Report of a case: Retroperitoneal mucinous cystadenocarcinoma with rapid progression

IntroductionRetroperitoneal mucinous cystic neoplasms are uncommon, and little is known about the etiology of the disease. Malignant forms of these are extremely rare. Here, we report a case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC), which demonstrated unexpectedly aggressive progression despite finding only a limited area of adenocarcinoma.Presentation of caseA 62-year-old woman with a complaint of abdominal discomfort was admitted to the hospital. Abdominal CT and MRI showed multiple large retroperitoneal cysts dislocating the right kidney nearly to the center of the abdomen. Transabdominal resection of the cysts was performed. Those cysts contained 1100 ml of mucinous fluids in total. Cytological examination of those fluids revealed no malignant cells. The cyst wall was lined with mucinous epithelial cells, and contained some ovarian-type stroma. Also, there was a focal area of adenocarcinoma in the cyst wall, and the lesion was diagnosed as primary retroperitoneal mucinous cystadenocarcinoma. Eight months later, the patient developed lumbar bone metastasis. Chemotherapy with S-1, an oral fluoropyrimidine, and docetaxel had been begun immediately; however, the disease had rapidly spread in the retroperitoneum. Eventually, the patient died of the disease 15 months after surgery.DiscussionRetroperitoneal mucinous cystic neoplasms are considered to be metaplasia of embryonal coelomic epithelium. Complete excision without rupture is essential. However, variance of biological aggressiveness might exist in PRMCs.ConclusionRetroperitoneal mucinous cystadenocarcinoma is a rare tumor, and it is urgently necessary to elucidate the etiology of an effective therapy for the disease.     

Mucin-producing bile duct carcinoma originating at the hepatic hilus

We describe a mucin-producing bile duct carcinoma that was resected and analyzed pathologically. Endoscopic retrograde cholangiography disclosed amorphous filling defects in the dilated extrahepatic bile duct, suggesting that a large amount of mucin was excreted from the tumor. An intraductal amorphous filling defect showed shape change at different examination times. Pathology examination revealed a papillary tumor which showed mucus production, in the right hepatic duct. The cut section of the resected specimen showed a protuberant papillary lesion, measuring 14×14mm in diameter, forming a cystic mucous lake within the duct wall. Many cancer cells were also found in the mucous lake, the contents of which were strongly positive for mucin stain. The mucous lake developed laterally and communicated with the peribiliary glands, suggesting that the tumor had originated in these glands.     

Pulmonary mucinous cystadenocarcinoma: an extremely rare tumor presenting as a cystic lesion of the lung

Pulmonary mucinous cystadenocarcinoma (PMC) is a rare tumor characterized by mucin production. It is similar to tumors of the same name arising in the ovaries and pancreas. Here we describe the 20th case of PMC reported in the English literature. The patient was a 75-year-old woman with a 3-day history of bloody sputum. Chest radiography and computed tomography revealed a cavitary mass 5 cm in diameter in the posterior segment of the right lung. ¹⁸F-fluorodeoxyglucose positron emission tomography demonstrated intense uptake in the wall of the lesion. Right lower lobectomy was performed, and the pathology examination revealed this tumor to be a PMC. The preoperative serum CA 19-9 level was 162.3 U/ml (cutoff 37 U/ml) and decreased to 22.8 U/ml after resection. No mutation of epidermal growth factor receptor or K-ras gene was detected. Thoracic surgeons should bear in mind this rare tumor for the differential diagnosis of a pulmonary cystic lesion.     

A case of cystadenocarcinoma of the liver

A case of cystadenocarcinoma of the liver is reported. The patient was a 73-year-old woman in whom a tumor was detected in the lateral segment of the liver during a health examination. Ultrasonograms and computed tomograms showed a multilocular cystic mass. Magnetic resonance imaging (MRI) showed a multilocular lowintensity mass, including a high-intensity portion and a portal branch compressed by the tumor. MRI with gadolinium showed an enhanced cyst wall. The cystic part of the tumor became smaller and the solid part became larger over a 1-month period, indicating that the tumor was malignant. Subsegmentectomy (S₃) was performed and cystadenocarcinoma with cystadenoma was diagnosed by histopathological examination. Identification of changes in the appearance of a tumor should be helpful for the differential diagnosis of cystadenoma and cystadenocarcinoma.     

Central papillary cystadenocarcinoma of the mandible: A case report and review of the literature

INTRODUCTION

Central papillary cystadenocarcinoma of the jaw is an extremely rare tumor with only three previously reported cases in the English literature. This tumor is a histologically low-grade cancer, affecting more commonly in the mandible than in the maxilla.

PRESENTATION OF CASE

A 65-year-old woman presented with a two months history of a rapidly growing, painless mass of the right ascending ramus of the mandible. The pathologic report from incisional biopsy was a papillary cystic tumor with a differential diagnosis of cystadenoma versus cystadenocarcinoma. Segmental mandibulectomy, parotidectomy and submandibular gland resection were performed. The final pathology was intraosseous papillary cystadenocarcinoma.

DISCUSSION

Clinical features of central papillary cystadenocarcinoma of the mandible mimic an odontogenic lesion and metastatic bone disease, careful review of radiograph and pathology should be done. Surgical excision with wide margins is the appropriate treatment. Postoperative radiation therapy should be considered in histologically aggressive or high-stage tumor.

CONCLUSION

This is the fourth case of central papillary cystadenocarcinoma of the mandible in the English literature. Although it is usually a low-grade cancer, en bloc resection with adjuvant postoperative radiotherapy in a high-stage disease, and long-term follow-up allow the patient to have a favorable prognosis.     

Role of frozen section assessment for intraductal papillary and mucinous tumor of the pancreas

Intraductal papillary mucinous neoplasms (IPMN) of the pancreas include a spectrum of dysplasia ranging from minimal mucinous hyperplasia to invasive carcinoma and are extensive tumors that often spread along the ductal tree. Several studies have demonstrated that preoperative imaging is not accurate enough to adapt the extent of pancreatectomy and have suggested routinely using frozen sectioning (FS) to evaluate the completeness of resection and also to check if ductal dilatation is active or passive, in order to avoid an excessive pancreatic resection. Separate main duct and branch duct analysis is needed due to the difference in the natural history of the disease. FS accuracy averages 95%. Eroded epithelium on the main duct, severe ductal inflammation mimicking dysplasia and reactive epithelial changes secondary to obstruction can lead to inappropriate FS results. FS results change the planned extent of resection in up to 30% of cases. The optimal cut-off leading to extend pancreatectomy is not consensual and our standard option is to extend pancreatectomy if FS reveals: (1) at least IPMN adenoma on the main duct; or (2) at least borderline IPMN on branch ducts; or (3) invasive carcinoma. However, the decision to extend resection must be taken after a multidisciplinary discussion since it does not exclusively depend on the FS result but also on age, general condition and expected prognosis after resection. The main limitation of using FS is the existence of discontinuous ("skip") lesions which account for approximately 10% of IPMN in surgical series and can lead to reoperation in up to 8% of cases.     

Intrahepatic bile duct dilatation with a liver cyst and hemangioma: Report of a case

We report a case of intrahepatic bile duct dilatation with a liver cyst and hemangioma. A 58-year-old woman was referred for investigation of a cystic lesion and peripheral intrahepatic bile duct dilatation in the left lateral segment of the liver. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) showed dilatation of the intrahepatic bile ducts in the left lateral segment, near a 4.5-cm cystic lesion. Early enhancement into the delayed phase was seen in the dorsal part of the cystic lesion. Celiac angiogram showed a belt-shaped hypervascular area, but no encasement or irregularity of the artery. To exclude malignancy, we performed a left lobectomy. Microscopically, the cyst was lined with a single layer of flattened epithelial cells and a spongy tumor was diagnosed as cavernous hemangioma, which compressed the bile duct. The histopathological diagnosis was biliary stenosis associated with cavernous hemangioma of the liver. Invasive surgery may be avoided by awareness of this unusual benign pathology.     

Carcinoma of the cystic duct protruding into the common bile duct: Report of a case

We report herein the case of 65-year-old man in whom a diagnosis of primary carcinoma of the cystic duct was made on the basis of Farrar's criteria. The patient was admitted with upper abdominal pain, and although there was no evidence of jaundice or a palpable mass, there was tenderness in his right upper quadrant. Carcinoma of the cystic duct was suspected on the basis of computed tomography and magnetic resonance imaging findings. Cholecystectomy with resection of the bile duct and lymph node resection was performed, and percutaneous transhepatic cholangiography revealed a filling defect in the common bile duct (CBD). The tumor was found to have arisen from the cystic duct and demonstrated papillary growth into the CBD intraluminally through the orifice of the cystic duct. Microscopically, the tumor was identified as papillary adenocarcinoma with invasion limited to the subserosal layer of the cystic duct. There were no signs of nodal metastasis.     

Neuroendocrine tumor of the small intestine diagnosed with trans-abdominal ultrasonography: A case report

IntroductionTumors of the small intestine are rare. In addition, clinical symptoms are nonspecific and neoplasm-related symptoms occur late. We report a case of neuroendocrine tumor (NET) of the small intestine that was diagnosed early with trans-abdominal ultrasonography (US).Presentation of caseThe patient was a 61-year-old man. Abdominal contrast-enhanced computed tomography (CT) was performed because the patient complained of abdominal pain. The CT showed a tumor lesion in the mesentery. Trans-abdominal US was undertaken to evaluate this tumor lesion, and a tumor lesion of the small intestine was found nearby. A diagnosis of lymph-node metastasis of a small-intestine tumor was made as a preoperative diagnosis. A laparotomy was performed with partial resection of the ileum, together with the small-intestine mesentery including an enlarged lymph node. Histological examination revealed NET of the ileum and lymph-node metastasis.DiscussionWith the application of trans-abdominal US, we could diagnose lymph-node metastasis of a small-intestine tumor relatively early and before surgery.ConclusionTrans-abdominal US is useful in the diagnosis of small-intestine NET.     

Endoscopic band ligation of small gastric stromal tumors and follow-up by endoscopic ultrasonography

Background Gastrointestinal stromal tumor (GIST) is a relatively common gastric submucosal tumor with potential for malignant transformation. The efficacy of a new method for resection of these tumors, endoscopic band ligation, was evaluated. Methods The study included 29 patients with small gastric stromal tumors arising in the gastric muscularis propria as determined by endoscopy, endoscopic ultrasonography (EUS), and deep endoscopic biopsies. A standard endoscope with a transparent cap attached to the tip was used. The cap was placed over the lesion, maximum sustained suction was applied, and an elastic band was released around the base. Beginning two weeks after banding, the lesions were observed endoscopically once per week until healing was complete. Thereafter, all patients underwent EUS every two to three months on schedule. Results The 28 GISTs sloughed completely. The mean time required for complete healing after band ligation was 4.8 weeks. One lesion did not slough because they were not completely ligated. The lesion was ligated for the second time and sloughed completely. Bleeding occurred in one patient three days after ligation because the lesion sloughed early. The bleeding was managed successfully with metallic clips. No perforation and other complications occurred. Followup ranged from 36 to 51 months, during which time only one recurrence was observed four months postoperatively. Conclusions Endoscopic band ligation with systematic followup by EUS is an effective and safe treatment for small GISTs.     

Malignant Carcinoid Tumor of the Common Bile Duct: Report of a Case

Carcinoid tumors of the extrahepatic bile duct are exceedingly rare and account for between 0.1% and 2% of all gastrointestinal carcinoid tumors, with most reported cases arising from the gallbladder. We herein present what we believe is only the 47th reported case of a primary carcinoid tumor occurring in the extrahepatic bile ducts. A 67-year-old woman sought treatment for obstructive jaundice accompanied by epigastric pain. Laboratory and imaging studies gave results that were consistent with a malignant obstruction in the common bile duct. We performed a pyrolus-preserving pancreaticoduodenectomy. Pathologically, an ill-demarcated mass was noted in the common bile duct measuring 1.6 × 1.5 × 0.5 cm in size. The tumor had invaded the adjacent pancreatic tissues. Immunohistochemically, the mass demonstrated chromogranin, synaptophysin, and CD56 positivity. The final pathologic diagnosis was well-differentiated carcinoid tumor of a malignant nature. The patient, who underwent a curative surgical resection, was alive and disease-free at the time of writing.     

The benefit of curative liver resection with a selective bile duct preserving approach for hepatocellular carcinoma with macroscopic bile duct tumor thrombus

BackgroundHepatocellular carcinoma (HCC) presenting with macroscopic bile duct tumor thrombus (BDTT) is an uncommon event. The role of a curative hepatic resection and associated long-term outcomes remain controversial. In addition the necessity for bile duct resection is still unclear. The aim of this study was to evaluate outcomes of hepatectomy with a selective bile duct preservation approach for HCC with BDTT in comparison to outcomes without BDTT.MethodsA total of 22 HCC with BDTT patients who had undergone curative hepatic resection with a selective bile duct preservation approach at our institute were retrospectively reviewed. These were compared to group of 145 HCC without BDTT patients. The impact of curative surgical resection and BDTT on clinical outcomes and survival after surgical resection were analyzed.ResultsAll HCC with BDTT cases underwent major hepatectomy vs. 32.4% in the comparative group. Bile duct preservation rate was 56.5%. The 1-, 3- and 5-year survival rates of HCC with BDTT patients in comparison to the HCC without BDTT group were 81.8%, 52.8% and 52.8% vs. 73.6%, 55.6% and 40.7% (P=0.804) respectively. Positive resection margin, tumor size ≥5 cm and AFP ≥200 IU/mL were significant risk factors regarding overall survival. However, it is unclear whether presence of a bile duct tumor thrombus has an adverse impact on either recurrence free survival or overall survival.ConclusionsBile duct obstruction from tumor thrombus did not necessarily indicate an advanced form of disease. Tumor size and AFP had greater impact on long-term outcomes than bile duct tumor thrombus. Major liver resection with a selective bile duct preserving approach in HCC with BDTT can achieve favorable outcomes comparable to those of HCC without BDTT in selected patients.     

Composite glandular-neuroendocrine carcinoma of the hilar bile duct: Report of a case

4 , S₅) and bile duct resection with lymphnode dissection were performed. A tumor measuring 6.0 3 3.0 cm was found to be located in the bile duct of the hepatic hilus. Histologically, the tumor was composed of well-differentiated adenocarcinoma and small cell neuroendocrine carcinoma cells, with a histological transition between the two components. Grimelius' method revealed the presence of diffuse positive tumor cells in neuroendocrine carcinoma. The neuroendocrine tumor cells were also diffusely immunoreactive to chromogranin A. To the best of our knowledge, only 22 previous cases of composite glandular-neuroendocrine carcinoma in the biliary tract have been reported; however, this is the first case report of a clearly composite tumor of the hilar bile duct. (Received for publication on Aug. 19, 1996; accepted on May 12, 1997)     

Low-Grade Intraductal Carcinoma of the Parotid Gland: A Case Report and Literature Review

Low-grade intraductal carcinoma is a rare neoplasia with an excellent prognosis, previously classified as low-grade cribriform cystadenocarcinoma and low-grade salivary duct carcinoma. The tumor mainly occurs in the parotid gland and presents a ductal phenotype and an intraductal/intracystic growth pattern. It resembles intraductal breast lesions such as atypical ductal hyperplasia, papillary and cribriform ductal carcinoma in situ. Despite its infrequency, discriminating low-grade intraductal carcinoma from other salivary gland tumors is crucial, especially because of its favorable prognosis. A 74-year-old woman with a history of neurofibromatosis underwent a superficial parotidectomy to remove a sharply demarcated multi-cystic mass, diagnosed as category 4 at FNAC. The histological examination revealed a demarcated but unencapsulated lesion composed of a bigger cyst surrounded by several smaller cysts, lined by a monolayer or bilayer epithelium alternated with a cribriform proliferation, characterized by “Roman-bridges”, with occasional micro-papillae. A myoepithelial component, with a basal disposition, was present, confirmed by intense staining for protein p63 and SMA. Immunohistochemical stains showed intense, strong uniform positivity for pan-cytokeratin, protein S100, and SOX10. The Ki67 proliferation index was low (< 10%). A diagnosis of Low-grade Intraductal Carcinoma (LGIC) of the parotid was made. We performed a literature search in PUBMED for “Intraductal carcinoma”, “Low-grade Intraductal Carcinoma”, “Cribriform Cystadenocarcinoma”, “Salivary Duct Carcinoma”, and “Low-Grade Salivary Duct Carcinoma”. We selected 17 papers published between 1983 and 2020; the most affected anatomical site was the parotid gland (77/90), followed by minor salivary glands (6/90), the intraparotid lymph nodes (3/90) and the submandibular gland (4/90). Their main histopathological features are reported in the paper. Here we present a case report and a review of scientific literature on this topic to provide some essential diagnostic tools to discriminate this rare entity.     

Testicular Metastases from Carcinoma of the Bile Duct: A Case Report

A 41 -year-old man was admitted to our hospital with chief complaints of painless tumor in the left scrotal contents and loss of body weight. As the tumor was suspected of being a neoplastic lesion, left radical inguinal orchiectomy was carried out. Histopathological examination showed metastatic adenocarcinoma of the bile duct. Metastatic carcinoma to the testicle, epididymis, and their tunics is unusual and testicular metastases from adenocarcinoma of the bile duct is extremely rare. A case of testicular metastases from carcinoma of the bile duct is reported and the literature reviewed.     

腹腔镜手术治疗阑尾粘液性肿瘤的临床体会(附11例报告)

目的:探讨腹腔镜手术治疗阑尾粘液性肿瘤的优势及作用。方法:回顾分析2010年5月至2016年10月收治的11例阑尾粘液性肿瘤患者的临床资料。结果:11例患者术前均拟诊为急性阑尾炎行腹腔镜手术治疗,经腹腔镜探查后术中均明确诊断,确诊率100%,8例行腹腔镜阑尾切除术,2例行腹腔镜右半结肠切除术,1例经腹腔镜探查后放置区域热循环治疗灌注管,术后应用洛铂及雷替曲塞等化疗药物行腹腔区域循环热灌注化疗3个疗程。术后均未出现严重并发症,11例均痊愈或好转出院。手术时间30~120 min,平均(48.18±31.64)min;出血量20~100 ml,中位数35 ml;肛门排气时间平均(2.09±1.04)d;住院3~8 d,平均(4.27±1.90)d,无中转开腹及围手术期死亡病例。结论:阑尾粘液性肿瘤罕见,无临床特异性,临床误诊率较高。腹腔镜手术治疗阑尾粘液性肿瘤探查确切,患者创伤小,术后康复快,住院时间短,是安全、有效的治疗手段。     

超声内镜对胰腺囊性肿瘤的诊断与治疗的应用进展

随着对胰腺囊性肿瘤认识的提高及影像学诊断仪器的普及,该病的检出率显著提高。由于胰腺囊性肿瘤种类繁多,病理分级可为良性、低度恶性及恶性,而且随着肿瘤体积的增大,病理分级也会不断改变。良性病变的准确诊断对于确定手术方式或内镜治疗的监测需求,以及避免对无潜在恶性的囊肿进行不必要的手术显得尤为重要。近年来,超声内镜(EUS)在胰腺囊性肿瘤的诊断及治疗方面取得了很大进展,本文综述了超声内镜对胰腺囊性肿瘤的诊断与治疗进展。     

Perioperative outcome of laparoscopic left lateral liver resection is improved by using staple line reinforcement technique: A case report

Current laparoscopic stapling technology still cannot prevent bile leakage (<55%) along the resection margin. A new staple line reinforcement technique was used in the present case. This technique incorporates an absorbable polymer membrane into the stapler system, such that it buttresses the transected solid organ. The objectives of this novel procedure are to decrease hemorrhage at the staple line and to prevent bile duct leakage after liver resection. A 47-year-old man followed for status post biliopancreatic diversion with duodenal switch presented with epigastric pain. On imaging, he was found to have a lesion in segment 2–3 of the left lobe of the liver, which measured at least 3 cm in diameter. He was admitted to the hospital to undergo a laparoscopic left lateral liver resection. This procedure involved laparoscopic ultrasonography of the liver and transection of the left liver lobe with endoscopic linear staplers. The staple height of 3.5, 60 mm long, reinforced with an absorbable polymer membrane was used for liver transection to catch the portal branches. This required multiple firings in the liver parenchyma and additional division of some tissue using the Harmonic scalpel. The larger branch in the middle of segment 2–3 and the left hepatic vein were both transected with the novel staple line reinforcement technique. Bleeding or any bile leakage in this area could not be visualized. No drains were left. The patient’s postoperative course was uncomplicated, and he was discharged on postoperative day 3. Pathology results showed a cavernous hemangioma of 4.5 cm in diameter. Staple line reinforcement with the absorbable polymer membrane has the potential to decrease staple line hemorrhage and bile leakage.