Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature

Gronchi, Alessandro; Diment, Judith; Colecchia, Maurizio; Fiore, Marco; Santinami, Mario

doi:10.1111/j.0309-0167.2004.01801.x

Cited by 14 publications

(9 citation statements)

References 33 publications

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“…This is further supported by the different anatomical distribution, being the genital areas prevalent in women independent of age and the involvement of the intestinal wall quite frequent in men (45%). In children, [6,[14][15][16][17][18][19][21][22][23]25,26,[28][29][30][32][33][34][35] 2 (focal)/21 MART-1 [14,18,33,36] 2/4 Melan-A [6,15,16,18,[20][21][22][23]25,26,28] 6/12 MiTF 22,23,28] 0/3 TFE3 [22,23,25,28] 5/5 Caldesmon [14,15,33] 0/3 Calponin [14,15,36] 3/3 SMA [14-18,20,21,23-26, 28,29,33,34,36] 7/18 Vimentin [17,…”

Section: Discussionmentioning

confidence: 99%

Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Alaggio

Cecchetto

Martignoni

et al. 2012

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 99%

Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Alaggio

Cecchetto

Martignoni

et al. 2012

Journal of Pediatric Surgery

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“…In this study, we analyzed 156 AMLs originating from different organs, which were classified into two groups (regular and epithelioid), and investigated whether the above proposal was suitable for distinguishing the cases with poor prognosis. In addition, although AMLs are mostly observed in the kidney and liver, other involved sites are sporadically reported in the hard palate (3), adrenal gland (4), anterior mediastinum (5), pancreas (6) and pelvis (7). Whether AMLs occurring in sites other than the kidney and liver have the same clinicopathological characteristics and prognosis was also investigated.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological analysis of 156 patients with angiomyolipoma originating from different organs

et al. 2012

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Abstract. Angiomyolipoma (AML) is a benign mesenchymal tumor composed of variable proportions of adipose tissue, spindle and epithelioid smooth muscle cells and abnormal thick-walled blood vessels. Approximately one-third of epithelioid AML (EAML) cases have been reported to have metastasis. Thus, it would be of interest to identify the adverse pathological parameters correlated with outcome. However, few studies have been conducted on large numbers of samples. The aim of this study was to highlight the clinicopathological features of AML and the morphological features of EAML, which were correlated with malignant behaviors in patients from a single institutional series analysis. One hundred and fifty-six consecutive AMLs, correlating with pathological characteristics, were analyzed between 1981 and 2010. The Chi-square test was performed to clarify the significance of the clinicopathological factors among the regular and epithelioid subtypes with or without atypia. The two organs most commonly involved were the kidney (77%, 120/156) and liver (14%, 22/156). Of the 156 AMLs, EAMLs (17.3%, 27/156) had more marked mitosis, hemorrhage and multinucleated giant cells compared with the regular AMLs (82.7%, 129/156). The 11 EAMLs with atypia (40.7%, 11/27) had more nucleoli and mitotic cells compared with the 16 EAMLs without atypia (59.3%, 16/27; p<0.05). Follow-up results of 79% of cases (124/156) were obtained, and of these, only one kidney AML (0.8%, 1/124) presented with liver metastasis one year after nephrectomy. This sizeable single institutional AML series analysis revealed that the kidney and liver were the two most commonly involved organs, and most of the cases presented a benign clinical course. Few EAMLs were malignant, although adverse features including atypical mitotic figures, blood vessel invasion and tumor embolus may be significant in predicting malignant behavior. IntroductionAngiomyolipoma (AML) is a relatively rare neoplasm, composed of variable proportions of adipose tissue, spindle and epithelioid smooth muscle-like cells and abnormal thick-walled blood vessels (1). Most AMLs are regarded as benign in clinical course, although certain cases have been reported to be recurrent or metastatic. Approximately 30% of epithelioid AMLs (EAMLs), which are partially or entirely composed of large epithelioid cells with abundant cytoplasm, vesicular nuclei and prominent nucleoli, have malignant potential (2). By contrast, an epithelioid morphology of the smooth muscle-like cells has been described in regular AMLs that are not associated with aggressive clinical behavior. This is analogous to the conventional description of smooth muscle neoplasm, in which epithelioid and conventional spindle cell leiomyomas exist; when referring to smooth muscle neoplasms, ʻepithelioidʼ does not necessarily indicate atypia or increased risk of malignancy (2). Therefore, in 2010, Brimo et al proposed the concept of atypical EAMLs, and suggested that although not all EAMLs have malignant potential, certain atypical morpholog...

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“…This lack of criteria is primarily a result of the tumour's rarity and the limited follow-up reported in most cases 12,17,18 . The limited number of malignant cases published thus far have described malignant spread as being characterized by a locally infiltrative growth pattern, vascular invasion, a high mitotic rate, necrosis, and tumour size exceeding 8 cm 4,14,16,[19][20][21][22] .…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2015

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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.

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Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature

Cited by 14 publications

References 33 publications

Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Clinicopathological analysis of 156 patients with angiomyolipoma originating from different organs

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

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