A Single-center Experience of Radiotherapy in Pediatric Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Chest Wall.

AIM: To evaluate the treatment results, prognostic parameters, and treatment-related toxicity in patients with Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET) of the chest wall who underwent surgery, chemotherapy, and radiotherapy (RT) in a tertiary referral center. METHODS: The data of 24 patients under 18 years of age with a histologic diagnosis of ES/PNET in the chest wall that received RT in our department between February 2003 and July 2020 were retrospectively evaluated. RT was applied to the primary site±whole involved chest wall and to the whole lung in patients with lung metastasis. RESULTS: The median age was 8.5 years (range: 1.5 to 17 y), 15 (63%) patients were female and 9 were male (37%). The tumor localization was extrathoracic in 18 (75%) and intrathoracic in 6 (25%) patients. Mediastinal lymph node and distant metastasis (DM) was present in 5 (21%) and 4 (16%) cases at diagnosis, respectively. The median follow-up after RT was 47 months (range: 11 to 162 mo). The 2-year and 5-year overall survival, event-free survival, local recurrence-free survival, and pleural recurrence-free survival were 83% and 48%, 48% and 42%, 74% and 48%, and 61% and 52%, respectively. The overall local control rate was 83% and the pleural control rate was 67%. RT was well tolerated, with 1 case of grade 3 acute dermatitis and 1 case of grade 3 subacute radiation pneumonitis. Late toxicity was observed in 3 (13%) cases. CONCLUSION: Long-term survival can be achieved with extended-field RT even in patients with ES/PNET of the chest wall with DM. The low toxicity rates allow us to draw the conclusion that RT with modern techniques is an effective and safe treatment modality for these patients.

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. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. CONCLUSIONS: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. LEVEL OF EVIDENCE: Case series, IV.

Cardiac rhabdomyomas: clinical progression, efficacy and safety of everolimus treatment.

BACKGROUND: Primary cardiac tumors are extremely rare. Cardiac rhabdomyoma is the most common primary cardiac tumor. 50-80% of solitary rhabdomyomas and all multiple rhabdomyomas are associated with tuberous sclerosis complex. Due to spontaneous regression, surgery is necessary only in severe hemodynamic compromise and persistent arrhythmias. Everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, can be used in the treatment of rhabdomyomas seen in tuberous sclerosis complex. We aimed to evaluate the clinical progression of rhabdomyomas followed-up in our center between the years 2014-2019 and evaluate the efficacy and safety of everolimus treatment on tumor regression. METHODS: Clinical features, prenatal diagnosis, clinical findings, tuberous sclerosis complex presence, treatment and follow-up results were evaluated retrospectively. RESULTS: Among 56 children with primary cardiac tumors, 47 were diagnosed as rhabdomyomas, 28/47 patients (59.6%) had prenatal diagnosis, 85.1% were diagnosed before one year of age and 42/47 patients (89.3%) were asymptomatic. Multiple rhabdomyomas were present in 51% and median diameter of tumors was 16mm (4.5 - 52 mm). In 29/47 patients (61.7%) no medical or surgical treatment were necessary while 34% of these had spontaneous regression. Surgery was necessary in 6/47 patients (12.7%). Everolimus was used in 14/47 patients (29.8%). Indications were seizures (2 patients) and cardiac dysfunction (12 patients). Regression in size of rhabdomyomas was achieved in 10/12 patients (83%). Although, in the long-term, the amount of tumor mass shrinkage was not significantly different between patients who received everolimus and untreated patients (p=0.139), the rate of mass reduction was 12.4 times higher in patients who received everolimus. Leukopenia was not detected in any of the patients, but, hyperlipidemia was noted in 3/14 patients (21.4%). CONCLUSIONS: According to our results, everolimus accelerates tumor mass reduction, but not amount of mass regression in the long term. Everolimus may be considered for treatment of rhabdomyomas which cause hemodynamic compromise or life-threatening arrhythmias before surgical intervention.

Primary ovarian tumors in children: a single center experience of 124 patients.

BACKGROUND: Primary ovarian tumors are rare in the pediatric age group. We reviewed our 40-year experience with ovarian tumors to evalute the clinical features and treatment results in a single institution. METHODS: Between January 1975 and October 2015, 124 girls with primary ovarian tumor were diagnosed and treated in our center. Tumors were identified with biopsy or total resection and/or serum markers. Seventy four children were included in the treatment analysis. RESULTS: Median age for 124 children was 11.0 years (0.73-17.63). The main complaint was abdominal pain in 85 patients (68.5%). One hundred and five patients (84.6%) had total one-sided salpingo-oophorectomy and five patients had bilateral salpingo-oophorectomy. Amongst 124 cases, 29 patients had mature teratoma, which was the most common tumor in this study. Dysgerminoma (n=21) was the most common malignant histopathologic type. Stage I disease was diagnosed in 57.2% and stage IV in 6.6% of the patients. Five year overall survival (OS) and event-free survival (EFS) for 124 children were 82.5% and 76.3% respectively. For 74 children who received treatment, 5-year OS and EFS were 75.2% and 67.1%, respectively. Age (p < 0.017), histopathological subgroup (p < 0.001), stage (p =0.003) and chemotherapy protocols (p =0.049) were significant prognostic factors for OS. CONCLUSIONS: The survival rates in children with ovarian tumors were comparable with studies in the literature. Although patients treated with platin based regimens had better survival rates, prognosis was still poor for the patients in advanced stages. This should be the focus for further studies and improvements.

Concerns and priority outcomes for children with advanced cancer and their families in the Middle East: A cross-national qualitative study.

Introduction: Palliative care access is limited in the Middle-East, with few specialist centers and forcibly displaced migrants facing additional struggles to access care. Little is known about the specificities of providing palliative care to children and young people (CYP) with cancer. They are rarely asked directly their concerns and needs, which limits the provision of quality patient-centered care. Our study aims to identify the concerns and needs of CYP with advanced cancer and their families, in Jordan and Turkey. Method: A qualitative cross-national study in Jordan and Turkey with framework analysis was conducted two pediatric cancer centers in Jordan and Turkey. In each country, 25 CYP, 15 caregivers and 12 healthcare professionals participated (N=104). Most caregivers (70%) and healthcare professionals (75%) were women. Results: We identified five areas of concern: (1) Physical pain and other symptoms (e.g. mobility, fatigue); (2) Psychological concerns and needs (e.g. anger, psychological changes); (3) Spirituality, uncertainty over the future and use of "Tawakkul" (e.g. use of religion as a coping mechanism); (4) Negative impact on social life (e.g. social isolation, loss of support); (5) Burden on caregiver and their families (e.g. financial issues, siblings left behind). Psychological concerns were a priority for both CYPs and caregivers (particularly for refugee and displaced families) but often overlooked during routine care. CYP were able to share their own concerns and care priorities. Conclusions: Advanced cancer care must ensure assessment and management of concerns across the concerns identified. Developing child- and family-centered outcomes would ensure monitoring the quality of care. Spirituality had a more important role compared to similar investigation in other regions.

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.

Palliative Care in 2020 & Beyond Workshop: an expert symposium to develop palliative care advocacy in Turkey.

BACKGROUND: In 2014, the World Health Assembly called for improved access to palliative care (PC) as a core component of healthcare systems. Still, in 2019 the development of PC activism in Turkey was patchy in scope, care provision is isolated and services are limited in relation to population size. This workshop was aimed to increase understanding of the PC approach in cancer and to discuss holistic strategies for implementing PC for cancer in Turkey. METHODS: The workshop hosted in February 2020 at Ankara was attended by 80 healthcare professionals, bureaucrats, and international PC experts. Panel discussions were held to determine the current status, shortcomings and specify solutions for future PC in cancer in Turkey. RESULTS: Positive developments in PC after 2010 were acknowledged. Yet PC services are insufficient and mostly unavailable in the less developed regions. PC centers embedded in oncology hospitals were run by oncologists and follows classical cancer treatment protocol. It has emerged that the future need for specialized pc will be greater than anticipated. The latest regulations and the National Pallia-Turk project will provide a framework to develop nationwide PC activism. The barriers are; limited training, lack of integration into cancer care, public ignorance, and legislative issues to Do-Not-Resuscitate (DNR) orders. The contextual suggestions are; all healthcare workers must acquire a minimum knowledge and skills of PC. Home-based PC should be timely and responsive, coordination among patient's care stakeholders, Hospital-based PC for intensive symptom control or reduce caregiver burden. simultaneous cancer and PC to avoid late referral, legal arrangements for advance directives and DNR orders, and public awareness via mass media initiatives. SIGNIFICANCE OF RESULTS: The workshop recommendation substantially contributes to the existence of PC policy and guidelines; will be useful for the development of comprehensive PC activism to address the future need of PC in Turkey.

Recurrent squamous cell carcinoma and a novel mutation in a patient with xeroderma pigmentosum: a case report.

BACKGROUND: Xeroderma pigmentosum is an extremely serious genetic disorder defined by sensitivity to sunlight, resulting in sunburn and pigment changes. If patients are not completely protected from ultraviolet radiation, xeroderma pigmentosum is characterized by a greatly increased risk of sunlight-induced cutaneous neoplasms. There is no standard therapy for skin cancer of xeroderma pigmentosum. However, immune checkpoint inhibitors were reported to increase response rates and improve outcomes and life expectancy in patients with various cancers, including squamous cell carcinoma in xeroderma pigmentosum. In this paper, we report on a patient with xeroderma pigmentosum from a consanguineous family with recurrent facial chemotherapy-resistant squamous cell carcinoma lesions treated successfully with an anti-programmed cell death protein 1 monoclonal antibody in both relapses. CASE PRESENTATION: A 7-year-old Turkish male was referred to our oncology department for recurring squamous cell carcinoma after local excision of the tumor over his nose. The lesion was a rapidly growing lesion, measuring 8 × 4 cm in size. Physical examination revealed that he also had hemorrhagic crusted plaques and nodules over both eyelids and upper lip, with multiple hypo- and hyperpigmented punctate lesions all over his body. After two more cycles of chemotherapy, progressive disease was noted, and a new lesion on the right eyelid caused blurred vision. Anti-programmed cell death protein 1 antibody treatment was planned with concomitant radiotherapy. He received nivolumab every 3 weeks for 4 months, improving his vision. No new lesions or active complaints have been observed in the current situation, and complete remission has been achieved. On the last admission, the patient was clinically diagnosed with xeroderma pigmentosum. Owing to the condition's genetic heterogeneity, whole-exome sequencing was performed with Ion Proton next-generation sequencing platform, and the c.2250 + 1G>A splice site mutation of the XPC gene was detected in the homozygous state. CONCLUSIONS: The clinical report emphasizes the importance of clinical awareness and crucial early diagnosis of xeroderma pigmentosum and presents a novel causative homozygous c.2250 + 1G>A splice site mutation. Our case proves that next-generation sequencing is an effective method for the rapid diagnosis and determination of xeroderma pigmentosum genetic etiology.

Ifosfamide-induced Encephalopathy With Rapid Response to Thiamine: A Pediatric Case.

Ifosfamide is an important chemotherapeutic agent used in the therapeutic protocols of many malignant tumors. Central nervous system toxicity of ifosfamide manifests with encephalopathy in 10% to 30% of patients treated with ifosfamide. Thiamine and methylene blue have been reported beneficial in the treatment and prevention of ifosfamide-induced encephalopathy (IIE). We describe an episode of encephalopathy developed at the third cycle of ifosfamide treatment in a child with Ewing sarcoma. With the administration of thiamin, the encephalopathy resolved and no episode was noted during subsequent courses of ifosfamide. Previous use of cisplatin, concomitant use of opioids, low levels of serum albumin and hemoglobin, and elevated levels of serum creatinine are potential risk factors for IIE. The current case illustrates the possibility of IIE even in the absence of such additional risk factors, treated successfully with thiamin and draws attention to the need for close neurological monitorization of patients treated with ifosfamide.

Diffuse Large B-Cell Lymphoma, Epstein-Barr Virus -Positive Kappa Monotypic Plasma Cell Proliferation and Invasive Carcinoma, Developing in a Child With Defective Mismatch Repair.

Constitutional mismatch repair deficiency (CMMRD) syndrome is characterized by biallelic mutations in a mismatch repair gene and is associated with development of childhood cancers and symptoms resembling neurofibromatosis type 1, like café-au-lait spots. We describe the extremely rare case of a 12-year-old male presenting with several light brown macular lesions on the skin, gastrointestinal diffuse large B-cell lymphoma, adenomatous polyposis throughout the gastrointestinal tract and an intra-abdominal invasive carcinoma derived from upper gastrointestinal system. All neoplasia, as well as normal tissues, showed loss of Msh6 expression with immunohistochemistry. Molecular studies showed pathogenic homozygous p.F1088Sfs*2 mutation in MSH6. Furthermore, signs consistent with immunodeficiency, namely decreased levels of IgG and IgA in the serum, nodular lymphoid hyperplasia and EBV-associated plasma cell proliferation with monotypic kappa light chain expression in the ileum, were also noted. Our case depicts the phenotypic diversity of CMMRD syndrome and emphasizes its association with immunodeficiency, raising awareness to a feature not widely recognized.

STK4 deficiency and EBV-associated lymphoproliferative disorders, emphasis on histomorphology, and review of literature.

Aberrations of the STK4 gene in humans result in an autosomal recessively inherited primary immunodeficiency. We identified three patients with STK4 deficiency who had presented to our hospital and reviewed their biopsy samples with the goal of detailing the characteristics of STK4 deficiency from a pathology perspective. Case 1 was a 20-year-old male who presented with cervical and supraclavicular lymphadenopathy which showed plasmacytic hyperplasia and a concurrent bronchial mass, with AA amyloidosis and EBV-associated "polymorphic lymphoproliferative disorder (LPD) resembling polymorphic post-transplant LPD." The second case was an 8-year-old girl with abdominal lymphadenopathy; biopsy revealed a complex lymphoproliferation which consisted of EBV-associated "polymorphic LPD resembling polymorphic post-transplant LPD," plasmacytic hyperplasia, granulomatous reaction, and a CD4- and PD-1-positive clonal T cell proliferation. The third was a 15-year-old girl with a laryngeal mass, representing a high-grade B cell lymphoma with prominent plasmacytic differentiation. Our cases emphasize the complex and challenging histopathology of lymphoid proliferations in patients with STK4 deficiency.

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. RESULTS: Between 1985 and 2021, 33 cases with appendiceal NETs (median age 11.8 years, range 7.8-16; male/female = 10/23) were identified. All but one patients presented with abdominal pain, six had vomiting, four had fever and they underwent appendectomies with presumed diagnosis of acute appendicitis. Abscess drainage and appendectomy was performed in a 16-year-old girl for suspected right ovarian mass, and tumour was positive in the omentum. Histopathological diagnosis was classical carcinoid tumour (NET) in all cases. Median tumour size was 0.9 cm (n = 26, range, 0.1-3.5 cm); tumours were ≤1 cm in 19 cases, 2 cm in one, 3.5 cm in another case. Tumours were located in the tip (n = 11), body (n = 6) and base of appendix (n = 1) (in others data unavailable). In 28 patients with data, tumour extended to submucosa in five, to tunica muscularis in seven, to subserosa in six, to serosa in six, to mesoappendix in three, to periappendiceal fat in one. Three cases were lost to follow-up, 31 cases were alive (median follow-up 53 months). CONCLUSIONS: Paediatric appendiceal NETs do not behave aggressively and appendectomy alone is sufficient for tumours ≤2 cm regardless of local invasion. The need for further extensive surgery in tumours >2 cm also remains controversial.

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.

Progress in palliative care for cancer in Turkey: a review of the literature.

BACKGROUND: The demographic transition in Turkey is shifting the burden of diseases towards non-communicable diseases including cancer. Palliative care (PC) as a component of Universal Health Coverage assures patient and family-centred care provision throughout the spectrum of cancer. OBJECTIVES: This study aimed to make a detailed evaluation of the progress achieved since the mid-90s and the current situation of cancer PC in Turkey. METHODS: A literature review was conducted in PubMed, Scopus, Embase, ScienceDirect, Web of Science, Google Scholar, The Turkish Academic Network and Information Centre databases, Ministry of Health documents, Council of Higher Education's thesis 01/1995 to 07/2020. The information was categorised into the six domains: history of the cancer PC; law and regulations; education and research; opioid use; patient care and palliative centres; public awareness, psychosocial support and end of life ethics. RESULTS: Of 27,489 studies, 331 met the inclusion criteria. The majority were published in the Turkish language and were journal articles. The findings showed that the development of PC in Turkey can be divided into three stages: early initiatives before 2000, the dissemination stage, 2000-2010 and the advanced stage after 2010. There is evidence of progress in terms of legal regulations, opioid use and number of PC services and research output. However, there is still a need for improvement in professional education, public awareness and end of life care. CONCLUSION: There is evidence of progress, barriers and opportunities. However, bringing research into practice is needed for scale-up and integration of PC in cancer care in Turkey.

Primary Paraspinal and Spinal Epidural Non-Hodgkin Lymphoma in Childhood.

Primary paraspinal/spinal epidural lymphoma (PPSEL) is rare in childhood. Here, we retrospectively evaluated patients with PPSEL treated in our department. We also reviewed the cases reported in the literature. Fifteen of 1354 non-Hodgkin lymphoma cases diagnosed over a 38-year period were PPSEPL. There were 11 male individuals and 4 female individuals with a median age of 13 years. Most common symptoms were pain and limb weakness. Physical examination revealed spinal cord compression in 80% of patients. The most common tumor location was the lumbar region. Histopathologic subtypes were lymphoblastic lymphoma in 6 and Burkitt lymphom in 5 patients. Subtotal or near-total excision of the tumor with laminectomy was performed in 6 patients. Thirteen and 9 patients received chemotherapy and radiotherapy, respectively. Neurologic recovery was observed in 70% of patients. Seven patients were alive without disease at a median of 88 months. Overall and event-free survival rates were 61.7% and 50.1%, respectively. We reviewed clinical features, treatment, and outcome of 69 PPSEL cases reported in the literature. Neurologic recovery and long-term survival was achieved in 66.7% of them. Heterogeneity in diagnostic methods and treatment have made it difficult to establish the prognostic indicators for neurologic outcome and survival. Multicenter prospective studies with more cases are necessary to determine the prognostic factors.

Primary intracranial germ cell tumors in children 36-year experience of a single center.

PURPOSE: Intracranial germ cell tumors (ICGCTs) comprise approximately 0.4%-3% of all brain tumors. In this study, we aim to evaluate clinical characteristics, treatment and outcomes of patients with ICGCT. PATIENTS AND METHODS: All patients with ICGCT diagnosed in Hacettepe University's Pediatric Oncology Department between January 1980 and January 2016 were evaluated, retrospectively. RESULTS: We identified 52 patients (male/female: 2.46) diagnosed with ICGT. Median age was 140 months. The median duration of symptoms was 3 months. Patients with endocrine symptoms were diagnosed later than others (P = 0.028). The primary site was pineal region in 20 patients, nonpineal region in 32 which included six bifocal involvements. Pineal location was more common in boys than girls (P = 0.02). Histopathological diagnosis was germinoma in 28 patients, nongerminomatous malignant germ cell tumors in 14 and immature teratoma in 4. The mean age for germinoma was higher than that of nongerminomatous tumors (P = 0.032). Patients treated with surgery and radiotherapy and chemotherapy. Median follow-up time was 52.5 months. Thirty-six patients were alive for 12-228 months. Relapsed/progressive disease was observed in 11 patients. Nongerminomatous tumors more frequently showed relapse/progression than germinoma (P = 0.06). Five-year overall and event-free survival rates for the whole group were 72.6% and 57.2%, respectively. Overall and event-free survival rates for germinoma were better than malignant nongerminomatous tumors. CONCLUSION: Although the ratio of ICGCTs to central nervous system tumors in our series was similar to western countries, some clinical features such as tumor location were similar to cases from East Asian countries. Although similar protocols were used survival rates lower than developed western and eastern developed countries.

Primary gastric lymphoma: A report of 16 pediatric cases treated at a single institute and review of the literature.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.

Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años / Outcome of 102 patients under 5 years of age with Hodgkin lymphoma

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

Background. Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. Objectives. The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. Methods. Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. Results. There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median followup time was 13 years. Twenty-three patients had B symptoms, 15 patients had 'bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of 'bulky disease' and B symptoms were associated with poor prognosis. Conclusion. Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.