervix, liver, kidney, lung, breast, cardiac septum, pancreas, prostate, thigh, and gastrointestinal tract. [ 1 , 9 – 13 ] These tumors all share a distinctive cell type, the PEC, which has no known normal tissue counterpart. [ 8 ]…”

Section: Discussionmentioning

confidence: 99%

“…In addition, there was a marked female preponderance. [ 9 , 12 , 13 ] In 2016, Chen et al [ 13 ] reported in a review study of gastrointestinal PEComa that the gastrointestinal tract is the second most frequent site of PEComa, with the most frequent site being the gynecological tract. They further indicated that gastrointestinal PEComas of the colon and rectum were most common.…”

Section: Discussionmentioning

confidence: 99%

“…They further indicated that gastrointestinal PEComas of the colon and rectum were most common. [ 13 ] In addition, there were several cases of PEComa in the gastrointestinal tract that were reported in both the pediatric [ 10 , 11 , 14 , 20 – 25 ] and adult populations [ 9 , 15 , 17 , 26 – 30 ] over the last 2 decades. In 2000, Prasad et al [ 25 ] reported the first child with PEComa involving the appendix.…”

Section: Discussionmentioning

confidence: 99%

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Successful management of perivascular epithelioid cell tumor of the rectum with recurrent liver metastases

et al. 2018

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Rationale:The perivascular epithelioid cell tumor (PEComa) is rare in young man and rarely occurs in the large intestine.Patient concerns:The clinical characteristics, diagnosis, and managements in a 28-year-old boy who presented with sudden onset of cramping and abdominal pain and intermittent melena with a blood pressure of 74/39 mm Hg was retrospectively reviewed. CT scan of the abdomen revealed a 8.9 × 7.2 cm mass in the pelvic floor.Diagnoses:Given the difficulty of obtaining a diagnostic specimen, surgical resection was performed. The pathology report of lower anterior resection was malignant PEComa of the rectum in 2006.Interventions:Treatment consisted of surgical resection only without additional adjuvant therapy. Over the next 49 months (until 2010) after surgery, abdominal CT showed a 0.6-cm hypodense mass over the liver with suspected liver metastasis. He refused any further evaluation and treatment. After 4 years (2014), abdominal CT showed that the original mass had increased from 0.6 to 1.5 cm and the number of tumors had increased from 1 to 3. In August 2014, he underwent a metastatic hepatectomy without additional chemotherapy or radiotherapy.Outcomes:We noted that the metastatic progression was slow in the 4 years after the first operation. At 28 months after metastatic hepatectomy, the patient was doing well. There was also no recurrence of the PEComa of the rectum at the 120-month follow-up in 2016.Lessons:To the best of our knowledge, this is the first report of a PEComa of the rectum with liver metastases treated with only surgical resection. At approximately 8.8 cm, this is the largest PEComa of the rectum reported in the recent literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Successful management of perivascular epithelioid cell tumor of the rectum with recurrent liver metastases

et al. 2018

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“…The age of the patients at the time of PEComa diagnosis is most commonly in the range of 38.9-56 years [7,8], but PEComa cases in children have also been described [9,10]. All reviews indicate more common PEComa occurrence in women (54-86.9% of cases) [11,12], also after exclusion of sex-specific locations from the analysis [3].…”

Section: Epidemiologymentioning

confidence: 99%

“…The basic pathological differential diagnosis for PEComa NOS (summarised in Table 1) encompasses: gastrointestinal stromal tumours (GIST), melanoma, renal cell, and adrenocortical carcinoma, especially the chromophobic type, clear cell sarcoma of tendon and aponeurosis -melanoma of the soft parts (CCS), alveolar soft part sarcoma (ASPS) [8,52], paraganglioma, angiomyolipoma, and also gynaecological tumours such as endometrial stromal sarcoma with clear cell features or uterine tumour resembling sex cord tumour and other tumours with a clear cell component [4]. It is also important to distinguish them from tumours derived from smooth muscle (epithelioid leiomyosarcoma -LMS and epithelioid leiomyoma).…”

Section: Pathomorphologymentioning

confidence: 99%

Diagnosis and treatment of malignant PEComa tumours

et al. 2020

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PEComa (PEC tumours; perivascular epithelioid cell tumours) is a family of rare tumours of mesenchymal origin, consisting of epithelial perivascular cells expressing melanocytic and myioid markers. This group includes benign tumours-such as angiomyolipoma (AML) of the kidney, and poorly differentiated malignant PEComa tumours with potential for an aggressive clinical course, which is the main focus of this review. PEComas are most often diagnosed in middle-aged women as extensive tumours located in the abdominal cavity or pelvis, manifesting as pain and complaints related to pressure on nearby organs. PEComa tumours should be differentiated from gastrointestinal stromal tumours (GIST), leiomyosarcoma, melanoma metastasis, chromophobic renal cell carcinoma, clear cell sarcoma, and other clear cell component tumours. Somatic inactivating mutations within the TSC1/TSC2 genes, resulting in excessive activation of the mTORC1 complex, are characteristic for this group of tumour. Recently, a separate PEComa subgroup has been distinguished, characterised by the presence of the TFE3 gene fusion, which also causes increased activity of the mTOR signalling pathway. Negative prognostic factors that indicate an increased risk of PEComa malignant biology are most often: tumour size > 5 cm, increased cytological and nuclear atypia, infiltration of surrounding tissues and blood vessels, presence of necrosis, and high mitotic activity. Radical resection remains the primary treatment method for PEComas because these tumours are characterised by high resistance to radiation and chemotherapy. In the case of locally advanced or metastatic disease, only single reports of short-term responses to palliative chemotherapy containing doxorubicin, gemcitabine, or ifosfamide are available in the literature. There are an increasing number of reports, in the form of several case reports and a few retrospective analyses, about the potential effectiveness of using mTOR inhibitors in unresectable cases. These drugs result in a reduction in primary tumour size and metastasis, as well as symptom relief, with controllable side effects. Unfortunately, case reports of complete resistance to mTOR inhibitor therapy are also available.

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