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Nuclear protein of the testis (NUT) midline carcinoma is genetically defined by rearrangement of NUT or by immunohistochemical expression of NUT. A 6-year old child had a NUT midline carcinoma of the lung. Despite aggressive therapy, the... more
Nuclear protein of the testis (NUT) midline carcinoma is genetically defined by rearrangement of NUT or by immunohistochemical expression of NUT. A 6-year old child had a NUT midline carcinoma of the lung. Despite aggressive therapy, the child died. NUT carcinoma, which can be diagnosed immunohistochemically, remains an aggressive tumor.
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Adequate nutrient intake should be provided for the cure of children diagnosed with cancer. The aim of this study was to evaluate serum trace elements and vitamins of children with cancer at diagnosis and during treatment. Children with... more
Adequate nutrient intake should be provided for the cure of children diagnosed with cancer. The aim of this study was to evaluate serum trace elements and vitamins of children with cancer at diagnosis and during treatment. Children with newly diagnosed cancer who were admitted to our center were evaluated for serum selenium, iron, ferritin, C-reactive protein, vitamin B12, folate, and 25-OH vitamin D levels at presentation, and at the third and sixth months of cancer treatment. Forty-two children (male/female: 15/27) with a median age of 8 years (range, 2 to 17) were included in the study. Mean serum B12, folate, and iron levels were within normal ranges, but selenium and 25-OH vitamin D were low at presentation and during the 6-month period. Serum ferritin levels were high in all 3 measures, but they decreased significantly at the sixth month (P=0.04). There was no relation between micronutrient deficiency and sex, or primary disease, or stage, or place of residence of the patient....
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We examined outcomes of 62 pediatric patients with relapsed or refractory non-Hodgkin lymphoma (rr-NHL) who underwent hematopoietic stem cell transplantation (HSCT). The overall survival (OS) and event-free survival (EFS) rates were 65%... more
We examined outcomes of 62 pediatric patients with relapsed or refractory non-Hodgkin lymphoma (rr-NHL) who underwent hematopoietic stem cell transplantation (HSCT). The overall survival (OS) and event-free survival (EFS) rates were 65% and 48%, respectively. Survival rates for patients with chemosensitive disease at the time of HSCT were significantly higher than those of patients with chemoresistant disease (69% vs. 37%, p = .019 for OS; 54% vs. 12%, p < .001 for EFS; respectively). A chemoresistant disease at transplantation was the only factor that predicted a limited OS (hazard ratio = 10.00) and EFS (hazard ratio = 16.39) rates. Intensive chemotherapy followed by HSCT could be an effective strategy for treating children with rr-NHL and may offer improved survival for a significant group of pediatric patients, particularly those with chemosensitive disease at transplantation.
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Research Interests:
... month. His physical examination revealed high blood pressure over 95 percentile for his age, streched penis length was above 90 percentile for age, his testes volumes were 5 mL/5mL and he had Taner stage 2 pubic hair. Other ...
Research Interests: Adolescent, Endoscopy, Biopsy, Biological Sciences, Complication, and 15 moreChild, End Stage Renal Disease, Female, Bacteria, Exploration, Dyspepsia, Epidemiology of Helicobacter pylori infection, Chronic Renal Failure, Adult, Gastritis, Chronic Kidney Failure, Gastroscopy, Duodenal Ulcer, Duodenum, and Gastric mucosa
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Wilms tumor (WT) is one of the most common solid tumors in children. It is the second most common extracranial solid tumor after neuroblastoma. WT has a strong tendency to invade blood vessels in the form of tumor thrombus, into the renal... more
Wilms tumor (WT) is one of the most common solid tumors in children. It is the second most common extracranial solid tumor after neuroblastoma. WT has a strong tendency to invade blood vessels in the form of tumor thrombus, into the renal veins, and inferior vena cava and even into the right atrium. Extension of tumor thrombus along to the renal vein into the inferior vena cava occurs in 4-10% of all patients. Tumor thrombus extending to the right atrium is less reported as 0.7-1%. WT with renal vein thrombus has been reported to be more common in the right kidney because of the shorter right renal vein. Most patients with tumor thrombus are asymptomatic and diagnosis is only made on imaging investigations. Several imaging modalities including computed tomography (CT), magnetic resonance imaging (MRI) and Doppler ultrasonography (USG) can demonstrate intravascular tumor thrombus before surgery. In addition to CT and MRI, Doppler USG is reliable in demonstrating the presence and exte...
Wilms tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5-7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more... more
Wilms tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5-7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more often in girls. Management of a child with bilateral WT is very challenging. In contrast to unilateral WT, there has not been uniform agreement about the therapeutic strategy in the management of bilateral WT. As surgery is a critical component in the treatment of WT, the aim is to achieve a high cure rate while maintaining adequate long-term renal function in patients with bilateral WT. In the past, radical surgical procedures which lead to the patients on dialysis have been traditionally recommended in these patients. After several multicentre trials, bilateral biopsies followed by pre-operative chemotherapy and then renal salvage surgery have been recommended. The management of bilateral WT has evolved from primary surgical extirpation to kidney-pre...
Although hemangiomas are the most common vascular tumors of the liver in infancy, data regarding hypothyroidism and heart failure related to hepatic hemangiomas are limited. Here, we present a 15- day -old girl who presented with... more
Although hemangiomas are the most common vascular tumors of the liver in infancy, data regarding hypothyroidism and heart failure related to hepatic hemangiomas are limited. Here, we present a 15- day -old girl who presented with prolonged jaundice at the age of 15 days. Because her TSH level was found to be 74 μIU/mL, she was initially diagnosed with congenital hypothyroidism and L-Thyroxine replacement therapy was initiated. On follow-up examination performed two months later, it was observed that her TSH level was not suppressed and a mass was noticed in the right upper abdomen on physical examination. Abdominal ultrasonography revealed multiple masses with sizes of about 3-3,5 cm covering the whole liver. When evaluated with clinical and radiological appearance, oral methylprednisolone at a dose of 5 mg/kg/day and propranolol at a dose of 2 mg/kg were initiated with a diagnosis of hepatic hemagioma/hemangioendothelioma. Consumptive hypothyroidism due to hepatic hemangioma and co...
Veno-occlusive disease (VOD) is a rare complication of chemotherapy in children with cancer. In the literature successful treatment of severe VOD has not been well determined. The authors report a 5-year-old boy with Willms tumor whose... more
Veno-occlusive disease (VOD) is a rare complication of chemotherapy in children with cancer. In the literature successful treatment of severe VOD has not been well determined. The authors report a 5-year-old boy with Willms tumor whose condition was complicated with VOD while receiving chemotherapy. He was treated successfully with high-dose methylprednisolone. After therapy, the clinical signs of VOD, such as abdominal ascites, oliguria, and jaundice, disappeared without any adverse effects of therapy.
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The aim of this study was to evaluate the clinical characteristics and treatment results of 17 children with cavoatrial tumor extension of Wilms tumor. Of the 360 Wilms tumors diagnosed between 1980 and 2000, 17 patients with intracaval... more
The aim of this study was to evaluate the clinical characteristics and treatment results of 17 children with cavoatrial tumor extension of Wilms tumor. Of the 360 Wilms tumors diagnosed between 1980 and 2000, 17 patients with intracaval thrombus were identified from the medical records at the pediatric oncology department of Hacettepe University. The following data were collected and reviewed: age, sex, presenting symptoms, tumor site, presence of anaplasia, stage, associated congenital anomalies, localization of tumor thrombus, radiologic findings, type and duration of preoperative chemotherapy, response to preoperative chemotherapy, recurrences, and survival. The frequency of cavoatrial extension in this group was 4.7% (15 in the inferior vena cava and 2 in the right atrium). Fourteen patients received preoperative chemotherapy consisting of two-drug regimen (vincristine and actinomycin D) ranging from 1 to 12 weeks (median 4 weeks). Since intravascular invasion is often asymptomatic, a careful radiologic examination to detect tumor thrombus before surgery is essential. There is no need for aggressive surgery in the presence of tumor thrombus. It may be resolved by preoperative chemotherapy. Surgical removal of the thrombus should be considered in the presence of life-threatening tumor thrombosis at diagnosis and in patients who had residual thrombus after chemotherapy.
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Research Interests:
ABSTRACT Multicentric Castleman disease is a rare lymphoproliferative disorder of unknown cause in children. Renal involvement of Castleman disease is uncommon. Proteinuria in association with multicentric Castleman disease has been... more
ABSTRACT Multicentric Castleman disease is a rare lymphoproliferative disorder of unknown cause in children. Renal involvement of Castleman disease is uncommon. Proteinuria in association with multicentric Castleman disease has been reported to occur ...
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Malnutrition is a common consequence of cancer in children, but the most effective methods of nutrition intervention are under debate. We aimed to evaluate the nutritional status of children diagnosed with cancer, and to investigate the... more
Malnutrition is a common consequence of cancer in children, but the most effective methods of nutrition intervention are under debate. We aimed to evaluate the nutritional status of children diagnosed with cancer, and to investigate the effect of oral nutritional supplements on anthropometric measurements, biochemical parameters, and outcome. A randomized clinical study of 45 newly diagnosed cancer patients was performed. Anthropometric and biochemical data and related factors were assessed at 0, 3, and 6 months after diagnosis. On initial anthropometric assessment, prevalence of malnutrition by weight or height was found to be lower as compared with body mass index (BMI), or weight for height (WFH), or arm anthropometry. Twenty-six of the patients (55%) received oral nutritional supplement. During the second 3 months after diagnosis, there was a statistically significant decrease in number of the patients with WFH <90th percentile and BMI <5th percentile (P = .003 and P = .04...
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Research Interests:
The focus of this study was to investigate anaplastic lymphoma kinase (ALK) expression by immunohistochemistry using a highly specific antibody. Distribution and frequency of ALK expression may provide a clue for ALK inhibitor use in... more
The focus of this study was to investigate anaplastic lymphoma kinase (ALK) expression by immunohistochemistry using a highly specific antibody. Distribution and frequency of ALK expression may provide a clue for ALK inhibitor use in small round cell tumors of childhood. The study group involved 76 small round cell tumors of childhood, which composed of 11 rhabdomyosarcomas, 13 Wilms tumors, 7 Ewing sarcoma/primitive neuroectodermal tumors, 34 peripheral neuroblastic tumors, and 11 acute lymphoblastic lymphoma. Anaplastic lymphoma kinase protein expression in small round cell tumors of childhood is poorly described in the literature. The findings of our study highlight a potential and possible role of targeting ALK in pediatric solid tumors by using ALK immunohistochemistry. Anaplastic lymphoma kinase may also have an oncogenic role in rhabdomyosarcomas and peripheral neuroblastic tumors, and they may possibly be treated with ALK inhibitors. Anaplastic lymphoma kinase expression in Wilms tumors is not reported in the literature, previously. Our study evaluated ALK expression in Wilms tumor samples.
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The authors describe a child with an unusual presentation of mesenteric inflammatory pseudotumor in association with leukemoid reaction. An 11-year-old-boy admitted with short stature was found to have an abdominal mass localized in the... more
The authors describe a child with an unusual presentation of mesenteric inflammatory pseudotumor in association with leukemoid reaction. An 11-year-old-boy admitted with short stature was found to have an abdominal mass localized in the right lower quadrant. The leukocyte count was 92,000/mm3 with neutrophilic leukemoid reaction. Abdominal ultrasonography and computed tomography revealed a massive calcified mass in the pelvis. Total resection of the mass was performed and the pathologic diagnosis of inflammatory pseudotumor of the mesentery was made. Leukemoid reaction dramatically resolved within a few days after surgical resection. Physicians should be aware of the association of inflammatory pseudotumor, leukemoid reaction, and massive calcification.
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Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. Extramedullary hematopoiesis (EMH) mimicking a mass may develop in the lymph nodes, kidneys, pleura, mediastinum, adrenal gland, and in particular... more
Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. Extramedullary hematopoiesis (EMH) mimicking a mass may develop in the lymph nodes, kidneys, pleura, mediastinum, adrenal gland, and in particular the spleen and liver. Other than EMH, B-cell lymphoma, acute lymphoblastic leukemia, and pancreatic schwannoma cases were reported in patients with HS. We present a 13-year-old female patient with HS and ganglioneuroma in the adrenal gland. This association is probably coincidental; however, with increasing cancer cases in HS and the genetic studies being made, this association will be clarified.
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Malignant rhabdoid tumors (MRT) mostly originate from the kidney and central nervous system. However, they may also originate from retroperitoneal and paravertebral regions, mediastinum, liver, chest wall, extremity, and neck, as well as... more
Malignant rhabdoid tumors (MRT) mostly originate from the kidney and central nervous system. However, they may also originate from retroperitoneal and paravertebral regions, mediastinum, liver, chest wall, extremity, and neck, as well as from the soft tissues. The most important method in the differential diagnosis is the analysis of cytogenetic alterations in the INI1 gene. A six-month-old girl presented with multiple conglomerated lymphadenopathies located in the anterior axillary line. MRT diagnosis was confirmed by loss of INI1 expression in the tumor tissue. This is the first case in the literature with unknown primary focus diagnosed from lymph node metastasis.
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Gastric adenocarcinoma is a rare entity in the pediatric population. Gastric hepatoid adenocarcinoma with elevated serum alpha-fetoprotein (AFP) is seen extremely rarely in children. A 12-year-old boy was admitted to the hospital with... more
Gastric adenocarcinoma is a rare entity in the pediatric population. Gastric hepatoid adenocarcinoma with elevated serum alpha-fetoprotein (AFP) is seen extremely rarely in children. A 12-year-old boy was admitted to the hospital with complaint of abdominal pain. X-ray revealed free air density below the diaphragm. Emergent laparotomy showed perforated stomach. Four weeks after the operation, he was readmitted with severe gastrointestinal obstruction symptoms. He underwent an explorative laparotomy, which revealed intestinal edema and diffuse small solid nodules covering the peritoneum. Serum AFP level was mildly elevated. Endoscopic evaluation of the upper gastrointestinal tract was performed, and a gastric mass was detected. All pathological findings were compatible with gastric carcinoma showing hepatoid differentiation. We report an unusual case of AFP-producing hepatoid gastric adenocarcinoma presenting with gastric perforation. This is, to the best of our knowledge, the first ...
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Research Interests:
Urticaria pigmentosa is the most common manifestation of mastocytosis, with the majority of cases undergoing spontaneous resolution, especially in children. Several reports have documented hematologic malignancies developing in patients... more
Urticaria pigmentosa is the most common manifestation of mastocytosis, with the majority of cases undergoing spontaneous resolution, especially in children. Several reports have documented hematologic malignancies developing in patients with urticaria pigmentosa. We present a 4.5-year-old boy with urticaria pigmentosa who developed Wilms tumor. To our knowledge, coexisting urticaria pigmentosa and Wilms tumor have not previously been described.
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Primary cardiac tumors are rare in childhood. The most common of these are rhabdomyomas. Considering that rhabdomyomas often show spontaneous regression, close follow-up may be sufficient in hemodynamically stable cases. However,... more
Primary cardiac tumors are rare in childhood. The most common of these are rhabdomyomas. Considering that rhabdomyomas often show spontaneous regression, close follow-up may be sufficient in hemodynamically stable cases. However, hemodynamically significant cardiac rhabdomyomas confer a risk of morbidity and mortality. Herein, we report a newborn infant with multifocal cardiac rhabdomyomas treated with everolimus. The optimal dose of the drug was 0.25 mg 2 times per day, 2 days per week. Patients with inoperable cardiac rhabdomyomas and with symptoms may be candidates for everolimus treatment.
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Research Interests: Comorbidity, Humans, Child, Male, Facial Features, and 15 moreBone marrow, Chimerism, Newborn Infant, Neutrophils, Multiple Infection, Ectodermal Dysplasia, Hematopoietic Stem Cell Transplantation, Antibody Response, Autosomal Dominant, Busulfan, Blood platelets, Liver neoplasms, Medical and Health Sciences, Paediatrics and reproductive medicine, and Immunologic deficiency syndromes
Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and... more
Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure.
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Peripheral neuropathy is one of the rarely reported neurological complications of interferon therapy. The authors report such a case in a 15-year-old boy during alpha-interferon therapy for Hodgkin&#39;s disease. He received... more
Peripheral neuropathy is one of the rarely reported neurological complications of interferon therapy. The authors report such a case in a 15-year-old boy during alpha-interferon therapy for Hodgkin&#39;s disease. He received alpha-interferon at a dose of 1.8 million units/day 5 times a week by subcutaneous injections as adjuvant immunotherapy post autologous stem cell transplant. Twenty months after the initiation of therapy, he complained of severe pain in his lower distal extremities. Neurological examination revealed the absence of deep tendon reflexes. A nerve conduction study showed a sensorial, polyneuropathic involvement in the lower extremities. Within 4 weeks after the stopping of interferon, his pain improved, and recovery was also seen by nerve conduction studies. Symptoms reappeared at the resumption of interferon treatment. This study suggests that peripheral neuropathy may rarely occur in patients given long-term interferon treatment at high cumulative dosage.