Alveolar soft part sarcoma in children: a clinicopathological study of 13 cases / 中华病理学杂志

To investigate the clinicopathological manifestations, molecular genetic, diagnostic histology and differential diagnosis of alveolar soft part sarcoma (ASPS) in children. A total of 13 cases of ASPS diagnosed at Beijing Children's Hospital from August 2009 to November 2018 were collected. HE staining, histochemical staining for PAS and D-PAS, immunohistochemical (IHC) staining for TFE3, INI1 and CD68 and florescence in situ hybridization (FISH) for TFE3 gene translocation were performed. There were four males and nine females, age ranged from 1 year and 2 months to 13 years and 8 months (mean 7.8 years); and four patients were under 5 years old. Histologically, the tumors showed a distinctive and characteristic nested or organoid growth pattern (11 cases) or solid, diffuse growth (2 cases). The tumor cells possessed abundant eosinophilic, or glycogen-rich and clear to vacuolated cytoplasm. The chromatin was relatively dispersed, with prominent and pleomorphic nucleoli; mitotic figures were rare. Vascular invasion was frequently seen. IHC staining showed specific nuclear TFE3 staining. The tumor cells were also positive for INI1,CD68 and vimentin; but were negative for MyoD1, Myogenin, CK and S-100 protein. Seven cases showed PAS and D-PAS staining, with fuchsia acicular or rod-shaped crystals in tumor cytoplasm. Nine cases showed TFE3 break-apart signals by FISH. ASPS is a rare soft tissue sarcoma in children. Compared with ASPA in adults, it has both similarities and unique clinicopathologic characteristics. The diagnosis needs to be confirmed by combining clinical, pathologic, IHC and genetic testing.

Clinicopathologic features of peripheral neuroblastic tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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).</p><p><b>CONCLUSIONS</b>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.</p>

Detection of group B streptococcus in the cases died of neonatal pneumonia / 中华儿科杂志

<p><b>OBJECTIVE</b>From the 1970s, group B streptococci (GBS) have been widely recognized as an important pathogen in neonatal infectious disease, and it emerged as the leading cause of neonatal morbidity and mortality in the Western world. However, there are few data on the prevalence of neonatal GBS infections in China. The aim of this retrospective study was to estimate whether GBS is an important pathogen in severe neonatal pneumonia, and to develop a method for detection of GBS infections in fatal neonatal pneumonia.</p><p><b>METHODS</b>A total of 234 neonatal cases (0 - 28 days) died in Beijing Children's Hospital from 1953 to 2004 were enrolled in this study. They were divided into two groups. Two hundred cases diagnosed as neonatal pneumonia were assigned to study group and the remaining 34 cases died of neonatal hemolysis or surgical operation without any confirmed infectious diseases were designated as control group. Formalin-fixed, paraffin-embedded lung tissues were used as source for total genomic DNA extraction. PCR and Southern blot analyses were applied to detect GBS specific cfb gene target sequence. And the clinical data of these cases were reviewed as well.</p><p><b>RESULTS</b>In the study group, 52 cases were detected positive for GBS DNA by PCR (26%), 130 cases were positive by Southern blot (65%). In the control group, 1 case was detected positive GBS DNA by PCR (3%), and 6 cases were positive by Southern blot (18%). The positive rate was significantly lower in the control group than that in the study group (PCR, chi(2) = 8.82, P < 0.01; Southern blot, chi(2) = 26.77, P < 0.01). The positive rate in the neonates younger than 7 days (early-onset) was significantly higher than that in neonates older than 7 days (late-onset) (PCR: 37% vs. 13%, chi(2) = 15.537, P < 0.01; Southern blot: 72% vs. 52%, chi(2) = 4.37, P < 0.05). In the positive early-onset cases, 39% of whom were born prematurely (29/74). Out of the 200 cases, 75 had complete clinical data. Neither blood nor lung culture for GBS was performed in any of these cases. But risk factors were identified for 35 cases, such as premature delivery, low birth weight, premature rupture of the membrane and abnormal amniotic fluid. GBS was positive in all these cases. Severe apnea appeared to be a common symptom and was present in most of the early-onset GBS-positive cases, while cough and wheezing were found in most of the late-onset GBS-positive cases. In the control group, one PCR positive case was suffered from malignant teratoma. The other 5 positive cases confirmed by Southern blot were diagnosed as kernicterus, hepatoma, aproctia complicating with cysti-urethral fistula, neonatal physio logical bleeding and aproctia complicated with archo-perineal fistula.</p><p><b>CONCLUSION</b>Group B Streptococcus is an important pathogen in fatal neonatal pneumonia, especially in early-onset cases. southern blot may be a sensitive method to detect GBS infection in archival tissues. In the clinical work, more attention should be paid to the neonates with GBS risk factors. And GBS detection and prevention in neonates should be put into clinical practice.</p>