Endoscopic techniques for diagnosis and treatment of gastroentero-pancreatic neuroendocrine neoplasms:Where we are

BACKGROUND The correct localization of the primary tumor site and a complete histological diagnosis represent the milestones for the proper management of gastro-enteropancreatic neuroendocrine neoplasms(GEP-NENs).AIM To analyze current evidence on the role of endoscopy in the diagnosis/treatment of GEP-NENs.METHODS An extensive bibliographical search was performed in PubMed to identify guidelines and primary literature(retrospective and prospective studies,systematic reviews,case series)published in the last 15 years,using both medical subject heading(MeSH)terms and free-language keywords:gastro-enteropancreatic neuroendocrine neoplasms;endoscopy;ultrasound endoscopy;capsule endoscopy;double-balloon enteroscopy;diagnosis;therapy;staging.RESULTS In the diagnostic setting,endoscopic ultrasonography(EUS)represents the diagnostic gold standard for pancreatic NENs and the technique of choice for the locoregional staging of gastric,duodenal and rectal NENs.The diagnosis of small bowel NENs(sbNENs)has been improved with the advent of video capsule endoscopy and double-balloon enteroscopy,which allow for direct visualization of the entire small bowel;however,data regarding the efficacy/safety of these techniques in the detection of sbNENs are scanty and often inconclusive.From a therapeutic point of view,endoscopic removal is the treatment of choice for the majority of gastric NENs(type 1/2),for well-differentiated localized nonmetastatic duodenal NENs<1 cm,confined to the submucosa layer and for<10 mm,stage T1–T2,rectal NENs.EUS-guided pancreatic locoregional ablative treatments have been proposed in recent studies with promising results in order to control symptoms or reduce tumor burden in selected patients.CONCLUSION Standard axial endoscopy and EUS still play a pivotal role in several GEP-NENs.Advanced techniques for increasing the rate of R0 resection should be reserved for high-volume referral centers.

Concomitant treatment of ureteral calculi and ipsilateral pelvic sciatic nerve schwannoma with transperitoneal laparoscopic approach: A case report

BACKGROUND Schwannomas are rare peripheral neural myelin sheath tumors that originate from Schwann cells.Of the different types of schwannomas,pelvic sciatic nerve schwannoma is extremely rare.Definite preoperative diagnosis of pelvic schwannomas is difficult,and surgical resection is the gold standard for its definite diagnosis and treatment.CASE SUMMARY We present a case of pelvic schwannoma arising from the sciatic nerve that was detected in a 40-year-old man who underwent computed tomography for intermittent right lower back pain caused exclusively by a right ureteral calculus.Subsequently,successful transperitoneal laparoscopic surgery was performed for the intact removal of the stone and en bloc resection of the schwannoma.The total operative time was 125 min,and the estimated blood loss was inconspicuous.The surgical procedure was uneventful.The patient was discharged on postoperative day 5 with the simultaneous removal of the urinary catheter.However,the patient presented with motor and sensory disorders of the right lower limb,caused by partial damage to the right sciatic nerve.No tumor recurrence was observed at the postoperative appointment.CONCLUSION Histopathological examination of the specimen confirmed the diagnosis of a schwannoma.Thus,laparoscopic surgery is safe and feasible for concomitant extirpation of pelvic schwannomas and other pelvic and abdominal diseases that require surgical treatment.

Survivin:Potential role in diagnosis,prognosis and targeted therapy of gastric cancer

Survivin is a protein that is highly expressed in a vast number of malignancies,but is minimally expressed in normal tissues. It plays a role as an inhibitor of cell death in cancer cells,thus facilitating the growth of these cells. In the case of gastric cancer,survivin is over-expressed in tumor cells and plays a role in the carcinogenesis process. Several studies on gastric cancer have indicated that there is a relationship between survivin expression and the ultimate behavior of the carcinoma. Since the expression pattern of survivin is selective to cancer cells,it has been described as an "ideal target" for cancer therapy. Currently,several pre-clinical and clinical trials are on-going to investigate the effects of interfering with survivin function in cancer cells as a biologic therapy. Survivin is a potentially significant protein in the diagnosis,prognosis and treatment of gastric tumors.

Advances in early diagnosis and therapy of pancreatic cancer

BACKGROUND: Pancreatic cancer remains a devastating disease with a 5-year survival rate of less than 5%. Recent advances in diagnostic methods and therapeutic approaches have increased the possibility of improving the existing poor prognosis. DATA SOURCES: English-language articles reporting early diagnosis and therapy of pancreatic cancer were searched from the MEDLINE and PubMed databases, Chinese-language articles were from CHKD (China Hospital Knowledge Database) RESULT: The current literature about pancreatic cancer was reviewed from three aspects: statistics, screening and early detection, and therapy. CONCLUSIONS: Early detection and screening of pancreatic cancer currently should be limited to high risk patients Surgical resection is the only curative approach available, with some recent improvement in outcomes. Gemcitabine has been a standard treatment during the last decade. Gemcitabine based combination treatment, especially combined with newer molecular targeted agents, is promising. The rationale for radiotherapy is controversial, but with the recent development of modern radiation delivery techniques, radiotherapy should be intensified. Patients with borderline pancreatic cancer could benefit from neoadjuvant therapy but more evidence is needed and the best neoadjuvant regimen is still to be determined.

Some recent works on diagnosis and treatment of gastric cancer

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Diagnosis and management of autoimmune pancreatitis:Experience from China

AIM： To determine the clinical, radiographic and pathologic characteristics, diagnostic and treatment modalities in patients with autoimmune pancreatitis （AIP）. METHODS： In this retrospective study, the data of patients with diagnosed chronic pancreatitis （CP） between 1995 and 2006 in Chinese PLA General Hospital were included to screen for the cases with AIP, according to the following diagnostic criteria： （1） diagnostic histopathologic features, and abound IgG4-positive plasma cells on pancreatic tissues; （2） characteristic imaging on computed tomography and pancreatography, together with increased serum IgG, y-globulin levels or presence of autoantibodies; （3） response to steroid therapy. The clinical, radiographic and pathologic characteristics, diagnostic and treatment modalities, and outcome of AIP cases were reviewed. RESULTS： Twenty-five （22 male, 3 female; mean age 54 years, 36-76 years） out of 510 CP patients were diagnosed as AIP, which accounted for 49% （21/43） of CP population undergoing surgical treatment in the same period. The main clinical manifestations included intermittent or progressive jaundice in 18 cases （72%）, abdominal pain in 11 （44%）, weight loss in 10 （40%）, and 3 cases had no symptoms. The imaging features consisted of pancreatic enlargement, especially in the head of pancreas （18 cases）, strictures of main pancreatic duct and intrapancreatic bile duct. Massive lymphocytes and plasma cells infiltration in pancreatic tissues were showed on pathology, as well as parenchymal fibrosis. Twenty-three patients were misdiagnosed as pancreaticobiliary malignancy, and 21 patients underwent exploratory laparotomy, theremaining 4 patients dramatically responded to steroid therapy. No pancreatic cancer occurred during a mean 46-mo follow-up period. CONCLUSION： AIP patients always are subjected to mistaken diagnosis of pancreatic cancer and an unnecessary surgical exploration, due to its similarity in clinical features with pancreatic cancer. The differential diagnosis with high index of suspicion of AIP would improve the diagnostic accuracy for AIR

Techniques to resect the distal ureter in robotic/laparoscopic nephroureterectomy

Treatment of clinically-organ confined high grade urothelial carcinoma of the upper tract has historically comprised open nephroureterectomy,with the distal ureter and bladder cuff mobilized through a separate open pelvic incision.To decrease morbidity,urologists have increasingly adopted laparoscopy and robotics in performing nephroureterectomy.In many published series of laparoscopic nephroureterectomy,the distal ureter and bladder cuff are detached from the bladder endoscopically by a variation of the“pluck”technique,with the resulting bladder defect left to heal by prolonged indwelling urethral catheter drainage.While the distal ureter and bladder cuff can be excised laparoscopically,it does require advanced laparoscopic skills.With the wrist articulation and stereoscopic vision in robotic surgery,robotic nephroureterectomy(RNU)and bladder cuff excision can be performed in antegrade fashion to mimic the open technique together with the ability to intracorporeally close the bladder defect in a watertight,mucosa to mucosa fashion after excising the bladder cuff.In this review,we discuss the published minimally invasive techniques in resecting the distal ureter and bladder cuff during laparoscopic and RNU.

Pancreatic tumor:DSA diagnosis and treatment

IM To study the clinical significance of the diagnosis and catheterized interventional treatment of digital subtraction angiography (DSA) for pancreatic tumors.METHODS Ninetytwo patients with pancreatic tumor, 69 males and 23 females, aged from 41 to 70 years (mean 571 years) were diagnosed with DSA. Sixtyone patients with pancreatic cancer were treated with transcatheter celiac and superior mesenteric arterial anticancer agents (MMC 20mg, EADM 40mg and 5FU 20g) infusion (TCSAI).RESULTS The DSA diagnoses were confirmed by operations and pathological examinations, with a coincidence 826% rate of, and a therapeutic effective rate of 426%.CONCLUSION DSA is of diagnostic value for pancreatic tumors, and helpful in understanding the course of the disease, judging the prognosis and selecting the therapeutic regimen, and could improve the chemotherapeutic effect as well..

Von Brunn’s Nests in the Ureter of Two Cats

The aim of this work is to emphasize the importance of a differential diagnosis of von Brunn’s nests in cats from other urothelial neoplastic disorders, as transitional cell carcinoma, for a subsequent optimal care. Von Brunn’s nests and cysts are submucosal benign urothelial processes, related to irritative stimuli (calculi and urinary infections), characterized by an invagination with buds and clusters of normal urothelium in the lamina propria. They are common findings in the urinary bladder of human beings, but few cases have been described in dogs and cats. We report macroscopic, histopathological and, in one case, immunohistochemical features of these rare forms in the left ureters of two cats. Macroscopic evidence during surgery of ureteral nodules in two cats was accompanied by histopathological diagnosis and, in one case, by immunohistochemical assessment. Histopathology was coherent with a rare condition characterized by nests and islands of normal urothelium in the lamina propria and submucosa, with formation of cysts and moderate focal subacute inflammation. In one case epithelial cells of the nests showed well differentiated urothelial cells with an intense immunoreactivity to pan-cytokeratin (CK AE1/AE3), CK19 in the first outer layers, a slight immunoreactivity to CK20 and a low proliferative activity using MIB-1 (Ki67).

The challenging management of malignant ureteral obstruction:Analysis of a series of 188 cases

Background:Malignant ureteral obstruction(MUO)is a common condition that complicates the course of advanced malignancies.The aims of this study are to analyze the causes,management,and survival of patients with obstructive nephropathy due to malignant ureteric obstruction and to determine prognostic factors.Furthermore,we studied the complications and outcomes in patients who underwent urinary diversion.Materials and methods:A retrospective study was conducted on patients with computed tomography-confirmed MUO between January 2016 and November 2020.Demographic,clinical,radiological,laboratory,and management data were collected.Survival curves were estimated using the Kaplan-Meier method,and univariate and multivariate Cox proportional hazards models were used to test the association between parameters and survival.Results:A total of 188 patients were included.The mean age was 69.01 years(SD,14.95 years),and the majority(54.8%)were male.The most common mechanism leading to MUO was compression by a pelvic mass(36.9%),and the 3 most frequent tumors causing MUO were prostate(17.6%),bladder(16.5%),and rectal cancer(11.7%).Forty-seven patients(25%)underwent urinary diversion:23(48.9%)underwent double-J stenting and 21(44.7%)underwent percutaneous nephrostomy.The most common reason for urinary diversion was acute kidney injury(53.3%).Recovery of renal function was observed in 55.8%of the patients after urinary diversion.The most frequently identified complications after urinary diversion were urinary tract infection(24.4%),hematuria(17.0%),and urinary sepsis(14.9%).The median survival after hydronephrosis diagnosis was 6.43 months(interquartile range,1.91-14.81 months).In patients who underwent urinary decompression,the median survival after urinary diversion was 8.67 months(interquartile range,2.99-17.28 months).In the multivariate analysis,a lower grade of hydronephrosis and cancer cachexia negatively impacted survival.Conclusions:Cancer patients with MUO have a poor prognosis;therefore,the risk-benefit ratio of urinary diversion should be carefully considered.Cachexia and hydronephrosis grade can be useful in selecting suitable candidates for urinary diversion.

唾液腺肿瘤诊治研究进展

唾液腺肿瘤是最常见的口腔颌面部肿瘤之一,根据国内7所口腔医学院口腔病理科的统计资料,在69902例口腔颌面部肿瘤中唾液腺上皮性肿瘤有23010例,占32.9%[1]。唾液腺肿瘤的诊断和治疗长期以来一直是北京大学口腔医院的临床特色项目,作者曾于2015年在本刊发表《北京大学口腔医院唾液腺肿瘤研究50年回顾》一文,对2012年前的50年唾液腺肿瘤研究工作做了系统性总结[2],本文介绍近10年本课题组唾液腺肿瘤诊治研究的新进展。

Gastrinoma and Zollinger Ellison syndrome:A roadmap for the management between new and old therapies

Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,providing potential diagnostic and therapeutic algorithms.

儿童胃肠道炎性肌纤维母细胞瘤的诊断与治疗

目的总结儿童胃肠道炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的临床特征及诊治经验。方法回顾性分析湖南省儿童医院普外科2010年1月至2021年12月收治的11例胃肠道IMT患儿临床资料,男7例,女4例;发病年龄8个月至15岁,收集患儿临床特点、影像学检查、病理学诊断、外科治疗及随访情况等。结果11例主要以腹痛、呕吐、发热、血便及腹部肿物就诊。10例行一期手术完整切除肿瘤,其中1例术后予化疗;1例经活检确诊后未予手术,仅行化疗。11例均病理诊断明确,均获随访(随访时间6~60个月),其中9例治愈,2例带瘤生存(1例于术后2年复发)。结论儿童胃肠道IMT临床少见,手术是首选治疗方法。复发、难治性IMT的治疗亟待进一步积累经验。

宫颈癌肉瘤一例并文献复习

宫颈癌肉瘤(cervical carcinosarcoma)是一种由恶性的上皮组织和间质成分组成的恶性肿瘤,患者通常以不规则阴道出血、流液就诊,其病因及发病机制尚不明确,临床上十分罕见,因此容易造成误诊和漏诊。现报告1例42岁宫颈癌肉瘤的病例资料,患者因接触性出血伴下腹痛收入院,盆腔磁共振成像示宫颈部体积明显增大,初步诊断为宫颈恶性肿瘤,遂行根治性全子宫切除+盆腔淋巴结切除+大网膜切除术,术后病理学明确诊断为宫颈癌肉瘤Ⅱb期。截至2024年3月2日,患者已行3次化疗,暂未发现复发及转移。宫颈癌肉瘤恶性程度高,临床表现无特异性,确诊主要依靠病理学检查和免疫组织化学检查,因此早识别、早治疗对改善患者的预后至关重要。

MRKH综合征合并卵巢恶性肿瘤一例

MRKH综合征(Mayer-Rokitansky-Küster-Hauser syndrome)是一种较为罕见的先天性女性生殖器官发育障碍综合征,以无阴道及子宫发育不全为特征。该病患者具有发生盆腔合并症的风险,但由于其发病率低,治疗上缺乏经验总结,容易发生误诊漏诊。报告1例MRKH综合征合并巨大卵巢浆液性乳头状癌病例的诊治情况。该类患者通常拥有正常的卵巢结构,妇科医生应当警惕卵巢病变的风险,术前配合磁共振成像检查可提高诊断敏感性,通过腹腔镜手术可更清晰地明确盆腔情况同时降低手术损伤;年轻的卵巢癌患者可考虑保留健侧卵巢以保证内源性激素,而残基子宫则建议切除以降低病变风险。该例患者术后肝功能明显异常,最终予诺雷得抑制卵巢功能以择期启动化疗;卵巢癌化疗方案仍适用于该类患者且随访疗效满意。通过结合文献分析,旨在提高妇科医生对MRKH综合征患者盆腔合并症的认识和警觉。

宫颈腺样囊性癌合并基底细胞癌及鳞状细胞癌一例

腺样囊性癌(adenoid cystic carcinoma,ACC)是一种来自涎腺组织的罕见恶性上皮肿瘤,发生于宫颈的ACC更为罕见,仅占所有宫颈腺癌的1%,其侵袭性强,预后较差,且尚未建立标准治疗方案。报告1例宫颈ACC合并基底细胞癌及鳞状细胞癌患者的诊治经过。患者为老年女性,因发现阴道口新生物伴阴道间断出血收治入院,阴道内新生物病理活检提示恶性肿瘤,符合ACC诊断,遂在腹腔镜下行盆腔淋巴结清扫术+广泛全子宫切除术+卵巢动静脉高位结扎术(双侧)+双侧卵巢和输卵管切除术+肠粘连松解术+肠系膜新生物切除术,术后病理提示宫颈混合癌,混合成分为ACC、腺样基底细胞癌及鳞状细胞癌,局部伴有非特异性梭形细胞肉瘤样间质,术后行2次化疗和1次放疗。患者术后恢复良好,随访至2024年1月22日规律复查未见转移及复发。

SMARCA4基因缺失的卵巢恶性肿瘤一例

SMARCA4基因缺失的卵巢肿瘤是一种罕见的恶性程度高、预后差的恶性肿瘤,患者早期症状不典型,确诊时往往是肿瘤晚期。报告1例SMARCA4基因缺失的卵巢恶性肿瘤病例,该患者以下肢肿胀、大便次数增多等症状入院就诊。CT检查示:左侧附件区占位,伴腹膜及网膜转移,腹膜后淋巴结肿大,腹腔及盆腔积液。2023年5月收治后结合影像学检查和肿瘤标志物检查结果考虑卵巢恶性病变,排除手术禁忌证后,在全身麻醉下行腹腔镜探查和盆腔病灶活检术,发现转移灶遍布腹腔、盆腔及盆腹腔内脏器表面,术后病理提示为SMARCA4基因缺失的卵巢恶性肿瘤,后进行化疗、放疗、转移灶微波消融等治疗。术后半年随访,患者生活质量有所提高。

外阴隆突性皮肤纤维肉瘤一例

隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)是一种少见的真皮间质肿瘤,而发生于外阴的DFSP在临床上更为罕见,病理学检查结合免疫组织化学染色是明确诊断的主要方法,常以手术治疗为主。本文报告1例外阴DFSP,该患者既往于当地医院行外阴肿物切除术,术后病理检查提示DFSP,遂转往上级医院行补充治疗。经手术切除残余病灶后送病理及免疫组织化学检查,再次明确诊断为外阴DFSP,术后给予补充化疗。通过探讨该病的病因及发病机制、临床表现、病理分型、诊断、鉴别诊断、治疗、预后及随访情况,以加深临床对该病的认识。

阔韧带子宫内膜样腺癌一例

阔韧带子宫内膜样癌是罕见的特殊部位的恶性肿瘤。其发病率极低,疾病的机制、诊断和治疗尚未明确。其临床表现缺乏特异性,术前常误诊为子宫或卵巢肿瘤。报告1例65岁阔韧带子宫内膜样癌患者的诊疗经过,术前误诊为卵巢肿瘤,经手术探查和术后病理明确诊断,另行全面分期手术。术后随访7个月,病情稳定,影像学检查未见异常,肿瘤相关指标未见异常。对于妇科检查扪及附件区肿物固定,影像学提示肿物边界清楚,需要考虑阔韧带肿瘤可能,应尽早手术。经病理诊断为阔韧带恶性肿瘤,应及时全面分期手术,术后长期随访。

宫颈脂肪平滑肌瘤17例临床分析

目的:提高对宫颈脂肪平滑肌瘤(cervical lipoleiomyoma)的认识及治疗水平。方法:回顾性分析2011年9月—2023年4月首都医科大学附属北京妇产医院(我院)收治的17例经手术、病理确诊的宫颈脂肪平滑肌瘤患者的病例资料,分析其发病机制、临床表现、诊断与鉴别诊断及治疗。结果:我院17例患者平均发病年龄为(44.6±9.6)岁。患者主要表现为下腹痛或腰痛、月经量增多或无明显临床症状。全部患者行超声检查,均未提示宫颈脂肪平滑肌瘤,5例(29.4%)患者提示肌瘤变性。6例患者行盆腔磁共振成像检查,2例患者检出脂肪成分。17例患者均行手术治疗,术中见宫颈肌瘤结节,直径2.0~15.0 cm不等,术后均经病理证实为宫颈脂肪平滑肌瘤。随访3~141个月,仅1例患者术后9个月再次发现宫颈肿物,行根治性手术,术后病理证实为宫颈平滑肌瘤。结论:宫颈脂肪平滑肌瘤的发生较为罕见,缺乏特异性临床症状和影像学表现,诊断主要依靠手术探查和术后病理,治疗方法主要是手术治疗,并需要术后长期随访。