Infantile myofibromatosis is a proliferative disorder occurring during infancy and early childhood, marked by the development of nodular or diffuse lesions consisting of various mesenchymal elements. Intracranial involvement is infrequently reported. Here, we present the case of a 3-year-old girl exhibiting a rare manifestation of IM with intracranial parenchymal involvement, displaying a histological pattern documented in existing literature on patients with infantile myofibromatosis. Subsequent MRI follow-up revealed no signs of recurrence.

BACKGROUND: Neurofibromatosis type I, also known as Von Recklinghausen disease, is a common phakomatosis affecting 1 in 2500-3000 live births; it may be associated with several common ocular findings, including Lisch nodules, plexiform neurofibromas, optic pathway gliomas, retinal astrocytic hamartomas and choroidal nodules.
METHODS: This report illustrates clinical evidence of simultaneous presence of retinal reactive astrocytic tumor (RRAT) and two retinal astrocytic hamartomas (RAH) in a 15 y/o patient with NF1, referred to our attention because of an asymptomatic fundus mass in his right eye of recent onset.
CONCLUSIONS: This case, in addition to considering NF1 as one of the ocular conditions associated with secondary RRAT, underlines the importance of early referral and continuous ophthalmological follow-up in preventing possible complications that could cause significant visual impairment in patients with NF1.

UNASSIGNED: Nursing management considers nursing quality management at its core. Evaluation indexes of nursing quality not only control nursing quality, measure work enthusiasm, and judge the difference in nursing levels but also help nurses accurately understand the satisfaction degree of patients.
UNASSIGNED: To analyze pediatric tumor nursing quality evaluation indexes and establish a scientific and rigorous index system of children\'s nursing quality.
UNASSIGNED:   Based on the \"structure-process-result\" in this study, the Delphi method was used to carry out questionnaire investigations and opinion collection for domestic experts in pediatrics, pediatric oncology internal medicine, and pediatric oncology surgical care and clinical aspects two times. In addition, the Kendall coordination coefficient and analytic hierarchy process were used to determine the content of indicators at all levels, set the weight, and finally establish the quality index system of pediatric tumor nursing.
UNASSIGNED: The response rate of 27 experts in the first round of the questionnaire was 100%, Ca0.77, Cs,0.73, Cr0.75, and the W value of the expert coordination coefficient was 0.315. In the second round of 27 expert consultations, the reply rate was 96%, Ca0.81, Cs0.80, Cr0.805, and the specialist coordination coefficient W value was 0.369. After two rounds of consultation, the importance evaluation scores of each indicator ranged from 3.31 to 4.88 and 3.29 to 5.00, respectively, and more than 94.7% of the indicators\' importance evaluation scores were higher than 3.50. Finally, 3 Level-1 indicators, 12 Level-2 indicators, and 54 Level-3 indicators are established.
UNASSIGNED: This study has established a simple, comprehensive, and scientific quality index system for pediatric tumor nursing, highlighted the characteristics of pediatric tumor nursing, and provided an evaluation system for specialized nursing of pediatric tumors to more standardized pediatric tumor nursing and contribute to the sustainable and scientific development of pediatric tumor nursing.

UNASSIGNED: Sacrococcygeal teratoma is a rare extragonadal germ cell tumor occurring at the lower end of the back. It is congenital, and can be diagnosed in utero or just after birth. These tumors are sporadic in nature, more common in females than in males. They can be solid, cystic or a mixture of the two in consistency. Authors present this case to share their experience because these congenital tumors are rare and a lapse in appropriate management can be devastating because of chances of recurrence and the possibility of a malignant nature of the disease.
METHODS: Authors in this case present a baby that was referred to their center 5 days post vaginal delivery with a huge mass on the sacrococcygeal region. MRI revealed sacrococcygeal teratoma type II. Wide local excision was done. Tumor margins were microscopically negative. The patient recovered well and was discharged. The patient did not require adjuvant treatment.
UNASSIGNED: Routine obstetric ultrasound can diagnose sacrococcygeal teratoma with 100 % sensitivity. Fetuses with tumors <5 cm in size can be delivered vaginally. Tumors larger than that should be delivered at term through cesarean section. High risk sacrococcygeal teratomas can be delivered at 28 weeks of gestation by cesarean section followed by ex-utero intrapartum therapy. MRI is useful to assess the intrapelvic extent of the tumor and to plan management. Surgical intervention should be done after delivery.
CONCLUSIONS: Intrauterine diagnosis is not an indication for termination of pregnancy. Surgical resection with negative margins provides a cure. Histological examination of the tumor is mandatory. For malignant sacrococcygeal teratomas, adjuvant chemotherapy is necessary for positive tumor margin after surgical resection. Positive margins may require chemotherapy. Neoadjuvant chemotherapy may be used for tumor debulking prior to surgery if the tumor infiltrates neighboring structures or is metastatic.

OBJECTIVE: In recent years, the use of robotic-assisted minimally invasive surgery in pediatric oncology has increased. Despite its benefits, its adoption remains limited. This single-center retrospective analysis examines technical nuances, indications, and surgical limitations to prevent complications.
METHODS: Data from cancer patients treated robotically in 2015-2016 (Group A) and 2020-2022 (Group B) were compared. Decision-making considered tumor characteristics and risks, guided by multidisciplinary tumor board discussions. Data collected included demographics, intra/post-operative details, and tumor classifications. Statistical analysis evaluated influencing factors.
RESULTS: Thirty-eight pediatric patients underwent robotic-assisted tumor resection, the median age was 5 years and weight 21.5 kg. Group A had higher median age and weight. Lesions included 23 malignant, 9 borderline, 5 benign cases; neuroblastoma (n = 19) was prevalent procedure and adrenalectomy was the predominant (28.94%). Open conversion occurred in 12 patients (31.58%), mainly due to vascular challenges (23.68%). Intraoperative complications were 10.53%, postoperative 7.9%. About 27% discharged by the third postoperative day; longer stays were needed for complex cases. All resumed post-op chemotherapy as scheduled, and all alive during follow-up.
CONCLUSIONS: Our study confirms the safety and efficacy of robotic-assisted tumor resections in pediatric oncology, even during the learning phase, emphasizing the importance of learning curve, patient selection, and trocar positioning.

OBJECTIVE: To report the surgical outcome of synovial osteochondromatosis, a rare tumor of the cervical spine, in a 6-year-old boy.
METHODS: A 6-year-old boy presented with muscle weakness in the right deltoid (2) and biceps (4) during a manual muscle test. Magnetic resonance imaging showed a 3 × 2 × 1.5 cm mass within the spinal canal at the C4-6 level, compressing the cervical spinal cord from the right side. Computed tomography revealed hyperintense areas within the tumor and ballooning of the right C4-5 and C5-6 facet joints.
RESULTS: After a biopsy confirmed the absence of malignancy, a gross total resection was performed. The pathological diagnosis of synovial osteochondromatosis was established. Postoperatively, muscle weakness improved fully in the manual muscle test, and there were no neurological findings after 3 months. However, the patient is under careful follow-up owing to the detection of a regrowth site within the right C4-5 and C5-6 intervertebral foramen 2 years postoperatively.
CONCLUSIONS: Synovial osteochondromatosis of the cervical spine in children is rare, and this is the first report of its regrowth after surgery. Synovial osteochondromatosis should be included in the differential diagnosis of pediatric cervical spine tumors.

Ependymomas are rare brain tumors that can occur in both children and adults. Subdivided by the tumors\' initial location, ependymomas develop in the central nervous system in the supratentorial or infratentorial/posterior fossa region, or the spinal cord. Supratentorial ependymomas (ST-EPNs) are predominantly characterized by common driver gene fusions such as ZFTA and YAP1 fusions. Some variants of ST-EPNs carry a high overall survival rate. In poorly responding ST-EPN variants, high levels of inter- and intratumoral heterogeneity, limited therapeutic strategies, and tumor recurrence are among the reasons for poor patient outcomes with other ST-EPN subtypes. Thus, modeling these molecular profiles is key in further studying tumorigenesis. Due to the scarcity of patient samples, the development of preclinical in vitro and in vivo models that recapitulate patient tumors is imperative when testing therapeutic approaches for this rare cancer. In this review, we will survey ST-EPN modeling systems, addressing the strengths and limitations, application for therapeutic targeting, and current literature findings.

UNASSIGNED: Medulloblastoma, a highly malignant embryonal tumor predominantly found in the pediatric population, typically arises within the cerebellum. This case report holds particular importance due to the rarity of medulloblastoma within the cerebellopontine angle (CPA). The distinct anatomical challenge posed by the CPA complex neurovascular structures, along with the absence of pathognomonic clinical or radiographic features, highlights the unique diagnostic and management challenge of this case.
UNASSIGNED: A 5-year-old boy presented with mild, progressively worsening headaches on CT/MRI imaging, which revealed a solid mass in the left CPA. Radiologically, the lesion closely resembled a CPA meningioma. The patient underwent a left retrosigmoid suboccipital craniectomy, utilizing a modified park bench position and careful burrhole creation. Intraoperatively, the tumor exhibited well-defined margins, firm adherence to cranial nerves, and complex tissue characteristics. Postoperatively, histopathological analysis identified nodular medulloblastoma, WHO grade IV, with immunohistochemical markers confirming its subtype.
UNASSIGNED: This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome. Uncommon tumor locations, such as the CPA, require tailored approaches, and the utilization of advanced diagnostic techniques, including immunohistochemistry, aids in accurate subtype classification.
UNASSIGNED: This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome.

BACKGROUND: Neuroblastoma (NB), a prevalent pediatric solid tumor, presents formidable challenges due to its high malignancy and intricate pathogenesis. The role of disulfidptosis, a novel form of programmed cell death, remains poorly understood in the context of NB.
METHODS: Gaussian mixture model (GMM)-identified disulfidptosis-related molecular subtypes in NB, differential gene analysis, survival analysis, and gene set variation analysis were conducted subsequently. Weighted gene co-expression network analysis (WGCNA) selected modular genes most relevant to the disulfidptosis core pathways. Integration of machine learning approaches revealed the combination of the Least absolute shrinkage and selection operator (LASSO) and Random Survival Forest (RSF) provided optimal dimensionality reduction of the modular genes. The resulting model was validated, and a nomogram assessed disulfidptosis characteristics in NB. Core genes were filtered and subjected to tumor phenotype and disulfidptosis-related experiments.
RESULTS: GMM clustering revealed three distinct subtypes with diverse prognoses, showing significant variations in glucose metabolism, cytoskeletal structure, and tumor-related pathways. WGCNA highlighted the red module of genes highly correlated with disulfide isomerase activity, cytoskeleton formation, and glucose metabolism. The LASSO and RSF combination yielded the most accurate and stable prognostic model, with a significantly worse prognosis for high-scoring patients. Cytological experiments targeting core genes (CYFIP1, EMILIN1) revealed decreased cell proliferation, migration, invasion abilities, and evident cytoskeletal deformation upon core gene knockdown.
CONCLUSIONS: This study showcases the utility of disulfidptosis-related gene scores for predicting prognosis and molecular subtypes of NB. The identified core genes, CYFIP1 and EMILIN1, hold promise as potential therapeutic targets and diagnostic markers for NB.

UNASSIGNED: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.
UNASSIGNED: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up.
UNASSIGNED: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.