The Mixed Epithelial and Stromal Tumor of the Kidney (MEST): a Case Report and Literature Review
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Case Report Clinics in Surgery Published: 15 Apr, 2021 The Mixed Epithelial and Stromal Tumor of the Kidney (MEST): A Case Report and Literature Review Chen Xu1, Ri-jin Song2, Min-hong Pan3, Min-Jun Jiang1* and Wei Zhang2* 1Department of Urology, The Ninth People’s Hospital of Suzhou City, China 2Department of Urology, The First Affiliated Hospital with Nanjing Medical University, China 3Department of Pathology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, China Abstract Purpose: To report a rare case of renal tumor and literature review. Methods: We reported a 55 years old female with a mass in her left retroperitoneum on color B-ultrasound. The Computed Tomography scan (CT-scan) revealed a mass of 6.5 centimeters lying on the upper pole of the left kidney with no invasion to the homolateral adrenal gland. A partial nephrectomy through a retroperitoneal laparoscopic approach was performed. Results: The patient had a quick and perfect recovery later, with a Length of Stay (LOS) of 72 h. The final pathological diagnosis was Mixed Epithelial and Stromal Tumor of the kidney (MEST). Five years follow-up showed no recurrence and metastasis. Conclusion: Mixed epithelial and stromal tumor is a rare tumor of the kidney and usually have a benign behavior. It has a distinct female preponderance. Surgical intervention is a preferred therapy. Keywords: Renal Tumor; MEST; Kidney OPEN ACCESS Introduction *Correspondence: The Mixed Epithelial and Stromal Tumor of the kidney (MEST) is a rare, typically benign tumor Wei Zhang, Department of Urology, that almost exclusively occurs in perimenopausal women [1-2]. It was first introduced by Michael The First Affiliated Hospital with and Syucek in 1998 and about a hundred cases have been reported in the literature [3-6]. Here, we Nanjing Medical University, Nanjing report a case of MEST and a review of literature. 210029, China, Case Presentation E-mail: [email protected] Min-Jun Jiang, Department of Urology, A 55 years old female was found with a mass in her left retroperitoneum on color B-ultrasound The Ninth people’s Hospital of Suzhou during routine checkup. The ultrasound revealed a hypoechoic tumor-like image between spleen and the left kidney. The ultrasound findings was further evaluated by a plain and enhanced CT-scan City, Suzhou 215200, China, (Figure 1), which also revealed a mass of 6.5 centimeter lying on the upper pole of the left kidney E-mail: [email protected] with no invasion to the homolateral adrenal gland. It was difficult to recognize the relationship Received Date: 23 Feb 2021 between the mass and the homolateral kidney due to infiltrative borders and we suggested the mass Accepted Date: 05 Apr 2021 as an untypically renal cell carcinoma. Therefore, a partial nephrectomy through a retroperitoneal Published Date: 15 Apr 2021 laparoscopic approach was performed. Citation: During the operation, we found the mass lying on the upper pole of the left kidney, outside the Xu C, Song R-J, Pan M-H, Jiang M-J, left adrenal gland. The capsule of the mass was intact and possibly a small base was found in the Zhang W. The Mixed Epithelial and homolateral kidney (Figure 2). The whole operative time was 70 min and the renal artery had not Stromal Tumor of the Kidney (MEST): been clamped. The operation was performed without any incidents and the patient had a quick and A Case Report and Literature Review. perfect recovery later with a Length of Stay (LOS) of 72 h. Clin Surg. 2021; 6: 3132. Pathological examination of the surgical specimen revealed a yellow well-circumscribed tumor Copyright © 2021 Min-Jun Jiang and macroscopically, with rounded contours, measuring 12 cm × 7 cm × 5 cm (Figure 3a). After the Wei Zhang. This is an open access specimen was longitudinally cut open, we discovered the tumor was composed of whitish, fish-like article distributed under the Creative tissue, with some vaguely nodules (Figure 3b). Commons Attribution License, which permits unrestricted use, distribution, Microscopically, the tumor consisted of epithelial and stromal component. The epithelial and reproduction in any medium, elements, composed of clusters of tubules with variable lining, are found scattered within the spindle provided the original work is properly cell stroma. Focally, large cysts display a lining made up of cells with abundant eosinophilic cytoplasm cited. uniformly in benign fashion. In some areas, tubules lined by bland-looking cells, containing luminal Remedy Publications LLC., | http://clinicsinsurgery.com/ 1 2021 | Volume 6 | Article 3132 Min-Jun Jiang and Wei Zhang, et al., Clinics in Surgery - Urology Figure 1: Computed Tomography Scan (A: Plain/B: Enhanced). Figure 4 (A): The tubules are lined by single-layered cuboidal cells, and stroma show leiomyoma like interlacing fascicles consisting of spindly cells with eosinophilic cytoplasm, some tubules contain luminal pink secretion (hematoxylin-eosin H and E, original magnification x100). (B): Immunohistochemical study demonstrated strong positivity for pancytokeratin in the epithelial cells (original magnification x200). Figure 2: The capsule of the tumor was intact and a small base was found in the left kidney (A). A retroperitoneal laparoscopic partial nephrectomy was performed perfectly (B). Figure 5: Immunohistochemical study demonstrated strong positivity for desmin in the stromal cells (original magnification x200). Figure 3 (A): Macroscopical Examination (B): Open Examination of the tumor. Discussion Mixed epithelial and stromal tumor of the kidney is a recently pink secretion, were also noted. The stromal component ranges in recognized distinct neoplasm that imaging studies are not diagnostic appearance from scar like fibrous tissue to leiomyoma like interlacing but reveal a solid or solid and cystic mass in most cases [2]. It had fascicles (Figure 4a). Both the lining epithelial cells and stromal cells been previously described as cystic hamartoma of the renal pelvis were relatively uniform and showed no significant cytologic atypia [7-8], adult type mesoblastic nephroma [9-11], cystic partially or mitosis. Immunohistochemically, the epithelial cells of cysts were differentiated nephroblastoma and cystic nephroma or mature positive for pancytokeratin, cytokeratin 7, cytokeratin 19, PAX2 but nephroblastic nephroma [12-14]. Patients with MEST range from 19 negative for cytokeratin 20, HMB45, and S100 protein, and spindle to 78 years old with a distinct female preponderance (male to female cells were positive for Smooth Muscle Actin (SMA), desmin, vimentin, ratio 1:6) [2]. The most common size of MEST is between 2 cm to 24 Estrogen Receptor (ER) and Progesterone Receptor (PR) (Figure 4b, cm, while Antic et al. [15] reported in an 8 cases series that the mean 5). The final histopathological diagnosis was Mixed Epithelial and size was 2.9 cm ranging from 0.5 cm to 10 cm. Although the etiology Stromal Tumor of the kidney (MEST). is still unknown, it is thought a possible hormonal pathogenetic mechanism involved in the process of MEST. Rare cases of men and Although malignant transformation, recurrence and metastasis pediatric patient were found in the overall reported cases of MEST in was rare for the natural history of MEST, the patient was originally the literatures [4-6,16,17]. placed on a strict follow-up schedule. At the scheduled 5-years follow-up visit, the patient was doing well and the follow-up CT scan Hormonal imbalance caused by long-term estrogen replacement was negative for recurrence or any other signs of malignancy. therapy for female perimenopausal patients and hormone therapy Remedy Publications LLC., | http://clinicsinsurgery.com/ 2 2021 | Volume 6 | Article 3132 Min-Jun Jiang and Wei Zhang, et al., Clinics in Surgery - Urology for men could be responsible for MEST. The change of hormone own larger cysts, thinner septa and lower prevalence of stromal to level could induce proliferation of ectopic or fetal mesenchymal cells epithelial ratio [2,18,26]. located in the kidney, with the ability to differentiate into epithelial (2) Congenital mesoblastic nephroma. It is generally a solid mass and stromal cells [18]. As the presence of estrogens and progesterone composed of spindle stromal cell elements with entrapped renal receptors in the MEST, S cells. However, in our case, as in the cases tubules and always involve the renal parenchyma. Recent study by reported by Tian and Minoda, patients with MEST did not have a Pierson et al. indicated that MEST lacks the genetic alteration of history of hormone therapy, no evidence of a hormonal receptor cellular congenital mesoblastic nephroma [22]. expression could be found, either [19-20]. Therefore, we suggest not all MEST cases implicate a hormonal mechanism. (3) Primary renal synovial sarcoma. On one hand, this tumor is characterized by gross or microscopic cysts and tubules lined by Clinically, patients with MEST usually present with a palpable hobnail epithelium and immunoreactivity for cytokeratin in the abdominal or flank mass, flank pain while it can also be found with epithelial element, which is similar to malignant MEST [27-28]. micro or gross hematuria or with symptoms suggestive of upper However, ovarian-like stroma cannot be observed in renal synovial urinary infection [2,20]. Recent studies showed most cases were sarcoma. On the other hand, strong immunohistochemical positivity found incidentally under the guidance of imaging studies, including for expression of estrogen receptor could be detected in MEST while our case [3,6,21,22]. Most cases were unilateral, while bilateral and it cannot be seen in synovial sarcoma [29]. multiple cases were rarely reported [20]. (4) Multicystic renal cell carcinoma. MEST lacks of the aggregates Macroscopically, the tumor is classically characterized by solid of clear cells when differentiated with multicystic renal cell carcinoma. and cystic components. It is common to see the tumor involve the renal hilum and compress the pelvicaliceal system [2]. In some cases, In most cases, MEST have a benign behavior.