Objective: To investigate the expression of glycoprotein non metastatic melanoma protein B (GPNMB) in renal eosinophilic tumors and to compare the value of GPNMB with CK20, CK7 and CD117 in the differential diagnosis of renal eosinophilic tumors. Methods: Traditional renal tumor eosinophil subtypes, including 22 cases of renal clear cell carcinoma eosinophil subtype (e-ccRCC), 19 cases of renal papillary cell carcinoma eosinophil subtype (e-papRCC), 17 cases of renal chromophobe cell carcinoma eosinophil subtype (e-chRCC), 12 cases of renal oncocytoma (RO) and emerging renal tumor types with eosinophil characteristics [3 cases of eosinophilic solid cystic renal cell carcinoma (ESC RCC), 3 cases of renal low-grade eosinophil tumor (LOT), 4 cases of fumarate hydratase-deficient renal cell carcinoma (FH-dRCC) and 5 cases of renal epithelioid angiomyolipoma (E-AML)], were collected at the Affiliated Drum Tower Hospital of Nanjing University Medical School from January 2017 to March 2022. The expression of GPNMB, CK20, CK7 and CD117 was detected by immunohistochemistry and statistically analyzed. Results: GPNMB was expressed in all emerging renal tumor types with eosinophil characteristics (ESC RCC, LOT, FH-dRCC) and E-AML, while the expression rates in traditional renal eosinophil subtypes e-papRCC, e-chRCC, e-ccRCC and RO were very low or zero (1/19, 1/17, 0/22 and 0/12, respectively); the expression rate of CK7 in LOT (3/3), e-chRCC (15/17), e-ccRCC (4/22), e-papRCC (2/19), ESC RCC (0/3), RO (4/12), E-AML(1/5), and FH-dRCC (2/4) variedly; the expression of CK20 was different in ESC RCC (3/3), LOT(3/3), e-chRCC(1/17), RO(9/12), e-papRCC(4/19), FH-dRCC(1/4), e-ccRCC(0/22) and E-AML(0/5), and so did that of CD117 in e-ccRCC(2/22), e-papRCC(1/19), e-chRCC(16/17), RO(10/12), ESC RCC(0/3), LOT(1/3), E-AML(2/5) and FH-dRCC(1/4). GPNMB had 100% sensitivity and 97.1% specificity in distinguishing E-AML and emerging renal tumor types (such as ESC RCC, LOT, FH-dRCC) from traditional renal tumor types (such as e-ccRCC, e-papRCC, e-chRCC, RO),respectively. Compared with CK7, CK20 and CD117 antibodies, GPNMB was more effective in the differential diagnosis (P<0.05). Conclusion: As a new renal tumor marker, GPNMB can effectively distinguish E-AML and emerging renal tumor types with eosinophil characteristics such as ESC RCC, LOT, FH-dRCC from traditional renal tumor eosinophil subtypes such as e-ccRCC, e-papRCC, e-chRCC and RO, which is helpful for the differential diagnosis of renal eosinophilic tumors.
Humans ; Kidney Neoplasms/pathology* ; Carcinoma, Renal Cell/pathology* ; Diagnosis, Differential ; Angiomyolipoma/diagnosis* ; Biomarkers, Tumor/metabolism* ; Leukemia, Myeloid, Acute/diagnosis* ; Membrane Glycoproteins

OBJECTIVE: To investigate the capabilities of classification models based on hierarchical fusion framework of multi-classifier using a random projection strategy for differentiation of renal cell carcinoma (RCC) from small renal angiomyolipoma (< 4 cm) without visible fat (AMLwvf). METHODS: We retrospectively collected the clinical data from 163 patients with pathologically proven small renal mass, including 118 with RCC and 45 with AMLwvf.Target region of interest (ROI) delineation was performed on an unenhanced phase (UP) CT image slice displaying the largest lesion area.The radiomics features were used to establish a hierarchical fusion method.On the projection-based level, the homogeneous classifiers were fused, and the fusion results were further fused at the classifier-based level to construct a multi-classifier fusion system based on random projection for differentiation of AMLwvf and RCC.The discriminative capability of this model was quantitatively evaluated using 5-fold cross validation and 4 evaluation indexes[specificity, sensitivity, accuracy and area under ROC curve (AUC)].We quantitatively compared this multi-classifier fusion framework against different classification models using a single classifier and several multi-classifier ensemble models. RESULTS: When the projection number was set at 10, the proposed hierarchical fusion differentiation framework achieved the best results on all the evaluation measurements.At the optimal projection number of 10, the specificity, sensitivity, average accuracy and AUC of the multi-classifier ensemble classification system for differentiation between AMLwvf and RCC were 0.853, 0.693, 0.809 and 0.870, respectively. CONCLUSION The proposed model constructed based on a multi-classifier fusion system using random projection shows better performance to differentiate RCC from AMLwvf than the AMLwvf and RCC discrimination models based on a single classification algorithm and the currently available benchmark ensemble methods.
Angiomyolipoma/pathology* ; Carcinoma, Renal Cell ; Diagnosis, Differential ; Humans ; Kidney Neoplasms/pathology* ; Retrospective Studies ; Tomography, X-Ray Computed/methods*

Adult ; Angiomyolipoma ; diagnosis ; genetics ; Exons ; genetics ; Humans ; Kidney Neoplasms ; diagnosis ; genetics ; Male ; Mutation ; genetics ; Tuberous Sclerosis ; complications ; Tuberous Sclerosis Complex 2 Protein ; genetics ; Young Adult

Angiomyolipoma (AML) is a rare benign mesenchymal tumor in the liver, which is composed of blood vessels, smooth muscle, and adipose cells. The proportion of each component varies, making a diagnosis difficult. This paper reports a case of AML in the liver without adipose tissue, mimicking a hepatocellular carcinoma (HCC), which was diagnosed by a surgical tissue biopsy. A 65-year-old woman was admitted for an evaluation of a hepatic mass that had been detected by ultrasonography. The serologic markers of viral hepatitis B and C were negative. The liver function tests and alpha fetoprotein level were within the normal limits. Magnetic resonance imaging revealed a 1.9 cm sized mass in segment 6 of the liver with early arterial enhancement and washout on the delayed phase accompanied by a rim-like enhancement, which is similar to the imaging findings of HCC. A frozen section examination during surgery indicated a hepatocellular neoplasm and suggested the possibility of HCC. On the other hand, the final pathologic diagnosis was epithelioid myoid type of AML with no adipose tissue component. The tumor cells were positive for human melanocyte B-45 and negative for cytokeratin and hepatocyte paraffin 1. This paper reports a very rare case of AML without adipose tissue in the liver mimicking HCC that was diagnosed by a surgical tissue biopsy.
Adipose Tissue ; Aged ; alpha-Fetoproteins ; Angiomyolipoma ; Biopsy ; Blood Vessels ; Carcinoma, Hepatocellular ; Diagnosis ; Female ; Frozen Sections ; Hand ; Hepatitis B ; Hepatocytes ; Humans ; Keratins ; Liver Function Tests ; Liver ; Magnetic Resonance Imaging ; Melanocytes ; Muscle, Smooth ; Paraffin ; Ultrasonography

Renal angiomyolipoma (AML) is a common benign tumor in the urinary system, mainly composed of adipose tissue, blood vessels and muscle tissue. Renal AML is sporadic in most of patients, while a few are associated with tuberous sclerosis. Classical renal AML occurs predominantly in middle-aged females. Most cases are found incidentally during imaging examinations. The fat content makes AML have unique imaging characteristics and is easy to be identified with other renal tumors. However, the amount of fat varies in each tumor. AML that contains only microscopically detectable fat and whose amount of intratumoral fat may be too small to be identified on unenhanced computed tomography (CT) images is termed minimal fat or fat-poor renal AML, which appears as a high density shadow in the renal parenchyma on unenhanced CT images. Thus, it can be difficult to distinguish it from renal cell carcinoma (RCC) on imaging. Since the imaging findings are atypical, the diagnosis depends on pathological results. In addition, a few of AML can mimic malignant neoplasms. Recent studies suggested that AML might involve to peri-renal or renal sinus fat, regional lymphatics and other visceral organs, as well as inferior vena cava, which further makes the diagnosis more difficult. However, there is currently no reports about involvement of regional limphatics in minimal fat renal AML. In this article, we report a 27-year-old female patient without family history of tuberous sclerosis, who came to visit the hematologist because a high density shadow near the left kidney was found during CT scan which was accompanied by neck, armpits, groin, abdominal cavity and retroperitoneal lymph nodes enlargement. She was suspected of lymphoma in the beginning and transferred to Department of Urology to perform laparoscopic left renal mass and retroperitoneal lymph node excision and pathological examination for a definitive diagnosis. Finally, pathologic results revealed AML. Postoperative continuous lymphatic fistula developed and the retroperitoneal drainage of chylous fluid was 100-200 mL per day, lasting for 12 weeks. The fistula was successfully closed after conservative treatment including fasting and rehydration. This article summaries and discusses the diagnosis and treatment of renal AML with lymph nodes enlargement and the management of postoperative refractory lymphatic fistula by reviewing the related cases and literature.
Adult ; Angiomyolipoma/pathology* ; Carcinoma, Renal Cell/diagnosis* ; Female ; Fistula ; Humans ; Kidney Neoplasms/pathology* ; Lymph Nodes/pathology* ; Middle Aged

A 31-year-old male visited a local hospital due to sudden-onset severe abdominal pain. Abdominal computed tomography revealed a solid cystic mass with a size of approximately 12 cm and exhibiting both hemorrhage and fluid collection in the pelvic cavity. Emergency angiography and embolization were performed, and a large hepatic tumor was subsequently surgically resected. The tumor cells stained positive for human melanoma black-45 and smooth-muscle actin, and the pathologic diagnosis was hepatic angiomyolipoma. This case report also discusses the spontaneous rupture of a hepatic angiomyolipoma.
Abdominal Pain ; Actins ; Adult ; Angiography ; Angiomyolipoma* ; Diagnosis ; Emergencies ; Hemorrhage ; Humans ; Liver ; Male* ; Melanoma ; Rupture, Spontaneous

The diagnosis of hepatocellular carcinoma (HCC) is based on imaging studies particularly in high-risk patients without histologic confirmation. This study evaluated the prevalence and characteristics of false-positively diagnosed HCC in a liver resection cohort for HCC. A retrospective review was performed of 837 liver resection cases for clinically diagnosed HCC between 2005 and 2010 at our institute. High-risk patients with tumors > 1 cm with one or two image findings consistent with HCC and tumors < 1 cm with two or more image findings consistent with HCC with persistently increased serum alpha-fetoprotein (AFP) levels above the normal range with underlying inhibited hepatitis activity underwent liver resection. The false-positive rate was 2.2% (n = 18). Of the 18 patients, 7 patients (0.8%) were diagnosed with benign conditions (one each of hemangioma, inflammation, cortical adenoma, dysplastic nodule, angiomyolipoma, bile duct adenoma, and non-neoplastic liver parenchyme) and 11 patients (1.3%) were diagnosed with malignancies (cholangiocarcinoma [n = 6], hepatoblastoma [n = 2], and one each of lymphoepithelioma-like carcinoma, ovarian cystadenocarcinoma, and nasopharynx carcinoma metastasis). The clinical characteristics of pathologically diagnosed HCC patients were similar (P > 0.05) compared to non-HCC patients except for higher rate of history of alcoholism (P < 0.05) observed in non-HCC patients. Four of 18 non-HCC patients (22.2%) showed diagnostic discordance on the dynamic imaging study. Despite the recent progression in diagnostic imaging techniques, 2.2% of cases were false-positively diagnosed as HCC in a liver resection patient cohort; and the final diagnosis was benign disease in 0.8% of liver resection patients clinically diagnosed with HCC.
Adenoma ; Adenoma, Bile Duct ; Alcoholism ; alpha-Fetoproteins ; Angiomyolipoma ; Carcinoma, Hepatocellular* ; Cohort Studies ; Cystadenocarcinoma ; Diagnosis* ; Diagnostic Imaging ; Hemangioma ; Hepatitis ; Hepatoblastoma ; Humans ; Inflammation ; Liver* ; Nasopharynx ; Prevalence ; Reference Values ; Retrospective Studies

Epithelioid angiomyolipoma (EAML) of liver is a rare neoplasm. Hepatic EAML is often misdiagnosed as other neoplasms such as hepatocellular carcinoma due to non-specific clinical and radiologic features. The morphologic features under microscope and immunohistochemistry staining profile are important in the diagnosis EAML. Here, we report a case of 52-year-old man who found 1.2 cm mass in liver by routine checkup. On the impression of hepatocellular carcinoma, lateral sectionectomy of the liver was done. Microscopically, the tumor is composed of predominant epithelioid cells with vascular component and foamy cells. These cells were positive for HMB45, MelanA, and smooth muscle actin and negative for epithelial membrane antigen. The final diagnosis was hepatic EAML.
Actins ; Angiomyolipoma* ; Carcinoma, Hepatocellular ; Diagnosis ; Epithelioid Cells ; Humans ; Immunohistochemistry ; Liver* ; MART-1 Antigen ; Middle Aged ; Mucin-1 ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms

OBJECTIVETo study the clinicopathological characteristics of hepatic angiomyolipoma (HAML) and to evaluate the correlation between clinicopathological parameters and tumor subtypes.

METHODSRetrospective analysis of clinicopathological features was conducted in 182 cases of HAML.

RESULTSHAML patients were predominantly female (M:F=1:4) and most commonly presented with non-specific symptoms. The median age at diagnosis was 46 years, ranged from 17 to 77 years. Tumor diameter was ranged from 0.3 to 32.0 cm with an average of 5.0 cm. Majority of the tumor was epithelioid type (112/165, 67.9%). Extramedullary hematopoiesis, multinucleated giant cells, intranuclear inclusions, nucleolus, cellular atypia, invasive growth pattern, multiple masses, hyperpigmentation and purpura-like changes mostly occurred in the epithelioid type (P<0.05). Extramedullary hematopoiesis was commonly seen in HAML, the significance of which was still uncertain.

CONCLUSIONSMost of HAML are epithelioid type, characterized by a proliferation of predominantly epithelioid cells, in which extramedullary hematopoiesis is commonly seen. Some morphologic features that may predict malignant such as necrosis, mitotic figures, and tumor emboli are only found in the epithelioid HAML. Mitotic activity, tumor necrosis, tumor thrombus, giant cells, periportal invasion, multiple lesions and tumors size over 10 cm are closely related with tumor recurrence and metastasis.

Adolescent ; Adult ; Aged ; Angiomyolipoma ; diagnosis ; pathology ; Epithelioid Cells ; cytology ; Female ; Gastrointestinal Neoplasms ; diagnosis ; pathology ; Giant Cells ; pathology ; Humans ; Middle Aged ; Necrosis ; Neoplasm Recurrence, Local ; Prognosis ; Retrospective Studies ; Young Adult

The prevalence of small renal masses (SRM) has risen, paralleling the increased usage of cross-sectional imaging. A large proportion of these SRMs are not malignant, and do not require invasive treatment such as nephrectomy. Therefore, differentation between early renal cell carcinoma (RCC) and benign SRM is critical to achieve proper management. This article reviews the radiological features of benign SRMs, with focus on two of the most common benign entities, angiomyolipoma and oncocytoma, in terms of their common imaging findings and differential features from RCC. Furthermore, the role of percutaneous biopsy is discussed as imaging is yet imperfect, therefore necessitating biopsy in certain circumstances to confirm the benignity of SRMs.
Abdominal Fat/pathology ; Adenoma, Oxyphilic/diagnosis/radiography/ultrasonography ; Angiomyolipoma/diagnosis/radiography/ultrasonography ; Carcinoma, Renal Cell/*diagnosis/radiography/ultrasonography ; Diagnosis, Differential ; Humans ; Kidney Neoplasms/*diagnosis/*radiography/ultrasonography ; Leiomyoma/diagnosis/radiography/ultrasonography