Primary malignant gastric PEComa – Diagnostic and technical dilemmas

Waters, Peadar S; Mitchell, David; Murphy, Ruth; McKenna, Michael J.; Waldron, R.

doi:10.1016/j.ijscr.2011.11.003

Cited by 12 publications

(8 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, there were also some cases with poor prognosis being described such as one patient who had surgery only and died of tumor in the third month. [ 36 ] Researchers also reported that integrated therapy of completed resection and chemotherapy was adopted in a case but the patient had local recurrence and liver metastasis in 22 months and subsequently, died of tumor in 27 months. [ 27 ] And we see that, even the most appropriate management strategy is yet to be defined, surgical resection now almost serves as the predominant treatment and chemotherapy or immunotherapy is considered in cases with trait of malignancy.…”

Section: Discussionmentioning

confidence: 99%

A systematic review

Chen

Han

et al. 2016

Medicine

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

A systematic review

Chen

Han

et al. 2016

Medicine

View full text Add to dashboard Cite

show abstract

“…The most common location of GI PEComas-NOS was the colon (n = 32), 5 – 14 , 36 followed by the small intestine (n = 20), 1 , 15 – 18 , 36 , 37 rectum (n = 7), 19 – 24 and stomach (n = 4). 25 , 26 , 36 Of the patients, 43 were females and 27 were males, with a ratio of 2:1. The age at diagnosis ranged from 5.5 to 71 years.…”

Section: Discussionmentioning

confidence: 99%

“…The majority of reported GI PEComas-NOS were considered to be benign or to have uncertain malignant potential, whereas only 22 cases (22/70) exhibited definite malignant behavior, with local recurrence in 4 cases, metastasis in 19 cases, and tumor-related death in 7 cases. 1 , 9 , 10 , 12 , 15 , 17 , 21 , 26 , 29 , 36 The proposed histologic features indicative of malignancy or high risk for aggressive clinical behavior in GI PEComas-NOS by Folpe et al 38 include infiltrative growth pattern, tumor size (>5 cm), high nuclear grade, tumor necrosis, high mitotic activity (>1/50 HPF), and lymphovascular invasion. A recent case series study of 35 GI PEComas has shown that malignant behavior was statistically significantly associated with marked nuclear atypia, diffuse pleomorphism, and mitoses ≥2/10 HPF, but not with tumor necrosis.…”

Section: Discussionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor of Gastrointestinal Tract

Wang²,

Zhang³

et al. 2015

Medicine

View full text Add to dashboard Cite

Perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas) are exceedingly rare, with only a limited number of published reports worldwide. Given the scarcity of GI PEComas and their relatively short follow-up periods, our current knowledge of their biologic behavior, molecular genetic alterations, diagnostic criteria, and prognostic factors continues to be very limited.We present 2 cases of GI PEComas, one of which showed an aggressive histologic behavior that underwent multiple combined chemotherapies. We also review the available English-language medical literature on GI PEComas-not otherwise specified (PEComas-NOS) and discuss their clinicopathological and molecular genetic features.Pathologic analyses including histomorphologic, immunohistochemical, and ultrastructural studies were performed to evaluate the clinicopathological features of GI PEComas, their diagnosis, and differential diagnosis. Immunohistochemistry, semiquantitative reverse transcriptase polymerase chain reaction, and DNA sequencing assays were carried out to detect the potential molecular genetic alterations in our casesMicroscopically, the tumors showed distinctive histologic features of PEComas-NOS, including fascicular or nested architecture, epithelioid or spindled cell type, and clear to eosinophilic cytoplasm. The tumor cells were immunohistochemically positive for melanocytic markers. Molecular pathological assays confirmed a PSF-TFE3 gene fusion in one of our cases. Furthermore, in this case microphthalmia-associated transcription factor and its downstream genes were found to exhibit elevated transcript levels.Knowledge about the molecular genetic alterations in GI PEComas is still limited and warrants further study.

show abstract

“…However it should be noted that such recurrence could be attributed to a few cases being treated with a combination of chemotherapy and rt after surgical resection 9 . The mean survival time for a malignant pecoma of the terminal ileum is estimated to be 28 months 23 .…”

Section: Discussionmentioning

confidence: 99%

PEComa of the Terminal Ileum Mesentery as a Secondary Tumour in an Adult Survivor of Embryonal Rhabdomyosarcoma

et al. 2015

View full text Add to dashboard Cite

Perivascular epithelioid cell tumours (pecomas) are rare mesenchymal tumours that are characterized by perivascular epithelioid cell differentiation and immunoreactivity to myogenic and melanocytic markers. These tumours can be classified as benign, uncertain malignant potential, or malignant. Because of the rarity of pecomas, their cause and clinical prognosis remain unclear.To the best of our knowledge, no reports in the literature describe a pecoma of the terminal ileum mesentery as a secondary tumour in an adult survivor of childhood embryonal rhabdomyosarcoma, let alone any childhood cancer. Here, we present the case of a 27-year-old man with a pecoma involving the mesentery of the terminal ileum. At the age of 5, he had been treated with a combination of chemotherapy and high-dose pelvic radiation therapy for embryonal rhabdomyosarcoma, most likely arising from the posterior bladder wall.During routine follow-up 22 years after this patient's initial treatment, computed tomography imaging revealed a mass within the terminal ileum mesentery. The tumour was successfully treated with surgical resection, and pathology examination determined the mass to be a pecoma with uncertain malignant potential.This first case of a pecoma of the terminal ileum mesentery arising within a high-dose radiation therapy field as a secondary tumour in an adult survivor of childhood cancer highlights the importance of screening and surveillance in high-risk childhood cancer survivors treated with high-dose radiation therapy. Further research to build a better understanding of this remarkably rare tumour is warranted.

show abstract

Primary malignant gastric PEComa – Diagnostic and technical dilemmas

Abstract: This case depicts the aggressive nature of malignant gastric PEComa's. The majority of PEComa's are benign in nature and have a better prognosis. We display here the challenges in ascertaining a definitive diagnosis and management of such patients due to limited clinical studies.

Cited by 12 publications

References 8 publications

A systematic review

A systematic review

Perivascular Epithelioid Cell Tumor of Gastrointestinal Tract

PEComa of the Terminal Ileum Mesentery as a Secondary Tumour in an Adult Survivor of Embryonal Rhabdomyosarcoma

Contact Info

Product

Resources

About