Precursor B-cell Lymphoblastic Lymphoma in Children: Hacettepe Experience.

The purpose of the study was to review the clinical and pathologic characteristics and treatment results of children with precursor B-cell lymphoblastic lymphoma. Of 530 children diagnosed with non-Hodgkin lymphomas between 2000 and 2021, 39 (7.4%) were identified as having precursor B-cell lymphoblastic lymphoma. Clinical characteristics, pathologic, radiologic, laboratory data, treatments, responses, and overall outcomes were recorded from hospital files and analyzed. The median age of 39 patients (males/females, 23/16) was 8.3 years (range 1.3 to 16.1). The most common sites of involvement were the lymph nodes. At a median follow-up of 55.8 months, 14 patients (35%) had a recurrence of disease (11 stage IV, 3 stage III); 4 were in complete remission with salvage therapies, 9 died of progressive disease and one died due to febrile neutropenia. Five-year event-free survival and overall survival rates were 65.4% and 78.3% for all cases, respectively. Survival rates were higher in patients with a complete remission at the end of induction therapies. The survival rates were lower in our study compared with other studies, which could be explained by the high relapse rate and higher incidence of advanced-stage disease due to bone marrow involvement. We demonstrated a prognostic impact of treatment response at the end of the induction phase. Cases with a disease relapse have poor prognosis.

Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.

Relapsed or refractory central nervous system (CNS) tumors still have poor prognosis, and, therefore, new treatment options are required. We retrospectively researched treatment results of patients with CNS tumors treated with nimotuzumab from 2010 to 2015. The study included nine patients with the diffuse intrinsic pontine glioma; eight with medulloblastoma; three each with anaplastic ependymoma, glioblastoma multiforme, and central nervous system primitive neuroectodermal tumor (CNS PNET); two patients with gliomatosis cerebri; and one patient each with other tumor types, including atypical teratoid rhabdoid tumor, thalamic astrocytoma, low-grade glial tumor, high-grade glial tumor, and cribriform neuroepithelial tumor. An objective response was observed in 10 of 33 patients with four patients showing a complete response, three a partial response, and three patients had stable disease. The 2-year overall survival (OS) and progression-free survival (PFS) rates were 35 ±9% and 19 ±8%, respectively. Due to the objective response in medulloblastoma, CNS PNET, and anaplastic ependymoma (MED group), survival rates of this group were analyzed. The 2-year OS and PFS for the MED group were 71 ±12% and 30 ±13%, respectively. The treatment was well tolerated. The treatment responses for medulloblastoma, CNS PNET, and anaplastic ependymoma have been promising. Likewise, some patients with relapsed or progressive CNS tumors may benefit through nimotuzumab-containing regimen.

Revaccination in Pediatric Oncology Patients: One Center Experience.

OBJECTIVE: After chemotherapy, cancer survivors suffer from acquired immunological defects and become vulnerable to vaccine-preventable diseases. There are no universally approved revaccination guidelines for non-transplanted oncology patients. This study aimed to share our experience of revaccination in childhood cancer survivors to plan future vaccination schedules. MATERIALS AND METHODS: This retrospective study was conducted in a Pediatric Oncology Department of a university-affiliated hospital. Patients who were diagnosed with malignancy other than leukemia constituted the study population. Patients were directed for revaccination 6 months after the cessation of treatment. Revaccination was performed according to patients' vaccination status before chemotherapy and seronegativity. RESULTS: Of the 64 patients in the study, 44 (68.75%) were boys. The mean age at the time of diagnosis and at start of vaccination was 8.8±5.3 years and 10.6±5.1 years, respectively. Hodgkin's lymphoma was the most common diagnosis. The vaccination schedule of 7 patients was interrupted because of chemotherapy; after completing the missing vaccine doses, the serology of 2 patients was negative for at least 2 antigens. The vaccination schedule of 57 patients was completed before beginning chemotherapy and 52 of them were seronegative for at least 1 antigen. No adverse reactions or life-threatening infections were observed because of vaccinations. CONCLUSION: There are different approaches when vaccinating the oncology patients after chemotherapy. Watching out for the four touchstones mentioned in our study will protect the patient and do no harm. More studies are needed to constitute universal and standardized revaccination guidelines for these patients.

Blue Rubber Bleb Nevus Syndrome: Promising Response To Sirolimus.

BACKGROUND: Blue rubber bleb nevus syndrome is a rare disease involving venous malformations. CASE CHARACTERISTICS: We present a 6-year-old female with the syndrome, and consumptive coagulopathy. INTERVENTION/OUTCOME: After management with sirolimus, symptoms resolved. MESSAGE: Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.

Lipoblastoma in children: Review of 12 cases.

BACKGROUND: Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization, and signs of compression of adjacent organs may be seen. METHODS: A total of 12 children with pathologically proven lipoblastoma from 2000 to 2014 were reviewed retrospectively for their clinical features, treatment and follow up. RESULTS: There were seven boys and five girls between 7.5 months and 7.5 years of age. The most common symptom was painless rapid-growing mass. The tumors were located in the neck, the abdomen, the groin, pelvis, axilla, glutea, labium majus, thigh and trunk. The largest tumor was in a retroperitoneal location and was 13 × 10 cm in size. Complete resection was performed in 10 patients. All of these patients were followed without any evidence of recurrence. Subtotal excision was performed in two patients. At a median follow up of 38 months (range, 1.8 months-10 years), all patients were disease free. CONCLUSIONS: Lipoblastoma should be considered in the differential diagnosis of rapidly growing mass in patients <3 years of age. Total excision is the treatment of choice with preservation of vital organs. The prognosis is excellent despite large tumor size and local invasion. Recurrence rate is high in incompletely resected tumors. Regular follow up is important for early detection of recurrence.

Renal Late Effects After the Treatment of Unilateral Nonsyndromic Wilms Tumor.

Wilms tumor is the most common renal malignancy of childhood. Because of the improvement in prognosis and the increase in survival rates, long-term consequences of the treatment for Wilms tumor are of greater concern. We investigated late renal effects of the treatment on 50 survivors of nonsyndromic unilateral Wilms tumor. After the second year since the cessation of treatment, the glomerular filtration rate (GFR), urinary protein excretion, urinary ß2 microglobulin levels, and blood pressure as well as the general health status were assessed. Results were analyzed for correlation with clinical variables, chemotherapy, and radiotherapy as possible risk factors. At a median follow-up time of 8.8 years (mean=10.9; range, 2.3 to 35.4 y), none of the patients developed end-stage renal disease. Compensatory hypertrophy was observed in 68% of the cases. The median maximum bipolar length was significantly higher in patients diagnosed after the age of 36 months. Eleven (22%) and 2 (4%) of the 50 patients were hypertensive at the time of the diagnosis and the study, respectively. Similarly, median GFR values were significantly lower at the time of diagnosis, although at the time of the study, all patients had normal GFR values. With longer follow-up intervals, especially after 10 years, a significant decreasing trend in the GFR was observed (P=0.002).

Fibrous hamartoma of infancy: A case report with typical ultrasonographic findings.

Fibrous hamartoma of infancy (FHI) is a rare, benign lesion which is commonly seen under two years of age. Clinical and radiological features of FHI can mimic malignant soft tissue sarcomas, it is important to make differential diagnosis. Ultrasound (US) examination reveals heterogeneous echogenicity that can be also suggestive for other soft tissue tumors but newly defined "serpentine pattern" of intervening hypoechoic portions in the hyperechoic mass with poorly defined margins and with poor vascularity is special for FHI. Here we report a 15-month-old-boy with FHI with serpentine pattern on US. He initially presented with a painless mass in his left axilla existing for approximately seven months. The mass was successfully excised and he has been followed for three years without any evidence of recurrence. Fibrous hamartoma of infancy should always be considered in differential diagnosis in children under two years of age with a firm and solitary mass in the axilla especially when US reveals serpentine pattern with poorly defined margins and with poor vascularity. If these clinical and ultrasonographic findings are seen in a child under two years old, surgery can be performed without any additional imaging modalities. Awareness and careful assessment are important in order not to misdiagnose this benign mass for which surgical excision is curative.