Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Intraosseous noninvoluting congenital hemangioma of the mandible in a neonate.

Hemangiomas are benign tumors of the capillary endothelium involved in varied clinical and pathologic entities. Congenital hemangioma is quite rare compared to infantile hemangioma. Hemangiomas rarely occur in bone. Intraosseous hemangiomas are most commonly found in the vertebral column and skull. The mandible is the most frequently affected skull bone. Intraosseous hemangiomas commonly present in the second decade of life. Here, we report a neonate with noninvoluting congenital hemangioma of the mandible. To our knowledge, she is the youngest patient reported in the literature.

Bleomycin induced hyperpigmentation with yolk sac tumor.

Hyperpigmentation is one of the cutaneous side effects of chemotherapautic agents, but it is usually accepted as a cosmetic problem. We report a child with yolk sac tumor who developed localized pigmentation after the first course of chemotherapy regimen that included cisplatin, etoposide and bleomycin. The hyperpigmentation was diffuse scattered, flagellate like and linear streaking which was thought to be mainly related to the skin toxicity of bleomycin.

Analysis of children with peripheral lymphadenopathy.

In this study, the clinical and laboratory features of children with lymphadenopathy were evaluated. Over a 3-year period, 126 patients were referred to the clinic for lymphadenopathy. Twenty-eight of cases have diseases mimicking lymphadenopathy; 98 (mean age: 86+/-55 months) have lymphadenopathy. Localized, limited, and generalized involvement was found in 52%, 30%, and 18% of patients. The most common localization was the head and neck region. The causes of lymphadenopathy were benign diseases in 75 patients. Sixty percent were reactive lymphadenopathy, 39% were lymphadenitis. Lymphadenitis was more frequently localized and bigger than 3 cm compared with reactive adenopathy (p=.02, p=.004). Twenty-three patients have malignant diseases whose mean age was higher than others (p=.002). The enlargement of supraclavicular nodes was more likely due to malignant disease (p=.001). The risk of malignant disease was higher in patients who had generalized lymphadenopathy, lymph nodes bigger than 3 cm, hepatosplenomegaly, and high lactate dehydrogenase levels. In conclusion, this study pointed out the important clues for the differential diagnosis, which were present in the history, physical, and laboratory findings.

Hodgkin lymphoma in a child with Diamond Blackfan anemia.

Diamond Blackfan anemia (DBA) is a rare disease characterized by aplasia or hypoplasia of erythroid lineage. Normochromic, usually macrocytic, but occasionally normocytic anemia and reticulocytopenia are characteristic findings of DBA. DBA is associated with an increased risk of malignancy. Most of the reported malignancies are acute myeloid leukemia. Solid tumors including hepatocellular carcinoma and osteosarcoma have also been identified. We could find 29 reported cases with DBA and malignancy. Two of them were diagnosed as Hodgkin lymphoma at 15 and 23 years, respectively. Here we report a 7-year-old boy with DBA who developed Hodgkin disease.

Thymic carcinoma in children.

Thymic epithelial neoplasms consist of thymomas, thymic carcinoids, and thymic carcinomas. Carcinomas are malignant tumors of the thymus characterized by obvious cytological anaplasia. They constitute only 4%-14% of thymic epithelial neoplams. Thymic carcinoma rarely occurs in children. Research in the English literature carried out for the present study revealed only 14 cases younger than 18 years-of-age. Here we have reported a 16-year old girl who presented with respiratory distress due to huge anterior mediastinal mass. Histological and immunohistochemical studies confirmed lymphoepithelioma-like thymic carcinoma. She received systemic chemotherapy and radiotherapy. However, she died within 15 months due to progressive disease.

Bone mineral metabolism and its relationship to leptin levels in survivors of childhood leukemia and lymphoma.

Leptin has important effects on bone metabolism. Possible relationships between leptin and bone mineral density were evaluated in the survivors of the childhood leukemia and lymphoma. Twenty patients were included the study. Anthropometric parameters, growth hormone response to provocative test, serum calcium, phosphorus, alkaline phosphates, osteocalcin, leptin levels, urinary calcium and deoxyypyridinoline levels, and bone mineral density were obtained. Anthropometric parameters of patients were not significantly different from those of a control group. Growth hormone provocative test was abnormal in 3 patients who received cranial radiotherapy. The osteocalcin levels and bone mineral density of patients were significantly lower than in the control group (p=.001, p=.02). Nine patients were in the osteopenic and 7 were in the osteoporotic range. The leptin levels of patients were significantly lower (p=.01) than in the control group. Bone mineral density (BMD) was significantly correlated with leptin level, age, body mass index, and Tanner stage in simple correlation analysis. However, in multivariate analysis only age was significant (p<.000, r: .752). Markers of bone metabolism, BMD, and leptin levels were not related with the growth hormone status of patients and did not present a correlation with the cumulative doses of drugs. There are a few studies evaluating the relationship between BMD and leptin levels in childhood cancer. Although this study did not find any correlation between the leptin level and BMD, detailed studies of larger numbers of patients are necessary to evaluate causes of decreased leptin level and the possible role of leptin on osteopenia observed in survivors of childhood cancer.

Adrenal myelolipoma in a child.

A case of adrenal myelolipoma is reported in a 14-year-old girl. Abdominal ultrasound examination revealed a left-sided ovarian cyst 13 cm in diameter and an 8 x 8-cm hyperechoic heterogenous solid mass localized in the right adrenal gland. The ovarian cyst and adrenal mass were removed surgically. Histological examination of the adrenal mass revealed a proliferation of mature adipose tissue with bone marrow-like hematopoietic elements. The ovarian cyst was a simple serous cyst.

Primary cerebral neuroblastoma: a case treated with adjuvant chemotherapy and radiotherapy.

Neuroblastoma is the most common extracranial solid tumor of childhood. A wide variety of tumor locations and clinical presentations have been described. However, neuroblastoma is rarely located in the central nervous system, except in the case of esthesioneuroblastoma. We report a child with primary central nervous system (CNS) neuroblastoma who admitted to the hospital in coma. The tumor could be partially removed in our patient. After surgery, we treated the patient successfully with combined radiotherapy and eight courses of cisplatin-based chemotherapy. Our patient was followed-up for 34 months without any evidence of relapse.

Castleman disease: a case with atypical presentation.

Castleman disease is a benign lymphoproliferative disorder characterized by enlarged lymph nodes. In children the disease is rare, usually localized, and asymptomatic. Resection of the node is almost always curative. A case is reported that was diagnosed as hyaline vascular-type Castleman disease at 1 year of age. The disease recurred from infraclavicular region in addition to primary site, even though total excision was performed. Although the disease is mullticentric after recurrence, the patient has no systemic symptoms.

Childhood cancer in developing countries.

The problem of childhood cancer cannot be evaluated separately from developmental levels and states of health of the countries. Rapid increase in population, poverty, poor hygiene, lack of education, and multitude health problems impede the development of pediatric oncology and success of the management of childhood cancer in developing countries. More than 85% pediatric cancer cases occur in developing countries that use less than 5% of world resources. The rate will exceed 90% in the next two decades, due to the increase of youth population in favor of developing countries. Incidence rates, pathology, and clinical characteristics of various cancers seem different in developing and developed countries. Different environments, life styles, dietary habits, and hygienic conditions are the main reasons for those differences. Unprecedented changes in diagnostic techniques, treatment methods and supportive care have occurred during the last decades. Consequently, management has improved and the mortality rates have decreased. Most of the children with cancer living in developing countries could not profit from those advances in pediatric oncology because of the cost.

Primary Burkitt's lymphoma only involving the chest wall at relapse in a child.

Burkitt's lymphoma primarily originating from the soft tissue is a very rare neoplasm. A five-year-old boy was admitted with a mass on the lateral side of the right breast. Radiological examination revealed a mass, which had originated from the soft tissue of the chest wall without any contiguous pleural and lung parenchyma disease. Histopathology showed this to be Burkitt's lymphoma. To our knowledge, this case is the first instance of Burkitt's lymphoma presenting as an isolated chest wall mass which originated from the soft tissue.

Wilms' tumor in a case with Möbius' syndrome associated with arthrogryposis and mega cisterna magna.

Möbius' syndrome is a rare congenital anomaly characterized by paralysis of the 7th and other cranial nerves and musculoskeletal abnormalities. We report a patient with Möbius' syndrome associated with arthrogryposis and mega cisterna magna in addition to the classic components of this syndrome. The case is interesting because she developed Wilms' tumor at 21 months of age. To our knowledge, this is the first case of this association reported in the literature.

Sphenoid sinus involvement in neuroblastoma: a case report and review of the literature.

Neuroblastoma is the most common extracranial solid tumors of childhood. The most frequent anatomic site of the primary tumor is abdomen. Metastatic spread of neuroblastoma to lymph node, bone, bone marrow and liver are common at presentation. However, primary or metastatic involvement of sphenoid sinus is extremely rare. Here, we report a case with stage IV neuroblastoma presents with the involvement of the sphenoid sinus. To our knowledge this is the fourth case of neuroblastoma involved sphenoid sinus in English literature.