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

Objective:To investigate the MRI features of hepatic nodular regenerative hyperplasia (NRH) induced by chemotherapy.Methods:The clinical data and MRI of 20 cases with hepatic NRH induced by chemotherapy and confirmed by pathology in Zhongshan Hospital Fudan University from August 2014 to May 2019 were retrospectively analyzed. There were 13 males and 7 females, with an average age of 49.8 ± 9.7 years. Contrast-enhanced MR scan with Gd-DTPA was performed eighteen patients, and two patients underwent contrast-enhanced MR scan with hepatobiliary specific contrast (Gd-EOB-DTPA). The image analysis includes the number, location, size, shape, signal intensity in plain scan and enhancement pattern of lesions. The apparent diffusion coefficient (ADC) values of the lesions and adjacent hepatic parenchyma were measured on the ADC map, and the difference was compared with paired sample t test.Results:A total of 36 lesions in 20 patients were rounded or oval, including 23 (63.9%) lesions in the right lobe, 12 (33.3%) in the left lobe and 1 (2.8%) in the caudate lobe. The average diameter of all lesions was 15.4 ± 6.4 (7.0-37.0) mm. The boundary was clear in 9 (25.0%) lesions and blurred in 27 (75%) lesions. In T1WI, 35 (97.2%) lesions showed slightly hypointensity, and in 1 (2.8%) lesion was iosintensity. All 36 lesions showed slightly hyperintensity in T2WI. 33 (91.7%) lesions showed slightly hyperintensity in DWI, and 3 (8.3%) lesions showed iosintensity. 31 lesions with Gd-DTPA enhanced MR scan were significantly enhanced in the arterial phase and showed slightly high signal intensity in early portal vein phase, late portal vein phase and equilibrium phase. 5 lesions with Gd-EOB-DTPA enhanced MR scan were also significantly enhanced in the arterial phase and showed slightly high signal intensity in early portal vein phase, late portal vein phase and equilibrium phase, then all lesions showed circular high signal intensity in hepatobiliary specific phase. The average ADC value of 29 lesions was (1.471 ± 0.253) × 10 -3 mm 2/s, and that of adjacent liver parenchyma was (1.460 ± 0.235) ×10 -3 mm 2/s. There was no significant difference between the two groups ( P > 0.05). Conclusion:MR findings of NRH induced by chemotherapy have certain characteristics, and the morphological manifestations, diffusion-weighted imaging, enhanced imaging and hepatobiliary specific phase features of the lesions can help to diagnose the disease.

Objective:To investigate the clinicopathological, molecular pathological features, and family genetic pedigree of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT).Methods:A total of 16 cases of SCCOHT diagnosed in Obstetrics and Gynecology Hospital of Fudan University from January 2013 to January 2023 were collected. The clinicopathologic features, SMARCA4/2/B1 protein expression, outcomes and SMARCA4 gene detection were reported. A follow-up study was also carried out.Results:The average age at diagnosis was 28.7 years (range 17-38 years). The preoperative calcium level was evaluated in 3 of 6 patients. The tumor was unilateral in all 16 cases, ranged from 8 to 26 cm (average 15.8 cm) in the greatest dimension. Extraovarian spread was present in 7 cases. In 10 cases, the tumors were initially misinterpreted as other ovarian neoplasms. BRG1 and BRM expression by immunohistochemistry were all lost in detected cases, while INI1 exhibited retained nuclear expression. All BRM-negative SCCOHTs also lacked BRG1 protein,but retained INI1 expression. SCCOHTs were only focally positive for EMA, CKpan, Calretinin, SALL4, and diffusely positive for WT1. Two of nine cases exhibited mutation-type p53 immunoreactivity. Ki-67 index was 58% on an average. ER, PR, FOXL2, α-inhibin, chromogranin A and LCA were negative in all the cases. SMARCA4 sequencing was available in 8 cases of SCCOHT, which revealed a germline SMARCA4 mutation in one patient, and others carried somatic mutation. Furthermore, two daughters, mother and an aunt of a patient with germline mutation were reported to be SMARCA4 mutation carriers. Follow-up was available for 15 patients, and the 6-month, 1-year and 2-year survival rate was 65.8%, 45.1%, and 22.6%, respectively. For patients in FIGO stages Ⅱ+Ⅲ, 6-month, 1-year survival rate was 53.6% and 35.7% respectively, compared to 80% (6-month) and 60% (1-year) in patients of staged I ( P=0.358). Conclusions:With dismal prognosis of SCCOHT, accurate diagnosis is necessary. The typical age distribution, a panel of various staining results, especially concomitant loss of BRG1 and BRM may be of diagnostic aid and can be used to distinguish SCCOHT from its histological mimics. After the diagnosis of SCCOHT, genetic testing and genetic counseling are recommended.

OBJECTIVE：To establ ish artificial neural netw orks（ANN）model to predict the interferon in the treatment of chronic hepatitis B （CHB），and to provide evidence for selecting suitable CHB therapy plan in clinic. METHODS ：The clinical data of 92 CHB patients treated by interferon ，from Guangzhou Eighth People ’s Hospital were retrospectively analyzed from Jul. 2011 to Dec. 2019. The basic information ，biochemical indexes ，blood routine indexes and virological markers of patients were collected. According to the effect of interferon ，the patients were divided into response group （73 cases）and non-response group （19 cases）. Minitab 18.0 software was used for multivariate Logistic regression analysis to screen the factors influencing the efficacy of interferon. Neurosolutions 5.0 software was used to randomly select 30％ of patients with CHB （27 cases）as the test group to establish and verify the ANN model. RESULTS ：The mean platelet volume ，platelet distribution width ，direct bilirubin ， hepatitis B e antigen and hepatitis B virus DNA more than 4×107 IU/mL had significant effect on interferon response （P＜0.05）. The accuracy ，specificity and area under characteristic curve of ANN test group were significantly higher than those of Logistic regression（P＜0.05）. CONCLUSIONS ：ANN model is accurate in predicting the efficacy of interferon in the treatment of CHB.

Objective: To compare the MRI features of peliosis hepatis and hepatic metastases following chemotherapy for gastrointestinal adenocarcinoma with the aim to improve the differential diagnosis. Methods: The clinical data of 33 patients with gastrointestinal adenocarcinoma treated from June 2014 to December 2017 at Zhongshan Hospital of Fudan University were retrospectively analyzed. Of the 26 males and 7 females aged (56.0±9.8) years, there were 11 patients with peliosis hepatis and 22 patients with hepatic metastases following chemotherapy in these patients. All patients underwent contrast-enhanced abdominal MRI scans. The differences in the MRI features, including morphology, margin, signal intensity on plain scanning and enhancement patterns were compared statistically. The apparent diffusion coefficient (ADC) values of peliosis hepatis, hepatic metastases and adjacent hepatic parenchyma were measured in an ADC map. Results: In 14 lesions of the 11 patients with peliosis hepatis, 10 lesions were ill-defined and 4 lesions were well-defined. In 31 lesions of the 22 patients with hepatic metastases, 5 lesions were ill-defined and 26 lesions were well-defined. Significant differences existed between peliosis hepatis and hepatic metastases in the margin (P<0.05). The ADC value of hepatic metastases was significantly lower than that of peliosis hepatis and the adjacent hepatic parenchyma (P<0.05). In all the 14 lesions of peliosis hepatis, 10 lesions showed gradual filling enhancement, and 4 lesions showed marked and persistent enhancement. In all the 31 lesions of hepatic metastases, 28 lesions showed a ring-shaped enhancement, and 3 lesions showed "quick in and quick out" enhancement. Conclusions The lesions of peliosis hepatis following chemotherapy for gastrointestinal adenocarcinoma were ill-defined, with no restriction of water diffusion in the diffusion weighted imagings, and with progressive enhancement. The MRI manifestations of peliosis hepatis helped to differentiate peliosis hepatis from hepatic metastases of gastrointestinal adenocarcinoma.

Objective: To investigate the clinicopathological characteristics, histogenesis, immunophenotypes, molecular genetic characteristics, diagnosis and differential diagnosis of calcifying fibrous tumors (CFT). Methods: A total of 32 cases of CFT (22 cases from Henan Provincial People′s Hospital and 10 cases from PLA Army Medical Center) diagnosed between June 2009 and February 2019 were reviewed. The clinical and pathologic data were analyzed. Results: There were 12 male and 20 female patients, aged from 15 to 63 years (mean 40.8 years). Eleven cases occurred in stomach, four cases in retroperitoneum, four cases in ovary, two cases in scrotum, two cases in mediastinum, two cases in head and neck, one case each in thoracic cavity, lung, adrenal gland, kidney, sigmoid colon, epididymis and mesosalpinx. All the tumors were solid masses with clear boundaries. The maximal dimension of the tumors ranged from 0.6 to 10.0 cm. Microscopically, there was hypocellular stromal sclerosis and wavy storiform coarse collagen with superimposed scattered or patchy lymphocytes and plasma cells; calcification or gravel formation were also detected. Immunohistochemistry showed that spindle cells were positive for vimentin and some were positive for CD34; and they were negative for calponin, SMA, desmin, S-100 protein, SOX10, STAT6, β-catenin, ALK, CD117, DOG1, CKpan, and EMA. No ALK rearrangement was detected by FISH in all cases. No C-KIT and PDGFRA mutation was detected in all the tested 11 cases of stomach, four cases of retroperitoneal and one case of sigmoid colon CFT. MDM2 was not amplified by FISH in all four tested cases of retroperitoneal CFT. Conclusions CFT is a rare benign tumor of fibroblastic cell origin. The diagnosis mainly depends on histomorphologic analysis and immunophenotyping. CFT should be differentiated from other benign and malignant spindle cell mesenchymal tumors.