Tumours in children with Gorlin-Golts syndrome: rare case report

Gorlin-Golts syndrome is a genetic determined disease, characterized by multisystem features and associated with different malignancies, which are more aggressive with very unfavorable prognosis. By literature data this syndrome is a rare pathology. Observation and treatment of patients with Gorlin-Golts syndrome include syndromic correction of clinical presentations and detailed observation for early malignancies detection. In the current issue a survey of modern literature about Gorlin-Golts syndrome in children and clinical case of patient sent to Federal State Budgetary Institution» N. N. Blokhin National Medical Research Center of Oncology «of the Ministry of Health for malignancy diagnosis are presented. By global and own clinical experience about Gorlin-Golts syndrome it is necessary a genetic verification and make us perform a multidisciplinary control for such patient health with obligatory examination and observation of pediatric oncologist.

Cắt gan trung tâm ở trẻ 6 tháng tuổi bị u nguyên bào đã điều trị hóa chất

TÓM TẮT Bệnh nhi 6 tháng tuổi được chẩn đoán u nguyên bào gan, ban đầu được xem là không thể cắt bỏ được vì khối u có kích thước lớn và lan tỏa. Bệnh nhân được điều trị 4 đợt hóa chất cisplatin với thời gian cách nhau 2 tuần. Kết quả chụp cắt lớp vi tính sau 4 đợt hóa trị cho thấy khối u đã thu nhỏ lại và có thể cắt bỏ. Khối u đã được cắt bỏ bằng phương pháp phẫu thuật cắt gan trung tâm. Bệnh nhân đã có thể ăn uống trở lại vào ngày thứ tư và xuất viện vào ngày thứ bảy sau mổ. Bác sĩ chuyên khoa nhi ung bướu đã theo dõi bệnh nhân bằng siêu âm gan, alpha - fetoprotein và tiếp tục điềutrị thêm 2 chu kỳ cisplatin. ABSTRACT CENTRAL HEPATECTOMY IN A 6 - MONTH - OLD CHILD WITH HEPATOBLASTOMA FOLLOWING CHEMOTHERAPY A hepatoblastoma in a 6 - month - old child was initially considered unresectable because of diffuse liver involvement. The patient received 4 courses of cisplatin with an interval time of 2 weeks. A computed tomography scan after 4 courses of chemotherapy showed shrinking of the tumor, which made it resectable, and the tumor was removed by central hepatectomy. The patient was able to eat a regular diet on the fourth day and was sent discharge on the seventh day, after the operation. The pediatric oncologist followed the patient with liver ultrasonography and alpha - fetoprotein and administered 2 more cycles of cisplatin. Keywords: Central Hepatectomy, Hepatoblastoma, children.

Problems of clinical and morphologic diagnosis of orolabial melanosis in children

Pigmented neoplasms of the skin and mucous membranes are a heterogeneous group of benign and malignant neoplasms, some of which are extremely rare in the practice of pediatric oncologist. Orolabial melanosis refers to a benign pigmented pathology of the skin and mucous membranes, requires differential diagnosis with ephelids, melanoma, and when localized on the tongue – with pigmented fungiform papillae of the tongue.This article presents a clinical case of orolabial melanosis with localization on the tongue in a 9-year-old child that was not previously described in the domestic literature. The clinical, dermatoscopic, immuno-morphological and differential diagnostic features of this pathology are described in detail.

Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.

Central Hepatectomy in a 6-Month-Old Child with Hepatoblastoma following Chemotherapy

A hepatoblastoma in a 6-month-old child was initially considered unresectable because of diffuse liver involvement. The patient received 4 courses of cisplatin with an interval time of 2 weeks. A computed tomography scan after 4 courses of chemotherapy showed shrinking of the tumor, which made it resectable, and the tumor was removed by central hepatectomy. The patient was able to eat a regular diet on the fourth day and was sent home on the seventh day, after the operation. The pediatric oncologist followed the patient with liver ultrasonography and alpha-fetoprotein and administered 2 more cycles of cisplatin.

New Procedures in Pediatric Oncology and Hematology: Too Many Questions

The article presents a legal analysis of the procedure for providing medical care in pediatric oncology and hematology which regulates the provision of medical care to children with neoplasms and diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism. The place of the pediatric oncology and hematology profile in the system of existing regulatory legal acts and the role assigned by the new procedure to the pediatric oncologist are determined. The requirements to the structure of medical organizations providing medical care in pediatric oncology and hematology profile, the procedure of oncological and hematological diseases diagnosis and management are considered. Attention is drawn to the lack of timescales of pediatric oncologist-hematologist consultation and taking biopsic material. The algorithm of medical organizations actions on entering data into information systems is analyzed.

A Case Study on Retinoblastoma Associated Orbital Cellulitis

A 20-months-old male child was brought to the Eye clinic with swelling of right upper eyelid, discharge sticking the eyelids in the right eye and redness in both eyes of three days duration. On detailed examination of anterior segment and fundus, the diagnosis of bilateral retinoblastoma with conjunctivitis was made. The conjunctivitis was cured with ciprofloxacin eye drops and eye ointment. CT scan of orbits and brain confirmed the diagnosis of retinoblastoma with calcification in both eyes. Optic nerve on both sides was normal and there was no metastasis in the brain. Since it was a bilateral case of retinoblastoma, chemoreduction followed by enucleation in the right eye, and salvaging the left eye with chemotherapy in order to save the vision was planned. Intravenous triple drug chemotherapy with carboplatin, etoposide and vincristine (six cycles) was started by pediatric oncologist in pediatric ward. After two weeks of completing the first cycle of treatment, enucleation of right eye was done. Postoperative period was uneventful. The chemotherapy was continued. The child developed marked swelling of left upper eyelid few days before the sixth cycle of chemotherapy. Examination of left eye showed signs of aseptic orbital cellulitis which was treated with oral prednisolone and topical eye drops of combination of gentamycin and dexamethasone. The inflammatory signs subsided completely in ten days time. The sixth cycle of chemotherapy was completed. On the follow up visit two weeks after discharge, the left eye ball was normal. In the first follow up, the child could pick up the toys thrown in front of him. Unfortunately the child defaulted follow up. Retinoblastoma should be excluded in all young children with orbital cellulitis because misdiagnosis is life threatening.

ALGORITHM FOR EARLY CANCER DETECTION IN CHILDREN

Background. Over the past decades, there has been a steady increase in the incidence of childhood cancer. The most childhood cancers are detected at advanced stages. The incidence of childhood cancer increased by 12.8 % (from 11.7 per 100,000 in 2007 to 13.2 per 100,000 in 2017) and the prevalence increased by 37.9 % (from 63.9 in 2007 to 88.1 in 2017 per 100,000). The purpose of the study was to develop algorithms for early cancer detection in children.Material and Methods. In the Arkhangelsk region, the algorithm for referring a patient to a consultation with a pediatric oncologist was introduced into pediatric clinical practice in 2016. To evaluate the effectiveness of the algorithm, we compared two groups of patients with histologically verified solid malignant neoplasms. Group I included 49 patients, who received treatment in 2011–15 (before performing the experiment), and group II consisted of 51 patients, who received treatment in the Department of Pediatric Oncology in 2016–18 (the control group).Results. The use of the algorithm for referring a patient to a consultation with a pediatric oncologist led to the increase in the 3-year survival time from 25.04 ± 2.05 months to 30.3 ± 1.57 months (р=0,045). The time parameters of various stages preceding the start of specialized treatment were statistically significantly reduced: the time from the visit of a pediatric oncologist to verification of the diagnosis decreased from 9.0 (7.0; 14.0) to 7.0 (5.0; 9, 0) days (p˂0.001); the time from diagnosis verification to the beginning of specialized treatment – from 12.0 (8.0; 16.0) to 8.0 (6.0; 10.0) days (p˂0.001); the time from the visit of a pediatrician to referral to a pediatric oncologist – from 11.0 (6.0; 17.0) to 2.0 (1.0; 3.0) days (p˂0.001); the time from the visit of a pediatrician until the beginning of specialized treatment – from 23.0 (17.0; 32.0) to 9.0 (8.0; 12.0) days (p˂0.001).Conclusion. The introduction of a structural and functional model for organizing medical care for children with cancer was shown to be effective.