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Objectives: This was a retrospective analysis of liver transplant for pediatric patients with liver cirrhosis and hepatocellular carcinoma., Materials and Methods: Fourteen pediatric patients with chronic liver disease and hepatocellular carcinoma underwent liver transplant from 2004 to 2021. Preexisting diseases were tyrosinemia (n = 6), progressive familial intrahepatic cholestasis type 2 (n = 2) and type 3 (n = 3), cryptogenic cirrhosis (n = 2), hepatitis B and D (n = 1), and biliary atresia (n = 1)., Results: Mean age was 9.43 ± 4.9 years (range, 13 months to 16 years). Three patients had 1 tumor, 4 had 2 tumors, and 7 had multiple (≥3) lesions. Six patients were classified as Pretreatment Extent of Disease Staging System for Hepatoblastoma (PRETEXT) stage IV, 3 as stage II, and 5 as stage I. Some patients received systemic chemotherapy before (n = 4) or after transplant (n = 3) or transarterial chemoembolization and microwave ablation pretransplant (n = 1). Hepatocellular carcinoma posttransplant recurrence was observed at 23, 47, and 108 months in 3 patients (21%). Recurrence sites were omentum (n = 1) and liver graft (n = 2). One patient was treated with hepatic resection, radiofrequency ablation, and radiotherapy, while the other received radiofrequency ablation and chemotherapy for graft tumor recurrence. Relapse-free patient survival rates were 92%, 82.5%, and 72.2% at 2, 4, and 10 years, respectively. Four recipients (28.5%) died; posttransplant cause of death was infection at 19 (n = 1) and 188 months (n = 1) or hepatocellular carcinoma recurrence at 79 (n = 1) and 165 months (n = 1). Median follow-up was 178 months (range, 13-204 months). Mean estimated survival was 171.25 ± 16.6 months. Overall patient posttransplant survival was 100%, 92.3%, 92.3%, 83%, and 72% at 1, 2, 5, 10, and 15 years, respectively., Conclusions: Hepatocellular carcinoma was mainly associated with inherited liver diseases in our pediatric series. Liver transplant provided a long-term survival advantage to pediatric patients with preexisting cirrhosis and hepatocellular carcinoma.

Heritable diseases associated with childhood tumors are sometimes defined as a probable etiologic factor or a coincidence. First of all, we must know the actual number of patients. Herein a case with medulloblastoma associated with glutaric aciduria type II [corrected] is reported for this purpose. A 5-year-old boy was admitted with nausea, vomiting, and lethargy. In medical history, consanguinity and siblings with mental-motor retardation and epilepsy are remarkable. Growth retardation, macrocephaly, lethargy, tremor, bilateral nistagmus, and papilledema were prominent features in physical examination. Noncontrast computed tomography of the brain showed a hyper dense mass in the cerebellar vermis. Gross total resection was made and the histopathology of the tumor was medulloblastoma. Besides medical history and physical findings, radiologic white matter changes in the subcortical, periventricular regions, bilateral basal ganglia, and caudate nuclei in magnetic resonance images other than tumor led us to investigate the child for glutaric aciduria type II [corrected]. The level of the 2-OH glutaric acid was determined as being 12-fold high in the urine. Chemo-radiotherapy was performed after surgery. Our case was the third patient with medulloblastoma in the literature and is still alive with no evidence of the disease 19 months after the initial diagnosis.

We evaluated the incidence of congenital malformations in 566 children (median age: 8, M:F 1.3) with lymphomas and solid tumors using patient records. In this study, 12.7% of children either had a congenital malformation (7.8%) or a birthmark (4.9%). The incidence of patients with a childhood cancer syndrome was 3% and these cases developed typical tumors. The rate of consanguineous marriages was 12.6%, and family history of cancer was positive in 31.2%. Median age at cancer diagnosis, gender, maternal age, history of stillbirth and missed abortion, consanguinity of parents, and family history of cancer were not significantly different in cases with and without a congenital malformation. The most frequent cancers were central nervous system tumors and lymphomas. No remarkable association between a particular anomaly and a specific cancer type could be shown. The high incidence of congenital anomalies in this study may stimulate future large cohort studies in our country.

Aneurysmal bone cyst is a rare benign tumor of the bone that can be difficult to distinguish from malignant tumors, especially when it presents in an unusual location. Herein, we report a six-year-old girl with a primary aneurysmal bone cyst in an uncommon location. It originated from the 4th rib and she presented with a huge chest wall mass. Despite the large size of the cyst, the only symptom was persistent cough. She was successfully treated with total excision of tumor without any complication. The patient has been followed up for 56 months without any recurrence.

An inflammatory myofibroblastic tumor is an uncommon benign tumor located in various organs that can be misdiagnosed as a malignant neoplasm. We herein present two patients with ileocecal inflammatory myofibroblastic tumors. An abdominal mass was detected in a 13-year-old girl and a 15-year-old boy who presented with paleness, fatigue, intermittent fever, and night sweating. The radiological findings confirmed a mass originating from the ileocecal region. The presumptive diagnosis was Burkitt's lymphoma. The histopathological diagnosis was inflammatory myofibroblastic tumor. After a surgical resection, all systemic symptoms rapidly resolved. Inflammatory myofibroblastic tumor is a rare pseudosarcomatous clinical and pathological entity. Although this tumor is more commonly reported in the lung, it can be detected in extrapulmonary sites, including the mesentery. Because the choice of treatment for this tumor is conservative surgery, an accurate preoperative analysis is important to avoid any unnecessary aggressive surgical intervention or other therapeutic approaches.

In this study, 136 febrile neutropenic episodes were overviewed retrospectively. Factors affecting treatment success and cost were analyzed. Twenty percent of the episodes were microbiologically documented and 51 % of the bacterial isolates were gram negatives. The most commonly used empirical therapies in febrile episodes were the combination of two drugs (58.0%), monotherapy (14.8%), and antibiotics plus fluconazole (20.6 %). In lymphoproliferative tumors duration of fever and discharge from the hospital were longer Administration of the hematopoietic growth factors shortened neither the duration of neutropenia nor fever and hospitalization. Treatment costs were higher in lymphoproliferative tumors, in bacteremia, and in episodes where glycopeptides, antifungal drugs, and hematopoietic growth factors were used. In conclusion, duration of neutropenia was a significant independent predictive factor for duration of fever In the lymphoproliferative tumors, duration of fever was longer and cost of treatment was more than in the solid tumors.

Malignant mesothelioma is a very rare tumor in childhood. Presently, treatment of this disease continues to be frustrating and prognosis remains poor. We here report a pediatric case of malignant pleural mesothelioma who gave a complete response to ICE-VAC chemotherapy regimen and achieved a long-term survival. An eight-year-old girl underwent exploratory thoracotomy and decortication because of a unilateral loculated and multicystic pleural effusion. Histopathological diagnosis was sarcomatoid pleural malignant mesothelioma. After decortication, chemotherapy with ICE (ifosfamide, carboplatin, etoposide) - VAC (vincristine, adriamycin, cyclophosphamide) combination was started. Six courses of chemotherapy resulted in complete clinical and radiological tumor response. She did not receive any further therapy and remains disease-free three years after the first remission. ICE-VAC chemotherapy combination resulted in a complete tumor response and a long-term disease-free survival for the presented case. The efficacy of this chemotherapy regimen in malignant mesothelioma needs to be documented in future trials.

Since a large variety of disorders may lead to lymph node enlargement determining the cause of peripheral lymphadenopathy (LAP) in children can be difficult. This retrospective study evaluated 200 children who were admitted to an Oncology-Hematology department because of lymphadenopathy and aimed to determine the clinical and laboratory findings that were valuable for differential diagnosis. A specific cause for lymphadenopathy was documented in 93 (46.5%) cases. One hundred forty (70%) children were classified as having a benign cause for lymph node enlargements. Fourteen (10%) of these cases underwent an excisional lymph node biopsy, and histopathological examination showed a reactive hyperplasia. Sixty (30%) cases were classified as having a malignant disease-causing lymphadenopathy. In terms of differential diagnosis, some associated systemic symptoms, physical findings, and laboratory investigations showed significant difference between benign and malignant lymphadenopathy groups. The following findings were determined as being important to alert the physician about the probability of a malignant disorder: location of the lymphadenapathy (supraclavicular and posterior auricular), duration of the lymph node enlargement (>4 weeks), size of the lymph node (>3 cm), abnormal complete blood cell findings, abnormalities in chest X-ray, and abdominal ultrasonography.

The authors report their experience with high-dose oral methylprednisolone therapy (HDMP) in 15 infants with complicated hemangiomas. The starting dose for methylprednisolone was 30 mg/kg/day for 5 days, then the dose was tapered gradually every 5 days to 20, 10, 5, 2.5, and finally to 1 mg/kg/day. Therapy was then stopped and the patients were followed. An initial response was evident in 12 patients. Nine out of 12 responders showed regrowth signs. After regrowth, 4 cases received prednisolone at doses between 1 to 5 mg/kg/day and 3 patients received a second course with HDMP as additional corticosteroid therapy. Overall, 9 out of 15 cases were responders; very good and good responses were obtained in 5, partial response in 4, and therapy failure in 5 cases. One child was not available for evaluation of response. A very rapid initial response was observed in subglottic and periocular hemangiomas. Side effects were not serious and resolved after discontinuation of treatment. Although the number of patients is small in this study, overall response rate with HDMP regimen seems not to be superior to the regimens that use lower doses (5 mg/kg/day), but it provides a high initial response rate and the duration of therapy is short. Therefore, it may be useful for treating hemangiomas that fail to respond with low doses, especially in centers with limited resources where other treatment modalities cannot be used at the moment.

We report a pediatric case who developed bleomycin-induced hyperpigmentation and hypersensitivity reactions to both etoposide and vinblastine while receiving chemotherapy for germ cell tumor. Skin hyperpigmentation related to chemotherapeutic agents has been reported only rarely in pediatric patients. This patient developed a characteristic skin hyperpigmentation which was "flagellate" in appearance. Two features of the hyperpigmentation were noteworthy: development at a low cumulative dose of bleomycin and persistence after cessation of chemotherapy. Additive effect of cisplatinum-induced hyperpigmentation was suggested. Although hypersensitivity reactions to etoposide have been previously reported, hypersensitivity reactions to vinblastine are almost unknown. To our knowledge, this is the first report of hypersensitivity reaction to vinblastine in a child in English literature.

Osteosarcoma of the cranial facial region is uncommon and only rarely involves the ethmoid or sphenoid bones. The authors report on an unusual case of a 17-year-old male presenting with chondroblastic osteosarcoma of the maxillary, ethmoid, and sphenoid sinuses who remains well and disease-free at 46 months. He was treated with anterior craniofacial resection followed by postoperative radiotherapy to the sight of the primary tumor. He did not receive chemotherapy because of emerging hepatitis-B infection and vasculitis. The literature on extragnathic craniofacial osteosarcomas is reviewed with particular emphasis on treatment options of this rare tumor.

Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor. Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major. The phenotypic features of the patient's family fulfill the criteria for Li-Fraumeni syndrome. The patient was treated with chemotherapy and radiotherapy and died with recurrent disease. To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia. The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.

We evaluated the antiemetic efficacy of tropisetron for control of acute emesis during grade 3 or 4 emetogenic chemotherapy in children. Tropisetron was administered as a single intravenous dose of 0.2 mg/kg on the first day and intravenously or orally with the same dose on subsequent days. A total of 125 courses of highly emetogenic chemotherapy was administered to 22 children with a median age of 14 years (range: 3-18 years). All 22 patients received tropisetron for at least two courses. Overall complete response on day 1 was observed in 80 out of 125 courses (64%). The response rates were consistent over multiple courses; a complete and major response rate on the first day of Course I (n: 22 courses) and Course II (n: 22 courses) was observed in 73 and 77 percent of cases, respectively. When the results were analyzed according to the daily schedules, overall complete response for grade 4, grade 3 and grade 1-2 emetogenic treatment days was 59, 85 and 75 percent, respectively. In this study, cost effectiveness for tropisetron was also determined; the cost per successfully controlled course was 162 USD. No side effects of tropisetron other than mild diarrhea and dry-mouth were documented in this study. In conclusion, the results of this study confirmed that tropisetron is a safe, well tolerated and effective antiemetic drug for the prevention of acute emesis in children and adolescents during highly emetogenic chemotherapy.

The research presented here investigated platelet activation in cyanotic and acyanotic congenital heart diseases (CHD). Children with cyanotic CHD are prone to both thrombosis and hemorrhage. However, patients with acyanotic CHD may also have a mild bleeding disorder. The platelet activation in CHD was investigated in support of a hypothesis that platelet activation may play a role in the hemostatic abnormalities reported in these patients. Platelet activation was determined by using flow cytometry with anti-CD62 monoclonal antibody (mAb), which has been shown to be a specific marker of platelet activation. Thirteen children with cyanotic CHD, 33 children with acyanotic CHD and 17 healthy children serving as controls were studied. Platelet activation was significantly higher in the cyanotic group and also in the acyanotic group compared with the healthy children (P = 0.0000 and P = 0.019, respectively). In the cyanotic group, platelet activation showed a direct correlation with arterial O2 saturation (SaO2) (P = 0.014). There was no correlation between platelet activation and erythrocyte related parameters in either group. Platelet activation occurs in CHD, particularly in patients with cyanotic CHD (even in patients with no evidence of clinical thrombosis) and it may play a role in the pathogenesis of thrombotic disorders seen in these patients.

A 14-year-old boy had a 1-month history of diplopia (due to a VI nerve palsy), motor ataxia and dizziness. Brain MRI showed a 1.5-cm mass posterior to the pons. Histopathological examination of a biopsy specimen showed the lesion to be of viral origin. After 3 months, the ataxia and dizziness had resolved and the MRI findings returned to normal. By 5 months the abducens paralysis had also resolved. Viral encephalitis should be considered in the differential diagnosis of posterior fossa tumours.

Nonlymphoid gastrointestinal malignancies are not frequently encountered malignant tumors of childhood. From 1972 to 1994, at Hacettepe University Children's Hospital, there were 6,774 malignant tumors encountered in patients from birth to age 17. Thirty-five of them (0.5%) originated from the gastrointestinal tract. There were five carcinoid tumors, four pancreas tumors (two were solid-cystic tumors), four APUD-omas, three leiomyosarcomas, one mesothelioma, two carcinomas of the stomach and duodenum, and 16 colon and rectum carcinomas (12 were mucin-producing). These rare tumors are discussed according to the characteristics of the patients, therapy modalities applied, and outcome of the patients.

The occurrence of immune thrombocytopenic purpura (ITP) in Hodgkin's disease is uncommon. This report describes a patient who developed ITP twice before splenectomy, and for the third time several years later, preceding an abdominal relapse of the disease. We suggest that patients with a history of Hodgkin's disease undergo diligent searches for active disease when ITP is diagnosed. ITP may be the only manifestation of active disease and may precede histologic documentation of Hodgkin's disease by months or years.

Ileo-colic intussusception was diagnosed in a nine-month-old male infant who presented with abdominal distention, irritability, and bilious vomiting. After reduction of the invaginated segment, a mass measuring one cm was palpated at the antimesenteric border of the terminal ileum. Pathological examination of the mass revealed ectopic pancreatic tissue, which most likely caused the intussusception.

Two unrelated infants, 1 female and 1 male, with Pearson's syndrome are presented. Both patients presented with severe macrocytic refractory anemia starting early in infancy. Investigation of the mitochondrial (mt), DNA showed that one of the patients had a 4,977 bp deletion, and the other had a 4.5 kb mtDNA deletion. It is concluded that Pearson's syndrome should be borne in mind in the differential diagnosis of refractory anemia especially when there are accompanying gastrointestinal disturbances and metabolic acidosis.

From 1972 through 1991, 404 patients with renal tumor (Wilms' tumor and clear cell sarcoma) were seen at the Pediatric Oncology Unit of Hacettepe Children's Hospital. The genital abnormalities and renal diseases in these patients were evaluated retrospectively. Eight patients with renal tumor had genital abnormality and/or renal disease of various types and degrees. One of these patients had a clear cell sarcoma while all the others had Wilms' tumor. Two patients had all components of the Drash syndrome. Two patients with Wilms' tumor had genital abnormality and mild proteinuria. Another two patients had only Wilms' tumor and renal disease. Two patients had only renal tumor and genital abnormality of whom one was the patient with clear cell sarcoma.

Since multiple primary malignant tumors are rare in children, their presence can be a diagnostic and therapeutic problem. In this report, we present a six-year-old boy with Langerhans cell histiocytosis and Hodgkin's disease. On admission, the patient had lytic lesions and a periosteal reaction on the left trochanter major from which an open bone marrow biopsy was performed. The biopsy revealed Langerhans cell histiocytosis. Eight months later, the child presented with enlarged left cervical lymph nodes and the biopsy demonstrated Hodgkin's disease. Although there was an eight-month interval between the two histopathological diagnoses, the diffuse pulmonary parenchymal infiltration observed on the first admission, was later confirmed by an open-lung biopsy as Hodgkin's disease. The patient was said to have two concurrent lymphoreticular malignancies. To our knowledge, this is the youngest case reported with this association in the English language literature.

Of 806 non-Hodgkin's lymphoma cases, only one primary subconjunctival lymphoma case was observed in Hacettepe Children's Hospital over a period of 16 years. The patient was a 12 year-old-boy with a subconjunctival mass on the upper part of his right eye. Biopsy revealed malignant lymphoma. Extensive investigations disclosed no other site of tumor. He was given the modified LSA2-L2 protocol. He has been in remission for 22 months. This may be the first report of a primary subconjunctival lymphoma in the childhood period.

A case of hyperpyrexia induced by procarbazine in a child with Hodgkin's disease, neurofibromatosis, and pectus excavatum deformity is presented. After the diagnosis of stage IIIS Hodgkin's disease, combined COPP chemotherapy was initiated. One week later she presented with high fever. After a diagnosis of infection was made, chemotherapy was stopped and antibiotics were given. Nearly the same picture recurred three times after reinstituting chemotherapy. On the fourth occasion, the patient had to be hospitalized because of hyperpyrexia and arrhythmia. There was no obvious reason for fever, and cytotoxic-induced fever was considered. The drugs were given one at a time. When a test dose (10 mg) of procarbazine was given, she developed a high fever with severe nausea and vomiting. The reaction was controlled by antihistaminics and steroids. To our knowledge this is the second report on hyperpyrexia due to procarbazine administration.

This study was aimed at detecting the early appearance of myelofibrosis by bone marrow biopsy examination in children with vitamin D-deficiency rickets. Twelve children, aged 4 to 18 months, were evaluated. Only a minimal increase of the reticulin was shown in rachitic children without anemia in whom no other laboratory evidence of myelofibrosis was present. Early signs of myelofibrosis with increase of reticulin were present in rachitic infants with anemia. In patients of the same age with iron deficiency anemia, the bone marrow reticulin findings were normal. Bone marrow biopsy after successful treatment of rickets could be repeated in one patient with myelofibrosis; results indicated that the myelofibrosis with anemia associated with vitamin D-deficiency rickets is reversible by vitamin D treatment.

The effects of low-dose and short-time acyclovir therapy in 14 children with malignant disease of ages 4-18 years who had developed varicella zoster virus infections while receiving aggressive chemo-/+radiotherapy are reported. Ten of them had chickenpox and 4 herpes zoster. Acyclovir 5 mg/kg was infused IV every 12 h in 9 patients and every 8 h in 5 patients for a median of 4 days' duration. We resumed the primary therapy when the patients' lesions had dried out and became crusted and new lesions had not reappeared. The period of initiation of the acyclovir therapy to the resumption of oncological treatment was 8.4 +/- 2.7 days for chickenpox and 12.0 +/- 3.4 days for herpes zoster patients. After restarting the oncological therapy, no adverse effects of acyclovir or complication of infection were observed. The efficiency of early, short-term, and relatively low dose acyclovir therapy is discussed and compared to the results in the relevant literature.

An unusual case of non-Hodgkin's lymphoma with primary renal involvement in a four-year-old boy is presented. The diagnosis was established by renal biopsy. The findings of an exploratory laparotomy and an extensive staging procedure showed no other primary site. The patient has been followed up for 14 months and no evidence of recurrence has been observed.