Clinical Features and Treatment Results in Children With Head and Neck Rhabdomyosarcoma.

Rhabdomyosarcoma constitutes 3% to 4% of childhood cancers, with nearly half seen in the head and neck location. We aimed to investigate the clinical features and treatment outcomes of 65 children diagnosed and treated for head and neck rhabdomyosarcoma (RMS) between 2004 and 2018. The median age was 5.

A giant ovarian mucinous cystadenoma in a postmenarchal adolescent girl.

Background: Giant mucinous cystadenomas are rare in adolescents and young adults.

Case Presentation: We report a mucinous cystadenoma in a 16-year-old postmenarchal girl presented with abdominal distention and pain, and elevated serum CA-125 levels. Radiological evaluations showed a large cystic mass originating from the right ovary.

Prospective Analysis of Serum Zinc and Selenium Levels in Children with Cancer.

Serum zinc and selenium concentrations might be altered by various disease conditions including malignancies. We aimed to prospectively investigate the serum levels of both elements in pediatric cancers. Children <18 years with newly diagnosed cancers were eligible.

Multidisciplinary Tumor Board Evaluation of Pediatric Patients with Adrenocortical Tumors Across Seven International Centers.

Background: The management of rare tumors at reference centers with specialized multidisciplinary tumor boards (MTBs) improves patient survival. No international standardized diagnostic and treatment recommendations for pediatric patients with adrenocortical carcinoma (ACC) exist so far. The aim of this case-based study is to determine how congruent treatment recommendations are in different specialized institutions in different countries.

Evaluation of prognostic factors in advanced pediatric ACC.

Therapeutic options of advanced pediatric adrenocortical carcinoma (pACC) are limited, and achieving valuable risk stratification remains challenging. We refined the value of prognostic factors with an emphasis on resection status. Retrospective international data from 106 patients with advanced pACC from various collaborating centers of the international pACC working groups ENSAT-PACT, IC-PACT and/or from individual international collaboration diagnosed were collected.

Inflammation-based score in pediatric adrenocortical carcinoma.

Inflammation-based scores have been demonstrated to be independent prognostic factors in predicting outcomes in adult adrenocortical carcinoma (ACC). We aimed to investigate the prognostic role of these scores in pediatric adrenocortical carcinoma (pACC) patients. An international multicenter analysis was conducted on a pediatric cohort from 21 ACC centers.

Non-Hodgkin lymphoma in Williams syndrome: A coincidence or an association?

To date there have been multiple clinical reports of pediatric patients with Williams Beuren syndrome developing non-Hodgkin lymphoma. However, there is no clear evidence of an association between this microdeletion syndrome and lymphoma risk and as of yet there are no recommendations for cancer surveillance in the follow-up of these patients. In this report we descibe two patients with Williams Beuren syndrome, both of whom were diagnosed with non-Hodgkin lymphoma, one at the age of 2 years and the other at the age of 6 years during clinical follow-up.

Assessment of Antibody Levels and Vaccine-induced Serologic Responses After Completion of Cancer Treatment in Pediatric Patients: A 6-Year Experience in Turkey on HAV, HBV, VZV, and MMR Vaccinations.

Objective: Childhood cancer treatment disrupts vaccination schedules and weakens or eliminates vaccine-induced immunity. In addition, post-treatment vaccine responses vary. This study aimed to assess post-treatment serum antibody levels and vaccine responses in children.

Pulmonary involvement in children with Langerhans cell histiocytosis.

Background: Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH.

Methods: We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022.

Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity.

Indolent lymphomas are rare in children and mostly consist of pediatric type follicular (PTFL) and pediatric marginal zone lymphomas (PMZL) and extranodal marginal zone lymphoma (ENMZL). Twenty children with indolent lymphoma (10 PTFL, 6 PMZL, 3 ENMZL, 1 mixed type) among 307 Non-Hodgkin Lymphoma (NHL) were retrospectively evaluated. The mean age of the entire group was 10.

Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort-a work from the ENSAT-PACT working group.

Objective: Pediatric adrenocortical carcinoma (pACC) is rare, and prognostic stratification remains challenging. We aimed to confirm the prognostic value of the previously published pediatric scoring system (pS-GRAS) in an international multicenter cohort.

Design: Analysis of pS-GRAS items of pACC from 6 countries in collaboration of ENSAT-PACT, GPOH-MET, and IC-PACT.

International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects.

Objective: Mitotane is an important cornerstone in the treatment of pediatric adrenal cortical tumors (pACC), but experience with the drug in the pediatric age group is still limited and current practice is not guided by robust evidence. Therefore, we have compiled international consensus statements from pACC experts on mitotane indications, therapy, and management of adverse effects.

Methods: A Delphi method with 3 rounds of questionnaires within the pACC expert consortium of the international network groups European Network for the Study of Adrenal Tumors pediatric working group (ENSAT-PACT) and International Consortium of pediatric adrenocortical tumors (ICPACT) was used to create 21 final consensus statements.

Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases.

Background: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs.

Methods: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified.

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.

An unexpected intracardiac location of yolk sac tumor.

Purpose: In the pediatric population, intracardiac tumors are rare, usually benign, and mostly diagnosed as rhabdomyoma. Yolk sac tumors (YSTs) are a rare malignant type of germ celltumor that typically occurs in gonads. It can also be seen in midline locations but the intracardiac location is extremely rare.

Radiotherapy for pediatric adrenocortical carcinoma - Review of the literature.

Background And Purpose: Pediatric adrenocortical carcinoma (pACC) is a rare disease with poor prognosis. Publications on radiotherapy (RT) are scarce. This review summarizes the current data on RT for pACC and possibly provides first evidence to justify its use in this setting.

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.

Background: Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon. Here, we present an interesting pediatric case with a PSIEES diagnosis confirmed by the presence of a specific fusion protein in the tumor tissue and who then developed a cerebellar recurrence. We also reviewed the PSIEES cases in childhood reported in the literature.

Paediatric appendiceal neuroendocrine tumours: a review of 33 cases from a single center.

Background: Appendiceal neurendocrine tumours (NETs) are rare neoplasms and diagnosis is commonly incidental following appendectomy. We aimed to review our experience with appendiceal NETs.

Methods: Records of children with appendiceal NETs were reviewed and data concerning demographic characteristics, clinical findings, surgical procedures, histopathological findings, management and outcomes were recorded.

Spindle cell hemangioma of nasal passage and ethmoidal sinus in a 4-month old infant.

Spindle cell hemangioma (SCH) is a benign unusual vascular neoplasm. It does not have gender predilection and can occur at all ages. The disease affects dermis and subcutis of distal extremities predominantly; head and neck involvement is very rare, paranasal sinus involvement has not been reported before.

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

Background: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management.

Methods: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively.