Readiness for hospital discharge and its association with post-discharge outcomes among oesophageal cancer patients after oesophagectomy: A prospective observational study.

AIM: To examine the level and influencing factors of discharge readiness among patients with oesophageal cancer following oesophagectomy and to explore its association with post-discharge outcomes (post-discharge coping difficulty and unplanned readmission). BACKGROUND: Oesophageal cancer is common and usually treated via oesophagectomy in China. The assessment of patient's discharge readiness gradually attracts attention as patients tend to be discharged more quickly. DESIGN: Prospective observational study. The STROBE statement was followed. METHODS: In total, 154 participants with oesophageal cancer after oesophagectomy were recruited in a tertiary cancer centre in Southern China from July 2019 to January 2020. The participants completed a demographic and disease-related questionnaire, the Quality of Discharge Teaching Scale and Readiness for Hospital Discharge Scale before discharge. Post-discharge outcomes were investigated on the 21st day (post-discharge coping difficulty) and 30th day (unplanned readmission) after discharge separately. Multiple linear regressions were used for statistical analysis. RESULTS: The mean scores of discharge readiness and quality of discharge teaching were (154.02 ± 31.58) and (138.20 ± 24.20) respectively. The quality of discharge teaching, self-care ability, dysphagia and primary caregiver mainly influenced patient's discharge readiness and explained 63.0% of the variance. The low discharge readiness could predict more risk of post-discharge coping difficulty (r = -0.729, p < 0.01) and unplanned readmission (t = -2.721, p < 0.01). CONCLUSIONS: Discharge readiness among patients with oesophageal cancer following oesophagectomy is influenced by various factors, especially the quality of discharge teaching. A high discharge readiness corresponds to good post-discharge outcomes. IMPLICATIONS FOR THE PROFESSION AND PATIENT CARE: Healthcare professionals should improve the discharge readiness by constructing high-quality discharge teaching, cultivating patients' self-care ability, mobilizing family participation and alleviating dysphagia to decrease adverse post-discharge outcomes among patients with oesophageal cancer. PATIENTS OR PUBLIC CONTRIBUTION: Patients with oesophageal cancer after oesophagectomy who met the inclusion criteria were recruited.

Synchronous Hepatoblastoma and Neuroblastoma in Two Chinese Infants.

Background: Commonly, pediatric solid tumors occur independently. Only two patients with synchronous hepatoblastoma (HBL) and neuroblastoma (NBL) have been reported. Case reports: Two Chinese infants presented with abdominal mass at 10 and 8 months. Computed tomography (CT) scans in both revealed hepatic masses with additional mediastinal or adrenal masses. Pathology confirmed synchronous HBLs in the liver and NBLs in the mediastinum and adrenal. Next generation sequencing (NGS) found no remarkable germline mutations. Both patients received gross total resections with chemotherapy before or after surgery. They were followed up for 36 and 8 months, and recovered well. Conclusion: These two cases of synchronous HBL and NBL tumors lacked significant genetic alterations.

[Clinicopathologic features of peripheral neuroblastic tumors].

OBJECTIVE: To study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features. METHODS: The clinical and pathologic findings were retrospectively reviewed in 121 cases of peripheral neuroblastic tumor. The clinical outcomes of patients were evaluated. The three-year event-free survival rate was analyzed, with respect to age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index. RESULTS: The median age at diagnosis was 2.7 years; and 96 cases (79.3%) occurred in patients younger than 5 years old. The number of cases in Evan's staging I, II, III, IV and IVs was 24, 39, 24, 29 and 5, respectively. There were 82 cases of neuroblastoma (NB) (including 2 cases of undifferentiated NB, 52 cases of poorly differentiated NB and 28 cases of differentiating NB), 9 cases of ganglioneuroblastoma, intermixed type (GNBi), 19 cases of ganglioneuroma, maturing type (GN) and 11 cases of ganglioneuroblastoma, nodular type (GNBn). Forty-nine cases were in the favorable histology subgroup and 72 cases in the unfavorable histology subgroup. The overall three-year event-free survival rate of the 121 cases was 73.0% ± 4.3%. The three-year event-free survival rates were associated with age (P = 0.002), Evan's staging (P = 0.000), histologic category (P = 0.000), mitosis-karyorrhexis index (P = 0.043), prognostic subgroup (P = 0.000). CONCLUSIONS: Most of the peripheral neuroblastic tumors occur in the children younger than 5 years old. It is composed of NB, GNBi, GN and GNBn. The three-year event-free survival rate is approximately 70%. Significant prognostic parameters include age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy.

Desmoplastic small round-cell tumors (DSRCT) frequently develop in the retroperitoneum, pelvis, omentum, and mesentery. Here, we present an unusual case of primary DSRCT in the liver. The patient was an 11-year-old boy with multiple solid masses in the liver parenchyma. The tumor in the needle biopsy had a histology revealing a small round cell morphology and desmoplasia. It shows the immunohistochemical features of DSRCT and documentation of EWSR1-WT1 fusion.A potential diagnostic pitfall is exerted when evaluating liver biopsy, in which DSRCT is a great mimicker and may be easily confused with more common liver malignancies of childhood, such as hepatoblastoma, calcifying nested stromal-epithelial tumor, undifferentiated embryonal sarcoma, and other small round cell tumors, as well as the fibrolamellar variant of hepatocellular carcinoma. This distinction is critical because an accurate therapeutic approach requires a correct diagnosis.

Relationship between tumor viability during treatment and the clinical outcomes of patients with bladder/prostate rhabdomyosarcoma: a single-center experience.

BACKGROUND: To analyze the relationship between tumor viability in specimens retrieved at second-look procedures (SLPs) and clinical outcomes in patients with bladder/prostate rhabdomyosarcoma (BP RMS). METHODS: We retrospectively analyzed patients` characteristics, times between diagnoses and SLPs, types of SLPs, the pathological findings, and clinical outcomes between January 2003 and May 2014. RESULTS: A total of 29 patients underwent at least one SLP before completing chemotherapy, including 24 boys and 5 girls. The mean age was 36 months. No patients with clinical/radiographic complete responses (CRs) had viable tumor cells and 7/18 patients (38.9%) without CR had no viable tumor cells. Seven patients experienced tumor relapse, progression, and metastasis, and three of these survived. Five-year event-free survival (EFS) rates were 88.5% in 18 patients without viable tumor at SLPs and 54.5% in 11 patients with viable tumor (Cox proportional hazards adjusted P=0.045). The respective five-year overall survival (OS) rates were 94.1% and 72.7% (Cox proportional hazards adjusted P=0.175). CONCLUSIONS: EFS was increased in patients with BP RMS having no viable tumor cells; however, OS was comparable in patients with and without viable tumor cells. Patients who achieved CR during the treatment generally had no viable tumor cells.

Immediate postoperative experiences before discharge among patients with rectal cancer and a permanent colostomy: A qualitative study.

PURPOSE: Patients with rectal cancer with a permanent colostomy often have issues in physical, psychological and social domains. Since discharge is an important transition period, the patient experience at that time is worthy of attention. The aim of this study was to explore the immediate postoperative experiences before discharge among patients with rectal cancer and a permanent colostomy in China. METHODS: A qualitative design was used. Semi-structured interviews were conducted with 18 patients newly living with colostomy in China, who were asked open-ended questions about their postoperative experience. The thematic analysis approach was used to analyze the data. RESULTS: Four themes and twelve sub-themes were identified from the interviews: (1) psychological reactions (stoma self-acceptance, negative emotion and social isolation), (2) daily life concern (daily life misunderstandings, sexual life compromise and work restriction), (3) stoma care consideration (strong stoma self-care willingness, decreased stoma self-care confidence and access to high-quality stoma care), and (4) support from others (enterostomal nurses, family members and stoma friends). CONCLUSION: Colostomy patients experience various obstacles in physiological, psychological, and societal aspect. Nurses should provide effective discharge guidance to correct misunderstandings about the stoma, refer patients to psychological care, and promote transitional care.

Inflammatory Myofibroblastic Tumor After Treatment of Wilms Tumor in a 6-Year-Old Boy: A Case Report and Literature Review.

Inflammatory myofibroblastic tumor (IMT) is a rare tumor with an indolent course. It is less often reported as a second tumor that occurs after treatment of malignant tumors in pediatric patients. Here, we report a case of IMT following Wilms tumor (WT), and conduct a literature review concerning IMTs and WT to evaluate the diagnostic possibility of IMT as a second tumor. The coexistence of the 2 tumors may cause confusion as to whether they share genetic links or that IMTs may appear as late effects of the treatment of WT.

Inflammatory Myofibroblastic Tumor of the Urinary Bladder and Ureter in Children: Experience of a Tertiary Referral Center.

PURPOSE: To present the clinicopathological characteristics and outcome of children with bladder and ureteral inflammatory myofibroblastic tumors (IMTs) in our center. METHODS: We reviewed the medical records of patients with bladder and ureteral IMTs from 2010 to 2018. We recorded patients' demographic data, presentation, hemoglobin level, presence of hydronephrosis, tumor size, treatment, and outcomes. RESULTS: Eight patients with bladder IMTs and 3 with ureteral IMTs were treated at our center during this period. The mean age was 7.1 years. Four patients presented with anemia at diagnosis with the mean hemoglobin level 84.5 g/L. Among patients with bladder IMTs, 5 were male and 3 were female. The most common symptom was lower urinary symptoms in 6 patients, followed by hematuria in 4 patients. 2 patients had complications of hydronephrosis and hydroureter. Among patients with ureteral IMTs, 2 were male and one was female. The most common symptom was abdominal pain, and 3 patients presented with upper urinary tract dilation. All patients underwent surgery. A total of 81.8% were positive for anaplastic lymphoma kinase. Cytokeratin (CK) expression was present in all patients with bladder IMTs, while it was negative in 2 patients with ureteral IMTs. During mean follow-up of 43.4 months, all patients survived event-free. CONCLUSION: The presence of hydronephrosis and hydroureter is rare in patients with bladder IMTs. Anemia caused by hematuria should be raised the index of suspicion for IMTs. Children with bladder and ureteral IMTs had excellent prognosis. The expression pattern of CK varied between bladder and ureteral IMTs.

[Clinicopathologic features of systemic EBV-positive T-cell lymphoproliferative disease of childhood].

OBJECTIVE: To study the clinicopathologic features, diagnosis and differential diagnosis of systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood (CSEBV(+)T-LPD). METHODS: Thirty cases of CSEBV(+)T-LPD were retrospectively studied by light microscopy, immunohistochemistry and in-situ hybridization for EBV-encoded RNA (EBER). The clinical information and follow-up data were analyzed. RESULTS: Nineteen of the 30 patients were males and 11 females. The median age of disease onset was 9 years (range = 1.5 to 32 years). The average duration between disease onset and diagnosis was 14 months. The major clinical manifestations were fever (96.7%), lymphadenopathy (83.3%) and hepatosplenomegaly (66.7%). Cutaneous manifestations were not uncommon, which included hypersensitivity to mosquito bite (13.3%) and skin rash (20.0%). Six of the 20 patients died on follow up. Histologically, the lymph nodes showed expansion of T zone, with diminished or effaced lymphoid follicles. The lymphoid cells were of small to medium size. Scattered large lymphoid cells were also identified in the expanded T zone. Furthermore, the liver and spleen showed mild to marked sinusoidal infiltration. In some cases, various degrees of sinus histiocytosis with erythrophagocytosis were present. Skin biopsies showed mild to marked degree of lymphocytes infiltration in dermis. Immunohistochemical study and in-situ hybridization showed that the EBER-positive cells were of T lineage and CD3 positive. They also expressed cytotoxic molecules granzyme B and TIA-1. Seven of the 8 cases examined were CD8 positive, while the remaining case was mainly CD4 positive. Thirteen of 15 cases were shown to be CD56 negative. The number of EBER-positive cells ranged from 5 to more than 500 per high-power field. These cells included small to large lymphoid cells located mostly in the expanded T zone and sometimes in the germinal centers. Nine of the 30 cases, which consisted mainly of medium to large-sized lymphoid cells, were also EBER positive. CONCLUSIONS: Systemic EBV-positive T-cell lymphoproliferative disease of childhood occurs most often in children and young adults, with a median age of 9 years. It has a subacute or chronic clinical course. Most of the patients have evidence of systemic disease, often with lymph node, liver, spleen and skin involvement. It carries a poor clinical outcome and can be life-threatening. The disease is characterized by a clonal proliferation of EBV-infected T cells with cytotoxic immunophenotype. Definitive diagnosis requires correlation between clinical, pathologic and ancillary investigation findings.

Thrombotic storm in a 4-year-old boy with a thrombus in the right atrium.

Thrombotic storm (TS) is a rare disease, especially with thrombus in the heart of pediatric patient. We present a case of a 4-year-old boy, who was diagnosed with TS during his first hospitalization due to lower extremity deep venous thrombosis, pulmonary embolism, and thrombosis of the inferior vena cava, cerebral, left internal jugular, portal, renal, and iliac veins. He was eventually prescribed with rivaroxaban to control thrombosis after 30 days of successive use of low-molecular-weight heparin, unfractionated heparin, and warfarin, which were demonstrating little effect on preventing thrombosis, and the patient was intolerant to argatroban. While his lupus anticoagulant ratio was slightly above the normal range and no other potential causes such as congenital thrombophilia, severe infection, malignancy, and trauma were confirmed, we suspected antiphospholipid antibody syndrome and prescribed glucocorticoid and rituximab to control the disease. After 36 days of admission, ultrasonography showed recanalization of the former thrombus. One month after discharge, a tumor embolus resembling a mass emerged in his right atrium under effective anticoagulant therapy. During his second admission, he underwent surgical thrombectomy, and pathological examination confirmed the mass to be a platelet-rich thrombus rather than tumor embolus or infection. Considering the suspected antiphospholipid antibody syndrome as the cause of the TS, we prescribed aspirin combined with rivaroxaban to prevent thrombosis. In this case, surgery and pathology shed light on the type of thrombus that emerged from the inferior vena cava and traveled to the heart, which is the possible potential cause of TS. It also changed our therapeutic strategy to antiplatelet therapy combined with anticoagulant therapy to control the disease.

Clinical characteristics of hemolytic uremic syndrome secondary to cobalamin C disorder in Chinese children.

BACKGROUND: The present study was undertaken to investigate the clinical characteristics of hemolytic uremic syndrome (HUS) secondary to cobalamin C disorder (cbl-C disorder). METHODS: We reviewed retrospectively the medical records of 3 children with HUS secondary to cbl-C disorder who had been treated between April 1, 2009 and October 31, 2013. RESULTS: The 3 patients with HUS secondary to cbl-C disorder presented with progressive hemolytic anemia, acute renal failure, thrombocytopenia, poor feeding, and failure to thrive. Two of the 3 patients once had high blood pressure. The mutations of c.609G>A (p.W203X), c.217C>T (p.R73X) and c.365A>T (p.H122L) in the methylmalonic aciduria (cobalamin deficiency) cbl-C type, with homocystinuria gene were detected in the 3 patients. In these patients the levels of lactate dehydrogenase and homocysteine in serum were elevated and the level of methylmalonic acid (MMA) in urine was also elevated. After treatment with hydroxocobalamin, 2 patients were discharged with no obvious abnormal growth and neurological development and 1 patient died of multiple organ failure. CONCLUSIONS: The results of this study demonstrated that cbl-C disorder should be investigated in any child presenting with HUS. The high concentrations of homocysteine and MMA could be used for timely recognization of the disease. Once the high levels of plasma homocystein and/or plasma or urine MMA are detected, the treatment with parenteral hydroxocobalamin should be prescribed immediately. The early diagnosis and treatment would contribute to the good prognosis of the disease.

[Study on MYCN gene amplification and CD44 expression in neuroblastoma].

OBJECTIVE: To assess the relationship between MYCN amplification in neuroblastoma (NB), tumor stage and prognosis; and to evaluate the usefulness of CD44 in predicting prognosis of NB. METHODS: Differential polymerase chain reaction (D-PCR) with serial dilution assay was used to quantify the MYCN gene copy number in 33 paraffin-embedded tissue samples of NB. All the samples were also studied by immunohistochemistry for CD44. The results were correlated with various prognostic factors of NB, including patient age, tumor stage, pathologic type and MYCN gene amplification. RESULTS: MYCN amplification was identified in 10 of the 33 samples studied (30.3%), which all were in high clinical stage (stage III or IV) and occurred in patients older than 1 year of age. MYCN amplification also significantly correlated with poor clinical outcome (P < 0.01). CD44 was positive in 21 cases and often occurred in patients below 1 year of age, in low clinical stage, with favorable histology and without MYCN gene amplification. The two-year survival rate of CD44-positive group (57.1%) was higher than that of CD44-negative group (8.3%, P < 0.01). Stronger CD44 expression was also associated with better prognosis (P < 0.01). CONCLUSIONS: MYCN gene amplification is significantly associated with advanced tumor stage and poor prognosis in patients with NB. CD44 expression is a reliable marker for better prognosis and is complementary to MYCN amplification assay. D-PCR with serial dilution assay is also suitable for clinical use in quantifying MYCN copy number in NB.

[Clinicopathological study of 145 childhood rhabdomyosarcoma cases].

OBJECTIVE: To study the clinicopathological, immunohistochemical and electron microscopic characteristics of pediatric rhabdomyosarcomas (RMS). METHODS: One hundred and forty-five cases of pediatric rhabdomyosarcomas were studied by routine histological, immunohistochemical and electron microscopic studies. RESULTS: There were 97 male and 48 female patients with ages ranging from 4 months to 13 years and a mean of 4.2 years. The follow-up period of 100 patients was from 1 year to 20 years with a mean of 5 years after diagnosis. All cases were subtyped into the following histological categories: embryonal RMS, botryoid RMS, spindle cell RMS, alveolar RMS and solid RMS. Histopathological subtypes, tumor site and tumor stage correlated significantly with the patients' 5 years survival. The best prognosis was observed in spindle cell and botryoid RMS. Embryonal RMS carried an intermediate prognosis. Patients with alveolar RMS and solid RMS had the worst prognosis. Tumors involving bladder, head and neck carried a favorable clinical outcome. Patients with tumors involving trunk extremities retroperitoneum and pelvis did poorly. Immunohistochemically, all cases were positive for Vimentin. The positive staining rates for desmin, SMA and myoglobin were 78%, 75% and 37%, respectively. All tumors were negative for NSE, CD99 and LCA. Electron microscopy study showed features of myofilament and sarcomere in 10 of 15 cases. CONCLUSIONS: RMS is the most common soft tissue sarcoma of childhood. Immunohistochemistry and electron microscopy are helpful in diagnosis and classification of RMS.

[Pathological changes in rat model of urinary calculus induced by melamine].

OBJECTIVE: To investigate melamine-induced pathological changes in the kidney. METHOD: Wistar rats were fed with a diet containing 0, 1% and 2% melamine for 15 weeks. After melamine feeding was stopped, various outcome measures were observed for 4 weeks. RESULT: Rats fed with melamine showed reduced caloric intake, slower weight gain and impaired renal function. The blood urea nitrogen of group A and B [(13.23 ± 5.10) mmol/L and (18.30 ± 5.90) mmol/L, respectively] and serum creatinine levels of group B [(19.90 ± 2.90) mmol/L] were higher than that of group C [(8.23 ± 2.30) mmol/L and (10.04 ± 1.73) mmol/L](P < 0.01, respectively). Additionally, the kidney coefficients of group A and B were higher than that of group C (P < 0.01, respectively). Crystals, tubular ectasia and interstitial inflammation and fibrosis were found in the kidneys of melamine fed rats. Four weeks after discontinuation of feeding with melamine-contained diet, the caloric intake and weight of the rats increased, the coefficients of the kidney decreased, and the blood urea nitrogen of group A and B [(17.96 ± 2.04) mmol/L and (19.20 ± 3.36) mmol/L, respectively] and serum creatinine levels of group B [(24.20 ± 5.28) mmol/L], which became worse than 4 weeks before (P < 0.01;P < 0.05, respectively), and were still higher than that of group C [(8.30 ± 1.79) mmol/L and (9.87 ± 2.71) mmol/L, P < 0.01, respectively]. Crystals remained inside the kidney, changes in the renal interstitium did not improve. CONCLUSION: (1) Melamine-induced urinary calculus rat model can be established by feeding 3-week old male Wistar rats with a diet containing 2% melamine for 15 weeks. The main constituent of the urinary calculus was melamine (> 90%), with a little uric acid and traces of cyanuric acid. (2) Melamine damaged the renal function, formed renal crystals, and led to the pathological changes of kidneys. All the influences seemed to be dose-depended and was related with the obstruction of the crystals or calculus in the kidney. (3) The renal function and the pathological changes did not improve 4 weeks after discontinuation of feeding with melamine-contained diet.