PEComa: morphology and genetics of a complex tumor family

Thway, Khin; Fisher, Cyril

doi:10.1016/j.anndiagpath.2015.06.003

Cited by 185 publications

(223 citation statements)

References 189 publications

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“…Angiomyolipoma is a member of the family of neoplasms that derive from perivascular epitheloid cells (PEC) (PEComa family) (Thway & Fisher 2015). It could occur in the settings of tuberous sclerosis complex.…”

Section: Adrenal Angiomyolipomamentioning

confidence: 99%

Lipomatous tumours in adrenal gland: WHO updates and clinical implications

Lam¹

2017

Endocrine-Related Cancer

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Adrenal lipomatous tumour is a group of adrenal tumours with a significant component of adipose tissue. According to the current World Health Organization (WHO) classification of tumours of endocrine organs, adrenal myelolipoma is the only entity amongst the group of tumours being described. In the literature, other more recently documented adrenal lipomatous tumours included 24 lipomas, 32 teratomas and 16 angiomyolipomas. Rare fatty tumours of the adrenal gland comprised liposarcoma, hibernoma, adrenocortical tumours with fat component and rare adrenal tumours with fat component. Myelolipoma comprises approximately 3% of primary adrenal tumour. It is noted more commonly in females and in the right adrenal gland. Approximately 40 bilateral myelolipomas were reported. The tumour is most frequently recorded in patients between fifth and seventh decades of life. Adrenal lipomas are often seen in males and in the right adrenal gland. They were commonly noted in patients in the sixth decade of life. The diagnosis could only be possible on examination of the surgically removed specimen. Adrenal teratomas were more common in females and with a bimodal age distribution. Slightly over 60% of the patients with adrenal teratoma are symptomatic. Adrenal angiomyolipomas were often symptomatic, more common in females and in the fifth decades of life. To conclude, adrenal lipomatous tumour is uncommon. They are often benign and non-functional. It is important to recognize the features of this group of lipomatous tumours in the adrenal gland as they are being detected on increasing incidence as a result of the wide-spread use of modern imaging modalities.

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Section: Adrenal Angiomyolipomamentioning

confidence: 99%

Lipomatous tumours in adrenal gland: WHO updates and clinical implications

Lam¹

2017

Endocrine-Related Cancer

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“…The most sensitive markers are desmin and HMB-45, followed by SMA and caldesmon (8). In the present case, HMB-45 and cytokeratin were positively expressed, whereas the present samples were negative for S-100 protein.…”

Section: Hepatic Multiple Perivascular Epithelioid Cell Neoplasm: a Cmentioning

confidence: 56%

“…Although PEComas exhibit a wide spectrum of biological behavior, experts classified it into three types: i) Benign; ii) of uncertain malignant potential; iii) malignant (8,9). The imaging features of malignant PEComas include signs of tumor size >5 cm, infiltrative growth pattern, high nuclear grade and hypercellularity, a high rate of mitosis, >1/50 high-power fields, coagulative necrosis and vascular invasion (10,11).…”

Section: Hepatic Multiple Perivascular Epithelioid Cell Neoplasm: a Cmentioning

confidence: 99%

Hepatic multiple perivascular epithelioid cell neoplasm: A case report and literature review

Lan

Hua

2016

Molecular and Clinical Oncology

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Abstract. Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor type and primary hepatic multiple perivascular is rarer still. The present case report investigated the case of a 40-year-old woman who was admitted to The Second Xiangya Hospital for hepatic multiple tumor by physical examination without any history of abdominal pain or body weight loss. Abdominal plain computed tomography revealed three lesions in the liver. All lesions exhibited a well-defined boarder and only one mass contained fatty tissue in S6 of the liver. An enhanced scan revealed that all lesions were heterogeneous, and were enhanced on the artery phase and on portal vein phase. On delay phase, the lesions revealed continued enhancement, which is isoattenuating to the normal hepatic parenchyma. Following excision of the three masses, the patient was pathologically diagnosed with hepatic multiple PEComa. Short-term re-examination revealed no recurrence.

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“…Patient 3, whose disease progressed, had a tumor lacking smooth muscle actin or desmin positivity, which has been noted in previous studies in PEComas with a transcription factor binding to IGHM enhancer 3 (TFE3) fusion gene product instead of mTOR pathway alterations (13), and this may explain the lack of response to temsirolimus. Additionally, approximately 75% of sporadic PEComas have alterations in TORC2, which in renal cell carcinomas is relatively more resistant to mTOR inhibitors (14,15). Testing for the TFE3 fusion gene product (Xp11) should be considered in patients with malignant uterine PEComas, as combination treatment with vascular endomethial growth factoror phosphatidylinositol-3-kinase-directed biological therapies may be more effective in this group.…”

Section: Discussionmentioning

confidence: 99%