Vitamin K prophylaxis in neonates: comparing two different oral regimens.

OBJECTIVE: This prospective study compared PIVKA-II and PT-INR levels in infants who received two vitamin K (VK) prophylactic regimens. METHODS: A single institution administered 119 healthy newborns 2 mg of VK syrup. Infants were assigned to a 3-time regimen (n = 56) with VK at birth, five days (5D), and 1-month-old (1 M), or a 13-time regimen (n = 63) with VK at birth, 5D, and then weekly for 11 weeks. RESULTS: The 13-time regimen significantly lowered PIVKA-II and reduced PT-INR at 1 M in both breastfed (PIVKA-II: 18-16 mAU/mL, p = 0.02; PT-INR: 1.37-1.13, p < 0.01) and formula-fed infants (PIVKA-II: 18-15 mAU/mL, p = 0.01; PT-INR: 1.54-1.24, p < 0.01), compared to baseline measurements taken at 5D. The 3-time regimen did not significantly alter PIVKA-II levels and only improved PT-INR (2.00-1.50, p < 0.01) in formula-fed infants. CONCLUSION: The 13-time VK regimen significantly enhanced coagulation profiles more effectively than the 3-time regimen.

Relationship between ETCO2 and PaCO2 under Changing Capnogram in Ventilated Infants with NAVA: An Observational Study.

This observational study evaluated the validity of end-tidal CO2 (ETCO2) as a surrogate for arterial PCO2 (PaCO2) in infants on neurally adjusted ventilatory assist (NAVA), particularly considering the influence of variable spontaneous breathing on capnography waveforms. The study involved 16 infants, analyzing 50 paired ETCO2 and PaCO2 values. Deming regression analysis highlighted a notably stronger correlation for maximum ETCO2 (r2 = 0.6783, p <0.0001) compared to mean ETCO2 (r2 = 0.5686, p <0.0001) and demonstrated a significantly weaker association for minimum ETCO2 (r2 = 0.1838). These findings emphasize the superior predictive value of maximum ETCO2 in estimating PaCO2, advocating its reliable use in clinical monitoring, especially given the dynamic capnography associated with NAVA's variable pressures. The results suggest ETCO2's potential to enhance noninvasive respiratory management, reduce the frequency of blood sampling, and improve overall care for infants requiring mechanical ventilation.

Timing of Elective Cesarean Section and Neonatal Outcomes in Term Singleton Deliveries: A Single-Center Experience.

OBJECTIVE: This study aimed to evaluate the timing of elective cesarean sections at 37 to 41 weeks from a tertiary hospital in Japan. The primary outcome was the rate of adverse neonatal outcomes, especially focusing on neonates delivered at 38 weeks of gestation. STUDY DESIGN: The study population was drawn from singleton pregnancies delivered following planned cesarean birth at the Fukuda Hospital from 2012 to 2019. Information on deliveries was obtained from the hospital database, which contains clinical, administrative, laboratory, and operating room databases. RESULTS: After excluding women with chronic conditions, maternal complications, indications for multiple births, or a neonate with an anomaly, 2,208 neonates remained in the analysis. Among adverse neonatal outcomes, the rate was significantly higher in neonates delivered at 37 weeks of gestation (unadjusted odds ratio [OR] = 13.22 [95% confidence interval [CI]: 6.28, 27.86], p < 0.001) or 38 weeks of gestation (unadjusted OR = 1.82 [95% CI: 1.04, 3.19], p = 0.036) compared with neonates delivered at 39 to 41 weeks. The adjusted risk of any adverse outcome was significantly higher at 380-1/7 weeks (adjusted OR = 2.40 [95% CI: 1.35, 4.30], p = 0.003) and 382-3/7 weeks (adjusted OR = 1.89 [95% CI: 1.04, 3.44], p = 0.038) compared with neonates delivered at 39 to 41 weeks, respectively. CONCLUSION: Our findings suggest that elective cesarean sections might be best scheduled at 39 weeks or later. When considering a cesarean at 38 weeks, it appears that 384/7 weeks of gestation or later could be a preferable timing in the context of reducing neonatal risks. However, as the composite outcome includes mostly minor conditions, the clinical significance of this finding needs to be carefully interpreted. KEY POINTS: · Timing of elective cesarean sections from 37 to 41 weeks was evaluated in a Japanese tertiary hospital.. · Neonates delivered at 37 and 38 weeks had higher adverse outcome rates compared with 39 to 41 weeks.. · Scheduling elective cesarean sections at least 384/7 weeks or later may reduce neonatal risk..

Effect of tidal volume and end tracheal tube leakage on end-tidal CO2 in very low birth weight infants.

OBJECTIVE: To examine the extents to which low tidal volume (VT) and endotracheal tube (ETT) leakeage influence the accuracy of ETCO2 for estimating arterial PCO2 (PaCO2) in very low birth weight (VLBW) infants with mechanical ventilation. STUDY DESIGN: An observational study. We evaluated a total of 287 paired ETCO2 and PaCO2 values as well as VTs obtained from 22 VLBW infants with ventilation. Deming regression, quadratic discriminant analysis, and Bland-Altman analysis were performed. RESULT: ETCO2 and PaCO2 were correlated (r2 = 0.5897, p < 0.0001). A quadratic discrimination analysis of the VT and the percentage of leak yielded 70.4% [95%CI, 65.1 to 75.7] discrimination for the agreement between ETCO2 and PaCO2. ETCO2 was strongly correlated with PaCO2 in the discriminant function Z > 0 group (r2 = 0.7234, p < 0.0001). CONCLUSION: Our results indicate that ETCO2 is a good surrogate for PaCO2 when VT is high and ETT leak is low.

Feasibility of neurally synchronized and proportional negative pressure ventilation in a small animal model.

RATIONALE: Synchronized positive pressure ventilation is possible using diaphragm electrical activity (EAdi) to control the ventilator. It is unknown whether EAdi can be used to control negative pressure ventilation. AIM: To evaluate the feasibility of using EAdi to control negative pressure ventilation. METHODS: Fourteen anesthetized rats were studied (380-590 g) during control, resistive breathing, acute lung injury or CO2 rebreathing. Positive pressure continuous neurally adjusted ventilatory assist (cNAVAP+ ) was applied via intubation. Negative pressure cNAVA (cNAVAP- ) was applied with the animal placed in a sealed box. In part 1, automatic stepwise increments in cNAVA level by 0.2 cmH2 O/µV every 30 s was applied for cNAVAP+ , cNAVAP- , and a 50/50 combination of the two (cNAVAP± ). In part 2: During 5-min ventilation with cNAVAP+ or cNAVAP- we measured circuit, box, and esophageal (Pes) pressure, EAdi, blood pressure, and arterial blood gases. RESULTS: Part 1: During cNAVAP+ , pressure in the circuit increased with increasing cNAVA levels, reaching a plateau, and similarly for cNAVAP- , albeit reversed in sign. This was associated with downregulation of the EAdi. Pes swings became less negative with cNAVAP+ but, in contrast, Pes swings were more negative during increasing cNAVAP- levels. Increasing the cNAVA level during cNAVAP± resulted in an intermediate response. Part 2: no significant differences were observed for box/circuit pressures, EAdi, blood pressure, or arterial blood gases. Pes swings during cNAVAP- were significantly more negative than during cNAVAP+ . CONCLUSION: Negative pressure ventilation synchronized and proportional to the diaphragm activity is feasible in small animals.

Combined subtotal gastrectomy and splenectomy after partial splenic embolization for a patient with gastric cancer and immune thrombocytopenic purpura: A case report.

INTRODUCTION: Immune thrombocytopenic purpura is an acquired thrombocytopenia. Preoperative management of thrombocytopenia is important in patients with gastric cancer. Partial splenic embolization can be effective for patients with thrombocytopenia, but could lead to ischemic necrosis of the remnant stomach when performing subtotal gastrectomy with splenectomy. PRESENTATION OF CASE: The patient is an 84-year old woman evaluated for anemia. Endoscopy revealed an advanced gastric cancer with bleeding. The patient also had immune thrombocytopenic purpura with a platelet count <50,000/µL. Administration of platelets did not increase the platelet count. Partial splenic embolization was performed followed by administration of high-dose immunoglobulin. The platelet count was over 50,000/µL preoperatively. The patient underwent combined subtotal gastrectomy and splenectomy, followed by an uneventful course. DISCUSSION: Patients with immune thrombocytopenic purpura and advanced gastric cancer can have anemia. Partial splenic embolization has been used to treat patients with refractory immune thrombocytopenic purpura as an alternative to splenectomy. Preoperative partial splenic embolization and high-dose immunoglobulin therapy resulted an increased platelet count in this patient. Elderly patients with gastric cancer have a high risk of postoperative complications. Patients with gastric cancer undergoing total gastrectomy have an impaired postoperative quality of life compared to those who undergo subtotal gastrectomy. We performed a subtotal gastrectomy and splenectomy as a function-preserving operation, completed safely by maintaining blood flow to the remnant stomach. CONCLUSION: Partial splenic embolization is effective for patients with immune thrombocytopenic purpura and gastric cancer. Combined subtotal gastrectomy and splenectomy is achieved by preserving blood flow to the remnant stomach.

Polymyxin B-immobilized fiber column direct hemoperfusion and continuous hemodiafiltration in premature neonates with systemic inflammatory response syndrome.

BACKGROUND: There have been no previous studies regarding whether combined use of Polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP) and continuous hemodiafiltration (CHDF) is helpful in the treatment of preterm infants with systemic inflammatory response syndrome (SIRS) and hypercytokinemia. METHODS: A retrospective review was carried out of 18 SIRS infants born at gestational week 24-28. Eight with blood interleukin (IL)-6 ≥ 1000 pg/mL were treated actively with 2 h PMX-DHP followed by 2 h PMX-DHP and CHDF. Ten with IL-6 < 500 pg/mL were treated conventionally (with neither PMX-DHP nor CHDF) and served as controls. RESULTS: Demographic characteristics were similar except for IL-6, arterial-to-alveolar oxygen tension ratio (a/APO2 ), and number of immature neutrophils between the two groups. Baseline a/APO2 was significantly lower in infants with than without active treatment (0.44 vs 0.67, respectively, P = 0.002). After 4 h treatment, the IL-6 decreased to < 500 pg/mL in all eight infants, and a/APO2 improved significantly to 0.62 (P = 0.006). Bronchopulmonary dysplasia occurred in a similar proportion (63%, 5/8 vs 80%, 8/10, respectively), but the number of days on inhaled oxygen (30 vs 47 days, respectively, P = 0.033) and tracheal intubation (36 vs 51 days, respectively, P = 0.040) was significantly lower in infants with than without active treatment. Prevalence of adverse events was similar (13%, 1/8 vs 50%, 5/10 for active vs conventional treatment, respectively). CONCLUSION: Active treatment with PMX-DHP and CHDF was helpful in the reduction of days on inhaled oxygen and tracheal intubation in preterm SIRS infants with hypercytokinemia. Further prospective randomized studies are warranted.

Feasibility of neurally adjusted positive end-expiratory pressure in rabbits with early experimental lung injury.

BACKGROUND: During conventional Neurally Adjusted Ventilatory Assist (NAVA), the electrical activity of the diaphragm (EAdi) is used for triggering and cycling-off inspiratory assist, with a fixed PEEP (so called "Triggered Neurally Adjusted Ventilatory Assist" or "tNAVA"). However, significant post-inspiratory activity of the diaphragm can occur, believed to play a role in maintaining end-expiratory lung volume. Adjusting pressure continuously, in proportion to both inspiratory and expiratory EAdi (Continuous NAVA, or cNAVA), would not only offer inspiratory assist for tidal breathing, but also may aid in delivering a "neurally adjusted PEEP", and more specific breath-by-breath unloading. METHODS: Nine adult New Zealand white rabbits were ventilated during independent conditions of: resistive loading (RES(1) or RES(2)), CO2 load (CO2) and acute lung injury (ALI), either via tracheotomy (INV) or non-invasively (NIV). There were a total of six conditions, applied in a non-randomized fashion: INV-RES(1), INV-CO2, NIV-CO2, NIV-RES(2), NIV-ALI, INV-ALI. For each condition, tNAVA was applied first (3 min), followed by 3 min of cNAVA. This comparison was repeated 3 times (repeated cross-over design). The NAVA level was always the same for both modes, but was newly titrated for each condition. PEEP was manually set to zero during tNAVA. During cNAVA, the assist during expiration was proportional to the EAdi. During all runs and conditions, ventilator-delivered pressure (Pvent), esophageal pressure (Pes), and diaphragm electrical activity (EAdi) were measured continuously. The tracings were analyzed breath-by-breath to obtain peak inspiratory and mean expiratory values. RESULTS: For the same peak Pvent, the distribution of inspiratory and expiratory pressure differed between tNAVA and cNAVA. For each condition, the mean expiratory Pvent was always higher (for all conditions 4.0 ± 1.1 vs. 1.1 ± 0.5 cmH2O, P < 0.01) in cNAVA than in tNAVA. Relative to tNAVA, mean inspiratory EAdi was reduced on average (for all conditions) by 19 % (range 14 %-25 %), p < 0.05. Mean expiratory EAdi was also lower during cNAVA (during INV-RES(1), INV-CO2, INV-ALI, NIV-CO2 and NIV-ALI respectively, P < 0.05). The inspiratory Pes was reduced during cNAVA all 6 conditions (p < 0.05). Unlike tNAVA, during cNAVA the expiratory pressure was comparable with that predicted mathematically (mean difference of 0.2 ± 0.8 cmH2O). CONCLUSION: Continuous NAVA was able to apply neurally adjusted PEEP, which led to a reduction in inspiratory effort compared to triggered NAVA.

Population-based study of esophageal and small intestinal atresia/stenosis.

BACKGROUND: The aim of this study was to describe the prevalence of esophageal atresia/stenosis and small intestinal atresia/stenosis in Nagano, Japan, together with associated anomalies, prenatal diagnosis and survival. METHODS: A population-based cohort study of the prevalence of esophageal atresia/stenosis and small intestinal atresia/stenosis was conducted in Nagano in January 1993-December 2011. The Mann-Whitney test, χ(2) test and Kruskal-Wallis test were used to compare variables. P < 0.05 was considered statistically significant. RESULTS: In total, 74 cases of esophageal atresia/stenosis and 87 cases of small intestinal atresia/stenosis (31 duodenal, 56 jejuno-ileal) were identified. Prevalences were 1.97 for esophageal atresia/stenosis and 2.23 for small intestinal atresia/stenosis (0.83 for duodenal atresia/stenosis and 1.49 for jejuno-ileal atresia/stenosis) per 10,000 births, respectively. The prevalence of esophageal atresia/stenosis increased significantly from 1993-2001 to 2002-2011 (relative risk [RR], 1.6), as did the prevalences of duodenal atresia/stenosis (RR, 2.2) and jejuno-ileal atresia/stenosis (RR, 3.1). Chromosomal anomalies, particularly trisomy 21, were seen significantly more often in association with duodenal atresia/stenosis (55%) than with esophageal atresia/stenosis (28%, P < 0.01) or jejuno-ileal atresia/stenosis (2%, P < 0.01). The proportion of patients associated with prenatally diagnosed chromosomal anomaly was higher compared to postnatal diagnosis (P < 0.01) in the esophageal atresia/stenosis group. CONCLUSION: The prevalence of esophageal and small intestinal atresia/stenosis increased significantly from 1993-2001 to 2002-2011. Prenatally diagnosed esophageal atresia/stenosis is associated with multiple anomalies, particularly chromosomal anomalies, compared to other small intestine atresia/stenosis.

Evaluation of hypercoagulability using soluble fibrin monomer complex in sick newborns.

BACKGROUND: Soluble fibrin monomer complex (SFMC) and fibrin monomer (FM) are well known as markers for hypercoagulability, but such measurements have not been investigated in detail for the neonate. To identify the presence of a hypercoagulable state in sick newborns, the behavior of SFMC with special reference to those of other coagulation tests, and the relationships with other parameters of blood coagulation as well as lactate, which is considered to be the gold standard for assessing tissue hypoxia, were studied. METHODS: Records of 216 sick newborns, who had undergone blood coagulation tests, were retrospectively studied based on their medical records. RESULTS: SFMC had a significant correlation with d-dimer in infants with birthweight <1500 g, but no correlation was observed with prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen, anti-thrombin or platelet count. In contrast, in infants with birthweight ≥ 1500 g, SFMC was correlated with PT, APTT, fibrinogen, and d-dimer, but no correlation was observed with anti-thrombin or platelet count. In addition, SFMC was significantly higher in the high lactate group (lactate ≥ 4 mmol/L), compared with the low lactate group (<4 mmol/L). CONCLUSION: Measurement of blood SFMC is useful to monitor hypercoagulable state in sick newborns with hypoxia.

Successful tracheobronchial reconstruction of communicating bronchopulmonary foregut malformation and long segment congenital tracheal stenosis: a case report.

Communicating bronchopulmonary foregut malformation (CBPFM) and congenital tracheal stenosis (CTS) are difficult developmental disorders especially when they are presented simultaneously in a patient. The authors report a case of a newborn boy born at 37 weeks of gestation weighing 2356 g with CBPFM (right esophageal lung) and long segment CTS. Staged surgical repair (by-force endotracheal intubation for securing the airway followed by bronchotracheal anastomosis for CBPFM, tracheostomy with handmade, length-adjustable tracheostomy tube, and slide tracheoplasty) was performed. He has been healthy without tracheostomy for 25 months after slide tracheoplasty. This is the first report of a successful tracheobronchial reconstruction for a patient with a long segment CTS and CBPFM preserving the affected lung function.

Retrospective analysis of risk factors for development of liver dysfunction in transient leukemia of Down syndrome.

About 20% of patients with transient leukemia (TL), which is a disease noted in Down syndrome, are reported to develop hepatic fibrosis, which has a poor prognosis. The clinical factors related to the poor prognosis of TL were retrospectively analyzed in 25 patients, and criteria for starting chemotherapy were established. The initiation of chemotherapy was recommended when two or more of the following categories were fulfilled in the process of the disease: (1) a reduced hepatic functional reserve estimated by direct bilirubin, prothrombin time, and the presence of ascites, (2) an elevated level of hyaluronic acid (>500 U/mL), (3) respiratory failure or poor sucking associated with hepatosplenomegaly, and (4) demonstration of fibrosis by liver biopsy. When these criteria were applied to our cases, all patients who received chemotherapy remained alive. Our criteria are useful for selecting patients with TL at high risk of developing hepatic fibrosis and for starting chemotherapy.

Vitamin K prophylaxis and late vitamin K deficiency bleeding in infants: fifth nationwide survey in Japan.

BACKGROUND: In 1980, the first nationwide survey on late vitamin K deficiency bleeding (VKDB) in infants was conducted in Japan, and it was followed by the second, third and fourth nationwide surveys in 1985, 1988 and 1991, respectively. The fifth nationwide survey was designed to ascertain the epidemiology of late VKDB between January 1999 and December 2004. PATIENTS AND METHODS: Questionnaires were sent to 2161 hospitals in Japan that employed members of the Japan Pediatric Society in March 2005. Responses were received from 1373 hospitals, for a response rate of 63.5%. RESULTS: The total number of reported cases was 71, including 21 idiopathic type and 16 secondary type. The incidence of late VKDB was estimated to be 1.9 cases per 100,000 births (95% confidence interval: 1.2-3.0) during this survey period. In 34 cases, the presence or absence of any underlying disease was not clarified. A total of 67/71 infants were entirely breast-fed. Intracranial hemorrhaging was observed in 26 (63.4%) out of 41 infants whose bleeding sites were described in the questionnaires. In 63 cases (88.7%) of late VKDB found in the present survey, however, vitamin K had been given at least once either during or after the neonatal period. CONCLUSIONS: A reevaluation of the current prophylaxis strategy for late VKDB in infants is necessary.

[Brain hypothermia therapy for newborns with severe birth asphyxia: an experience from a single neonatal intensive care unit].

The object of this study was to determine the efficacy and safety of brain hypothermia therapy (BHT) for neonates with severe birth asphyxia in our neonatal intensive care unit (NICU). We retrospectively reviewed medical records to analyze the prognosis and the factors affecting the prognosis of 21 patients who underwent BHT at the NICU between 2001 and 2007. The prognosis of those 21 patients at the time of discharge from the NICU was as follows: good-11 patients (52.4%); disability-5 patients (23.8%); and death-5 patients (23.8%). The ten poor prognosis patients (disability: 5, death: 5) had a shorter gestational period, a lower Apgar score, and a significantly higher blood lactate level in comparison with good-prognosis newborns. In particular, a gestational period of less than 34 weeks (3 patients) and a blood lactate level of at least 200 mg/dl (6 out of 7 patients) are considered to be factors for a poor prognosis. In addition, intraventricular hemorrhage was recorded in 7 patients of the 10 poor-prognosis patients and 4 of those patients developed acute renal failure during BHT. Consequently, these disorders are considered to worsen the prognosis. This study supports the efficacy and safety of BHT for neonates with severe birth asphyxia. On the other hand, BHT for the above mentioned types of high-risk patients still requires further consideration for the adoption and methods of BHT.

Fosfluconazole for antifungal prophylaxis in very low birth weight infants.

We conducted a retrospective case series study to evaluate the safety of fosfluconazole prophylaxis for preventing invasive fungal infection in VLBW infants with a central vascular access. Fosfluconazole was administered intravenously at a dose of 6 mg/kg everyday during which time a central venous catheter was placed. A total of 23 infants met the criteria for enrollment in our study. No cases of fungal infection were detected during the central venous catheter placement in the group. None of the infants had an elevated beta-D-glucan, and all of them were still alive at discharge. Regarding the liver and renal function, no statistically significant differences were observed before and at the end of fosfluconazole prophylaxis. The results of this study demonstrate that fosfluconazole prophylaxis in preventing invasive fungal infection was well tolerated by VLBW infants. This is a first report to describe antifungal prophylaxis using fosfluconazole for VLBW infants.

Effect of transfusion on the venous blood lactate level in very low-birthweight infants.

BACKGROUND: In recent years the blood lactate level can be easily and quickly measured with a small amount of blood, and the availability of an arterial blood lactate level has been reported as an indicator of oxygen deficit in adults. To determine whether venous blood lactate level can serve as such a marker for determining the indications for transfusion, blood lactate and hemoglobin level were monitored before and after transfusion. METHODS: The study subjects consisted of 12 very low-birthweight infants admitted to the neonatal intensive care unit and who had transfusion between June 2005 and June 2007. The data on the blood lactate and hemoglobin were collected retrospectively by referring to the clinical records. RESULTS: A total of 18 transfusions was performed. There was no significant relationship between venous blood lactate and hemoglobin concentration before transfusion. The subjects were classified into two groups according to the lactate level before transfusion: > or =3.3 mmol/L and <3.3 mmol/L. In the high-lactate group the lactate decreased significantly after transfusion (P < 0.01) and it continued to decrease thereafter. In the low-lactate group, however, the lactate remained unchanged. CONCLUSIONS: Venous blood lactate measurements may offer some additional information regarding the optimal time for performing a transfusion. To the authors' knowledge this is the first report to study the changes in lactate levels using venous blood sampling in red blood cell transfusion in very low-birthweight infants.

Anaplastic large cell lymphoma in leukemic presentation: a case report and a review of the literature.

It is extremely rare that a patient with anaplastic large cell lymphoma (ALCL) demonstrates circulating lymphoma cells. A 10-year-old Japanese boy was presented with high-grade fever and cough. The physical examination revealed marked hepatosplenomegaly with ascites and lymphadenopathy in the cervical and periauricular areas. The white cell count was 26.2x10(9)/L with 95% of abnormal lymphoid cells, which were small to medium-sized with a high nucleus/cytoplasm ratio, basophilic cytoplasm, condensed nuclear chromatins, and 1 or 2 distinct nucleoli, hemoglobin 6.4 g/dL, and platelet 0.9x10(9)/L. A flow cytometric analysis of abnormal cells in both the peripheral blood and bone marrow samples was strongly positive for CD30 on their cell membranes. Karyogram and fluorescent in situ hybridization showed abnormal cells to have a characteristic chromosomal translocation, t(2;5)(p23;q35). Reverse transcriptase-polymerase chain reaction of peripheral blood cell-derived mRNA also indicated the fusion gene product of anaplastic lymphoma kinase and nucleophosmin. Subsequently, the patient was diagnosed to have ALCL with a rare clinical feature of a peripheral leukemic presentation, and his disease revealed to be refractory to chemotherapy. On the basis of the 11 childhood cases of ALCL with leukemic presentation so far published and reviewed herein, the prognosis is very poor.

Primary Ewing's sarcoma family tumors of the lung a case report and review of the literature.

Ewing's sarcoma family tumors (ESFT) have been reported to originate in a variety of sites, most commonly in the extremities. We herein report a case of a primary ESFT of the lung presenting in an 8-year-old boy. A histological examination of hematoxylin-eosin stained sections showed a homogeneous population of closely packed small neoplastic cells. The tumor cells were strongly positive for CD99/MIC2 and negative for the leukocyte common antigen, myoglobin, desmin, epithelial membrane antigen, AE1/AE3 and synaptophysin. The patient was treated with neoadjuvant chemotherapy and surgery. Nine months later, he is in good condition and chest CT scans have revealed no evidence of either local recurrence or distant metastasis. Cases of ESFT of the lung have been reported in recent years but there are still few reports of primary ESFT of the lung. To date, only eight cases of ESFT of the lung have been reported in the literature. This is the first report of an ESFT of the lung occurring in a patient under 10 years of age. The clinical course and therapeutic management of ESFT are also discussed.