Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child.

Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an 11-year-old boy who presented to our hospital with no apparent cause of left shoulder pain for 6 months. On physical examination, tenderness was noted in the left chest wall and shoulder joint, which had a limited range of motion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest revealed an irregular soft tissue mass in the upper left thorax, with a wide base attached to the adjacent pleura and bone destruction of the adjacent left first rib. The patient's bone scan showed a dense focus of increased radiotracer accumulation in the left first rib. A subsequent CT-guided aspiration biopsy of the left pleural mass with histomorphology and immunohistochemical phenotyping led to a diagnosis of extraskeletal Ewing sarcoma. To inhibit tumor growth, alternating systemic chemotherapy cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and isocyclophosphamide and etoposide (IE) were administered at 3-week intervals. After completing three VDC and two IE cycles, the child's condition was well and the pain in the left shoulder joint was relieved. However, a repeat MRI of the chest showed that the mass did not shrink.

Three-in-one incidence of hepatocellular carcinoma, cholangiocellular carcinoma, and neuroendocrine carcinoma: A case report.

BACKGROUND: Primary hepatic neuroendocrine carcinoma (NEC) is rare, and a combination with hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) is extremely rare. To date, only four combination cases have been reported. The present paper describes the fifth patient. CASE SUMMARY: A 32-year-old Chinese man with chronic hepatitis B was hospitalized for persistent upper abdominal pain. Abdominal computed tomography (CT) examination revealed a liver mass. The tumor was located in the 7th and 8th segments of the liver, and CT and magnetic resonance imaging findings were consistent with the diagnosis of HCC. Laboratory examinations revealed the following: Alanine aminotransferase, 243 U/L; aspartate aminotransferase, 167 U/L; alpha-fetoprotein, 4519 µg/L. Laparoscopic right lobe hepatectomy was performed on the liver mass. Postoperative pathology showed low differentiation HCC plus medium and low differentiation CCA combined with NEC. One month after the surgery, the patient suffered from epigastric pain again. Liver metastasis was detected by CT, and tumor transcatheter arterial chemoembolization was performed. Unfortunately, the liver tumor was progressively increased and enlarged, and after 1 mo, the patient died of liver failure. CONCLUSION: This is a rare case, wherein the tumor is highly aggressive, grows rapidly, and metastasizes in a short period. Imaging and laboratory tests can easily misdiagnose or miss such cases; thus, the final diagnosis relies on pathology.

Primary signet-ring cell carcinoma of the extrahepatic bile duct: A case report.

BACKGROUND: Signet ring cell carcinoma (SRCC) is a specific type of mucinous secretory adenocarcinoma, which contains abundant mucus in the cytoplasm and pushes the nucleus to one side of the cell membrane, forming a round or oval, and the nuclear deviations give the cells a signet ring-like appearance. SRCC often originates in the gastrointestinal tract, especially in the stomach. However, primary SRCC of the extrahepatic bile duct is extremely rare. Therefore, little is known about its epidemiology, treatment, and prognosis. CASE SUMMARY: An 82-year-old female was admitted with abdominal pain, jaundice, and skin pruritus for 2 mo. She had no specific family history. Physical examination presented normal vital signs, icteric sclera, visible jaundice, and mild tenderness in the right upper abdominal quadrant. Tumor-related cell markers were within normal values. Contrast-enhanced computed tomography revealed a thickened wall of the common bile duct, strengthened with intrahepatic bile duct dilation and multiple round-like lesions in the liver. In addition, the lymph nodes in the hepatic hilum area, the pancreatic head area, and around the abdominal aorta were enlarged. Thus, a preoperative diagnosis of cholangiocarcinoma was established. To alleviate jaundice and prolong the overall survival, percutaneous transhepatic cholangiopancreatic drainage (PTCD) was performed. During the operation, segmental stenosis of the extrahepatic bile duct and a vine-like expansion of the intrahepatic bile duct was observed. Furthermore, a biliary biopsy was performed under fluoroscopy to determine the nature and origin of the lesion. The pathological diagnosis of the biopsy was SRCC. Finally, a diagnosis of primary SRCC of extrahepatic bile duct with distant lymph node metastasis and multiple liver metastases was made based on the radiographic, PTCD, and pathological characteristics. The tumor was diagnosed as T3N1M1 stage IV. Despite our aggressive approach, the patient died of liver failure after 1 mo. CONCLUSION: This is the only case report on primary SRCC of the extrahepatic bile duct with distant organ metastasis to date.

Removal of "ruptured" pulmonary artery infusion port catheter by pigtail catheter combined with gooseneck trap: A case report.

BACKGROUND: Implanted intravenous infusion port (IVAP) is indicated for patients undergoing chemotherapy, total parenteral nutrition and long-term antibiotic treatment. Among their complications, the rupture and migration of the catheter of an IVAP via internal jugular vein represents a very rare but potentially severe condition. CASE SUMMARY: A 43-year-old woman was identified with a spontaneous fracture and migration of catheter of an IVAP via right internal jugular vein after adjuvant chemotherapy for left breast cancer. A computed tomography showed the fractured catheter of the IVAP in the pulmonary artery. Therefore, we conducted an emergency procedure to remove the catheter fragment by a pigtail catheter combined with a gooseneck trap. CONCLUSION: When the fractured catheter of an IVAP was detected, the special shape of the pigtail catheter in combination with the gooseneck trap successfully facilitated the removal of the dislodged catheter.

Identification of hepatocellular carcinoma prognostic markers based on 10-immune gene signature.

BACKGROUND: Due to the heterogeneity of hepatocellular carcinoma (HCC), hepatocelluarin-associated differentially expressed genes were analyzed by bioinformatics methods to screen the molecular markers for HCC prognosis and potential molecular targets for immunotherapy. METHODS: RNA-seq data and clinical follow-up data of HCC were downloaded from The Cancer Genome Atlas (TCGA) database. Multivariate Cox analysis and Lasso regression were used to identify robust immunity-related genes. Finally, a risk prognosis model of immune gene pairs was established and verified by clinical features, test set and Gene Expression Omnibus (GEO) external validation set. RESULTS: A total of 536 immune-related gene (IRGs) were significantly associated with the prognosis of patients with HCC. Ten robust IRGs were finally obtained and a prognostic risk prediction model was constructed by feature selection of Lasso. The risk score of each sample is calculated based on the risk model and is divided into high risk group (Risk-H) and low risk group (Risk-L). Risk models enable risk stratification of samples in training sets, test sets, external validation sets, staging and subtypes. The area under the curve (AUC) in the training set and the test set were all >0.67, and there were significant overall suvival (OS) differences between the Risk-H and Risk-L samples. Compared with the published four models, the traditional clinical features of Grade, Stage and Gender, the model performed better on the risk prediction of HCC prognosis. CONCLUSION: The present study constructed 10-gene signature as a novel prognostic marker for predicting survival in patients with HCC.

[Hemophagocytic lymphohistiocytosis: tortuous experience in the diagnosis and treatment of one lymphoma].

OBJECTIVE: The etiology of hemophagocytic lymphohistiocytosis (HLH) is complicated and difficult to diagnose, unexplained HLH often with hematological malignancies. Invasive biopsy can help to find etiology, the results may be affected by the technique and the location of the puncture site. Multiangle puncture can improve the success rate, but the corresponding risk increases. A patient with HLH was admitted to Affiliated Hospital of Zunyi Medical College. The etiology was unknown. Active symptomatic support treatment was conducted, at the same time, finding the evidence of viral infection, autoimmune disease related detection, blood culture, bone marrow puncture smear and spleen biopsy were performed respectively to find the pathogen basis. Spleen hemorrhage was not being controlled after spleen biopsy in patients, and emergency splenectomy was adopted to stop bleeding for saving lives. Finally, the patients died of low protein, pulmonary edema and respiratory failure. The bone marrow puncture and spleen biopsy failed to provide the basis for tumor invasion, while the spleen pathological slices plus immunohistochemical indicate diffuse large B cell lymphoma (DLBCL) after splenectomy, which was identified as malignant tumor-associated hemophagocytic syndrome. Underscoring the high risk of bleeding after tumor-associated splenomegaly puncture and the importance of having emergency plans. Through analyzing the clinical characteristics, diagnosis and treatment of this patient, we hope to improve the clinicians' understanding of HLH and lymphoma.