Duodenal duplication cysts in adults are rare, and a preoperative diagnosis remains difficult because clinical manifestations are nonspecific and variable. We describe a case of a duodenal duplication cyst mimicking a pancreatic pseudocyst with repeated intracystic hemorrhage.A 47-year-old male who complained of upper abdominal pain and vomiting was referred to our hospital. He was a heavy drinker and had a past history of hospitalization for alcoholic chronic pancreatitis. Plain abdominal computed tomography (CT) showed a cystic lesion of 7 cm in size in the lumen near the second part of the duodenum. The cystic lesion showed high density inside. Gastrointestinal endoscopy revealed that the lumen of the duodenum was deformed by a submucosal tumor-like mass and the endoscope could not pass through it, but active bleeding was not seen in the lumen of the duodenum. On the fourth day of hospitalization, anemia progressed and contrast-enhanced CT demonstrated a high-density spot on the wall of the cystic lesion. A pancreatic pseudocyst complicated with intracystic hemorrhage was preliminarily considered. Angiography was immediately performed, and a pseudoaneurysm was identified in the branch of the anterior superior pancreaticoduodenal artery (ASPDA). Transcatheter arterial embolization (TAE) was performed. Anemia did not progress after that, and follow-up CT showed reduction in the size of the cystic lesion. Afterward, the same symptoms recurred twice and surgical treatment was performed for the pancreatic pseudocyst with repeated intracystic hemorrhage. Macroscopically, a cystic mass of 5 cm in size was adjacent to the second part of the duodenum on the pancreas side. A pinhole-sized communication was identified between the cyst and the duodenum lumen. Microscopically, the cyst wall was composed of gastric mucosa and shared a common proper muscle layer with the duodenum. Chronic ulcers and erosions were seen in the cyst. Based on these findings, a diagnosis of duodenal duplication cyst was made.Duodenal duplication cysts in adults have seldom been reported and should be considered as a differential diagnosis for a patient with a cystic lesion adjoining the duodenum. Duplication cysts are rare congenital anomalies of the gastrointestinal tract. Duodenal duplication cysts are rare, accounting for only 5 to 7% of gastrointestinal tract duplication cysts . They arA 47-year-old male who complained of upper abdominal pain and vomiting was referred to our hospital. He was a heavy drinker and had a past history of hospitalization for alcoholic chronic pancreatitis. Laboratory data revealed elevated levels of amylase 245 IU/L), CRP (14.99 mg/dl), and white blood cell count (14900/μL). Plain abdominal computed tomography (CT) showed a cystic lesion of 7 cm in size in the lumen near the second part of the duodenum. The cystic lesion showed high density inside. The pancreas was slightly enlarged, and the main pancreatic duct was dilated. Calcifications were seen in the uncus of the pancreas . After TAE, the size of the cyst decreased and symptoms were relieved. However, the same symptoms recurred 2 months later. We obtained informed consent for surgical treatment, and we performed subtotal stomach-preserving pancreatoduodenectomy (SSPPD). Intraoperatively, severe inflammatory adhesion was noted around the pancreas head and the border between the pancreas and the cystic lesion was unclear. Macroscopically, a cystic mass of 5 cm in size was adjacent to the second part of the duodenum on the pancreas side and was close to the ampulla Fig. a. A pinhDuplication cysts are rare congenital anomalies that can occur anywhere along the alimentary tract, and they are defined by the presence of epithelial mucosal lining, a well-developed smooth muscle layer, and an intimate attachment to the native gastrointestinal tract . DuplicaPreoperative diagnosis of a duodenal duplication cyst is difficult. CT is useful for determining the size and location of a cystic lesion. MRCP and endoscopic retrograde cholangiopancreatography (ERCP) are useful for visualizing the pancreaticobiliary tract and its relationship to the cystic lesion. However, all of the modalities only enable suspicion of the presence of an abnormal lesion, and the diagnosis is confirmed by laparotomy and pathologic results. Recently, endoscopic ultrasonography (EUS) has been reported as safe and accurate for diagnosis of duplication cysts. The typical appearance on sonographic examination is a “muscular rim sign” manifesting as inner echogenic mucosa and outer hypoechoic muscle layer. Muscular peristalsis of the cyst wall noted on EUS helps distinguish duplication cyst from other abdominal cysts , 9. UnfoDifferential diagnosis can include any cystic masses in the pancreaticoduodenal region. Among them, special attention is needed for choledochoceles, intraluminal duodenal diverticulum, and pancreatic pseudocyst . A choleIn our case, the duplication cyst had a pinhole-sized communication with the native duodenum lumen, and the pinhole size was too small to be observed by endoscopy. Upper gastrointestinal series demonstrated a filling defect in the duodenum, but the inside of the cystic lesion was not contrasted. We regarded this radiographic finding as severe compression by the pancreatic pseudocyst outside of the duodenum. In addition, the patient had a past history of alcoholic chronic pancreatitis. Based on these findings, we had no doubts that the cystic mass was a pancreatic pseudocyst associated with chronic pancreatitis before surgery. Consequently, a high-density spot within the cyst that was demonstrated by contrast-enhanced CT was thought to be intracystic hemorrhage. At first, TAE was performed for hemostasis. After all, surgical resection was performed because intracystic hemorrhage repeated after TAE. Pathological examination revealed that the cystic lesion was a duodenal duplication cyst and that the hemorrhage resulted from peptide ulceration of the ectopic gastric mucosa within the cyst. The mechanism why the cyst was decreased in size after TAE is thought that hemorrhage within the cyst was stopped by TAE and the accumulated blood in the cyst was drained from a pinhole-sized communication into duodenum lumen and the cyst resulted in shrinkage.Optimal treatment for a duodenal duplication cyst is complete resection, thus preventing any further risk that may arise from residual ectopic gastric mucosa as well as the possibility of the duplication cyst becoming malignant . SurgicaWe described a rare case of a duodenal duplication cyst mimicking a pancreatic pseudocyst with intracystic hemorrhage. Duodenal duplication cysts in adults have seldom been reported and should be considered as a differential diagnosis for patients with a cystic lesion adjoining the duodenum.


Peritoneal carcinomatosis is sometimes diagnosed by cytology or by biopsy, but routine radiologic and endoscopic workup may not reveal a primary site. This clinical condition is called adenocarcinoma of unknown primary site.The clinical findings of two patients with adenocarcinoma of unknown primary site were presented and the implications for management of similar patients were suggested.The patient had the diagnosis of adenocarcinoma of unknown primary site made when cancer was discovered at the time of umbilical hernia repair or laparoscopy. Radiologic and endoscopic tests were not definitive in providing the primary site for the malignancy. Exploratory laparotomy was performed which revealed an appendiceal mucinous adenocarcinoma as the cause of the peritoneal carcinomatosis. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) were used at the time of the exploratory laparotomy to treat the appendiceal adenocarcinoma with peritoneal metastases.Peritoneal carcinomatosis of unknown primary site may be caused by an occult appendiceal adenocarcinoma. This error in diagnosis may lead to suboptimal treatment. Surgical exploration to visualize a normal appendix should occur prior to making a definitive diagnosis of peritoneal carcinomatosis of unknown primary site. The primary tumor is identified and then definitive biopsy is performed. In some patients the first symptom or sign of malignancy may be peritoneal carcinomatosis. Usually, the primary cancer site is identified by radiologic or endoscopic procedures and definitive biopsy obtained. In some unusual patients peritoneal metastases are documented but the primary site for the disease cannot be determined. The ascites fluid or peritoneal nodules containing the cancer cells are documented establishing the carcinomatosis but the primary site of the process is not discovered by conventional diagnostic tests. This is then carcinomatosis of unknown primary site ,2.www.researchregistry.com website with UIN 4989.Data on these two patients was prospectively recorded and then retrospectively reviewed at an academic institution. This research work has been reported in line with the PROCESS criteria . This st22.1In May 2014, a 35 year old male had a new onset umbilical hernia repaired. Approximately one liter of mucinous fluid drained when the peritoneal space was entered. Biopsy from the hernia sac showed mucinous adenocarcinoma. Immunohistochemistry suggested origin in the gastrointestinal tract. Upper and lower gastrointestinal endoscopy was negative. CT showed ascites. The patient was given a presumptive diagnosis of adenocarcinoma of unknown primary site. He was started on 5-fluorouracil, leucovorin and oxaliplatin (FOLFOX) chemotherapy and completed six cycles over 3 months [In October 2014, CT showed a reduced volume of ascites. The patient was asymptomatic. He was taken to the operating room with plans for exploratory laparotomy with possible complete cytoreductive surgery and perioperative intraperitoneal chemotherapy with curative intent ,6. The p2 for 3 h was used [The right side of the abdomen was clearly exposed. The caecum and ascending colon including terminal ileum were reflected from left to right to clearly expose the retroperitoneum. By visual inspection the appendix was absent and thought to have been destroyed by adenocarcinoma. A stapler was used to divide the caecum just below the ileocaecal valve and this specimen was sent separately to pathology to look carefully for remnants of a primary appendiceal mucinous adenocarcinoma. Other palliative surgical procedures were a greater omentectomy and tumor debulking. A palliative intraperitoneal chemotherapy regimen of hyperthermic liposomal doxorubicin at 100 mg/mwas used .Pathology specimens all showed metastatic well differentiated mucinous adenocarcinoma. The specimen of caecum and appendix showed a mucinous adenocarcinoma of the appendix with peritoneal metastases. It was considered by the pathologist as the primary site for the disease. Five lymph nodes within the specimen were negative for cancer.The patient received additional FOLFOX chemotherapy. He is alive with disease in June of 2019.2.2In October 2014, a 53 year old male began complaining of increasingly severe mid-abdominal pain. A CT, MRI and then laparoscopy were performed. The laparoscopy showed widespread mucinous adenocarcinoma. No primary site for the cancer was seen at the time of laparoscopy. Biopsies showed atypical cells suspicious for a gastrointestinal malignancy. Colonoscopy and upper gastrointestinal endoscopy were negative.In November 2014, the patient was started on FOLFOX chemotherapy and he received 7 cycles (4). CEA was reduced from 125 prior to chemotherapy and 8 after its completion. On June 22, 2015, a repeat CT scan was performed which showed a reduction in the peritoneal metastases. It was thought that there was a complete or near complete response to systemic chemotherapy. On July 21, 2015, the patient was taken to the operating room where he had an exploratory laparotomy through a long midline abdominal incision. A greater omentectomy, cholecystectomy, and right colon resection with anastomosis was performed. The abdominal exploration showed fibrosis of the undersurfaces of the right and left diaphragm, right paracolic sulcus and pelvis. The small bowel was traced from the area of the ligament of Treitz to the ileocaecal valve. No obvious tumor masses were present on the stomach or large bowel. In reflecting the right colon from right to left, a diffuse mass was noted in the region of the appendix. The right colon and its adjacent lymph nodes were widely resected.Upon histopathological examination the resected greater omentum showed foci of acellular mucus. The right colon and terminal ileum contained a high grade mucinous adenocarcinoma involving the wall of the appendix and periappendiceal tissues. Sixty-one lymph nodes were present in the specimen and all were negative for cancer. A Meckel’s diverticulum present in the terminal ileum was resected as part of the right colon specimen.2, mitomycin C 15 mg/m2, and 5-fluorouracil 400 mg/m2 and leucovorin 20 mg/m2 intravenously (6). Postoperatively the patient received early postoperative intraperitoneal chemotherapy with 5-fluorouracil for 4 days at 600 mg/m2 (6).The patient received hyperthermic intraperitoneal chemotherapy with doxorubicin 15 mg/mThe patient recovered without adverse events. He received an additional 5 cycles of FOLFOX chemotherapy. He is alive without evidence of disease in June of 2019.33.1In the past a diagnosis of carcinomatosis of unknown primary site required a positive cytological biopsy, negative radiologic tests, usually an abdominopelvic CT, and negative upper and lower gastrointestinal endoscopy ,2. In th3.2There are several characteristic features of an appendiceal adenocarcinoma that result in great difficulties in diagnosis in the absence of exploratory laparotomy. Of course, an upper gastrointestinal endoscopy will be negative. Also, colonoscopy to the caecum is usually negative unless the adenocarcinoma is at the orifice of the appendix. For perforation and peritoneal seeding to occur, the adenocarcinoma is usually not at the appendiceal orifice but somewhere along the shaft of this structure. Here perforation would occur into the free peritoneal cavity with widespread dissemination of cancer cells into the peritoneal space. CT may be negative because of the small size of the primary tumor. An appendiceal adenocarcinoma, especially a mucinous malignancy, may cause extensive peritoneal metastases in the absence of a mass identified on CT. If the appendiceal primary adenocarcinoma is retrocaecal, especially if there is surrounding inflammation, laparoscopy may not be able to visualize the lesion. Finally, the occult primary appendiceal adenocarcinoma may not cause any symptoms and signs. The perforation of the appendix by cancer may occur in the absence of pain or localized tenderness typical of appendicitis. Bleeding from the primary cancer never seems to occur as is frequently observed in gastric cancer, small bowel adenocarcinoma, or colon cancer. Finally, because the malignancy is outside of the passage of enteric contents, bowel obstruction does not occur.3.3Other unusual malignancies may present with peritoneal metastases and a negative diagnostic workup. Ectopic gastric or pancreatic epithelium in a Meckel’s diverticulum may present as peritoneal metastases . Also, a3.4The surgeon who is responsible for the exploratory surgery must be prepared to definitively treat the peritoneal metastases. Knowing that a definitive diagnosis of an appendiceal primary cancer may occur, an appropriate treatment strategy needs to be available prior to exploration. A new standard of care for appendiceal neoplasms with peritoneal metastases has been reported and is globally accepted . Cytored3.5At the time of exploratory laparotomy, peritoneal carcinomatosis of unknown primary site should not be used as a diagnosis unless a normal appendix is visualized. The appendix cancer may not be obvious at the time of surgical exploration. A high grade appendiceal adenocarcinoma may totally destroy the appendix and peritoneal metastases occupy the anatomic site for the normal structure. Realizing this, the diagnosis of peritoneal carcinomatosis of unknown primary site is made only if a normal appendix is visualized. An absence of the appendix indicates appendiceal cancer that has destroyed the normal structure. This indicates an invasive process and a guarded prognosis may occur even with adequate treatment.In order to gain maximal information regarding the primary site, the appendix or the caecum and appendix must be resected including as much surrounding tumor as possible. Sometimes a right colon resection is indicated. The tissue specimen that contains a possible occult appendiceal malignancy must be oriented for the pathologist with specific instructions that there is a high index of suspicion that the cancerous mass may contain the primary cancer. If the tissue from the anatomic site of the appendix and caecum is sent along with a large cancerous mass from greater omentum and areas of tumor debulking, the occult appendix cancer is most likely to be missed by pathologic examination.4In order to optimize the treatment of cancer patients, an accurate diagnosis of the site of primary disease is necessary. The diagnosis of peritoneal carcinomatosis of unknown primary site is a diagnosis of exclusion. All possible primary sites for the disease need to be ruled out. An occult appendiceal adenocarcinoma may perforate and seed the abdomen with peritoneal metastases. An absence of symptoms and an absence of definitive radiologic and endoscopic tests may exist with appendiceal adenocarcinoma. An exploratory laparotomy and visualization of a normal appendix is a requirement to preserve a diagnosis of peritoneal carcinomatosis of unknown primary site.Foundation for Applied Research in Gastrointestinal Oncology.Data management and secretarial support provided by Local IRB-approval for this case report was not required:MedStar Health Institutional Review Board has determined that a case report of less than three (3) patients does not meet the DHHS definition of research (45 CFR 46.102(d)(pre-2018)/45 CFR 46.102(l)(1/19/2017)) or the FDA definition of clinical investigation (21 CFR 46.102(c)) and therefore are not subject to IRB review requirements and do not require IRB approval.This case series is of 2 patients.Written and signed consent was obtained from the patients.Paul H. Sugarbaker, MD: study concept or design, data collection, data analysis or interpretation, writing the paper.www.researchregistry.com website with UIN 4989.This study was registered as a case series on the Paul H. Sugarbaker, MD.Not commissioned, externally peer-reviewed.Paul H. Sugarbaker has no conflicts of interest to declare.


Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), is rarely reported in the liver as a primary site. We report a case of a previously healthy 56-year-old male, who presented with abdominal pain and jaundice. The patient was originally diagnosed with cholecystitis, treated with cholecystectomy, which was complicated by abdominal abscess. One week following discharge, the patient was readmitted with fever, chills, and leukocytosis. Computed tomography (CT) guided liver biopsies demonstrated an epithelioid to spindle cell neoplasm with markedly atypical nuclei and prominent necrosis infiltrating between hepatocytes. Immunohistochemical studies were negative for epithelial, melanocytic, and hematolymphoid differentiation. Positron emission tomography (PET) was performed, which showed a single markedly hypermetabolic central hepatic mass (14 x 8.5 x 8.5 cm) with likely central necrosis, consistent with primary malignancy. The patient was treated with one cycle of chemotherapy (doxorubicin and ifosfamide), refusing additional cycle due to medication side effects. The patient subsequently succumbed to complications associated with the malignancy and died within 19 days of diagnosis. Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), was first reported in 1964 by O'Brien and Stout . It has The first case of primary hepatic UPS was described in 1985 . Fewer t9/L (N: 4.5-11.0 x 109/L), Hb: 9.9 g/dL (N: 13.5-17.5 g/dL), serum Na+ 129 mEq/L (N: 135-145 mEq/L), serum K+ 3.4 mEq/L (3.5-5.0 mEq/L), serum albumin: 2.1 g/dL (N: 3.5-5.0 g/dL), lipase 303 U/L (N: 0-50 U/L), and AST/ALT 93/97 U/L (N: AST/ALT: 8-20/8-20 U/L). The patient was discharged on antibiotics after three weeks of treatment. One week later, he developed a fever, chills, and leukocytosis. He was readmitted into hospital. Abdominal CT showed multiple fluid collections within the liver parenchyma with the largest one being 2.2 x 2.0 cm in size. A CT guided liver biopsy of the presumed abscess was performed.A previously healthy 56-year-old man suffering from abdominal pain and jaundice was admitted with an initial clinical diagnosis of acute cholecystitis. The patient underwent an endoscopic retrograde cholangiopancreatogram (ERCP) and cholecystectomy. His symptoms did not improve and repeat imaging study indicated common bile duct narrowing. A 20 x 3.5 cm perihepatic abscess was found that required drainage and he underwent percutaneous transhepatic cholangiography (PTC) and biliary drainage. The cytologic examination was not performed on the drained material. Laboratory studies at that time revealed the following: WBC: 51.4 x 10The biopsy showed an epithelioid to spindle cell neoplasm infiltrating between hepatocytes with markedly atypical nuclei and prominent necrosis Figures . The tumBased on the inconclusive findings, a second liver biopsy was performed. The morphology was similar to the prior biopsy. Further staining was performed. The tumor cells were also negative for HMB-45, CD15, CD20, CD21, CD23, CD43, CD45, desmin, myogenin, calretinin, myeloperoxidase, D2-40, CD68, and clusterin . Based oFollowing the biopsies, the physician in charge ordered a PET/CT after reviewing the biopsy results in order to evaluate tumor size and potential metastasis . A largeThe patient was treated with one cycle of chemotherapy (adriamycin and ifosfamide) which caused severe confusion and further treatment was refused. The patient expired within 19 days of diagnosis.UPS refers to a group of pleomorphic sarcomas that lack any specific line of differentiation . In factPrimary hepatic UPS is a tumor of late adulthood with a mean age of 58 . SymptomGrossly, UPS is often white to pale yellow, with central hemorrhage and necrosis . The tumClinically, in the present case, possible peritoneal dissemination was noted and was suggested by the peritoneal implants in the PET image . CommonlFor the origin of UPS cells, many scientists and pathologists believe the “dedifferentiation theory.” In this model it is presumed that varying tumors with shared similar morphologic features become progressively more undifferentiated, ultimately resulting in a high grade undifferentiated pleomorphic sarcoma –16. OtheSeveral studies suggested that UPS and pleomorphic leiomyosarcoma potentially share a linage due to significant similarities between them , 18. BöhResearchers also compared UPS with myogenic differentiation (MD) and non-MD UPS in soft tissue hypothesizing that UPS with MD would be more aggressive than non-MD UPS as pleomorphic sarcoma would suggest. However, no survival rate difference was demonstrated between them .In the past several decades since UPS was firstly recognized, tremendous effort has been directed towards reclassification of UPS based on clinical behavior due to its significance for the treatment. Multiple strategies have been applied in recent years including comparative genomic hybridization (CGH) analysis , cDNA miMeanwhile, a number of recurrent chromosomal regions of gain and loss have been identified in soft tissue UPS . Some ofRecently, a study of a prognostic miRNA biomarker (miRNA138) for clinical validation was identified along with a RHO-ROCK cell adhesion pathway that modulates the UPS metastatic phenotype  which prIn sum, although hepatic UPS is a rare malignant mesenchymal tumor with a nonspecific clinical and radiologic presentation, it should be considered in the diagnosis of large liver lesions without evidence of differentiation. Surgical resection is the most effective means for treating this rare tumor and the prognosis usually is poor. Recent progress in UPS research has allowed for a potential classification system and potential therapeutic targets , 30–32.


Hepatocellular carcinoma (HCC) is considered the third-leading cause of cancer-related mortality worldwide. Most cases of HCC are usually associated with liver cirrhosis due to various causes such as alcohol or more commonly viral hepatitis. Usually, patients remain asymptomatic for a long time, and symptoms are usually related to the cirrhosis itself or secondary to tumor extension. Intra-cardiac involvement with HCC rarely develops with a very poor prognosis. The occurrence and recurrence of HCC in cirrhotic patients treated with direct-acting antiviral (DAA) therapy (sofosbuvir) have been discussed in a few trials so far with no valid answer.We are reporting a case of recurrent HCC with an accidentally discovered direct right atrial extension with tumor thrombus through the inferior vena cava (IVC) in a cirrhotic patient previously treated with DAA. Unfortunately, due to his critical general condition, he died within days.Cardiac involvement in HCC rarely occurs and usually develops in advanced stages of HCC with very poor reported prognosis. Data regarding the relation between DAA and HCC development is controversial. Hepatocellular carcinoma (HCC) is the fifth most common malignancy and the third most common cause of cancer death worldwide. It is often a rapidly progressive tumor with high potential for both direct and distant vascular extension. However, despite the high incidence of venous involvement and proximity to the heart, case reports of intracardiac metastasis are quite rare. Here, we are reporting a case of intracardiac extension of recurrent HCC in a previously treated hepatitis C virus (HCV) with DAA with a brief review of cardiac metastasis of HCC and debatable data related to DAA and occurrence of HCC.A 52-year-old hepatitis C virus (HCV)-positive male is our patient with a known history of transarterial chemoembolization (TACE) for HCC two times, 2 years and one and a half year ago. He was successfully treated with DAA (sofosbuvir) 1 year ago. He presented to our medical facility complaining of deep jaundice, massive ascites, bilateral lower limb edema, and dyspnea. Ultrasonography of the abdomen revealed a mass in the liver extending through the IVC, which is most likely a recurrence of HCC together with a massive amount of ascites. Transthoracic echocardiography (TTE) revealed a highly mobile large cauliflower mass measuring 4.5 × 2.5 cm at the right atrium Figs.  and 4. DGlobally, HCC is considered the fifth most common cancer and the third most frequent cause of cancer-related mortality . More thHCC is usually asymptomatic until late. Cardiac involvement in HCC rarely occurs and usually develops in advanced stages of HCC. The main mechanism of metastasis into the cardiac cavity is through a direct vascular extension of the tumor to the right side via the hepatic vein and IVC .TTE is a simple readily available noninvasive modality for initial assessment of cardiac involvement in any tumor defining its site, mobility, extension, and effect. Both computed tomography (CT) and magnetic resonance imaging (MRI) can provide further tissue characterization beyond the value of TTE . CardiopThere is controversial data regarding the relation between DAA and HCC development. Conte et al. concluded that in patients with HCV-related cirrhosis, DAA-induced resolution of HCV infection was not associated with a reduction of the incidence of HCC occurrence, and previously treated HCC patients have still a high risk of tumor recurrence, in the short term. For these reasons, all cirrhotic patients should be closely monitored and followed during and after antiviral therapy . while I


Giant colonic diverticulum (GCD) is a diverticulum that is larger than 4 cm in diameter most commonly arising from the sigmoid colon. Patients with GCD are typically asymptomatic, and clinical manifestations vary from a soft palpable abdominal mass to diarrhea, which is well described in the literature. However, few studies have demonstrated a presentation of GCD with acute diverticulitis. Herein, we report a case of a middle-aged patient presenting with sepsis due to acute diverticulitis as an initial presentation of a GCD. Giant colonic diverticulum (GCD), commonly referred to as giant gas cyst or giant colon cyst, is a rare entity that was first described in 1946 [3  with a neutrophilic predominance and lactic acid of 3 mmol/L . The patient underwent a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis revealing an isolated 4.5 cm diverticulum at the sigmoid colon with surrounding inflammatory changes consistent with acute diverticulitis (Figures A 63-year-old male with a history of multiple sclerosis and major depression presented to our hospital with generalized abdominal pain of one-day duration. There were no other associated symptoms. Vital signs were within normal limits except for sinus tachycardia, and fever of 101°F. Physical examination was remarkable for lower abdominal tenderness with no rigidity or guarding. Laboratory studies revealed leukocytosis of 23,400/mmA GCD is a rare complication of diverticular disease . It was The pathogenesis of GCD is not clearly understood. Two theories have been proposed. A ball and valve mechanism involving communication between the bowel lumen and diverticulum where a one-way valve permits the entry of air into the diverticulum, thus progressively raising its intraluminal pressure and causing persistent enlargement of the diverticulum . An alteHistologically, GCD can be classified into three types , 5. TypeThe entity is usually asymptomatic, but clinical manifestations are variable and non-specific when they occur. They include abdominal pain, constipation, the sensation of an abdominal mass, vomiting, diarrhea, and rarely, bleeding per rectum . UrinaryImaging studies usually suggest the diagnosis: abdominal X-ray may demonstrate a balloon sign in most cases, which appears as a sizeable gas-filled cyst that is either round or oval in appearance , 3. The The preferred treatment of an uncomplicated GCD consists of an en-bloc resection of the affected colon segment with primary colonic anastomosis . In hemoGCD is a rare complication of colonic diverticulosis. Clinical presentation is very non-specific with variable signs and symptoms and requires the use of imaging modalities to delineate the diagnosis. The gold standard of treatment in symptomatic GCD consists of surgical segment resection of the affected colon with primary anastomosis. Conservative measures should be preserved to individuals who are either asymptomatic or deemed to be at high surgical risk.


Primary sino-nasal neuroendocrine carcinomas (PSNNECs) are rare, with a wide spectrum of histological differentiation. Advanced tumors may invade the surrounding structures, such as the skull base, orbit, or brain. Here, we present a rare case of PSNNEC and its management by concurrent chemoradiation (CCRT) followed by adjuvant chemotherapy with the radiological resolution of the tumor mass. The sino-nasal tract (SNT) is the location of a diversity of malignant tumors among which squamous cell carcinoma is the most common, followed by adenocarcinoma, malignant lymphoma, sino-nasal undifferentiated carcinoma, malignant melanoma, and olfactory neuroblastoma, due to the anatomic complexity and tissue variability . MalignaMalignant SNT tumors are rare in most parts of the world -4 and co2 on Day 1 and etoposide 100 mg/m2 on Days 1 to 3 (every three-weekly cycle). The CT scan revealed an optimal response at Week 5 of RT ; examination revealed a mass in the left nasal cavity. Computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a heterogeneous soft tissue attenuation mass in the left anterior nasal cavity, causing the erosion of the medial wall of the left maxillary sinus, showing irregular speculated calcification with a small, extra-osseous soft tissue component in the anterior deep subcutaneous tissue of the cheek, causing the blockage of the left osteomeatal complex and the narrowing of the left inferior meatus with resultant soft tissue attenuation Figures -1B. BiopPSNNEC was first proposed by Silva et al. in 1982 -8.The limited number of cases published, difficulties of diagnosis, and heterogeneity of treatment approaches hamper evaluating the ideal treatment strategy -11. PredWith regards to the treatment protocol in our patient, CCRT has shown a good response in terms of radiological as well as subjective symptomatic control, hence, we recommend that such patients should be subjected to the upfront CCRT rather than subjected to mutilating surgeries, as described in the literature. This report should open the front for future randomized controlled trials to show the beneficial effect of CCRT over surgery.Neuroendocrine tumors of the sino-nasal tract are locally recurrent and destructive and a multimodality treatment approach is needed for its management. Neoadjuvant treatment in the form of concurrent chemoradiation followed by adjuvant chemotherapy should be the standard protocol because of the chemoradiosensivity. Surgery, being mutilating, should be the last resort if patients do not show a response to the CCRT protocol.


Primary renal Ewing’s sarcoma is a rare tumor with dismal prognosis. The main treatment modality of treatment is surgical excision with adjuvant or neoadjuvant chemotherapy.We present a case of primary renal EWS in 27-year-old male. He presented to our center complaining of three days history of severe left flank pain associated with nausea and vomiting. Computerized tomography (CT) revealed a 10 × 7 × 6.5 cm left lower pole renal mass. Left radical nephrectomy was performed and revealed a tumor with uniform round to oval nuclei cells. The cells stained strongly positive for CD 99. The diagnosis was established as primary renal Ewing’s sarcoma. The patient refused the chemotherapy. Fortunately, he was free of the disease on regular clinical and radiological follow-up, the last follow up was 30 months post-nephrectomy.This case highlights the importance of proper surgical treatment and its role in the managing this type of malignancy especially in localized disease at presentation. EWS ariPrimary renal (EWS) is a rare tumor in adults. In 1975, Seemayer and colleagues were the first who described primary renal EWS and has since been sporadically documented in the literature ,4. The cThis case study was performed and is being reported in line with the SCARE criteria .2A 27-year-old Asian male; not known to have any medical illness; presented to our emergency department complaining of three days history of severe left flank pain associated with nausea and vomiting. No family history of malignancy was detected. Physical examination revealed left costovertebral angle tenderness. Laboratory investigations were conducted and were remarkable for elevated white blood cell count . Urine analysis and creatinine level were normal.An ultrasound was performed and revealed the presence of a large heterogenous cystic mass replacing the lower pole of the left kidney measured 12 × 10 cm. Computerized tomography (CT) revealed a 10 × 7 × 6.5 cm left lower pole renal mass. Part of the mass appeared in subcapsular location and causing significant mass effect over the left kidney with invasion into the renal pelvis and perirenal fascia  and 2 . He was admitted and a DMSA renal scan disclosed a split function of 24% for the left and 76% for the right. Bone scan revealed no scintigraphic evidence of osteoblastic or osteolytic bone metastasis. Then the decision was to take percutaneous renal biopsy, which revealed a small cell neoplastic tumor. Wilms tumor, pheochromocytoma, primary renal Ewing sarcoma and primary renal neuroblastoma were included as differential diagnosis.Later, left radical nephrectomy was performed. The renal mass weighed 572 g, measured 16 × 10 × 6 cm, and was surrounded by a thin membranous capsule . On histopathological studies, the tumor was highly cellular; the tumor cells were uniform with round to oval nuclei, finely dispersed chromatin, inconspicuous nucleoli and ill-defined scant pink cytoplasm . The celHis post-operative course was uneventful other than some minor incisional pain. He was discharged on day 3 postoperative. He had no evidence of disease at 30 months after surgery and refused chemotherapy at that time.3EWS of the kidney is an exceedingly rare entity. However, the true incidence may have been underestimated as the number of case reports is increasing in recent years, probably due to advanced immunohistochemistry . PrimaryIt is essential to distinguish renal EWS from other malignant neoplasms of kidney due to the prognostic point of view . Small bImaging findings in EWS include a large, ill-defined renal mass, often with heterogeneous contrast enhancement with areas of necrosis and hemorrhage . The imaBecause of the rarity of this tumor, there is no standardized treatment strategy. The primary modality is surgical excision ,9. The 24We report yet another case of primary renal EWS, in hope of expanding the knowledge of a rare occurrence and increasing the demand for further research about the etiology, clinical manifestation, prognostic factors and treatment modalities. This case also highlights the importance of proper surgical treatment and its role in the managing this type of malignancy especially in localized disease at presentation.No funding.The institutional review board is not required.The patient gave his consent for publication of this Case report and any accompanying images. None of the images contains any patient`s identifiers.All authors contributed significantly and in agreement with the content of the article. Abdalla, Tashtush, and Aleshawi collected all data and photographs to draft the manuscript. Talahmeh was the urologist who perform the surgery. Abdalla and Alawneh were the radiologist. Tashtush, Abdalla, and Aleshawi wrote the manuscript for submission. All authors presented substantial contributions to the article and participated of correction and final approval of the version to be submitted.None.Dr Khalid Abdalla.Not commissioned, externally peer-reviewed.The authors declare that they have no competing interests.


Neurofibromas are a characteristic of the autosomal dominant disorder Neurofibromatosis type 1 (NF1), also known as von Recklinghausen’s disease. Appendiceal neurofibromas are extremely rare, and low-grade appendiceal mucinous neoplasms (LAMNs) have not previously been reported in NF1.A 62-year-old man with NF1 was scheduled for elective surgical treatment of an asymptomatic, enlarged and diffusely thickened appendix that remained after curative antimicrobial treatment of acute appendicitis. Laboratory analysis revealed all normal. Colonoscopy showed thickened appendiceal mucosa projecting into the cecum. A sample of the mucosa was found to be pathologically benign. The patient was preoperatively diagnosed with treated acute appendicitis with chronic appendiceal inflammation versus appendiceal neoplasms. Laparoscopic cecectomy was performed. Multiple neurofibromas were observed in the muscle layer, submucosa, and mucosa of the appendix on histopathological examination. Immunohistochemical examination showed positive staining for S-100. Pathologically, the patient was diagnosed with appendiceal neurofibroma consistent with NF1 with LAMNs. His postoperative course was unremarkable. He was discharged on post-operative day 3 and remained in good health 7 month after surgery.Appendiceal neurofibromas are often preoperatively diagnosed as appendicitis. Appendiceal neurofibromas should be considered in patients with NF1 who are suspected of having appendicitis. NF1 is a multisystemic disorder that can affect any organ in the body. The most typical clinical presentations are neurofibromas and café-au-lait spots . However23/μL; platelets, 17.9 × 104/μL; serum total protein, 7.9 g/dL; serum albumin, 4.8 g/dL; total bilirubin, 0.8 mg/dL; aspartate aminotransferase, 27 IU/L; alanine aminotransferase, 21 IU/L; and lactic acid dehydrogenase, 173 IU/L; C-reactive protein, 0.073 mg/dL. The serum levels of tumor markers were normal, including carcinoembryonic antigen, 3.2 ng/ml and carbohydrate antigen 19–9, 7.1 U/mL. Contrast-enhanced computed tomography (CT) demonstrated an enlarged and diffusely thickened appendix (A 62-year-old man with NF1 was scheduled for elective surgical treatment of an asymptomatic, enlarged and diffusely thickened appendix that remained after curative antimicrobial treatment of acute appendicitis because he hoped for an antimicrobial treatment 2 months ago. Physical examination revealed multiple neurofibromas and café-au-lait spots on the skin. He had no other co-morbidities other than having NF1. Laboratory analysis revealed hemoglobin, 15.9 g/dL; white blood cell count, 5.33 × 10appendix a, b. Colappendix . A samplappendix a. Histopappendix b. Multipappendix c. Micro appendix d. Pathol3NF1, also known as von Recklinghausen’s disease, is the most common autosomal dominant single-gene neurodevelopmental disorder, with an incidence of 1:2700 . GastroiGastrointestinal neurofibromas are often asymptomatic. However, when the lesions grow in size, they may present as constipation, abdominal pain, palpable abdominal masses or bowel obstruction . NeurofiSurgical resection is the standard treatment for appendiceal neurofibromas and is aimed at improving symptoms, preventing complications and avoiding the risk of malignant transformation . HoweverLAMNs are characterized by low-grade cytologic atypia and the absence of destructive invasion. These tumors have the potential for peritoneal spread giving rise to pseudomyxoma peritonei . Thus, LSurgical resection is the standard treatment for patients with LAMNs. However, the most appropriate surgical procedure for patients with LAMNs remains controversial. Generally, tumor involvement of a surgical margin is an indication for additional surgery. However, Arnason et al. reported that in patients with appendiceal LAMNs without discharge of mucin or exposure of the appendiceal serosa, involvement of the appendectomy margin by either neoplastic epithelium or acellular mucin was not associated with disease recurrence or peritoneal dissemination . TherefoThere are no guidelines for the optimal management strategy of appendiceal neurofibromas and LAMNs. Thus, the most appropriate surgical procedure for patients with appendiceal neurofibromas or LAMNs is unknown. At the very least, it is important to obtain negative resection margins. A larger number of long-term follow-up patients with appendiceal neurofibromas and LAMNs are required to establish optimal, evidence-based treatment.4We report herein a rare case of appendiceal neurofibroma with LAMN in NF1 patient. Patients with NF1 may have gastrointestinal tract involvement such as appendiceal neurofibromas. These cases are often preoperatively diagnosed as appendicitis. Therefore, it is necessary to suspect appendiceal neurofibromas in NF1 patient who have symptoms of appendicitis.The authors declare that they have no conflicts of interest.The authors declare that this study was not funded externally.The study such as this case report was exempted from ethical approval by the Institutional Review Board of Hiroshima City Asa Citizens Hospital.When obtaining informed consent for surgical procedures, general consent for publication and presentation was obtained from the patient.TK drafted the manuscript. TK and KO reviewed and edited the manuscript. TK, KO, TK, MY, NT, MK, AN, YA, MS, and MM participated in the care of the patients. MK and HM provided the histopathological examination and diagnosis. TK, JH, MF, HM, and NH participated in critical revision of the manuscript. All authors read and approved the final manuscript.This is case report not research study.Koichi Oishi.Not commissioned, externally peer reviewed.


Brain metastasis from prostate cancer may be becoming more common and may be associated with occurrence of diffuse systemic metastases. Brain metastasis from prostate cancer may be becoming more common and may be associated with occurrence of diffuse systemic metastases. Because of its rareness, brain metastasis is usually not considered for follow‐up in prostate cancer. However, improved overall survival in patients with prostate cancer might change this notion. In this report, three cases of brain metastasis from castration‐resistant prostate cancer (CRPC) are described. Whole‐brain radiotherapy (WBRT) was administered in all three cases, and the time from the diagnosis of CRPC to the detection of a brain metastasis was 6, 24, and 29 months, respectively. Overall survival after diagnosis of a brain metastasis was 1, 3, and 5 months, respectively. Brain metastasis in prostate cancer is a rare phenomenon, but it is more frequent in metastatic CRPC patients than in the past.22.1A 64‐year‐old man presented with a prostate‐specific antigen (PSA) level of 25.78 ng/mL. After diagnosis of metastatic prostate cancer cT4N1M1b) with Gleason score 7 (4 + 3), he was started on combined androgen blockade (CAB) with degarelix and bicalutamide in January 2018. For local control, he underwent intensity‐modulated radiation therapy (IMRT) for the prostate (66 Gy/33 fractions). Although the patient initially responded well to treatment (PSA nadir of 0.039 ng/mL), he was diagnosed with CRPC when we found that his PSA levels had increased 9 months after starting CAB. Abiraterone and prednisolone were administered and resulted in a 50% reduction of PSA. However, despite the reduction of PSA, the disease progressed. He had frequent repeated episodes of severe anemia, and bone marrow aspiration showed a bone marrow metastasis. In April 2019, the patient presented with a severe headache and nausea. Magnetic resonance imaging (MRI) showed multiple brain metastases with major lesions in the left middle cranial fossa  with Gleason score 7 (4 + 3), he started on CAB with leuprorelin and bicalutamide in December 2000. CAB was effective for 153 months. Subsequently, his anti‐androgen withdrawal response continued for 11 months, and flutamide was effective for 19 months. However, PSA levels gradually increased, and bone scintigraphy established findings of a bone metastasis. The patient was diagnosed with M1CRPC and was consequently treated with sequential abiraterone, enzalutamide, docetaxel, and cabazitaxel. After three cycles of cabazitaxel therapy, the patient presented at our hospital with a headache and double vision. MRI showed multiple brain metastases with major lesions in the right cavernous sinus  with Gleason score 7 (3 + 4), he underwent brachytherapy (144 Gy) in April 2007. Two years after brachytherapy, androgen deprivation with leuprorelin was administered because of the recurrence of prostate cancer. However, PSA levels gradually increased, and bone scintigraphy established findings of a bone metastasis. The patient was diagnosed with M1CRPC and was consequently treated with sequential bicalutamide, alpha radium, enzalutamide, abiraterone, docetaxel, and cabazitaxel. After seven cycles of cabazitaxel, the patient's Eastern Cooperative Oncology Group Performance Status score decreased to 3, so we ceased cabazitaxel therapy and administrated dexamethasone. Four months after discontinuation of cabazitaxel, the patient presented at our hospital with a headache, nausea, and aphasia. MRI showed multiple brain metastases with major lesions in the left middle cranial fossa  may benefit performance status without worsening survival.In the present cases, surgical debulking was considered prior to WBRT but abandoned because of the high number (>5) of metastases and the uncontrolled systemic cancer. WBRT is still preferred in the setting of numerous brain metastases, carcinomatous meningitis, or primary histologies prone to micrometastatic disease. In these instances, stereotactic radiosurgery (SRS) often cannot effectively target all the disease.The authors declare that they have no competing interests.YS: YS contributed to the conception, design, and drafting of the manuscript. YS wrote the manuscript. TH: TH contributed to the critical revision of the manuscript. YM: YM contributed to the writing and critical revision of the manuscript. HS: HS contributed to the design and critical revision of the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patients.


Benign schwannoma is the most common tumor of peripheral nerves while plexiform multiple schwannomas are rare conditions. This manuscript reported a case of multiple Schwannomas characterized by unusual location.We report a 34-year-old workman presented with multiple nodules in his forefinger, middle finger and palm respectively for 5 years. He felt pain combined with paraesthesias in fingers and palm. The symptoms could be eased through rest and drugs medication.Three Schwannomas were found in surgery respectively. Histologic findings confirmed the diagnosis of schwannomas.We removed all of the 3 schwannomas completely without damaging the continuity of the nerve.Six months after the surgery, the patient had had not experienced any symptom recurrence.Successful treatment of multiple schwannomas depends upon accurate diagnosis. Early surgery can improve the treatment outcome of multiple schwannomas. Schwannomas are the most common in 3rd and 6th decades with no sex predilection. They are usually solitary, slow growing, encapsulated neoplasm arising from the schwann cells of the myelin sheaths.Schwannomas, also known as neurilemmomas, are the most frequently arising benign tumors involving peripheral nerve.Schwannomas are generally represented as painless swellings for several years before diagnosed which lead to difficulties in clinical diagnosis and treatment. In the upper limb, they may be mistaken for ganglion or neurofibroma. Pain, paraesthesia, or other symptoms may occur when the tumors reach sufficient size to compress the involved nerve. However, it is unusual for a schwannoma to exceed 3 cm in diameter. MRI and ultrasonography (USG) are helpful in the diagnosis and localization of these tumors. Preoperative accurate diagnosis and careful plan can be used to avoid unnecessary resection of the significant nerve and optimum efficiency nerve recovery. Surgical removal is usually curative. Single schwannoma is the most common tumor of all peripheral nerves, however, multiple schwannomas of different nerves in the same upper extremity are rare. We report a case of multiple neurilemmomas of the common palmar digital nerves and two individual digital nerves in one hand. Only few cases have been reported in the literature. We reviewed the literature and discussed the clinical features and therapeutic options in this report.2A 34-year-old male labor worker presented with pain and paeresthesias on his right index, middle fingers and palm that had slowly increased in size over 5 years. The patient reported no preceding history of significant trauma or inflammation of the right hand. He complained of paeresthesias and pain in the area of the mass in the index, middle fingers and palm. These symptoms began approximately 2 years earlier. The pain got increased when pressure was applied on the nodules and with finger movements. The patient had no loss of sensation and he had the normal function of grip initially. And through rest and Non-Steroidal Anti-inflammatory Drugs medication , the patient's symptoms had been eased. On clinical examination, there were palpable, tender swellings in the middle phalanx of the index, middle fingers and the volar of third metacarpal respectively Fig. . There w2.1The surgical procedure was performed under brachial plexus block. A brachial tourniquet was used and the procedure was carried out with surgical loupes. With volar approach, a longitudinal incision was made on the middle of the second and third metacarpal. A 12 mm × 7 mm × 4 mm yellowish tan, firm mass was separated from common palmar digital nerves of median nerve Fig. . The ner2.2Grossly the tumor tissue appeared as soft, solitary, encapsulated with well-defined surface and had a yellowish color. Microscopically-well circumscribed tumor composed of spindle shaped cells arranged in a palisading fashion. There was no mitotic activity or malignancy features were seen. Immunostaining demonstrated strong extensive S-100 immunoreactivity of the nodules with CD34, Desmin, SMA, and EMA negativity Fig. . These f2.3Postoperatively, there were no motor or sensory deficits. No infection was found after surgery. One week after the surgery, the patient was able to perform full motion of his operated hand. Symptoms of pain and paraesthesias resolved, and his finger range of motion recovered to the normal level. Tinel-Hoffman sign was negative. The patient was followed up by clinic every 3 months. Two years after the surgery, the patient had not experienced tumor recurrence.3 The case we reported showed multiple localized schwannomas confined to three different nerves in one hand. To our knowledge, it is extremely rare in the literature.Schwannomas are typically benign slow growing nerve sheath tumors, which usually arise on the flexor aspect of the extremities. Generally, they present as solitary tumors, although there are reports of patients with multiple tumors in the literature. In material presented by Ogose et al, the incidence of multiple schwannomas had been reported as 4.6% of all patients diagnosed with schwannoma. Schwannoma may be misdiagnosed as neurofibroma, lipoma and ganglion preoperatively.,7 Schwannoma can arise in association with any peripheral nerve but it generally arises in the sensory portion of the nerve. Neurological symptoms like pain, paraethesia and numbness are presented when the tumors compress the involved nerve.,9 On clinical exam, the swelling can be mobile from side to side, but not in the longitudinal axis of the nerve. Percussion induces painful paraesthesiae in the area of the nerve of origin similar to Tinel sign. A full history and careful detailed examination can improve the preoperative diagnostic accuracy. In our case, symptoms such as palpable tumor mass, pain, paraesthesias, and positive Tinel-Hoffman sign enhanced the evidence to diagnosis schwannomas.Pain and swelling are the usual presentation in general clinical physical examination. The similar features sharing with other soft tissue tumors cause difficulties in diagnosis.,12 Therefore, schwannoma of the hand is hard to distinguish from a cystic lesion. Till now, MRI is still the most competent imaging modality used to localize the tumors and to plan for surgery. Schwannoma on MRI is revealed by high signal intensity on T2 scans and low signal intensity on T1 scans. Possibly, it can lead to misdiagnosis as nerve sheath tumors and ganglions that have same appearance with Schwannoma on MRI. However, we can identify schwannomas from ganglions through the administration of gadolinium in which ganglions do not show any enhancement. In addition, the location of the lump to the nerve is another key to separate them, where schwannomas is eccentric to the nerve while neurofibromas is central to the nerve.USG and MRI are potent for localizing the tumors in hand as they are noninvasive and easy to manipulate. Ultrasonography works as a first-line imaging examination to localize and evaluate lesions of the hand. Schwannoma is homogenous, hypoechoic mass which exhibit enhanced through transmission, target appearance, pseudocystic appearance on ultrasonography, which is rather similar to ganglion cysts. It is also a key characteristic to distinguish schwannomas from leiomyosarcoma and fibrosarcoma.Histology of schwannoma shows the typical features including areas of Antoni type A, which characterized as compact bundles of Schwann cells, or Antoni type B, difined as loose matrix of oval cells. Both of the 2 types and a distinct capsule could be seen in our case. Compared with neurofibromas, schwannomas shows greater cellularity in Antoni type A. The cellular areas of the schwannomas express positive S-100 protein strongly, while neurofibromas express variable S-100 protein. Proper microsurgical dissection is as important for success as in a bloodless surgery, such utilization of loupe or microscopical magnification is strongly advised to use in order to avoid the nerve fibers damage. It requires surgeons pay meticulous attention to preserve functionally important sensory and motor branches without making unnecessary sacrifice. To protect the nerve, we used surgical loupes to remove the schwannomas in our case, and ultimately no nerve damage was found. Successful treatment of multiple schwannomas depends upon accurate early diagnosis. Surgery can improve the treatment outcome of multiple schwannomas.Surgery is the main therapeutic strategy for schwannoma, the principle of which is enucleation of tumor with no nerve damage.First and foremost, I would like to show my deepest gratitude to my colleagues Dr Hui Lu and Dr Hui Shen who have provided me with valuable assist in every stage of writing this paper. Last but not least, I’ d like to thank all my friends, especially my lovely wife for her encouragement and support.Data curation: Shuai Jiang, Hui Shen.Funding acquisition: Hui Lu, Shuai Jiang.Methodology: Hui Shen.Writing – original draft: Shuai Jiang.Writing – review & editing: Shuai Jiang, Hui Lu.Shuai Jiang orcid: 0000-0002-4401-6595.


Aneurysms of the deep lower limbs veins are extremely rare. Diagnosis of such conditions may be confusing and suggest the presence of a neoplastic lesion.We herein report a case of a 68-year-old woman who was admitted with a large vein tumour revealed by sonography and computed tomography. A direct phlebography revealed a large venous aneurysm of the right common iliac vein with an adhering thrombus and a large collateral circulation. Anticoagulant treatment and compression with an elastic stocking were initiated because the patient refused surgical treatment. A 2-year follow-up showed no aneurysm growth or thromboembolic complications.We show herein that conservative management can be effective and safe in cases of this rare condition. Venous aneurysms are extremely rare anomalies within the veins. Superficial vein aneurysms may be misdiagnosed as inguinal hernias . The autVenous aneurysms are associated with deep vein thrombosis and pulmonary embolism. On radiologic examination , deep vein aneurysms may be misdiagnosed as neoplastic tumours in the abdominal cavity.Due to the rarity of the described pathology, a unified management protocol of such lesions has not yet been established. The treatment is mainly based on the clinicians’ experience.A 68-year-old woman was admitted by her general practitioner (GP) with chronic pain situated in the epigastrium lasting for several years. No complaints of nausea, vomiting, body weight loss, or other symptoms were noted. The patient had no history of oedema of the lower limbs, varices, and trauma. Her only prior surgery was a Fothergill-Manchester operation in 2004 due to genital prolapse and urinary incontinence. The GP performed an abdominal ultrasound revealing a fluid-filled tumour (5.5 × 5.5 cm) situated between the common iliac arteries Fig. .Fig. 1UlNo pathology was noted during the complete gynaecological examination. Subsequently, computed tomography (CT) of the abdomen and pelvis was performed revealing an enormous mass in the presacral space. The CT image clearly suggested the presence of a neoplasm in the abdominal cavity Figs.  and 3.FiAfter the CT, the patient was admitted to the Department of General and Vascular Surgery and Angiology. An arterial and venous Doppler ultrasound was performed that indicated proper arterial flow and no deep vein thrombosis in the lower limbs. The Doppler ultrasound was followed by a direct phlebography that revealed a large venous aneurysm of the right common iliac vein with an adhering thrombus and a large collateral circulation Fig. . All of She received anticoagulant treatment for 2 years, and no thromboembolic complications or aneurysm growth has been noted thus far.The definition of venous aneurysm is in fact difficult to establish. The authors assume that the extension of the deep vein  should be called a “venous aneurysm.” On the contrary, dilatation of the superficial veins  is itself varicose. We agree with the definition presented in the Rutherford textbook that varicose veins are subcutaneous veins wider than 4 mm  [On the other hand, Hach defined a venous aneurysm as a limited venous dilatation with an increased risk of thrombosis. However, that might also apply to superficial veins . It seemThe natural history and indications for the treatment of venous aneurysms are ambiguous because of their rare occurrence. The face and neck region is the most frequent localisation; the least frequent are veins in the abdominal cavity (iliac veins and the inferior cava vein) and thorax  . In otheDue to the presence of the thrombus in the lumen of the venous aneurysm, it can potentially be a source of pulmonary embolism and cause life-threatening conditions. According to Abbott’s classification, venous aneurysms can be divided into primary and secondary . PrimaryThe most important factor in the presented pathology is thrombosis in the aneurysmal sac (such as in this case) that does not cause typical symptoms of limb oedema and pain. The other possible complication is aneurysm rupture . The mosThe treatment is surgically based to reduce potentially life-threatening consequences such as the risk of rupture, thromboembolism, or other organ compression.Surgery should be performed in a vascular division and is based on ligation of the aneurysm, tangential excision with lateral venorrhaphy, autologous vein patch, or complete resection with interposition grafting , 13.Treating the described pathology is controversial due to the disease’s rare occurrence and the difficulty in reaching an acceptable consensus. Due to the risk of pulmonary embolism, most clinicians agree that surgery is the best form of therapy. The authors concur. In the described case, the patient did not agree to the procedure; hence, only conservative treatment was applied. We decided to present the results of conservative treatment because it is rarely utilised.Because the patient refused operative treatment, we decided to administer permanent oral anticoagulant drugs and compression with an elastic stocking with regular follow-up visits, including yearly CT scans of the pelvis and lungs.


A wide range of medical conditions may mimic multiple sclerosis. Among them, cerebrovascular diseases, including moyamoya disease, need to be excluded since they share common clinical features and radiographic findings with multiple sclerosis. A 44-year-old woman experienced transient numbness of her right sided face and arm and was referred to our unit due to small brain lesions in magnetic resonance imaging, with a possible diagnosis of multiple sclerosis. Neurological examination was unremarkable except for plantar reflexes and jerky deep tendon reflexes. Brain magnetic resonance angiography revealed findings typically seen in moyamoya disease, confirmed with digital subtraction angiography. Antiplatelet therapy started, but few days later, she developed suddenly global aphasia and right hemiparesis . Brain magnetic resonance imaging revealed acute infarct in the distribution of the left middle cerebral artery. At her discharge, she was significantly improved (NIHSS 3). Diagnosis of multiple sclerosis is often challenging. In particular, in young patients with transient neurological symptoms and atypical white matter lesions in magnetic resonance imaging, cerebrovascular disorders such as moyamoya disease should be considered in the differential diagnosis. Detailed clinical and neuroimaging evaluation are mandatory for the correct diagnosis. Multiple sclerosis (MS) is an immune mediated demyelinating disease of the central nervous system. MS has a female preponderance and its diagnosis relies on clinical symptoms and/or the presence of multiple white matter lesions in the magnetic resonance imaging (MRI) demonstrating dissemination in space and time , 2. A wiMoyamoya disease (MMD) represents a rare chronic occlusive cerebrovascular disease of unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery (ICA) and an abnormal vascular network at the base of the brain originating either from the basilar artery or extracranial blood vessels . MMD is We report a 44-year-old previously healthy woman a month ago, who experienced a transient numbness of her right sided face and arm. She was referred to our unit with a possible diagnosis of MS or of a systemic autoimmune disease due to small brain lesions in MRI. For years, she had been complaining of intermittent weakness of her left arm, but it had been attributed to psychosomatic problems.Upon presentation, the neurological examination revealed indifferent plantar reflexes and jerky deep tendon reflexes. Her brain MRI revealed various lesions of hyperintensity in T2 and Fluid Attenuated Inversion Recovery (FLAIR) sequence, in the subcortical white matter of the hemispheres and also periventricular, with mild linear cortical, enhancement of the left parietal lobe. Cerebrospinal fluid analysis and laboratory tests for inflammatory-autoimmune diseases were normal.However, a more thorough analysis of brain MRI revealed that lesions were mainly in the border zone of anterior cerebral artery (ACA) and middle cerebral artery (MCA) with no evidence of restricted diffusion . Given tMMD should be considered in the differential diagnosis of MS, as both affect young adults, cause intermittent neurological symptoms, and show multifocal abnormalities on brain imaging . DespiteThe clinical presentations of MMD include transient ischemic attacks (TIAs), ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment . Few pubBrain MRI can show lesions of T2 hyperintensity in the subcortical cerebral white matter in both disorders. Particularly, in MMD, lesions are especially localized in the frontal and parietal watershed regions and may also affect the deep or superficial grey matter. Additionally, in acute lesions restricted diffusion is particularly common in watershed areas . On the The key to diagnosis of MMD is imaging of intracranial vasculature. Definitive diagnosis requires catheter angiography in unilateral cases, while bilateral cases can be promptly diagnosed by either catheter angiography or MRA .Diagnosis of MS is demanding and several disorders should be considered in the differential diagnosis. In young patients with transient neurological symptoms suggestive of MS and atypical white matter lesions in MRI, vasoocclusive diseases such as MMD should be taken seriously into account in the differential diagnosis. Therefore, increased awareness is required, and detailed clinical and neuroimaging evaluation are mandatory, in order to prevent misdiagnosis and treatment delay.


Colovesical fistula is an atypical communication between the colon and the bladder. The most common causes of colovesical fistula are diverticulitis, inflammatory bowel disease, lymphoma and complication from radiation therapy. Patients with colovesical fistula present with recurrent urinary tract infections (UTI), dysuria, frequency, abdominal pain, pneumaturia, faecaluria, and hematuria. We present a case of a patient with stage IV lung adenocarcinoma presented with abdominal pain, dysuria, and faecaluria who was found to have a colovesical fistula. Although colovesical fistula may be sequelae of advanced colon or bladder cancer, it is a very uncommon presentation of metastatic cancer from distant sites. Our case is the first to show that colovesical fistula may present from metastatic lung adenocarcinoma. Clinical awareness of this very unusual presentation of metastatic cancer can lead to faster diagnosis and treatment, possibly minimizing excessive use of antibiotics. Colovesical fistula is an atypical communication between the lumen of the colon and the bladder. Most commonly, a colovesical fistula is formed as a result of diverticulitis which comprises approximately 60% of cases, colorectal cancer is responsible for around 20% of cases, inflammatory bowel diseases comprising nearly 10% of cases, less frequent etiologies include appendicitis, radiation therapy, and trauma [Non-small cell lung carcinoma (NSLC) is the most common form of lung cancer, and is broken down into 3 subtypes including adenocarcinoma, squamous cell carcinoma, and large cell carcinoma. Approximately 50% of all newly diagnosed lung cancers have distant metastasis at presentation . The mosPatient is a 79 year-old man with history of hypertension and coronary artery disease initially presented for evaluation of a palpable right neck mass with associated symptoms of dyspnea on exertion and 36lb weight loss. Workup revealed stage 4 adenocarcinoma with negative anaplastic lymphoma kinase (ALK), c-ros oncogene 1 (ROS1), epidermal growth factor mutation (EGFR) and positive for programmed death-ligand 1 (PD-L1). Positron emission tomography/computed tomography (PET/CT) revealed disease mostly restricted to the thoracic area, but there was also dense hypermetabolic activity with mural thickening in the sigmoid colon of unclear significance. Patient refused nivolumab systemic therapy and was treated with palliative radiation therapy to his right neck mass, right hilum and mediastinum with good palliative response.Two weeks after radiation therapy, patient presented to the hospital with dysuria, fecaluria and abdominal pain which did not improve after taking a course of antibiotics. CT of the abdomen and pelvis revealed a colovesical fistula between the sigmoid colon and bladder, large mesenteric lymph node just above the sigmoid colon mass and wall thickening in the sigmoid colon Figure . Cystoscopy revealed edematous mucosa and exudation of feculent material from the fistula site. Sigmoidoscopy revealed a fistula in the distal to mid sigmoid colon about 25 cm from dentate line and a 3 cm area of extrinsic compression.Patient was taken to surgery for resection of colovesical fistula mass, sigmoidectomy with colostomy, drainage of the intraabdominal abscess and repair of a complicated bladder fistula. Pathology of the tumor revealed metastatic poorly differentiated lung adenocarcinoma with an associated colovesical fistula and extensive lymphovascular invasion. Comparison of resected colovesical fistula mass to primary lung cancer biopsy showed similar morphology by Haemotoxylin and Eosin staining (H&E) and lung cancer specific thyroid transcription factor-1 (TTF1) marker consistent with the colovesical fistula mass being metastatic lung cancer Figures -D. Our case is the first in the literature to show that colovesical fistula can develop from a lung cancer metastasis. Our patient did not have evidence of diverticulitis or inflammatory bowel disease on colonoscopy or pathological evaluation, ruling out these common causes of colovesical fistulas. He does not have any history of radiation to the abdomen or pelvis.The most common symptoms of colovesical fistula are urinary urgency, dysuria, tenesmus, suprapubic pain, pneumaturia, fecaluria, hematuria, and UTI. Colovesical fistula is more common in males than in females since the uterus and the broad ligaments serve as a barrier between sigmoid colon and the bladder . ClinicaManagement of colovesical fistula can be divided into conservative management and surgical management. Conservative management is reserved for patients with multiple comorbidities or elderly who cannot tolerate general anesthesia. Patients can be managed with medical therapy with periodic administration of antibiotics and supportive care with urethral catheter drainage . For surOur case is the first in the literature to show that colovesical fistula can develop from a lung cancer metastasis. Clinical awareness of this very unusual presentation of metastatic cancer can lead to faster diagnosis and treatment, possibly minimizing excessive use of antibiotics.


Head and neck chondrosarcomas are incredibly rare with documented cases arising from skull base, maxilla, larynx, and nasal septum. We present the first reported case of chondrosarcoma arising from the lower lateral cartilage of the nose treated with surgical resection and primary reconstruction. Sarcomas of the head and neck are rare, comprising less than one per cent of all head and neck neoplasms . Of thesA 68-year-old man presented with a ten-year history of a lump in the right nasal tip, with a significant increase in size over a six-month period. Medical history included adenocarcinoma of the rectum treated with surgery and radiotherapy two years previously, polycythaemia, chronic alcohol abuse and a 50-pack per year smoking history. Clinical examination revealed a large bulbous firm swelling of the right lower third of the nose causing significant cosmetic asymmetry and distorting the right ala . Rigid nAfter discussion at the multidisciplinary head and neck meeting, the patient underwent primary resection via an external rhinoplasty approach. The skin and soft tissue envelope (SSTE) was not adherent to the tumor, which was well-circumscribed and easily dissected from the surrounding soft tissue with no evidence of local invasion. Macroscopic resection of a 3 cm × 3 cm × 3 cm mass arising from the lateral crus of the right LLC was performed . Soft tiHistopathological review of the specimen confirmed an intermediate grade chondrosarcoma with clear resection margins. As the tumor was resected from an easily observable area, the multidisciplinary head and neck meeting recommendation was for surveillance only, with no postoperative radiotherapy at this stage.There were no post-operative complications. Sutures and splints were removed one week following surgery. The SSTE was contracted and adhered to underlying tissue without development of seroma or haematoma. At five-week review, the right nasal airway was patent with no dynamic collapse of the right nasal ala. At four months , there wAs demonstrated in this case, chondrosarcoma is histopathologically difficult to differentiate from benign neoplasms of cartilaginous origin, such as enchondroma, osteochondroma, and synovial chondromatosis . DiffereSurgical resection is the primary modality of treatment for chondrosarcomas ,5. A sysPrimary reconstruction at the time of resection was both ideal and convenient with native septal cartilage harvested from an adjacent surgical site. The decision was made not to resect the excess SSTE to minimize the risk of wound breakdown, particularly in view of his ongoing smoking history.As the condition is rare, the existing literature is retrospective in nature, and therefore, data regarding treatment, outcome and prognosis are variable. A wide range of five-year survival rates from 44 to 88% has been reported ,10,11. RA recent retrospective study of 47 patients with grades I and II skull base chondrosarcoma reported local recurrence was 33% in patients receiving surgery alone compared to surgery and adjuvant proton therapy (11%), however, there was no significant difference in 10-year disease-specific survival between the groups . There wAlthough rare, chondrosarcoma must be considered in cases of a lower nasal mass as there is a significant risk of histological misdiagnosis of a benign lesion on biopsy. Surgical access through an external rhinoplasty approach for resection is ideal for both resection and primary reconstruction of tumors located at this site.


Primary malignant cardiac tumors are extremely rare and can present with the same nonspecific characteristics as benign primary cardiac tumors. We herein describe a middle-aged female with an intracavitary, irregular atrial mass who experienced partial surgical resection. The atrial mass which was recognized as myxoma before surgery was finally diagnosed as dedifferentiated liposarcoma (DDLPS) by postoperative pathological examination.The patient, a 61-year-old female, presented to the emergency room because of progressive chest congestion and shortage of liberties for 6 months and orthopnoea and paroxysmal nocturnal dyspnea for 3 days. The laboratory examinations confirmed no abnormalities. The thoracic computed tomography (CT) scan showed massive hydropericardium, pleural effusion and left atrium occupying lesion. The transesophageal echocardiography (TEE) confirmed an intracavitary and irregular left atrial mass, limiting the mitral valve inflow and pulmonary venous blood reflux. The positron emission tomography/computed tomography (PET/CT) revealed high grade fluorodeoxyglucose uptake only in the intracavitary mass which near the mitral valve. According to operative exploration, the intracavitary mass had invaded the mitral annulus and posterior wall of left ventricle which cannot be resected completely, we did merely partial surgical resection to relieve the patient’s symptoms. Postoperative immunohistochemical stain confirmed the diagnosis of DDLPS. The patient was transferred to the oncology department for further therapy. Unfortunately, the patient was detected with brain metastasis 1 month later and died within 5 months after the surgery.Primary cardiac DDLPS is an extremely rare histological subtype of undifferentiated pleomorphic sarcomas which present the same nonspecific characteristics as benign primary cardiac tumors. Even though surgical resection combined with chemotherapy or radiotherapy remains the mainstream treatment strategy, the prognosis of cardiac malignancy is poor with high mortality. Novel management strategies need to be further explored.The online version of this article (10.1186/s13019-019-0973-0) contains supplementary material, which is available to authorized users. The presence of a heart tumor was first identified in 1559. However, it was not until 1934 that the first clinical diagnosis of a primary heart sarcoma was reported , 3. The 9/L). Thoracic CT scan revealed occupying lesion in left atrium, accompanied with massive hydropericardium and pleural effusion. The patient received emergency surgical intervention under cardiopulmonary bypass since her clinical status deteriorated quickly. The pericardium effusion was drained firstly after pericardiotomy. Operative exploration confirmed complete resection could not be achieved, since the broad intracavitary mass surrounded with fibrotic density had invaded the mitral annulus and posterior wall of left ventricle Fig. e. The inAfter the surgery, the removed left atrium mass was sent for further histopathological examination. Hematoxylin–eosin staining showed the mass was composed of markedly atypical cells which lack specific morphological features of differentiation Fig. a. ImmunoDDLPS is one of the most common types of soft tissue sarcoma which represents over 60% of all liposarcoma  and is aIn primary cardiac DDLPS, no sex predilection is apparent. Although cases have been identified among a wide range of ages, most patients that are affected are younger than 45 years of age , 12. TheThe DDLPS in cardiac chambers often appear as large, irregular, low-attenuation lesions on multidetector CT, and can be either noninvasive/focal or demonstrate infiltration into the myocardium , 15. As Even though cardiac tumors present a particular challenge for cardiac surgeons, surgical resection should be attempted in the setting of localized disease, provided the patient has acceptable performance status, in that it seems to provide the best option for palliative care and potential cure , 17. ComWith a better understanding of the molecular and genetic factors promoting the growth of tumors, more specific and patient-targeted agents such as palbociclib  (a cycliIn conclusion, primary cardiac DDLPS is an extremely rare histological subtype of undifferentiated pleomorphic sarcomas which present the same nonspecific characteristics as benign primary cardiac tumors. Even though surgical resection combined with chemotherapy or radiotherapy remains the mainstream treatment strategy, the prognosis of cardiac malignancy is still poor with high mortality. Novel management strategies such as molecular targeting therapy in the treatment of cardiac sarcomas needs to be further explored.


Endometriosis is a pathological condition whereby endometrial glands and stroma are located outside the physiologically expected location, the uterus. A lesion is usually located in the pelvis but can also occur in the diaphragm, bowel, pleural cavity, or surgical scar. It is a benign estrogen-dependent condition. Here, we present the case of a 40-year-old woman with ectopic endometriosis. Surgical scar endometriosis is a rare condition that is very difficult to diagnose because of its nonspecific symptoms. The patient may present with pain at the incision site from previous surgery . The pain may be cyclical, intensifying during the menstrual period. Some patients may present, in addition to the symptoms above, with some leakage from the scar area . Here, wA 40-year-old woman, who has had one pregnancy and has delivered once (G1P1), was referred by her gynecologist to our surgical oncology clinic for evaluation of a lower midline abdominal wall mass. The patient reported having abdominal pain and discomfort in this area for more than five years, with no definite sense of lump or mass, and worsening pain immediately prior to and during her menses. Personal history revealed that she had a planned lower segment cesarean section several years ago. She was recently diagnosed with dysfunctional uterine bleeding. She is not taking any medication at home.Her physical examination revealed a palpable tender subcutaneous mass located under the incision scar. Sonography and Doppler examination of the abdomen revealed a 5 cm x 3 cm heterogeneous hypoechoic mass, with an otherwise normal examination of the uterus and ovaries Figure . ComputeFine needle aspiration revealed amorphous debris, hemorrhage, and hemosiderin cystic degeneration, negative for malignant cells. An ultrasound-guided core needle biopsy of the abdominal wall mass was conducted by an interventional radiologist, and pathology revealed benign endometrial glands and stroma compatible with endometriosis with active inflammation and fibrosis. Test results were negative for epithelial dysplasia.She was managed with hormonal therapy (Lupron®). Six months into treatment with hormonal therapy, her abdominal pain, and vaginal bleeding resolved, and she had no awareness or feeling of an abdominal mass. A CT scan revealed marked shrinkage of the abdominal mass Figure .At the 12-month follow-up, the patient was discontinued on hormonal therapy for approximately two months, her menses were regular, and she had no abdominal pain. A CT scan revealed further shrinkage of the abdominal mass Figure .At that time, we discussed options such as observation, radiation therapy, or surgical resection. Surgical resection would have been an extensive procedure given the location and a full-thickness involvement of the medial aspect of the bilateral rectus muscles. The patient elected to continue with observation and follow-up every six months with a CT scan.Due to its asymptomatic nature in some women, determining the prevalence of ectopic endometriosis can be a daunting task. However, surgery is the definitive diagnostic means . EndometIn decreasing order of frequency, the most common anatomic sites for this lesion are ovaries, posterior and anterior cul-de-sac, posterior broad ligaments, uterosacral ligaments, uterus, fallopian tubes, sigmoid colon, and appendix and round ligaments .Associated risk factors for ectopic endometriosis include nulliparity  and prolSonography and Doppler examinations can reveal heterogeneous hypoechoic mass with echogenic spots. Diagnosis can be confirmed with excision and analysis of tissue samples from the area, and examination will reveal fibrosis of the tissue that involves the fascia of the area as well .Surgery is the definitive treatment for endometriosis and must be performed with clear margins and followed up with reconstruction of the damaged tissue. Hormone suppression has also been suggested since hormone induction due to surgical procedure is a suspected cause of the condition . PreventSurgical scar endometriosis is a rare condition that is difficult to diagnose. Surgery is the definitive diagnosis and management. However, in carefully selected patients, hormonal suppression has been used to successfully manage this condition, as evidenced in our case presentation.


The presence of the vermiform appendix, whether inflamed or not, inside a hernial sac is known as Amyand's hernia. Due to nonspecific signs, preoperative diagnosis is not common and requires a high index of suspicion along with awareness of this rare entity. It is more commonly mistaken for a strangulated or incarcerated inguinal hernia. Most cases of reported Amyand's hernia with appendicitis were in pre-term babies, infants, and post-menopausal women. We present a similar case in a 19-day-old, full-term baby presenting with inguinoscrotal edema, erythema, and without a palpable inguinoscrotal mass. Amyand's hernia is defined as an inguinal herniation of a normal, perforated, or acutely inflamed appendix. It was first described by Claudius Amyand in 1735, a French born surgeon working at St George's Hospital in London. On December 6, 1735, he performed the first recorded successful appendicectomy in an 11-year-old boy who had an inguinal hernia combined with an acutely inflamed appendix.Our patient is a 19-day-old, full-term baby, born in good general condition who presented to our emergency department with 1-day history of inguinoscrotal erythema and persistent irritability. The patient was struggling with achieving full feeds, although not vomiting and managing to open bowels normally. Examination revealed a distended, soft, nontender abdomen and bilateral scrotal edema along with moderate inguinoscrotal erythema and tenderness . ExaminTwelve hours later, our patient exhibited bilious vomiting and worsening inguinoscrotal edema. Abdominal examination showed signs of generalized rigidity and guarding. With no clinical evidence of an incarcerated hernia, the exclusion of testicular torsion by Doppler, and the development of an acute abdomen, we opted for an exploratory laparotomy through a right upper quadrant incision. This allowed us to be able to exclude any source of intra-abdominal sepsis while at the same time providing adequate surgical access to deal with the possibility of a missed irreducible hernia. The bowel was inspected and the appendix was found to be incarcerated in the right internal ring with the evidence of inflammation and suppuration . The apNeonatal appendicitis is a rare condition associated with comorbidities such as prematurity or other diagnoses like Hirschsprung's disease, meconium plug syndrome, and cystic fibrosis.Although the exact pathophysiology of this entity is unknown, one of the theories is the presence of a congenital band extending from the appendix to the right testis going through the inguinal canal.Most authors consider diagnosis of this condition to be intricate. This is because of the nonspecific manifestations, as in this case, where no obvious signs of irreducible hernia or intestinal obstruction were found. Cankorkmaz et al described a series of 12 cases, where only one was diagnosed preoperatively by US. All patients presented with an inguinal nonreducible mass and only a few exhibited fever, vomiting, or intestinal obstruction symptoms. These patients were taken to theater with a working diagnosis of incarcerated hernia. Intraoperatively they found six cases of Amyand's hernia with inflamed appendices: two normal appendices and four of equivocal nature.Due to the majority of cases being incidentally found on surgical exploration, treatment is mainly by primary hernia repair and appendectomy through an inguinal incision. In cases with unclear diagnosis, reports of laparotomy as a primary incision, combined abdominal and inguinal incisions, and even scrotal explorations were described.Diagnosing this rare entity requires a high index of suspicion by clinicians and when imaging is inconclusive, resorting to diagnostic laparoscopy should be considered to avoid laparotomy or multiple surgical incisions. While a tender, irreducible inguinal mass is the most common presentation, we report a case of Amyand's hernia with concurrent appendicitis presenting only with inguinoscrotal erythema and edema.


The most common cause of LETM is neuromyelitis optica spectrum disorders (NMOSD) and other inflammatory demyelinative disease. Neuromelitis optica (NMO) is a demyelinating syndrome of the nervous system by attacks of optic neuritis and myelitis. Intramedullary tumors such as astrocytoma, ependymoma, and intravascular lymphoma are other causes of LETM, and intramedullary metastasis is very rare. However, development of progressive myelopathy in the absence of antecedent neurological symptoms and without signs indicating dissemination beyond the spinal cord is a diagnostic challenge, and rule out of other causes of myelitis should be done.2-5 and on the contrary, we report a rare case of renal cell carcinoma (RCC) presenting by LETM without manifestation of primary tumor. There have been many reports of patients with demyelinating disorders mimicking spinal cord tumors,A 70-year-old farmer without any other medical history was referred in October 2014 with right hand paresthesia and neck pain that radiated to shoulders. The first cervical magnetic resonance imaging (MRI) showed cervical spondylosis without abnormality in spinal cord parenchyma. The patient was treated for cervical spondylosis.Two months later he noticed atrophy of first interosseous muscle and brain MRI was performed, which revealed a few small vessel ischemia and electromyography study showed motor neuron disease.In May 2015, he was referred to our hospital with paraparesis (left more than right). At physical examination, mental examination was normal and paraparesis with a motor grade 3/5 in left and 4/5 in right side was detected without sphincteric problem.The patient underwent whole spine MRI, which revealed an extensive intramedullary hyperintensity in T2-weighted MRI involving predominantly central spinal cord and extended until medulla location .Routine investigations including hematology, biochemistry, electrocardiography (ECG), echocardiography, and chest X-ray showed no abnormality. The patient complained of dyspnea. LETM due to inflammatory demyelinated and primary spinal cord tumor were main differential diagnosis. High-dose methyl prednisolone was started and weakness and dyspnea was recovered dramatically after 5 days of treatment. Serum sample was referred for vasculitis tests and NMO antibody. Brain and cervical MRI with gadolinium and computed tomography (CT) scan of thorax and abdomen with contrast was done. After discontinue of corticosteroid, motor function in both lower extremities deteriorated without sphincter problem.The second MRI of the cervical spine revealed an ill-defined T1 sequence isointense and T2 hyperintense intramedullary lesion extending from medulla to T2 levels with cord enlargement. There were two lesion enhancements . Abdomin1LETM is one of the common presentations of NMOSD. MRI of the spine is very helpful in diagnosing NMOSD. In acute phase, it typically shows LETM and gadolinium enhancement; in chronic stage, the enhancement and expansion resolve while the T2 signal persists, but the LETM regresses to less than three segments and breaks up in to shorter fragments.Many neoplastic lesions are misdiagnosis as intramedullary spinal cord myelitis such as NMOSD. More specifically, the fallowing misdiagnoses are made: infectious disorders, demyelination [multiple sclerosis (MS) and NMOSD], granulomatous disease, vascular lesions, and syringomyelia. 3It is difficult to distinguish these lesions from neoplastic spinal cord only by history and neurological examination. On brain MRI, most lesions are non-specific and asymptomatic, except for optic nerve enhancement in optic neuritis. The age onset of NMO ranges from childhood to late adulthood. The incidence tapering off after the fifth decade. However, there are many reports of very late onset of NMOSD.4Otherwise, neoplastic diseases can cause LETM through intramedullary tumor or metastasis. Although intramedullary metastasis is rare, lung, breast, and RCCs are common underlying causes. So, it seems reasonable to consider intramedullary metastasis in patients with history and manifestation of neoplasm. On the other hand, tumors can cause LETM as paraneoplastic lesions before detection of malignancy, particularly RCC.5RCC is unique among the genitourinary malignancies in that close to one third of affected patients show symptoms of a paraneoplastic syndrome in addition to central nervous system metastasis. Such as neuromyopathies have been described in cases of nonmetastatic RCC. Degrees of severity vary from nonspecific myalgias to bilateral phrenic nerve paralysis. There is evidence to suggest that some RCCs may secrete a substance that may mediate such neuromyopathies.We have to acknowledge limitation in this case due to lack of autopsy result. Actually, history of disease, age of patient, and antecedent neurological or systemic symptoms beyond the spinal cord were so helpful for diagnosis. This case report suggests that LETM may be a rare presenting manifestation of malignancy, which may be due to paraneoplastic etiology or intramedullary spinal cord metastasis. It is recommended that in an elderly patient with LETM, possibility of any underlying malignancy should be investigated, and the NMOSD be noticed as the second diagnosis.