Adult Cystic Nephroma Research Articles

Смешанная эпителиальная и стромальная опухоль в сочетании с онкоцитомой почки: редкий клинический случай и обзор литературы.

Introduction. Mixed epithelial and stromal tumors (MEST) of the kidney are rare neoplasms and, along with adult cystic nephroma, are grouped together called mixed epithelial and stromal tumors (World Health Organization Classification of Tumors of the Kidney, 2022). Clinically and radiologically, they mimic other cystic renal neoplasms, thus definitive diagnosis requires histological and immunohistochemical examination. Despite the benign course, MEST can undergo malignancy; isolated cases of recurrence have also been documented. Clinical case. Patient X, 65 years old, underwent robot-assisted left-side partial nephrectomy for cystic formations of the left kidney with a hypervascular soft tissue component along the medial semicircle of one of the cysts. According to histological examination, MEST of the left kidney in combination with oncocytoma was diagnosed. Conclusions. This article presents probably the first published case of MEST in combination with oncocytoma of the kidney.

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.

Mixed Epithelial and Stromal Tumor Family of Kidney (Adult Cystic Nephroma, Mixed Epithelial and Stromal Tumor): Retrospective Clinicopathological Evaluation.

Tumors belonging to the mixed epithelial stromal tumor family (MESTF) are rare; thus clinicopathological experience about them are limited. Each epithelial and stromal component shows different patterns in these tumors. Clinicopathological features of 11 MESTF cases that were diagnosed between 2000 and 2021 at a single center were evaluated. Ten of the 11 patients were female (F:M = 10:1). The mean age of the females was 47 (31-63) years; the male patient was 45 years old. The mean tumor diameter was 6.7 (3.5-19) cm. All tumors had varying proportions of cystic and solid components. Eight cases were well circumscribed, and the others had distinct but irregular borders. Two of the tumors with irregular borders were bulging into the renal sinus. The epithelial component was dominant in most cases. In the epithelial component, macrocyst, microcyst, and tubules were the most common patterns and the most common types of lining epithelium were flat, cuboidal and hobnail. The stromal component was variable in most cases and included hypocellular (mostly collagenous) and cellular areas. In most cases, the cellular stroma had an ovarian-like appearance. Among the other features observed, hyalinization and dystrophic calcification were common. The positivity for estrogen and progesterone receptor in the stromal component was observed in almost all female cases. MESTF, which has distinctive features, should be considered in the differential diagnosis of cystic kidney tumors.

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 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.

Tubulocystic renal cell carcinoma: a review of literature focused on radiological findings for differential diagnosis.

Tubulocystic renal cell carcinoma (TC-RCC) has been classified as an independent subtype according to the 2016 World Health Organization (WHO) classification. It is a rare subtype that predominantly affects men. Although few in number, radiological imaging reports have suggested that TC-RCC is characterized by multilocular cystic lesions, which are categorized as the Bosniak classification II-IV, with signature pathological characteristics comprising numerous small cysts or a tubular structure. The Bosniak classification system facilitates patient management; however, the differentiation of cystic tumors exhibiting similar imaging findings remains impossible; in fact, the differentiation of multilocular cystic RCC, adult cystic nephroma, and mixed epithelial and stromal tumor remains challenging. This review aims to discuss TC-RCC with a focus on implications of radiological findings in the differential diagnosis of TC-RCC.

Ultrasound Imaging of Cystic Nephroma.

Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term “cystic nephroma” represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections. In this review, we summarize the ultrasound imaging features of cystic nephroma and describe the characteristics of the most common renal cystic lesions and the differential diagnosis of cystic nephroma with other renal cystic lesions.

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 ...

Mixed Epithelial and Stromal Tumor of the Kidney: Radiologic-Pathologic Correlation

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare, typically benign lesion that occurs predominantly in perimenopausal women. At computed tomography (CT), it typically manifests as a multiloculated cystic renal mass with a variable proportion of solid and cystic components and containing internal septa that demonstrate heterogeneous and delayed contrast material enhancement. MEST may mimic a variety of benign and malignant renal lesions, such as adult cystic nephroma, complex renal cyst, and cystic renal cell carcinoma. The preoperative diagnosis of MEST can be problematic, and most cases are treated surgically. However, CT with two-dimensional multiplanar reformation and three-dimensional volume rendering helps define the diagnostic features of MEST and can assist in surgical planning.

Adult Cystic Nephroma and Mixed Epithelial and Stromal Tumor of the Kidney Are the Same Disease Entity: Molecular and Histologic Evidence

Adult Cystic Nephroma and Mixed Epithelial and Stromal Tumor of the Kidney Are the Same Disease Entity: Molecular and Histologic Evidence

Clinicopathologic analysis of mixed epithelial and stromal tumor of kidney and adult cystic nephroma

To study the clinicopathologic features, immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney (MEST) and adult cystic nephroma (CN). Five cases of MEST and 4 cases of CN were retrospectively analyzed. Immunohistochemical study was carried out and the literature was reviewed. All of the five patients with MEST were females. Their median age was 45 years. For CN, there were 3 males and 1 female and their median age was 41 years. All patients presented with loin pain and hematuria. On gross examination, MEST was well-circumscribed but non-encapsulated. There was no evidence of haemorrhage or necrosis. Three of the cases were solid in nature. One was composed of a mixture of solid and cystic elements, while the remaining case showed a multicystic cut surface bridged by thick fibrous septa. On the other hand, CN were well-circumscribed and encapsulated. They were multiloculated cystic in nature. The cystic spaces were separated by thin septa and there was no significant solid or necrotic component. Histologically, MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns. Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified. The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium. In contrast, the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen. The spindle cells in MEST expressed vimentin (5/5), smooth muscle actin (3/5), desmin (4/5), CD10 (5/5), estrogen receptor (4/5) and progesterone receptor (4/5). They were negative for HMB45, CD34, CD117 and S-100 protein. On the other hand, the spindle cells in CN were variably positive for vimentin (4/4), smooth muscle actin (4/4), desmin (1/4), estrogen receptor (3/4) and progesterone receptor (1/4). They were negative for CD10, HMB45, CD34, CD117 and S-100 protein. Both MEST and CN are uncommon renal neoplasm. Most of them run a benign clinical course. The stromal cells in MEST show smooth muscle or myofibroblastic differentiation. Areas demonstrating Müllerian features also existed in some cases. MEST and CN share overlapping histological and immunohistochemical features, and may represent spectrum of the same group of lesions.

Adult Cystic Nephroma and Mixed Epithelial and Stromal Tumor of the Kidney Are the Same Disease Entity

Adult cystic nephroma (CN) and mixed epithelial and stromal tumor of the kidney (MEST) are considered as separate entities in the 2004 World Health Organization classification of renal neoplasms. Recent studies suggested that the two share clinicopathologic features and may represent the same disease process of varying morphology. However, definitive genetic evidence is lacking. We examined their relationship using gene expression profiling and histologic analysis. Gene expression profiles of 3 CN and 3 MEST were analyzed using HGU133 Plus 2.0 microarrays (Affymetrix) and were compared with each other and also with 48 other renal tumors and 13 normal kidneys. Histologic examination of 26 CN and 13 MEST focused on the cystic septal thickness, cyst-to-stroma ratio, stromal cellularity and composition, types of epithelial cells lining cysts and glands, and estrogen and progesterone receptors expression. Patients' age, sex distribution, and tumor size were similar between the two. They also shared many histologic features, including lining epithelium of cysts and glands, stromal cellularity and composition. Unsupervised clustering of mRNA expression profiles demonstrated that they had very similar expression profiles that were distinct from other renal tumors. By microarray analysis, progesterone receptor expression was significantly higher in CN and MEST relative to both normal and other renal tumors, while estrogen receptor expression was not. By immunohistochemistry, expression of both receptors was similar between CN and MEST. This study provides the most convincing molecular evidence that CN and MEST represent different parts of the morphologic spectrum of the same disease.

Adult cystic nephroma: A case report and a review of the literature

A 23-year old women who underwent radical nephrectomy due to right renal mass is presented. The histopathological examination is reported as adult cystic nephroma, a rare benign lesion of the kidney. The epidemiology, differential diagnosis, histopathological features, and treatment alternatives are discussed and the literature is reviewed.

Adult Cystic Nephroma and Mixed Epithelial and Stromal Tumor of the Kidney: Clinical, Radiographic, and Pathologic Characteristics

Adult cystic nephroma (CN) and mixed epithelial and stromal tumor (MEST) are benign renal tumors readily distinguished from cystic renal cell carcinoma and other malignant variants based on histopathology. Clinical and radiographic data regarding these lesions are sparse, especially with respect to factors providing clinical suspicion for CN or MEST. Pathology of 22 CN (21 patients) and 10 MEST (9 patients) treated between 1987 and 2005 was re-reviewed according to 2004 WHO classification. Nineteen CN patients (90%) and 9 MEST patients (100%) were female and median ages were 55 (range: 39 to 79) and 52 (range: 39 to 67) years, respectively. Twenty-two patients (73%) presented symptomatically. CN were commonly Bosniak III lesions (77%), whereas 70% of MEST had solid enhancing components. An intrapelvic component was present in 5 CN (23%) and no MEST. Preoperative radiologic suspicion was documented in 36% of CN, but in only 1 patient with MEST. Nephron-sparing surgery was performed for 16 tumors (50%), including 5 suspected preoperatively and 3 that were 7 cm or greater. There was 1 case each of bilateral CN and MEST. Two patients with CN had concomitant renal cell carcinoma. One patient with MEST had sarcomatous component. At mean follow-up of 4.5 years (range: 1 to 18), no patient had novel or recurrent tumors develop. CN and MEST typically present symptomatically in perimenopausal women. Pedunculation of a multiloculated cystic lesion may allow for preoperative suspicion of these lesions in the proper clinical setting. Nephron-sparing surgery is the preferred treatment even for large and/or central lesions, if amenable.

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

Mixed epithelial stromal tumor of the kidney (MEST)/adult cystic nephroma (CN) is a lesion characterized by epithelial lined tubular or cystic structures interspersed within a variably prominent, distinctive spindle-cell stroma. Although typically benign, cases with malignant features have been reported. Herein, we report a MEST/CN with malignant stromal features and rhabdoid differentiation arising in the left kidney of an 84-year-old woman. Histologically, the tumor displayed multiple tubules and variably sized cystic structures lined by benign epithelium with an intervening malignant-appearing spindle-cell stroma. The malignant stroma displayed condensation in the regions surrounding the epithelial component consistent with the ovarian-like stroma typically observed in MEST/CN. In addition, the stromal cells displayed extensive rhabdoid differentiation. Immunohistochemical analysis revealed strong expression of cytokeratin 7, CAM 5.2, AE1/AE3, wide-spectrum keratin, and epithelial membrane antigen by the epithelial component. The stromal component displayed strong immunohistochemical expression of WT-1, CD-99, CD-56, INI1, and estrogen receptor; focal actin positivity; and was negative for desmin, myogenin, and progesterone receptor. Analysis by reverse transcriptase polymerase chain reaction failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma. To our knowledge, this represents the first report in the literature of malignant MEST with rhabdoid features and suggests that this entity should be considered in the diagnosis of renal stromal malignancies with prominent rhabdoid features.

Cystic nephroma (multilocular cyst) and mixed epithelial and stromal tumor of the kidney: a spectrum of the same entity?

The recently described mixed epithelial and stromal tumor (MEST) of the kidney and adult cystic nephroma (CN) (multilocular cyst) are rare benign cystic renal neoplasms that are composed of epithelial and stromal elements. Consensus criteria for distinguishing these entities have not been well established. Our objective in this study was to evaluate cases of CN and MEST to define the morphological, immunophenotypic, and clinical features of these two entities. Eleven cases from the files of a single institution diagnosed from 1996 to the present as either CN or MEST were reviewed. Architecturally, all lesions were composed of multiple noncommunicating cysts lined by a single layer of epithelial cells. All cases had areas with increased stromal cellularity and 8 cases had ovarian-like stroma present at least focally within the tumor. No stromal or epithelial cell atypia, blastemal elements, or increased mitotic activity was appreciated. The immunoprofile was also similar in the 7 cases stained and included epithelial reactivity with keratin and CAM 5.2 and stromal reactivity with estrogen receptor, progesterone receptor, smooth muscle actin, WT-1, vimentin, and focal desmin. All cases have acted in a benign fashion with no history of recurrence or metastasis. We propose that CN and MEST of the kidney represent a spectrum of the same entity. If the diagnosis of CN is limited to cases that are comprised entirely of thin fibrous-walled cysts, all 11 of our cases would be classified as MEST.