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Objective: To study the long-term clinical effect of multicenter multidisciplinary treatment (MDT) in children with renal malignant tumors., Methods: A retrospective analysis was performed on the medical data of 55 children with renal malignant tumors who were diagnosed and treated with MDT in 3 hospitals in Hunan Province from January 2015 to January 2020, with GD-WT-2010 and CCCG-WT-2016 for treatment regimens. A Kaplan-Meier survival analysis was used to analyze the survival of the children., Results: Of the 55 children, 10 had stage I tumor, 14 had stage Ⅱ tumor, 22 had stage Ⅲ tumor, 7 had stage IV tumor, and 2 had stage V tumor. As for pathological type, 47 had FH type and 8 had UFH type. All children underwent complete tumor resection. Of the 55 children, 14 (25%) received preoperative chemotherapy. All children, except 1 child with renal cell carcinoma, received postoperative chemotherapy. Among the 31 children with indication for radiotherapy, 21 (68%) received postoperative radiotherapy. One child died of postoperative metastasis. The incidence rate of FH-type myelosuppression was 94.4%, and the incidence rate of UFH-type myelosuppression was 100%. The median follow-up time was 21 months and the median survival time was 26 months for all children, with an overall survival rate of 98% and an event-free survival rate of 95%., Conclusions: Multicenter MDT has the advantages of high success rate of operation and good therapeutic effect of chemotherapy in the treatment of children with renal malignant tumors, with myelosuppression as the most common side effects, and radiotherapy is safe and effective with few adverse events. Therefore, MDT has good feasibility, safety, and economy.

Objective: To investigate the clinicopathological characteristics and molecular genetics of atypical renal cysts. Methods: Six cases of atypical renal cysts were collected from Zhejiang Provincial People's Hospital, Hangzhou, China, between February 2014 and February 2019. The clinicopathological characteristics and disease progression were analyzed. The 3p deletion and trisomy of chromosomes 7 and 17 were detected using fluorescence in situ hybridization (FISH). Results: All of the 6 patients were male, aged 43-63 years (median: 52 years). Preoperative Bosniak classification showed 4 cases of grade Ⅱ, 1 case of grade Ⅰ and 1 of grade Ⅲ. Histologically, atypical renal cysts appeared as unilocular or multilocular cysts, lined by multilayered flattened or cuboidal-shaped clear or eosinophilic cells. They often showed short papillary projections, and lacked solid or nodular growth of the lesional cells within the wall or septa of the cysts. Histologically, these cysts could be classified into three categories: acquired cystic disease-associated renal cell carcinoma (ACKD-RCC)-like (3 cases), clear cell type (2 cases), and eosinophilic papillary type (1 case). Two cases of ACKD-RCC-like atypical renal cysts were accompanied by clear cell renal cell carcinomas. On immunohistochemical staining, ACKD-RCC-like atypical renal cysts were focally CK7+/AMACR+/CD57+, the clear-cell type atypical renal cysts were CK7+/CAⅨ+, and eosinophilic papillary type atypical renal cysts were CK7+/AMACR+. FISH analyses showed that one case of ACKD-RCC-like atypical renal cysts had trisomy 17 and one case of clear cell type had 3p deletion, while no signal abnormality was detected in the other cases. The six patients were followed up for 13 to 70 months (median: 27 months), and no evidence of renal cell carcinoma was noted. Conclusion: Atypical renal cysts are a group of lesions that are heterogeneous in clinical, histological and immunophenotypical and molecular genetic features. FISH analyses suggest that a subset of the cases may be precursors of currently known renal cell carcinomas. Extensively sampling and careful observation of the histological characteristics of the cyst wall are important for distinguishing atypical renal cysts from extensively cystic renal cell carcinomas.

Objective: To investigate the clinicopathological features,diagnosis and differential diagnosis of renal angiomyolipoma with epithelial cysts(AMLEC). Methods: Four cases of renal AMLEC diagnosed between January 2014 and June 2019 at the Department of Pathology,Zhejiang Provincial People's Hospital were subjected to clinicopathological, histological and immunohistochemistry analyses along with a literature review. Results: All the four patients were females and aged from 19 to 52 years (mean 34.5 years). Three cases were accidentally discovered by physical examination, and the medical history was 1 to 6 years. The preoperative imaging Bosniak classification was grade Ⅲ in 3 and grade Ⅳ in 1 case. The maximum diameter of the tumor ranged from 2.5 to 9.0 cm (average 5.0 cm). Histologically, all of the 4 tumors showed three histological components: (1) simple epithelial cysts lined by a layer of cuboidal/low-columnar to occasionally, hobnailcells; (2) a thin, compact subepithelial "cambium-like" layer of cellular, mullerian-like short spindle cell stromas with prominent admixedchronic inflammation; (3) a outermost layer of thick, long-fascicles of smooth muscle-like stromas, often surrounded by dysplastic, tortuous thick-walled blood vessels. There was often a prominent lymphatic channel network in the smooth muscle component forming slit like branched and curvilinear spaces. None of the 4 tumors had fat content.Immunohistochemically, the epithelial cells lining the cysts strongly expressed PAX8 and CK7. The subepithelial "cambium-like" stromas strongly expressed melanocytic markers (HMB45, Melan A, Cathspin K, MiTF) and mullerian markers (ER,PR,CD10), and were negative for smooth-muscle markers(SMA,desmin,calponin). The outermost layer of smooth muscle-like stromas strongly expressed smooth-muscle markers, and were only focally positive for melanocytic and mullerian markers. Follow-up information was obtained in 3 cases, among which no evidence of tumor recurrence or metastasis was found at 3, 5, and 66 months of follow-up, respectively. Conclusions: Renal AMLEC is a rare histological subtype of angiomyolipoma with benign biological behavior, and has characteristic histological and immunophenotypic characteristics.Pathologists should be familiar with the clinicopathological appearances of AMLEC and include it in the differential diagnostic spectrums of renal tumors with biphasic epithelial and mesenchymal features.

Objective: To investigate the clinicopathologic features, diagnostic and differential diagnostic aspects of pigmented microcystic chromophobe renal cell carcinoma (ChRCC). Methods: Five cases of pigmented microcystic ChRCC were collected at Zhejiang Provincial People's Hospital from January 2013 to January 2018. The clinical features, gross and histological appearances, immunohistochemistry and prognosis were analyzed and the relevant literature was reviewed. Results: There were 3 men and 2 women with age range of 45 years to 72 years (mean 57 years). All tumors were incidentally identified by imaging examinations. Grossly, the tumors were well-demarcated and showed diameters ranging from 1.8 cm to 4.0 cm(mean 2.9 cm). On cross section, the tumors were brown to gray tan with solid cut-surface mixed with multiple cysts of variable sizes. Hemorrhage was common, central scar was not seen. Microscopically, the tumors were composed predominantly of irregular and variable-sized microcystic or tubulocystic patterns, with extensive cribriform structures formation and focal adenomatous rearrangements seen in one case each, and focal pseudo-papillary structures (lacking true fibro-vascular cores) seen in two cases. Microscopic calcifications and psammoma bodies were present in all tumors. Four tumors composed mostly of eosinophilic cells whereas 1 predominated in plant-like cells. Brown pigmentations, either intracytoplasmic or extracytoplasmic, were noted in all five cases. The tumor cells had irregular, low-grade nuclei (Paner grade: 1) frequently with binucleation and perinuclar halos. Tumor necrosis or sarcomatous transformation was not seen. By immunohistochemistry, the tumor cells expressed CK, EMA, and E-cadherin diffusely and strongly in five cases; and CK7 and CD117 diffusely in four cases. They were negative for vimentin, CD10, CA9, AMACR/P504s, TFE3, HMB45, Melan A, S-100 protein, synaptophysin and chromogranin. Partial nephrectomies were performed for all five patients; there was no tumor recurrences or metastases at a follow-up of 2 to 55 months (mean, 17 months). Conclusions: Pigmented microcystic ChRCC is a rare histological variant of ChRCC with relatively indolent behavior, and shows morphologic heterogeneity which can elicit a wide range of differential diagnoses. Careful attentions to search for typical features of classic ChRCC with the use of immunohistochemistry can help to distinguish this tumor from its many mimickers.

Objective: To investigate the clinicopathologic and differential diagnostic features of glomus tumor of the kidney. Methods: Four cases of glomus tumor of the kidney were collected from the archives of Peking University Third Hospital, the Second Hospital of Tianjin Medical University, Ningbo Yinzhou Second Hospital and Zhejiang Provincial People's Hospital between January 2012 to June 2017; the clinical and radiologic features, histomorphology, immunohistochemistry, ultrastucture and prognosis were analyzed and the relevant literature was reviewed. Results: Patients consisted of 2 men and 2 women with ages ranging from 37 years to 66 years (mean 55 years). Three patients had history of hypertensive disease (grade Ⅱ, 3 to 10 years). The tumors measured in maximum diameter from 3.0 cm to 4.0 cm (mean 3.6 cm) and showed gray-white to yellow and tan on cut surface. Macroscopical examinations showed all tumors were circumscribed but non-encapsulated. Histologically, 1 tumor presented as glomus tumor with extensive myxoid change, 1 as cellular and solid pattern glomus tumor, 1 as glomangioma with focal myopericytoma-like pattern and 1 as symplastic glomus tumor with areas resembling myopericytoma. The tumor cells in two cases showed scant cytoplasm and uniform, bland-appearing nuclei without mitoses. In one case, the tumor cells were epithelioid with abundant eosinophilic cytoplasm and relatively well-defined cell borders. There was an increased mitosis of 4/50 HPF; however, no evidence of atypical mitosis or nuclear atypia was noted. In the symplastic glomus tumor the tumor cells showed frequently nuclear pleomorphism without mitoses. By immunohistochemistry, all tumors showed strong and diffuse reactivities to at least 3 of the 4 muscle-associated markers (SMA, h-Caldesmon, MSA and Calponin), 3 tumors strongly and diffusely expressed collagen Ⅳ, 2 expressed CD34 and 1 focally expressed desmin; whereas markers including epithelial, neuroendocrine, nephrogenic, melanoma-associated, STAT6, S-100 protein, CD117 and β-catenin all were negative in all the 4 tumors. Ultrastuctural analysis was done in 2 cases and showed prominent cytoplasmic actin bundles and pericellular basement membrane, and lacking of rhomboid renin crystals in both tumors. The hypertension persisted after surgical resection for all the 3 patients with this medical history. Follow-up information (range: 6-64 months, mean: 44 months)showed that no evidence of local recurrence or distant metastasis was identified in all 4 patients. Conclusions: Glomus tumor rarely occurs in the kidney and usually has a good prognosis. Careful attention to its morphology with the judicious use of immunohistochemistry and ultrastuctural analysis can be helpful for its diagnosis and differential diagnosis.

Objective: To investigate the clinical and histopathologic characteristics, diagnosis, differential diagnosis and prognostic features of anastomosing hemangioma. Methods: Five cases of anastomosing hemangioma of the kidney and adrenal gland were collected, the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed with review of literature. Results: Three patients were male and two were female with ages ranging from 47 to 77 years; three were located in adrenal gland and 2 originated from the kidney. Clinically, 4 tumors were incidentally identified, 1 presented as edema of lower extremity. By radiography, all presented as a well-demarcated, oval, solid and low-density mass. Grossly, the tumors ranged in maximum diameter from 1.6 to 2.5 cm (mean 2.1 cm). Microscopically, the tumors consisted of anastomosing sinusoidal capillary-sized vessels lined by a single layer of flattened, cubical to hobnail endothelial cells, setting in an pauci-cellular stroma of edematous and hyaline changes. Other commonly seen features included vaguely lobular growth pattern (3/5), hemorrhage and thrombosis (5/5), intravascular growth pattern (5/5), eosinophilic intracytoplasmic hyaline globules (1/5) and extramedullary hematopoiesis (3/5). The tumor cells were typically bland-appearing and mitoses were scarce, with 1 case demonstrating cellular foci of tumor with slight pleomorphism and increased mitoses (2/50 HPF). Immunohistochemical studies showed the tumor cells expressed endothelial cells markers. Follow-up information was available for all 5 patients and showed no evidence of tumor recurrence or metastasis within 6 to 52 months (mean 30 months). Conclusions: Anastomosing hemangioma is a rare, benign subtype of capillary hemangioma that predominantly affects the urologic organs and adrenal glands; it is needed to distinguish it histologically from a series of benign or malignant tumors that feature a richly vascular stroma. Careful attentions to its characteristic morphology with the judicious use of immunohistochemistry can help distinguish this tumor from its many mimickers.

Mucinous tubular and spindle cell renal cell carcinoma is a rare, recently described variant of renal cell carcinoma characterized by an admixture of cuboidal cells in tubules and sheets of spindle cells, and variable amounts of mucinous stroma. It has been recognized as a distinct entity in the 2004 World Health Organization tumor classification. Since then, several dozen of these tumor have been reported with additional complementary morphologic characteristics, immunohistochemical profile, and molecular genetic features that have further clarified its clinicopathologic aspects. Although originally considered as a low grade renal cell carcinoma on the basis of its bland appearing nuclear features and indolent clinical course, mucinous tubular and spindle cell renal cell carcinoma has currently been proven to be a tumor that has a histological spectrum ranging from low to high grade that includes sarcomatoid differentiation. In this review, we present a detailed summary of the current knowledge regarding the clinicopathologic, immunohistochemical, molecular genetic, and prognostic characteristics, as well as differential diagnoses of mucinous tubular and spindle cell renal cell carcinoma.