The Chinese guidelines for the diagnosis and treatment of pancreatic neuroendocrine neoplasms (2020) / 中华消化外科杂志

Pancreatic neuroendocrine neoplasms (pNENs) are highly heterogeneous, and the management of pNENs patients can be intractable. To address this challenge, an expert committee was established on behalf of the Chinese Pancreatic Surgery Association, Chinese Society of Surgery, Chinese Medical Association, which consisted of surgical oncologists, gastroenterologists, medical oncologists, endocrinologists, radiologists, pathologists, and nuclear medicine specialists. By reviewing the important issues regarding the diagnosis and treatment of pNENs, the committee concluded evidence-based statements and recommendations in this article, in order to further improve the management of pNENs patients in China.

Preliminary value of CT radiomics in predicting anaplastic lymphoma kinase fusion gene expression in lung adenocarcinoma / 中华放射学杂志

Objective@#To explore the value of quantitative CT radiomics features in predicting the anaplastic lymphoma kinase (ALK) mutation status in lung adenocarcinoma patients.@*Methods@#This retrospective study reviewed one hundred and ninety-five lung adenocarcinoma patients (including 60 patients with ALK mutation) whose ALK genetic test results were available from Nov 2015 to May 2018 in PUMCH. VOIs were labeled by an automatic pulmonary nodule detection and segmentation algorithm and were later revised and confirmed by two senior radiologists. The PyRadiomics tools were used to resample the labeled regions, followed by image pre-processing (Wavelet filter or Laplacian of Gaussian (LoG) filter) and feature extraction. Normalized features were selected based on their representativeness on Dr. Wise research platform. Multivariate logistic regression was performed to develop prediction models of ALK mutation gene based on different image pre-processing techniques and different radiomics feature types. The results were validated by ten runs of five-fold cross validation. ROC curve analysis and Delong test were used to compare the predictive performance among models.@*Results@#Fifteen radiomics features with the highest representativeness were selected from the original 1 232 features. The prediction model based on these radiomics features showed good performance (AUC=0.88 in the training set and 0.78 in the validation set) and was not significantly different from the prediction models based on radiomics features of different pre-processing images (AUC=0.76, P=0.1, original CT images; AUC=0.75, P=0.3, Wavelet-filtered images; AUC=0.76, P=0.2, LoG-filtered images). Among the models built with radiomics features of different types, the one based on GLCM feature (a subtype of texture feature) showed the best performance in predicting ALK genetic status (AUC=0.83, accuracy=0.74, sensitivity=0.85 and specificity=0.69). The model based on first-order statistic features had an AUC of 0.80.@*Conclusion@#Quantitative CT radiomics features have a good potential to anticipate the expression of ALK fused gene in patients with lung adenocarcinoma.

Clinical and immunohistopathologic study of phosphaturic mesenchymal tumor / 中华病理学杂志

Objective@#To study the clinicopathological characteristics and immunohistochemical phenotype of phosphaturic mesenchymal tumor (PMT) .@*Methods@#The clinicopathological data and immunohistochemical profiles were obtained retrospectively from 206 patients diagnosed with PMT at Peking Union Medical College Hospital (PUMCH) during July 2008 to September 2017, with a review of literature.@*Results@#The mean age of PMT patients was 42 years (range 13 to 70 years), with a male to female ratio of 1.1∶1.0. All patients presented with different degree of bone pain, muscle weakness, shorten of stature, thoracic deformity and pathological fractures, with hypophosphatemia and high serum ALP. Phosphatemia returned to normal within 1 week after operation in all cases underwent complete tumor resection. The duration of osteomalacia before resection (documented in 197 cases) ranged from 20 days to 40 years (average 5.7 years). The average blood phosphorus concentration raised from 0.49 mmol/L to 0.92 mmol/L before and after tumor resection (P<0.01), with 147 cases (84.0%, 147/175) returned to normal range within 2 weeks. The rate or blood phosphorus concentration recovery in 15 days after operation was 79.6% in average, displayed significant differences between patients with complete resection and those with partial resection (85.4% vs. 21.1%, P<0.01). PMT lesions mainly involved lower extremities (55.8%), followed by head and neck (29.1%). In immunohistochemical study, all cases were positive for vimentin (100.0%), while most cases were positive for NSE (96.3%), CD56 (94.2%), FGF23(88.4%), CD68 (88.3%), D2-40 (70.9%), CD34 (23.1%), SMA (55.5%), bcl-2 (59.8%) and CD99 (47.1%). The Ki-67 positive index of tumor varied from less than 2% (51.4%), 3% to 10% (41.3%) to >10% (7.2%).@*Conclusions@#PMT mainly occurs in lower limbs or head and neck, with unique clinical characteristics and blood biochemical indexes. The tumor expresses a variety of immunohistochemical markers, indicating the potential of multi-directional differentiation. Clinical profile, blood biochemistry testing and immunohistochemical phenotype is helpful for diagnosis of PMT.

Primary carcinoid of ovary: a clinicopathologic analysis of 17 cases / 中华病理学杂志

Objective@#To study the clinicopathologic feature, diagnostic strategy and prognostic significance of primary carcinoid of the ovary (PCOTO).@*Methods@#A series of 17 patients previously diagnosed as PCOTO at Department of Pathology, Peking Union Medical College Hospital during the period from 2002 to 2017 were evaluated with clinical data analysis, histopathology and immunohistochemistry, and the patients were followed up and the relevant literatures were reviewed.@*Results@#The age of patients ranged from 24 to 64 years (mean, 42 years). Fourteen patients were found a pelvic mass for a health check-up, and only 3 patients presented with pain in the lower abdomen. The left ovary was involved initially in ten patients, and the right in seven. The major axis of the tumor ranged from 2 to 14 cm. The surface of these tumors was smooth. Seven of the tumors were solid-cystic, 6 were cystic and 4 were solid. Under light microscope, 6 cases were diagnosed as strumal carcinoid, 4 cases were insular carcinoid, 4 cases were trabecular carcinoid, 3 cases were insular and trabecular mixed type carcinoid and 1 case was mucinous carcinoid. The mitotic figures were no more than 1/10 HPF.There were 11 cases complicated with other ovarian tumors, including 10 cases with teratoma, and 1 case with mucinous cystic adenoma. The paraffin-embedded tissues of all cases showed immunoreactivity for NSE and Syn, and the positive propotion of CgA was 10/17. TTF1 was positive in thyroid follicles and negative in strumal carcinoid. The positive index of Ki-67 was no more than 2%. Follow-up of 13 to 188 months showed 16 patients without recurrence and 1 patients were loss to follow-up.@*Conclusions@#PCOTO is very rare. Most of the patients are found with a mass during health check-up in unilateral ovary and without obvious clinical symptoms. Histologically, the tumor always exists with other ovarian tumors, including teratoma and mucinous cystic adenoma, with relatively good prognosis.

MRI Features and Pathological Basis of Aggressive Angiomyxoma in Pelvic and Perineal Areas / 中国医学影像学杂志

Purpose To summarize the clinical features, MRI features and pathological features of aggressive angiomyxoma (AAM) occurring in pelvic and perineal areas. Materials and Methods The clinical, MR and pathological data of 7 patients with AAM pathologically confirmed were retrospectively analyzed. Results Seven cases of AAM were all females, aged from 12 to 45 years old. Among them, 5 cases were treated due to perineal area tumors, and 2 cases due to relapses after surgical treatment of perineal area AAM in other hospitals. MRI findings: 7 cases were single occurrence with the maximum diameter of 5.9-15.7 cm; 6 cases were in irregular shape, with no envelope and unclear borders with adjacent tissues or organs; 1 case was in long column shape with clear boundary and pseudocapsule visible. On T1WI, AAM showed equal signal (4/7) or equal, low mixed signal (3/7). On T2WI, AAM showed high and low mixed signals with 5 cases of "vortex sign". 5 cases underwent routine enhancement examinations, all demonstrating significant enhancement, 4 cases of which displayed heterogeneous enhancement ("vortex sign" was detected in 2 cases) and 1 case homogeneous enhancement. On diffusion weighted imaging, AAM manifested as homogeneous (3/7) or heterogeneous (4/7) high signal. Pathological examination showed that a large amount of mucin-like matrix was contained in AAM, and there were scattered fusiform and astral tumor cells and abundant arterial and venous blood vessels seen locally. Tumor cells infiltrated the peripheral fat or muscle tissues at the border. Conclusion The characteristic MRI of AAM is manifested as "vortex sign", which corresponds to its pathological features such as sparse tumor cells, mucin-like matrix in large amount, and locally rich blood vessels, suggesting great significance for imaging diagnosis.

Clinicopathologic features of gastric adenocarcinoma based on the revised Lauren′s classification / 中华病理学杂志

Objective@#To compare the clinicopathologic features and prognosis of the subtypes in a consecutive series of gastric cancers (GC) patients basing on the revised Lauren′s classification so as to better understand the biological behavior of GC.@*Methods@#The surgically resected GC from Peking Union Medical College Hospital during 2003-2005 were reviewed for patients′ age, gender, tumor size, location, Borrman classification, depth of invasion, lymph node metastasis, vascular invasion, and tumor growth pattern (Ming classification).@*Results@#One hundred and sixty-six GC cases were enrolled and classified into four groups: intestinal GC (30, 18.1%), diffuse GC (56, 33.7%), solid GC (9, 5.4%), and mixed GC (71, 42.8%). Intestinal GC patients were older[ (63.2±11.3) years], with a male predominance, and were more frequently found in the antrum. Intestinal GC was the most common subtype for early GC, and tend to develop liver metastases. Diffuse GC patients were youngest [(52.2±12.7) years], with no gender difference, and were usually found in the antrum. Microscopically, diffuse GC were more likely infiltrative (51/56, 91.1%), and tended to metastasize by lymphatic pathway. Solid GC were usually large[ (6.4±2.2) cm], with a male predominance, and the most frequent site was the body/fundus. Solid GC were more likely to show expansile growth pattern with greater depth of invasion (8 of 9 cases were T3/4), but lower rate of lymphatic metastasis. Mixed GC also showed a male predominance, usually found in the antrum, and showed an infiltrative growth pattern (49/71, 69.0%). Though there was fewer T3/4 than solid and diffuse GC, mixed GC were more likely to show lymph nodes, vascular metastases and liver metastases (13/71, 18.3%). TNM staging, lymph nodes metastasis, lymphovascular invasion and revised Lauren′s classification were four independent prognostic factors on multivariate analysis(P<0.05). The survival of patients with mixed GC were significantly worse than patients with other histological types.@*Conclusion@#Revised Lauren′s classification for GC has four distinct subgroups and can be used as independent prognostic factors.

Study on the overexpression of succinate dehydrogenase B and MIB-1 in pheochromocytoma/paraganglioma tissue / 中华泌尿外科杂志

Objective We aimed to investigate the overexpression of succinic dehydrogenase (SDH) B and MIB-1 in patients with pheochromocytoma/paraganglioma(PHEO/PGLs) and its significance for predicting the clinical malignant behavior.Methods From August 2008 to April 2016,the clinical characteristics of 93 patients with PHEO/PGLs were analyzed retrospectively.There were 57 males and 36 females,with an average of 34 years,ranging 8-73 years old.There were 68 cases of adrenal pheochromocytoma and 25 cases of paraganglioma.There were 79 cases with hypertension and 14 cases of adrenal accidental tumors.Sixty-six cases with typical hyper-catecholamine secretion symptoms and 27 cases with non-functional PHEO/PGL.Benign PHEO/PGLs were 77 cases and malignant 16 cases.The tumor was located on the left side in 39 cases,on the right side in 32 cases and multiple lesions in 22 cases.The diameter of the PHEO/PGL tumor was (6.8 ± 2.7) cm.The 24 h urine catecholamine was measured before operation,which showed epinephrine was (42.6 ± 5.1) μg/24 h,norepinephrine was (167.5 ± 13.5) μg/24h and dopamine was (246.4 ± 71.2)μg/24h.Six cases wihtout hereditary diseases of urinary system were selected as normal control group.SDHB,SDHAF2,SDHC,SDHD,VHL and RET gene mutations were detected in all patients.Immunohistochemical panel has been performed to detect the expression of SDHB,MIB-1,EPAS1,VEGF-1 receptor (VEGF-1 R),and chromain A (CgA) in 93 specimens of PHEO/PGL tissue.The positive granular cytoplasm staining ＞ 50％ was strongly positive (+ + +),11％ to 50％ was moderately positive (+ +),1％ to 10％ was weak positive (+) and the negative was compared with the known positive internal reference,that is,there was less than 1％ or no stain completely.Results SDHB,SDHAF2,SDHC,SDHD,VHL and RET gene mutations in 27 cases (29.5％).Nine patients with SDHB gene mutation (9.7％).RET proto-oncogene mutations in 8 cases (8.6％).3 cases had VHL mutation (3.2％).Immunohistochemical staining showed that MIB-1 positive expression was found in 7 of 9 patients with SDHB gene mutation.Six cases in the control group were negative for gene detection and MIB-1,EPAS1,CgA and VEGF-1R immunohistochemical results.EPAS1 showed moderately positive in patients with PHEO/PGL and strong positive in patients with malignant PHEO/PGL.In 9 cases with SDHM mutation,EPAS1 was noticed positive in seven cases,which showed the relationship with CgA,MIB-1 and VEGF-1R.Conclusions The SDHB gene mutation is usually shown as a paraganglioma focus outside the adrenal gland.And 9.8％ of the paragangliomas were associated with a mutation of the SDHB gene with an increase in malignant risk.The SDHB mutation caused over-expression of MIB-1 and the positive expression of EPAS1 and VEGF-1R in PHEO/PGL tissues,which was associated with invasion and metastasis of malignant PHEO/PGL.

Placental transmogrification of the lung: two case report and systematic review of the literature / 中华胸心血管外科杂志

Objective Placental transmogrification of the lung(PTL) is rare;summarizes the reported cases and add our two cases, to explore the best diagnosis and treatment strategy.Methods Review of the cases reported in the literature, combined with the 2 cases described in this article, summarizes the characteristics of PTL and analyzed the best diagnosis and treatment strategy.Results We reported two cases of placental transmogrification of the lung, both presented in the right lower lobe, imaging performance as a giant bulla with a cystic nodule.VATS lobectomy was performed in both cases, no complication after operation.Combined with literature review of 34 cases of patients to analyze the best diagnosis and treatment strategy.Conclusion Grossly and microscopically, the lesion resembles placental tissue, with formation of placental villus-like papillary structures covered by epithelial cells.The most common imaging manifestation of PTL is a bullous emphysema pattern or with a mixed pattern of thin-walled cystic lesions and nodules.Early diagnosis and surgical operation should be performed as soon as possible, these lesions are best treated by minimally invasive surgery, leaving as much normal lung tissue and avoiding pneumonectomy if possible.Surgical treatment is usually curable and leads to successful improvement of symptoms and quality of life.

Expression of CD133 in rectal cancer tissues and its relationship with neoadjuvant chemoradiotherapy / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate the association of CD133 expression in rectal cancer tissues with neoadjuvant chemoradiotherapy (nCRT) and tumor regression grading (TRG) after nCRT.</p><p><b>METHODS</b>Radical resected rectal cancer specimens and clinicopathological data of 105 patients, including 60 men and 45 women with median age of 59 years, diagnosed as locally advanced rectal cancer in Peking Union Medical College Hospital from January 2008 to December 2014 were collected retrospectively. Thirty-nine and 66 cases were histologically classified as good-moderate and poor differentiation respectively. Sixty-eight and 37 cases were clinically graded as stage I(-II( and III(-IIII( in preoperative assessment respectively. NCRT was administered in 61 cases before surgery (nCRT group). The nCRT consisted of preoperative pelvic radiotherapy using 50 Gy (2 Gy once, for 25 sessions) with FOLFOX regimen (5-fluorouracil plus oxaliplatin) for 2-3 cycles or XELOX regimen (capecitabine plus oxaliplatin) for 2 cycles. Patients underwent surgery after 6 courses of nCRT, and then received the same previous chemotherapy regimen. In nCRT group, biopsy specimens before nCRT were obtained in 45 cases. Forty-four cases received surgery alone without nCRT (surgery alone group). CD133 expression was tested by immunohistochemical Envision two-step methods. The histological TRG evaluation was performed in the nCRT group. TRG score 0-2 was defined as insensitivity to nCRT, whereas TRG score 3-4 was defined as sensitivity. CD133 expression in rectal cancer samples before and after nCRT was compared. Association of CD133 expression with TRG after nCRT was examined.</p><p><b>RESULTS</b>No significant differences of baseline parameters were found between nCRT group and surgery alone group (all P>0.05). The positive rate of CD133 in nCRT group was 70.4%(43/61,) which was significantly higher than that in surgery alone group (47.7%, 21/44)(χ(2)=5.566, P=0.018) and that in biopsy samples before nCRT group (44.4%, 20/45)(χ(2)=7.287, P=0.007). Twenty-two cases (36.1%, 22/61) in nCRT group had TRG score of 3-4 . Among these 22 cases, 11 cases were negative CD133, and constituted 61.1% (11/18) of all CD133-low expression cases in nCRT group, whereas the other 11 cases were positive CD133, and constituted 25.6%(11/43) of all CD133-high expression cases in nCRT group (χ(2)=6.974, P=0.008).</p><p><b>CONCLUSION</b>The CD133 expression up-regulates markedly in rectal cancer after nCRT and nCRT may have potential positive modulation on CD133 expression. CD133-positive cancer reveals lower response to nCRT, suggesting CD133 may be a potential target for improving efficacy of nCRT in rectal cancer.</p>

Diagnostic value of MYB protein expression in adenoid cystic carcinoma and status of MYB gene copy number / 中华病理学杂志

<p><b>OBJECTIVE</b>To explore the diagnostic value of MYB protein expression for adenoid cystic carcinoma and its differential diagnosis from other salivary gland tumors, and to further investigate the status of MYB gene copy number.</p><p><b>METHODS</b>MYB expression was studied by immunohistochemistry in 34 adenoid cystic carcinomas, 55 non-adenoid cystic carcinomas (other salivary gland tumors) including 10 pleomorphic adenomas, 10 basal cell adenomas, 10 epithelial-myoepithelial carcinomas, 9 basal cell adenocarcinomas, 8 mucoepidermoid carcinomas, 4 carcinoma in pleomorphic adenomas, and 4 polymorphous low-grade adenocarcinoma. MYB gene copy number status was detected by FISH in MYB protein-positive cases.</p><p><b>RESULTS</b>82.4% (28/34) of adenoid cystic carcinomas were MYB protein-positive, compared with 9.1% (5/55) of non-adenoid cystic carcinomas, and the difference between the two groups was statistically significant (P < 0.01). 2/18 of adenoid cystic carcinomas had duplication of MYB gene by FISH, and all non-adenoid cystic carcinomas were negative although the difference was not statistically significant (P = 0.435).</p><p><b>CONCLUSIONS</b>MYB protein expression is a useful diagnostic marker for adenoid cystic carcinomas in its separation from other salivary gland tumors. In addition, duplication of MYB gene is no a major mechanism for the MYB protein overexpression.</p>