Abstracts
Mixed epithelial and stromal tumor (MEST) represents a recently described biphasic kidney neoplasm, which predominantly affects perimenopausal females. The authors report the case of a young male patient with a MEST exhibiting positivity for estrogen and progesterone receptors. Computed tomography/magnetic resonance imaging (CT/MRI) showed an expansive lesion affecting the right kidney. Grossly, a solid-cystic tumor was identified, which measured 5.7 × 3.5 × 2.4 cm. On microscopic examination, a biphasic tumor constituted by stromal and epithelial elements, without significant atypias, was identified. The stromal element was composed of spindle cells revealing positive immunoexpression for actin, desmin, vimentin, and estrogen receptors. The epithelial component exhibited a predominantly tubular pattern showing positive immunoreaction for cytokeratins. The diagnosis of MEST was then established.
kidney; neoplasias; mixed epithelial and stromal tumor; pathology; immunohistochemistry
O tumor epitelial e estromal misto (TESM) representa uma neoplasia renal bifásica descrita recentemente que afeta predominantemente mulheres na perimenopausa. Os autores relatam o caso de um paciente jovem, do sexo masculino, com TESM exibindo positividade para receptores de estrogênio e progesterona. A tomografia computadorizada/ressonância magnética (TC/RM) mostrou lesão expansiva no rim direito. Ao exame macroscópico, identificou-se tumor sólido-cístico medindo 5,7 × 3,5 × 2,4 cm. À microscopia, foi encontrado tumor bifásico constituído por elementos estromais e epiteliais, sem atipias significativas. O componente estromal era composto por células fusiformes, exibindo imunoexpressão positiva para actina, desmina, vimentina e receptores de estrogênio. Os elementos epiteliais mostraram padrão predominantemente tubular e exibiram imunorreação positiva para citoqueratinas. O diagnóstico de TESM foi então estabelecido.
rim; neoplasias; tumor misto epitelial e estromal; patologia; imuno-histoquímica
INTRODUCTION
Tumors of the kidney amount to 2% of the total human cancer burden, and renal cell
carcinoma represents, on average, over 90% of all malignances of the kidney. Mixed
epithelial and stromal tumor (MEST) is a complex renal neoplasm composed of a mixture of
stromal and epithelial elements(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.
2 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.-33 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and
PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with
epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal
tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.). These rare
lesions have been termed cystic hamartoma of renal pelvis, adult mesoblastic nephroma,
leiomyomatous renal hamartoma, and mesoblastic nephroma(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,44 Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors
of the kidney: evidence for a single cell of origin with capacity for epithelial and
stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.
5 Michal M, Hes O, Bisceglia M, et al. Mixed epithelial and stromal tumors
of the kidney. A report of 22 cases. Virchows Arch.
2004;445(4):359-67.-66 Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and
stromal tumours of the kidney: a report of the first two cases with a fatal clinical
outcome. Histopathology. 2004;44(3):302-4.). MEST is
four to six times more common in women than in men. All patients have been adults, with
a mean age of 46 years. Owing to the disparity between incidence rates in women and men,
a hormonal influence on the development of MEST has been suggested(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.
2 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.-33 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and
PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with
epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal
tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.,66 Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and
stromal tumours of the kidney: a report of the first two cases with a fatal clinical
outcome. Histopathology. 2004;44(3):302-4.). Herein the
authors report the case of a male patient with a MEST exhibiting positive
immunoexpression of estrogen and progesterone receptors, and discuss clinical and
morphologic findings of this uncommon tumor.
CASE REPORT
A 26-year-old male patient was admitted to the nephrology service due to a 30-day history of macroscopic hematuria episodes. On physical examination, there were no clinical changes. There was no previous history of relevant disease. On laboratory investigation, glucose, hemoglobin, erythrocytes, creatinine, urea, liver enzymes, and cholesterol were within normal plasma levels. Serologic tests for hepatitis B virus, hepatitis C, and HIV were negative. Qualitative urinalysis revealed the presence of several red blood cells per field. Abdominal computed tomography/ magnetic resonance imaging (CT/MRI) showed an expansive round lesion affecting the inferior pole of the right kidney. CT/ MRI of the chest has not identified significant alterations. The patient underwent radical nephrectomy. On gross examination, an ovoid well-circumscribed solid-cystic pale tumor was identified, which measured 5.7 × 3.5 × 2.4 cm (Figure 1). The process did not compromise the renal capsule or the perinephric tissues.
Mixed epithelial and stromal tumor of the kidney: a circumscribed, light brown, solid tumor, with cystic areas
On microscopic examination, a biphasic tumor was found. The stromal element was composed of spindle cells arranged in short fascicles (Figure 2), and varying from hypocellular to fibrotic areas. The epithelial element was constituted by cuboidal to columnar cells showing clear or acidophilic cytoplasm and ovoid central nuclei. Epithelial cells were distributed in immature tubules exhibiting various degrees of luminal dilation, and/or forming micro- and macrocysts. Neither significant atypia nor necrosis was identified. The stromal element exhibited positive immunoexpression for actin, desmin, vimentin, and estrogen (Figure 3) and progesterone receptors. The epithelial component showed positive immunoreactions for cytokeratins (AE1/AE3) and vimentin. The tumor exhibited negative immunoexpression for melanocyte antigen (melan-A), and human melanoma black 45 (HMB-45). The diagnosis of kidney MEST was then established.
Mixed epithelial and stromal tumor: spindle cells arranged in short fascicles, hematoxylin-eosin, 200×.
Mixed epithelial and stromal tumor A) a biphasic tumor composed by spindle cells and epithelial elements; B) positive immunoexpression for estrogen receptor in the stromal component, streptavidin-biotin, 400×; C) positive control for estrogen receptors in breast invasive ductal carcinoma, streptavidinbiotin, 200×; D) epithelial covering cystic formations, hematoxylin-eosin, 40×.
DISCUSSION
MEST comprises a rare tumor of the kidney that is more common in women than in men (6:1,
respectively). In general, patients are adults, with a mean age of 45 years. MEST lacks
the translocation characteristic of cellular congenital mesoblastic nephroma, and most
reported cases are benign tumors(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,77 Durham JR, Bostwick DG, Farrow GM, et al. Mesoblastic nephroma of
adulthood. Report of three cases. Am J Surg Pathol.
1993;17(10):1029-38.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.). The presenting symptoms are flank pain, hematuria, or symptoms
of the urinary tract. The lesion corresponds to an incidental finding in 25% of the
patients(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,77 Durham JR, Bostwick DG, Farrow GM, et al. Mesoblastic nephroma of
adulthood. Report of three cases. Am J Surg Pathol.
1993;17(10):1029-38.
8 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.-99 Menéndez CL, Rodríguez VD, Fernández-Pello S, et al. A new case of
malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous
transformation. Anal Quant Cytopathol Histopathol. 2012;34(6):331-4.). On gross
examination, MEST is a centrally located, circumscribed, solid-cystic kidney lesion that
frequently extends into the renal pelvis. A partial or complete capsule is often
present. The tumor infrequently shows an infiltrative border. Mean tumor size reported
in the literature was 6 cm(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor
of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.
9 Menéndez CL, Rodríguez VD, Fernández-Pello S, et al. A new case of
malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous
transformation. Anal Quant Cytopathol Histopathol. 2012;34(6):331-4.
10 Mohanty SK, Parwani AV. Mixed epithelial and stromal tumors of the
kidney: an overview. Arch Pathol Lab Med. 2009;133(9):1483-6.
11 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor.
In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary
structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p.
185.
12 Pierson CR, Schober MS, Wallis T, et al. Mixed epithelial and stromal
tumor of the kidney lacks the genetic alterations of cellular congenital mesoblastic
nephroma. Hum Pathol. 2001;32(5):513-29.
13 Srigley JR, Delahunt B, Eble JN, et al. The International Society of
Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. Am J Surg
Pathol. 2013;37(10):1469-89.-1414 Wang CJ, Lin YW, Xiang H, et al. Mixed epithelial and stromal tumor of
the kidney: report of eight cases and literature review. World J Surg Oncol.
2013;11(1):207.).
Microscopically, MEST can be composed of cysts, microcysts, tubules, and complex
branching glandular formations. In some, there are leaflike arrangements resembling
those seen in phyllodes tumor. Epithelial component ranges from low cuboidal to columnar
or hobnail, with clear to pale, eosinophilic, or vacuolated cytoplasm(22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.
3 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and
PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with
epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal
tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.-44 Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors
of the kidney: evidence for a single cell of origin with capacity for epithelial and
stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.,1515 Lopez-Fontana G, Gallegos I, Sepúlveda F, et al. Mixed epithelial and
stromal tumor of the kidney (MEST). Arch Esp Urol. 2012;65(7):713-6.,1616 Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the
kidney. Pathol Res Pract. 1998;194(6):445-8.). Ciliated cells, urothelium, presence of mucin, or epithelial
cells exhibiting müllerian findings have been described. The architecture of the
microcysts varies from simple microcysts with abundant stroma to densely packed clusters
of microcysts, or even to complex branching channels which may be
dilated(33 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and
PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with
epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal
tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.,44 Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors
of the kidney: evidence for a single cell of origin with capacity for epithelial and
stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.,1010 Mohanty SK, Parwani AV. Mixed epithelial and stromal tumors of the
kidney: an overview. Arch Pathol Lab Med. 2009;133(9):1483-6.,1111 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor.
In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary
structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p.
185.,1515 Lopez-Fontana G, Gallegos I, Sepúlveda F, et al. Mixed epithelial and
stromal tumor of the kidney (MEST). Arch Esp Urol. 2012;65(7):713-6.,1717 Pawade J, Soosay GN, Delprado W, et al. Cystic hamartoma of the renal
pelvis. Am J Surg Pathol. 1993;17(11):1169-75.
18 Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal
tumor of the kidney. Virchows Arch. 2001;439(5):700-2.-1919 Van Velden DJ, Schneider JW, Allen FJ. A case of adult mesoblastic
nephroma: ultrastructure and discussion of histogenesis. J Urol.
1990;143(6):1216-9.). The stromal component exhibits
spindle cells with plump nuclei and abundant cytoplasm. Stromal areas can vary from
hypocellular, collagenous and fibrotic to densely cellular, with a woven pattern
resembling ovarian stroma(22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.,1111 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor.
In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary
structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p.
185.,1616 Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the
kidney. Pathol Res Pract. 1998;194(6):445-8.
17 Pawade J, Soosay GN, Delprado W, et al. Cystic hamartoma of the renal
pelvis. Am J Surg Pathol. 1993;17(11):1169-75.-1818 Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal
tumor of the kidney. Virchows Arch. 2001;439(5):700-2.,2020 Sukov WR, Cheville JC, Lager DJ, et al. Malignant mixed epithelial and
stromal tumor of the kidney with rhabdoid features: report of a case including
immunohistochemical, molecular genetic studies and comparison to morphologically
similar renal tumors. Hum Pathol. 2007;38(9):1432-7.). Fascicles of smooth muscle cells or
areas exhibiting a myxoid stroma can be found. Adipose tissue is occasionally
present(22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,33 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and
PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with
epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal
tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.,44 Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors
of the kidney: evidence for a single cell of origin with capacity for epithelial and
stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.,1515 Lopez-Fontana G, Gallegos I, Sepúlveda F, et al. Mixed epithelial and
stromal tumor of the kidney (MEST). Arch Esp Urol. 2012;65(7):713-6.,1616 Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the
kidney. Pathol Res Pract. 1998;194(6):445-8.,2020 Sukov WR, Cheville JC, Lager DJ, et al. Malignant mixed epithelial and
stromal tumor of the kidney with rhabdoid features: report of a case including
immunohistochemical, molecular genetic studies and comparison to morphologically
similar renal tumors. Hum Pathol. 2007;38(9):1432-7.,2121 Teklali Y, Piolat C, Durand C, et al. Mixed epithelial and stromal renal
tumour in a 12-year-old boy. J Pediatr Urol. 2010;6(3):320-7.). Neither
the stromal nor the epithelial component shows mitotic figures or cytological atypia.
Mitotic figures, hemorrhage, and necrosis have not been found in most cases of indolent
MEST(22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal
tumor of the kidney of possible müllerian origin. J Urol.
2001;166(4):1381-2.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal
tumor of the kidney with polypoid component extending into renal pelvis and ureter.
Acta Radiol Short Rep. 2012;1(1):3.,1111 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor.
In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary
structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p.
185.,1616 Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the
kidney. Pathol Res Pract. 1998;194(6):445-8.
17 Pawade J, Soosay GN, Delprado W, et al. Cystic hamartoma of the renal
pelvis. Am J Surg Pathol. 1993;17(11):1169-75.-1818 Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal
tumor of the kidney. Virchows Arch. 2001;439(5):700-2.,2020 Sukov WR, Cheville JC, Lager DJ, et al. Malignant mixed epithelial and
stromal tumor of the kidney with rhabdoid features: report of a case including
immunohistochemical, molecular genetic studies and comparison to morphologically
similar renal tumors. Hum Pathol. 2007;38(9):1432-7.). The Table shows cases of MEST found in the international literature and
comparable to the present report.
Spindle cells show positive immunoexpression for actins, desmin, vimentin, smooth muscle actin, and estrogen and progesterone receptors. The epithelial component exhibits positive immunoexpression for cytokeratins and often vimentin. Positivity for estrogen receptor has been described in the epithelial element, and it has also been noted in male patients who have received antiandrogenic therapy for prostate cancer. MEST has been considered a cystic nephroma that has acquired cellular, ovarian-like stroma secondary to hormonal influences(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,33 Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.,1414 Wang CJ, Lin YW, Xiang H, et al. Mixed epithelial and stromal tumor of the kidney: report of eight cases and literature review. World J Surg Oncol. 2013;11(1):207.,1919 Van Velden DJ, Schneider JW, Allen FJ. A case of adult mesoblastic nephroma: ultrastructure and discussion of histogenesis. J Urol. 1990;143(6):1216-9.,2323 Ekici AID, Ekici S, Gürel B, et al. Benign mixed epithelial and stromal tumor of the kidney. ScientificWorldJournal. 2006;6:615-8.,2727 Suzuki T, Hiragata S, Hosaka K, et al. Malignant mixed epithelial and stromal tumor of the kidney: report of the first male case. Int J Urol. 2013;20(4):448-50.,2828 Zou L, Zhang X, Xiang H. Malignant mixed epithelial and stromal tumor of the kidney: the second male case and review of literature. Int J Clin Exp Pathol. 2014;7(5):2658-63.). In the present report, authors described immunopositivity for estrogen receptor in the stromal component of a male patient who developed a MEST of the kidney. Some authors consider this unexpected finding associated with tumor loss differentiation and the activation of estrogen-related protein p29, which is a possible factor of influence on some gender-specific estrogen-associated tumors(44 Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors of the kidney: evidence for a single cell of origin with capacity for epithelial and stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.,66 Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and stromal tumours of the kidney: a report of the first two cases with a fatal clinical outcome. Histopathology. 2004;44(3):302-4.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal tumor of the kidney with polypoid component extending into renal pelvis and ureter. Acta Radiol Short Rep. 2012;1(1):3.,99 Menéndez CL, Rodríguez VD, Fernández-Pello S, et al. A new case of malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous transformation. Anal Quant Cytopathol Histopathol. 2012;34(6):331-4.,1414 Wang CJ, Lin YW, Xiang H, et al. Mixed epithelial and stromal tumor of the kidney: report of eight cases and literature review. World J Surg Oncol. 2013;11(1):207.,1818 Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal tumor of the kidney. Virchows Arch. 2001;439(5):700-2.,2323 Ekici AID, Ekici S, Gürel B, et al. Benign mixed epithelial and stromal tumor of the kidney. ScientificWorldJournal. 2006;6:615-8.). The histogenesis of MEST is unknown. Hormonal imbalance during the perimenopausal period can be related to the activation of a misplaced immature or fetal mesenchyme, which harbors the capacity for a dual, epithelial and mesenchymal differentiation. Some authors suggest the hypothesis of müllerian remnants developing neoplastic transformation due to excessive hormonal stimulation(11 Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol. 2000;24(7):958-70.,22 Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal tumor of the kidney of possible müllerian origin. J Urol. 2001;166(4):1381-2.,66 Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and stromal tumours of the kidney: a report of the first two cases with a fatal clinical outcome. Histopathology. 2004;44(3):302-4.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal tumor of the kidney with polypoid component extending into renal pelvis and ureter. Acta Radiol Short Rep. 2012;1(1):3.,1111 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor. In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p. 185.,1818 Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal tumor of the kidney. Virchows Arch. 2001;439(5):700-2.,2323 Ekici AID, Ekici S, Gürel B, et al. Benign mixed epithelial and stromal tumor of the kidney. ScientificWorldJournal. 2006;6:615-8.,2727 Suzuki T, Hiragata S, Hosaka K, et al. Malignant mixed epithelial and stromal tumor of the kidney: report of the first male case. Int J Urol. 2013;20(4):448-50.).
The differential diagnosis includes adult nephroblastoma, cystic nephroma, and sarcomatoid renal cell carcinoma. The lack of cellular anaplasia rules out these tumors. The absence of blastema excludes nephroblastoma, and cystic nephroma typically shows a thin and fibrous stroma. MEST have been treated with surgical excision, and a few cases of recurrence, malignant transformation, and metastases development have been reported(66 Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and stromal tumours of the kidney: a report of the first two cases with a fatal clinical outcome. Histopathology. 2004;44(3):302-4.,88 Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal tumor of the kidney with polypoid component extending into renal pelvis and ureter. Acta Radiol Short Rep. 2012;1(1):3.,99 Menéndez CL, Rodríguez VD, Fernández-Pello S, et al. A new case of malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous transformation. Anal Quant Cytopathol Histopathol. 2012;34(6):331-4.,1111 Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor. In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p. 185.,1313 Srigley JR, Delahunt B, Eble JN, et al. The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. Am J Surg Pathol. 2013;37(10):1469-89.,1515 Lopez-Fontana G, Gallegos I, Sepúlveda F, et al. Mixed epithelial and stromal tumor of the kidney (MEST). Arch Esp Urol. 2012;65(7):713-6.,1616 Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the kidney. Pathol Res Pract. 1998;194(6):445-8.,2323 Ekici AID, Ekici S, Gürel B, et al. Benign mixed epithelial and stromal tumor of the kidney. ScientificWorldJournal. 2006;6:615-8.,2424 Hou CP, Chiang YJ, Chu SH, et al. Mixed epithelial and stromal tumor of the kidney - a case report. Chang Gung Med J. 2010;33(6):693-8.,2727 Suzuki T, Hiragata S, Hosaka K, et al. Malignant mixed epithelial and stromal tumor of the kidney: report of the first male case. Int J Urol. 2013;20(4):448-50.).
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1Adsay NV, Eble JN, Srigley JR, et al. Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol. 2000;24(7):958-70.
-
2Beiko DT, Nickel JC, Boag AH, et al. Benign mixed epithelial stromal tumor of the kidney of possible müllerian origin. J Urol. 2001;166(4):1381-2.
-
3Karafin M, Parwani AV, Netto GJ, et al. Diffuse expression of PAX2 and PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal tubular differentiation. Am J Surg Pathol. 2011;35(9):1264-73.
-
4Kum JB, Grignon DJ, Wang M, et al. Mixed epithelial and stromal tumors of the kidney: evidence for a single cell of origin with capacity for epithelial and stromal differentiation. Am J Surg Pathol. 2011;35(8):1114-22.
-
5Michal M, Hes O, Bisceglia M, et al. Mixed epithelial and stromal tumors of the kidney. A report of 22 cases. Virchows Arch. 2004;445(4):359-67.
-
6Nakagawa T, Kanai Y, Fujimoto H, et al. Malignant mixed epithelial and stromal tumours of the kidney: a report of the first two cases with a fatal clinical outcome. Histopathology. 2004;44(3):302-4.
-
7Durham JR, Bostwick DG, Farrow GM, et al. Mesoblastic nephroma of adulthood. Report of three cases. Am J Surg Pathol. 1993;17(10):1029-38.
-
8Horikawa M, Shinmoto H, Kuroda K, et al. Mixed epithelial and stromal tumor of the kidney with polypoid component extending into renal pelvis and ureter. Acta Radiol Short Rep. 2012;1(1):3.
-
9Menéndez CL, Rodríguez VD, Fernández-Pello S, et al. A new case of malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous transformation. Anal Quant Cytopathol Histopathol. 2012;34(6):331-4.
-
10Mohanty SK, Parwani AV. Mixed epithelial and stromal tumors of the kidney: an overview. Arch Pathol Lab Med. 2009;133(9):1483-6.
-
11Murphy WM, Grignon DJ, Perlman EJ. Mixed epithelial and stromal tumor. In: Murphy W, Grignon DJ, editors. Tumors of the kidney, bladder, and related urinary structures - AFIP Atlas of Tumor Pathology. 4th Series, Washington: ARP; 2004, p. 185.
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12Pierson CR, Schober MS, Wallis T, et al. Mixed epithelial and stromal tumor of the kidney lacks the genetic alterations of cellular congenital mesoblastic nephroma. Hum Pathol. 2001;32(5):513-29.
-
13Srigley JR, Delahunt B, Eble JN, et al. The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. Am J Surg Pathol. 2013;37(10):1469-89.
-
14Wang CJ, Lin YW, Xiang H, et al. Mixed epithelial and stromal tumor of the kidney: report of eight cases and literature review. World J Surg Oncol. 2013;11(1):207.
-
15Lopez-Fontana G, Gallegos I, Sepúlveda F, et al. Mixed epithelial and stromal tumor of the kidney (MEST). Arch Esp Urol. 2012;65(7):713-6.
-
16Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the kidney. Pathol Res Pract. 1998;194(6):445-8.
-
17Pawade J, Soosay GN, Delprado W, et al. Cystic hamartoma of the renal pelvis. Am J Surg Pathol. 1993;17(11):1169-75.
-
18Svec A, Hes O Michal M, et al. Malignant mixed epithelial and stromal tumor of the kidney. Virchows Arch. 2001;439(5):700-2.
-
19Van Velden DJ, Schneider JW, Allen FJ. A case of adult mesoblastic nephroma: ultrastructure and discussion of histogenesis. J Urol. 1990;143(6):1216-9.
-
20Sukov WR, Cheville JC, Lager DJ, et al. Malignant mixed epithelial and stromal tumor of the kidney with rhabdoid features: report of a case including immunohistochemical, molecular genetic studies and comparison to morphologically similar renal tumors. Hum Pathol. 2007;38(9):1432-7.
-
21Teklali Y, Piolat C, Durand C, et al. Mixed epithelial and stromal renal tumour in a 12-year-old boy. J Pediatr Urol. 2010;6(3):320-7.
-
22Demiralay E, Çmunoglu1C, Özdemiret H, et al. Mixed epithelial and stromal tumor of the kidney: a case report. Surg Science. 2011;2(2):66-8.
-
23Ekici AID, Ekici S, Gürel B, et al. Benign mixed epithelial and stromal tumor of the kidney. ScientificWorldJournal. 2006;6:615-8.
-
24Hou CP, Chiang YJ, Chu SH, et al. Mixed epithelial and stromal tumor of the kidney - a case report. Chang Gung Med J. 2010;33(6):693-8.
-
25Rao HD, Sriram S, Srinivas BH, et al. Mixed epithelial stromal tumor of the kidney. Indian J Urol. 2011;27(2):284-7.
-
26Richter M, Meyer W, Küste J, et al. Exophytic benign mixed epithelial stromal tumour of the kidney: case report of a rare tumor entity. Diagn Pathol. 2010;5:16.
-
27Suzuki T, Hiragata S, Hosaka K, et al. Malignant mixed epithelial and stromal tumor of the kidney: report of the first male case. Int J Urol. 2013;20(4):448-50.
-
28Zou L, Zhang X, Xiang H. Malignant mixed epithelial and stromal tumor of the kidney: the second male case and review of literature. Int J Clin Exp Pathol. 2014;7(5):2658-63.
Publication Dates
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Publication in this collection
Jan-Feb 2015
History
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Received
30 Apr 2014 -
Reviewed
10 Dec 2014 -
Accepted
11 Dec 2014