Mixed Epithelial And Stromal Tumor Research Articles

Immunophenotypic analysis of angiomyolipoma with epithelial cysts, comparison to mixed epithelial and stromal tumors and epithelial and stromal elements of normal kidney and ovaries.

Angiomyolipoma with epithelial cysts (AMLEC) is a rare variant of renal angiomyolipoma (AML). It is characterized by a conventional AML component admixed with epithelial cysts within an "ovarian-like" stroma. Mixed epithelial and stromal tumor (MEST) is another renal neoplasm featuring epithelial cysts and "ovarian-like" stroma. While there is consensus that in MEST the epithelial and stromal components are neoplastic, in AMLEC it has been hypothesized that the epithelial component may represent renal tubular entrapment or ovarian-like transdifferentiation of tumor cells. The aim of this study was to compare the immunophenotypes of the epithelial-stromal components of AMLEC and MEST, with normal kidney and ovary to provide additional insights into the pathogenesis and relationships of these entities. In this study, we analyzed eight cases of AMLEC and 14 cases of MEST from 2003 to 2023. We used tissue microarrays, full sections, or unstained slides with an immunohistochemical panel including renal and ovarian markers: SF1, ER, PR, AR, PAX8, WT1, GATA3, CA-IX, p16, inhibin A, and BCL2. We compared these cases with ten non-neoplastic ovary and kidney samples. Our findings indicate that the epithelial component of AMLEC and MEST resembleshormone receptor positive renal tubular epithelium (AR + /ER - /PR -). AMLEC's stromal component resembled hormone receptor positive renal stroma, while MEST's resembled ovarian stroma, supporting mullerian transdifferentiation. Our study showed that the epithelial and stromal components of AMLEC and MEST are immunophenotypically different and also differ from normal tissues. Our findings suggest that in AMLEC, the epithelial-stromal component represents a hormonally driven proliferation of non-neoplastic renal elements within a dysregulated tumor microenvironment.

Multilocular cystic nephroma in an adult: a diagnostic quandary.

Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological featuresthat causes diagnostic dilemma. MLCN lies in the spectrum of mixed epithelial and stromal tumor (MEST) family of kidney. According to World Health Organization (WHO 2016 classification), MEST encompasses spectrum of tumors ranging from predominantly cystic tumors, adult cystic nephroma (ACN) to tumors that are variably solid (MEST), thus creating diagnostic dilemma. Moreover, it has several benign and malignant differentials due to its several overlapping histomorphological features which when not cautiously dealt with may result in misdiagnosing it as malignant lesion. We hereby present a case of a woman in late twenties who presented with left flank swelling and pain since 6months which was misdiagnosed as renal cell carcinoma on radiology which turned out to be ACN on histology and further verified on immunohistochemistry.

Mixed Epithelial and Stromal Tumor (MEST): Case Report of An Unusual Presentation Causing Diagnostic Dilemma

ABSTRACT Mixed epithelial and stromal tumor (MEST) are rare tumors of kidney accounting to approximately 0.2 percent of all renal tumors. They have striking female predominance seen in peri-menopausal females. They present as clinically palpable masses hematuria with few being diagnosed incidentally. Most of them are benign with few cases reported with its malignant transformation. We present a case of 41-year-old female who came with the complaints of abnormal uterine bleeding since one and a half years, for which she was further evaluated. Radiology revealed a multiloculated solid-cystic mass lesion in the right kidney with bilateral ovarian cysts which prompted a clinical suspicion of renal neoplasm with metastasis. Right radical nephrectomy revealed a single capsulated solid-cystic tumor. Microscopy was diagnostic of mixed epithelial and stromal tumor (MEST). Due to its rarity, MEST is least suspected clinically. The benign nature of this tumor and its characteristic histopathology leads to accurate diagnosis and appropriate management of patients.

Locally Aggressive Mixed Epithelial and Stromal Tumour of Kidney Leading to Nephron Loss.

Mixed epithelial and stromal tumour (MEST) is a rare benign renal tumour. It is mainly found in perimenopausal women. We present a case of a 42-year female with no known comorbid who was presented in the outpatient clinic for the right flank pain. Contrast-enhanced CT scan revealed a complex renal cyst with internal septations. Considering a large symptomatic cyst and the presence of internal septations, she was planned for cyst excision. Peroperatively, significant disease progression and loss of renal parenchyma were noted contrary to preoperative scan. Histopathology of the specimen revealed MEST. We demonstrate that MEST of the kidney may have an aggressive local behaviour leading to nephron loss. Key Words: Mixed epithelial and stromal tumour, Kidney, Benign, Renal neoplasm.

A two-generation hyperparathyroidism-jaw tumor (HPT-JT) syndrome family: clinical presentations, pathological characteristics and genetic analysis: a case report

BackgroundHyperparathyroidism-Jaw Tumor (HPT-JT) is caused by inactivating germline mutations of CDC73. This hereditary disease can present with a range of symptoms. Jaw ossifying fibroma (OF) is one of the most important clinical presentations, affecting 30% of HPT-JT patients. However, OF is easily confused with other fibro-osseous lesions (FOLs) of the jaw. The correct diagnosis of HPT-JT is a real challenge and must be confirmed by genetic testing.Case presentationA female proband and her father suffered from multiple and recurrent FOLs in the jaw. Considering well demarcated margin and heterogeneous calcified substance lying in a variable density of fibrous stroma, we reached the diagnosis of jaw OF through radiologic and microscopic analyses. Additionally, the proband presented with chronic anemia resulting from menorrhagia, as well as renal mixed epithelial and stromal tumor (MEST). Two patients both presented with no evidence of Hyperparathyroidism (HPT). A germline start codon mutation (c.1A > G) of CDC73 was identified in them. Copy number loss at the CDC73 gene locus was verified in the jaw tumor sample of the proband.ConclusionRegardless of whether HPT manifestations are present, patients with heritable jaw OF may be at risk for HPT-JT. Genetic testing should be adopted to confirm the diagnosis. Early recognition of HPT-JT helps to better develop tailored treatment plans and surveillance programs.

Hidronefrotik fonksiyon göstermeyen böbrekli genç erkek olguda mikst epitelyal stromal tümör: Literatür bulguları ile karşılaştırma

The mixed epithelial and stromal tumor (MEST) family is comprised of adult cystic nephroma with a dominant cystic component and MEST with a dominant solid component. MEST is the rarely observed benign, biphasic tumor of the kidney with a mixed epithelial and stromal component. In the examinations of a 24-year-old male patient who presented with abdominal pain, grade-4 hydronephrosis was detected. In the microscopic examination of his nephrectomy specimen, there were tubular structures with cuboidal epithelium in different diameters within the ovarian stroma. The patient was diagnosed with MEST via morphological and immunohistochemical findings. We aimed at comparing the clinical and histopathological characteristics of our case, which we diagnosed with MEST, which is encountered in limited numbers in young men and has never been reported together with a nonfunctional kidney before, to the literature data. Keywords: Mixed epithelial and stromal tumor, nonfunctional kidney, male.

A case report of mixed epithelial and stromal tumor of the kidney

Mixed epithelial and stromal tumor (MEST) represents a recently described biphasic kidney neoplasm, which predominantly affects perimenopausal females. We describe case of a 35 year male patient with a MEST. Computed tomography (CT scan) showed an expansive lesion affecting the right kidney. Grossly, Greyish-white, a solid-cystic tumor was identified, which measured 3x 3 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 SMA (α-Smooth Muscle Actin) and CD10. The epithelial component exhibited a predominantly tubular pattern showing positive immunoreaction for PAX8. The diagnosis of MEST was then established.

Unusual Presentation of Mixed Epithelial and Stromal Tumor of Ureter Posing Diagnostic Dilemma

The mixed epithelial and stromal tumor (MEST) family comprises a spectrum of tumors varying from predominantly cystic tumors such as adult cystic nephroma, to variably solid tumors such as MESTs. These rare renal tumors typically affect women in their perimenopausal age. Most of these tumors are benign; however, at some rare occasions, they are associated with malignant transformation. The current article reports a rare case of MEST arising from the proximal ureter in a perimenopausal woman, manifested as a cord-like structure in the ureter, and posing the diagnostic dilemma.

A PATHOLOGIST'S EXPERIENCE WITH THE HEAVIEST RENAL TUMOR EXCISED TILL DATE

Mixed epithelial and stromal tumor (MEST) was described in 1973 by Block et al. It was introduced in WHO in 2002 and was defined as a complex renal neoplasm composed of a mixture of stromal and epithelial elements. Microscopically it shows abundant stroma that is thrown into broad papillae with microcysts embedded within. This gives it a phyllodes-like appearance. The tumor is benign. There are no reported recurrences of MEST. We here report the histological finding of the heaviest renal mass excised to date and emphasize on the role of careful histological examination to not miss out rare benign conditions like MEST.

A MEST up classification? Review of the re-classification of mixed epithelial and stromal tumor and adult cystic nephroma for the abdominal radiologist.

Mixed epithelial and stromal tumor (MEST) and the tumor formerly known as adult cystic nephroma (ACN) are uncommon renal tumors that have historically been described as separate entities in terms of histologic and imaging findings. However, these entities share many epidemiologic, radiologic, and pathologic features. While recent surgical and pathological literature has supported classifying MEST and ACN within the same tumor family, most radiologists and radiology texts continue to describe MEST and ACN as separate entities.

Renal mixed epithelial and stromal tumor (MEST) family: a clinicopathological study of 9 cases

目的： 探讨肾混合性上皮和间质肿瘤（MEST）家族的临床病理学特征。 方法： 对MEST家族中2例成人囊性肾瘤和7例MEST的临床资料、组织病理学、免疫组织化学进行分析。 结果： 9例患者中，围绝经期妇女5例，男性3例和孕妇1例。可双肾多发。肿瘤大体为囊实性结构。镜下呈上皮和间质的双相性病变，上皮细胞为立方或扁平上皮、柱状上皮，胞质透明或嗜酸性，核圆形、杆状或多形性，单层或假复层排列成巢团状、腺样、乳头状、簇状等，腔内可见嗜酸性分泌物；间质由伴有平滑肌分化的梭形细胞组成，类似卵巢样间质。上皮细胞细胞角蛋白、PAX8阳性，CD10阴性；间质细胞雌激素受体、孕激素受体、波形蛋白、CD10、肌动蛋白、结蛋白、平滑肌肌动蛋白可为阳性，HMB45、Melan A为阴性。 结论： MEST家族是一种具有形态学差异、与性别相关肿瘤家族，病变中存在正常的肾小球，对肿瘤发病机制有重要提示价值。.

Renal Cystic Nephroma: Histologic and Immunohistochemical Analyses and Review of Literature

Background: A case of renal Cystic Nephroma (CN) was reported in 32 years-old women presenting with an incidental renal mass on radiological imaging. Methods: On CT-scan examination, a renal cell carcinoma was suspected and right nephrectomy was performed. The resected tumor measured 17 × 10 × 7 cm. Macroscopically, the tumor was a multilocular mass with cysts ranging from 0.5 cm to 4.5 cm. The tumor was well delineated relative to non-tumoral renal parenchyma, which was physiological in appearance with a cortex at 1 cm without evidence of infiltration of the tumor. At the hilum, the arterial, venous and ureter were normal. No lymph node was identified. Microscopically, the lesion was well defined by a fibrous capsule, consisting of multiple cystic formations of variable sizes bordered by a mono-stratified epithelium, which ranged from flat to cubic. The lining epithelial cells had a hobnail appearance, abundant cytoplasm and an oval nucleus associated with a nucleolus. No mitosis were founded. The cysts were subtended by cellular fibrous septa of ovarian type. On immunohistochemical examination, the epithelial cells were positive for cytokeratins 7 and 19; stromal cells were positive for estrogen receptors, progesterone receptor and CD10. In the septa, there were some bundles of smooth muscle cells positive for desmin and smooth muscle actin. Conclusion: The patient was alive 8 years after treatment, without metastasis or recurrence. Histologic and immunohistochemical analyses may be helpful in differential diagnosis between benign cystic renal neoplasms, CN, mixed epithelial and stromal tumor (MEST) and malignant tumors such as clear cell renal carcinoma (CCRC) in adults.

Bilateral and multiple mixed epithelial and stromal tumors of the kidney: A case report.

Mixed epithelial and stromal tumor (MEST) of the kidney is a benign tumor occurring predominantly in older women. Histologically, MEST comprises tubuloglandular and cystic structures in an ovarian-like stroma. Bilateral and multiple cases are rare. We herein present a case of bilateral and multiple MEST in a 43-year-old woman. The patient presented with gross hematuria and was referred to a primary care physician. A computed tomography scan revealed 5 tumors in the right and 2 in the left kidney. Renal cell carcinoma was suspected, and left partial nephrectomy followed by right radical nephrectomy was planned. After the left partial nephrectomy, the histopathological diagnosis was MEST, which was consistent with the results obtained following needle biopsy. Therefore, the contralateral tumor was also suspected to be MEST, and needle biopsy was performed. The histopathological diagnosis was identical and the right kidney was spared. The patient is currently periodically monitored. As MEST is a benign tumor, preoperative diagnosis is crucial for avoiding overtreatment.

MP55-01 MIXED EPITHELIAL AND STROMAL TUMOR FAMILY: A SINGLE INSTITUTIONAL EXPERIENCE WITH THIS RARE RENAL ENTITY

You have accessJournal of UrologyKidney Cancer: Localized: Surgical Therapy III1 Apr 2017MP55-01 MIXED EPITHELIAL AND STROMAL TUMOR FAMILY: A SINGLE INSTITUTIONAL EXPERIENCE WITH THIS RARE RENAL ENTITY Mahyar Kashan, Mazyar Ghanaat, Maria Becerra, Andreas M. Hötker, Brandon Manley, Jozefina Casuscelli, Nicole Benfante, Shawn Mendonca, Satish Tickoo, Oguz Akin, Paul Russo, Jonathan Coleman, and A Ari Hakimi Mahyar KashanMahyar Kashan More articles by this author , Mazyar GhanaatMazyar Ghanaat More articles by this author , Maria BecerraMaria Becerra More articles by this author , Andreas M. HötkerAndreas M. Hötker More articles by this author , Brandon ManleyBrandon Manley More articles by this author , Jozefina CasuscelliJozefina Casuscelli More articles by this author , Nicole BenfanteNicole Benfante More articles by this author , Shawn MendoncaShawn Mendonca More articles by this author , Satish TickooSatish Tickoo More articles by this author , Oguz AkinOguz Akin More articles by this author , Paul RussoPaul Russo More articles by this author , Jonathan ColemanJonathan Coleman More articles by this author , and A Ari HakimiA Ari Hakimi More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2017.02.1694AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES In 2016 the World Health Organization coined the term mixed epithelial and stromal tumor family (MESTF) to encompass adult cystic nephroma (ACN) within the classification of mixed epithelial and stromal tumors (MESTs) on the basis of overlapping clinical and pathologic profiles. MESTs have traditionally been regarded as benign, with only a few reports of malignant transformation or recurrence in the literature. Diagnosis requires histopathological evaluation, as radiologic imaging cannot accurately determine whether such tumors are benign or malignant. We aim to characterize our institutional experience with this rare neoplasm. METHODS We identified all patients with a pathological diagnosis of MEST or ACN from our prospectively collected institutional database between Jan 1995 - Dec 2015. Available imaging was re-reviewed by a single expert radiologist (AH). Demographic, radiologic, and clinical characteristics were recorded. RESULTS Data was available for 40 patients. The median age at diagnosis was 48.8 years (31.5-73.4). Thirty-seven (92.5%) patients were female and three (7.5%) were male. Imaging was available for re-review for 29 (72.5%) patients. The mean diameter of the tumor on preoperative imaging was 5.8 cm (1.9-16.1). Patients presenting with symptoms at diagnosis had a mean diameter of 7.4 cm (4-16.1). On imaging, 25 (86.2%) tumors where characterized as Bosniak 3 lesions and four (13.8%) were described as Bosniak 4 lesions. All patients underwent surgical resection, with partial nephrectomy performed in 72.5% of cases. Mean pathological tumor size was 5.9 cm (1.5-15). Median follow-up was 71.6 months (1-217). Two (4.9%) patients died from non-tumor related causes. At last follow-up, all patients showed no evidence of disease. CONCLUSIONS MESTF are indolent tumors with a female predominance. They are usually detected incidentally as Bosniak 3 or 4 lesions. Partial nephrectomy should be performed whenever possible to resect the tumor and preserve renal function. Given the low likelihood of recurrence following excision, we believe that, once pathologically identified, patients have an excellent long-term prognosis and require minimal surveillance imaging on follow-up. Genomic characterization is currently underway. © 2017FiguresReferencesRelatedDetails Volume 197Issue 4SApril 2017Page: e730 Advertisement Copyright & Permissions© 2017MetricsAuthor Information Mahyar Kashan More articles by this author Mazyar Ghanaat More articles by this author Maria Becerra More articles by this author Andreas M. Hötker More articles by this author Brandon Manley More articles by this author Jozefina Casuscelli More articles by this author Nicole Benfante More articles by this author Shawn Mendonca More articles by this author Satish Tickoo More articles by this author Oguz Akin More articles by this author Paul Russo More articles by this author Jonathan Coleman More articles by this author A Ari Hakimi More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

52. Mixed epithelial and stromal tumour of kidney with prominent growth into pelvicaliceal system mimicking urothelial carcinoma

Mixed epithelial and stromal tumour (MEST) of the kidney is rare neoplasm with low malignant potential, most commonly found in middle-aged women who had long-term history of oestrogen replacement. With complete surgical excision of the tumour the outcome is usually favourable. Nonetheless a few cases of malignant transformation of the stromal component have been described in recent literature. The unusual case of MEST was encountered in our department with prominent vegetation into pelvicalyceal system suggestive papillary urothelial neoplasm, which was the preoperative diagnosis. However microscopic examination showed typical for MEST biphasic tumour with marked growth under reactive urothelium. Immunostains of the renal neoplasm showed positive staining of epithelial cells with CK7, AE1/AE3, PAX8, weak staining with CD10 and S100, Ki67 low labelling. It also revealed strong staining of Stromal cells with CD10, ER, PR and Smooth Muscle Actin with negative staining for MelanA, HBM 45, S100 and Ki67 low labelling. Histogenesis of mixed epithelial and stromal tumour of kidney remains questionable. It is believed that both components of tumour are neoplastic with possibly common cell of origin. As mentioned above, surgical excision is curable in most of the cases, however malignant transformation is possible and the definition of malignancy is problematic in this entity.

Renal mixed epithelial and stromal tumor: case report

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.

Mixed Epithelial and Stromal Tumor of the Kidney: A Rare Case Report

Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare kidney tumor that is composed of both epithelial and mesenchymal components with solid and cystic architecture. Up to the present, approximately 100 cases have been reported. It was introduced in the World Health Organization 2004 renal tumor classification. Here, we report a 49-year-old female with mixed epithelial and stromal tumor (MEST) in her kidney with no cystic components and no history of hormone use. J Med Cases. 2014;5(6):362-365 doi: http://dx.doi.o rg/10.14740/jmc1797 w

Expanding the Morphologic Spectrum of Adult Biphasic Renal Tumors—Mixed Epithelial and Stromal Tumor of the Kidney With Focal Papillary Renal Cell Carcinoma

Mixed epithelial and stromal tumor (MEST) is a distinctive adult biphasic neoplasm of the kidney characterized by the presence of solid and cystic areas composed of spindled stroma and epithelium lining tubules and cystic spaces respectively. Most MESTs are benign although sarcomatous transformation has rarely been reported. It has not been clearly established whether the epithelial component represents entrapped tubules or constitutes a true neoplastic component. We report an unusual case of a biphasic tumor of the kidney with a benign stroma and a focal component of papillary carcinoma arising in one of the cysts and discuss its pathogenesis.

Mixed epithelial and stromal tumor of the kidney (MEST) simulating

We present a rare and interesting case of a mixed epithelial andstromal tumour (MEST) of the kidney. The case is unique as itinvolves a male patient with no history of hormonal therapy presenting with a filling defect in the renal collecting system and positive urine cytology. The patient was diagnosed with transitional cell carcinoma of the renal pelvis and subjected to nephroureterectomy, which revealed a solid tumour arising from the lower calyces and extending into the renal pelvis and upper ureter. Pathology revealed a MEST. The patient was disease-free at the 6-month follow-up.

Mixed epithelial and stromal tumor of the kidney

Mixed epithelial and stromal tumor (MEST) of the renal pelvis or the older terminology cystic hamartoma of renal pelvis is a rare benign tumor and recently been recognized as a distinct clinical and pathological entity. These complex neoplasms are composed of a mixture of stromal and epithelial elements and are seen predominantly in women (ratio of women to men 6:1) and are generally seen in perimenopausal women with presenting symptoms of flank pain, hematuria or symptoms of urinary tract infection. We present here a case of MEST in a 48-year-old female who presented with a mass per abdomen associated with a mild dragging pain of 6 months duration. The ultrasonography report was of a multi-cystic lesion in the right kidney. Surgical excision of the lesion showed a mass measuring 18 × 14.5 × 7 cm with attached normal ureter. The outer surface was bosselated and showed multiple-cystic nodules with pale brown to dark brown smooth and glistening surface. The tumor was well circumscribed and there were multiple cysts of varying sizes separated by stroma arising from the dilated pelvicalyceal system. The wall of the cysts are covered by papillary excrescences. On histological examination, a well delineated tumor composed of cysts of varying sizes lined by columnar, cuboidal and transitional epithelium. Surrounding stroma showed a mixture of ovarian type of stroma and spindle shaped cells of smooth muscle type having plump nuclei. Sections from the renal-tissue adjacent to the tumor showed normal glomeruli and tubules.