Malignant Monotypic Epithelioid Angiomyolipoma of the Retroperitoneum

Lau, Sean K.; Marchevsky, Alberto M.; McKenna, Robert J.; Luthringer, Daniel

doi:10.1177/106689690301100313

Cited by 23 publications

(27 citation statements)

References 33 publications

(82 reference statements)

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“…This independent rare subtype of AML has malignant potential and imaging characteristics similar to RCC. Some cases of epithelioid AML metastasized to the lung, liver or bone have been previously reported but our patient wasn't metastatic and was asymptomatic (65)(66)(67). As the proper treatment for epithelioid AML is still discussed, we decided to perform an additional thermal ablation of the tumor while other authors recommend a more aggressive strategy using surgery with or without chemotherapy (68).…”

Section: Discussionmentioning

confidence: 99%

Selective arterial embolization of symptomatic and asymptomatic renal angiomyolipomas: a retrospective study of safety, outcomes and tumor size reduction

Bardin¹,

Chevallier²,

Bertaut³

et al. 2017

Quant. Imaging Med. Surg.

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Background: Angiomyolipoma (AML) is the most common renal benign tumor. Treatment should be considered for symptomatic patients or for those at risk for complications, especially retroperitoneal bleeding which is correlated to tumor size, grade of the angiogenic component and to the presence of tuberous sclerosis complex (TSC). This study reports our single-center experience with the use of selective arterial embolization (SAE) in the management of symptomatic and asymptomatic renal AMLs. Methods:In this retrospective mono-centric study, all demographic and imaging data, medical records, angiographic features, outpatient charts and follow-up visits of patients who underwent prophylactic or emergency SAE for AMLs between January 2005 and July 2016 were reviewed. Tumor size and treatment outcomes were assessed at baseline and after the procedure during follow-up. Computed tomography (CT), magnetic resonance imaging (MRI) or ultrasonography was used to evaluate AML shrinkage. Renal function was measured pre-and post-procedure.Results: Twenty-three patients (18 females, 5 males; median age, 45 years; range, 19-85 years) who underwent SAE either to treat bleeding AML (n=6) or as a prophylactic treatment (n=17) were included.Overall, 34 AMLs were embolized. TSC status was confirmed for 6 patients. Immediate technical success rate was 96% and 4 patients benefitted from an additional procedure. Major complications occurred in 3 patients and minor post-embolization syndrome (PES) in 14 patients. The mean AML size reduction rate was 26.2% after a mean follow-up was 20.5 months (range, 0.5-56 months), and only non-TSC status was significantly associated with better shrinkage of tumor (P=0.022). Intralesional aneurysms were significantly more frequent in patients with hemorrhagic presentation (P=0.008). There was no change in mean creatinine level after SAE.Conclusions: SAE is a safe and effective technique to manage renal AMLs as a preventive treatment as well as in emergency setting, with significant reduction in tumor size during follow-up. A multidisciplinary approach remains fundamental, especially for TSC patients. In addition to size, the presence of intralesional aneurysms should be considered in any prophylactic treatment decision.

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Section: Discussionmentioning

confidence: 99%

Selective arterial embolization of symptomatic and asymptomatic renal angiomyolipomas: a retrospective study of safety, outcomes and tumor size reduction

Bardin¹,

Chevallier²,

Bertaut³

et al. 2017

Quant. Imaging Med. Surg.

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“…10 The cell of origin of EAML is considered to be a perivascular epithelioid cell (PEC), which is characterised by immunoreactivity for HMB-45. 1 Other tumours derived from PECs include lymphangiomyomatosis, clear cell tumours of various organs, clear cell myomelanocytic tumours and abdominal pelvic sarcomas of PECs. 1 There does not seem to be any histological finding that predicts malignant behaviour in AMLs.…”

Section: Discussionmentioning

confidence: 99%

“…1 Other tumours derived from PECs include lymphangiomyomatosis, clear cell tumours of various organs, clear cell myomelanocytic tumours and abdominal pelvic sarcomas of PECs. 1 There does not seem to be any histological finding that predicts malignant behaviour in AMLs. The presence of mitotic activity in the epithelioid areas is a feature common to many of the reported cases of metastatic AML 6 ; however, it is often observed in otherwise benign EAMLs.…”

Section: Discussionmentioning

confidence: 99%

“…Infection-associated haemophagocytic syndrome associated with recurrent acute myeloid leukaemia/myelodysplastic syndrome: an autopsy case 1 We report a fatal case of IAHS arising in a patient with recurrent acute myeloid leukaemia (AML)/myelodysplastic…”

Section: Authors' Affiliationsmentioning

confidence: 99%

“…It is considered a benign neoplasm, even though it may exhibit atypical histological features, involve regional lymph nodes and be multifocal. [1][2][3][4][5] Recently, an epithelioid variant of AML (EAML) has been described, and is thought to be more aggressive. We describe an EAML of the retroperitoneum that metastasised to the liver and bone, and present a literature review.…”

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confidence: 99%

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Metastatic retroperitoneal epithelioid angiomyolipoma

et al. 2007

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Angiomyolipoma (AML) is a rare benign neoplasm that usually arises in the kidneys, but may rarely originate in sites such as the retroperitoneum, liver and bone. It is characterised by an intimate admixture of blood vessels, smooth muscle and fat. This multiphasic composition allows for its recognition on ultrasound and CT scan examination. Metastases are exceedingly uncommon, and only one other case of metastatic extrarenal AML exists in the literature. Histological variants of AML such as epithelioid angiomyolipoma (EAML) are considered to be locally aggressive. A unique case of an EAML of the retroperitoneum that metastasised to the liver and bone in an 80-year-old woman is described.

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PEComas: the past, the present and the future

et al. 2007

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The perivascular epithelioid cell (PEC) is a cell type constantly present in a group of tumors called PEComas. PEC expresses myogenic and melanocytic markers, such as HMB45 and actin. Recently, recurrent chromosomal alterations have been demonstrated in PEC. At present, PEComa is a widely accepted entity. In the past 10 years, the use of this term has allowed to report and describe numerous cases permitting to start highlighting the biology of this group of lesions. PEComas are related to the genetic alterations of tuberous sclerosis complex (TSC), an autosomal dominant genetic disease due to losses of TSC1 (9q34) or TSC2 (16p13.3) genes which seem to have a role in the regulation of the Rheb/mTOR/p70S6K pathway. There are some open questions about PEComas regarding its histogenesis, the definition of epithelioid angiomyolipoma and the identification of the histological criteria of malignancy. An innovative therapeutic trial using rapamycin is under way for tumors occurring in TSC such as renal angiomyolipoma and lymphangioleiomyomatosis. Its success could provide the rationale for the use of the same drug in other lesions composed of PECs, especially in the malignant ones.Keywords PEComa . PEC . Angiomyolipoma . Lymphangioleiomyomatosis . Sugar tumor . TSC . mTOR . RapamycinWhat is the perivascular epithelioid cell?The perivascular epithelioid cell (PEC) (Fig. 1) is a cell type constantly present in a group of tumors including angiomyolipoma (AML), clear-cell "sugar" tumor (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres and rare clear-cell tumors of other anatomical sites.It has morphologic, immunohistochemical, ultrastructural and genetic distinctive features such as an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus and an inconspicuous nucleolus. PEC has mild to any atypia and a typical perivascular location [13]. At present, PEC has not a known normal counterpart.Immunohistochemically, PEC expresses myogenic and melanocytic markers, such as HMB45, HMSA-1, MelanA/ Mart1, microophtalmia transcription factor (Mitf), actin and, less commonly, desmin [13,55,126]. Its immunoreactivity for vimentin is usually incospicuous.At ultrastructural analysis, PEC contains microfilament bundles with electron-dense condensation, numerous mithocondria and membrane-bound dense granules [11,118].It is thought that PEC can modulate its morphology and immunophenotype: given all the characteristics described above (Fig. 2), PEC can show muscular features with a spindle shape and a stronger positivity for actin than for HMB45 or it can have an epithelioid feature with a strong positivity for HMB45 and a mild, if any, reaction for actin (Fig. 3). PEC can also become vacuolized acquiring the feature of an adipocyte. Progesterone receptor positivity has been described in PEC with spindle morphology; this suggests a possible role of progesterone in this morphologic modulation [...

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Malignant Monotypic Epithelioid Angiomyolipoma of the Retroperitoneum

Cited by 23 publications

References 33 publications

Selective arterial embolization of symptomatic and asymptomatic renal angiomyolipomas: a retrospective study of safety, outcomes and tumor size reduction

Selective arterial embolization of symptomatic and asymptomatic renal angiomyolipomas: a retrospective study of safety, outcomes and tumor size reduction

Metastatic retroperitoneal epithelioid angiomyolipoma

PEComas: the past, the present and the future

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