2020
DOI: 10.1007/s00428-020-02910-9
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Renal cell carcinoma with leiomyomatous stroma in tuberous sclerosis complex: a distinct entity

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Cited by 12 publications
(7 citation statements)
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“…An awareness of the spectrum of AML histologies, such as “fat-poor” AML, angiomyolipoma with entrapped epithelial cysts (AMLEC), sclerosing AML, and AML with oncocytic features, can prevent misdiagnosis [ 24 , 39 , 40 , 41 ] ( Figure 1 ). Epithelioid AML (epithelioid PEComa) is a rare variant composed of ≥80% epithelioid cells ( Figure 1 ).…”
Section: Renal Tumors With Tsc/mtor Pathway Gene Alterations and Thei...mentioning
confidence: 99%
See 1 more Smart Citation
“…An awareness of the spectrum of AML histologies, such as “fat-poor” AML, angiomyolipoma with entrapped epithelial cysts (AMLEC), sclerosing AML, and AML with oncocytic features, can prevent misdiagnosis [ 24 , 39 , 40 , 41 ] ( Figure 1 ). Epithelioid AML (epithelioid PEComa) is a rare variant composed of ≥80% epithelioid cells ( Figure 1 ).…”
Section: Renal Tumors With Tsc/mtor Pathway Gene Alterations and Thei...mentioning
confidence: 99%
“…The name “renal cell carcinoma with fibromyomatous stroma” (RCC FMS) was officially endorsed by the GUPS in 2021 [ 34 ]. RCC FMS is commonly observed in the sporadic setting, but morphologically, immunohistochemically, and molecularly identical tumors have also been described as the most common RCC in TSC patients [ 1 , 2 , 24 , 41 ]. In the sporadic setting, RCC FMS is often associated with mutations involving the TSC1 / TSC2 or MTOR genes [ 24 , 34 , 47 ].…”
Section: Renal Tumors With Tsc/mtor Pathway Gene Alterations and Thei...mentioning
confidence: 99%
“…This entity is difficult to distinguish from a variety of benign and malignant neoplasms, such as clear cell renal cell carcinoma with degenerative fibrosis or hemangioma-like changes, sarcomatoid renal cell carcinoma, clear cell (tubulo)papillary RCC, renal angiomyoadenomatous tumors (RAT), epithelioid angiomyolipoma, and mixed epithelial stromal tumor of the kidney [14]. The distinction between these entities often requires an immunohistochemical study, the profile of which has varied greatly in the different cases published until today.…”
Section: Discussionmentioning
confidence: 99%
“…The majority of these tumors are seen in a sporadic setting; 10 however, identical tumors have been found in patients with TSC, 17 suggesting the existence of hereditary and sporadic counterparts of this tumor. RCCFMS occurs more frequently in women (M: F=1:2) and is seen in adults of a broad age range.…”
Section: Renal Cell Carcinoma With Fibromyomatous Stromamentioning
confidence: 95%
“…16 Recent studies have demonstrated that RCCFMS indeed represents a distinct entity that can be separated from other RCC which exhibit clear cells, as well as tubulopapillary morphology and prominent smooth muscle/fibromatous stroma, such as clear cell RCC and clear cell papillary renal cell tumor (CCPRCT). 9,10 The majority of these tumors are seen in a sporadic setting; 10 however, identical tumors have been found in patients with TSC, 17 suggesting the existence of hereditary and sporadic counterparts of this tumor. RCCFMS occurs more frequently in women (M: F = 1:2) and is seen in adults of a broad age range.…”
Section: Renal Cell Carcinoma With Fibromyomatous Stromamentioning
confidence: 98%