Pulmonary blastoma: A rare case of childhood malignancy

A case of pulmonary blastoma is presented with review of the radiographic, ultrasonographic and CT appearances of this rare malignant tumor of children.Pulmonary blastoma arises from primitive lung mesenchyma and histologically resembles fetal lung. The clinical presentation is usually respiratory distress. The prognosis of the disease is poor even if surgical resection, supplemental chemotherapy and radiation therapy are performed [1]. We present a case of pulmonary blastoma who died two days after surgical resection.     

Chylous effusion: a rare presentation of childhood malignancy

Two boys, aged 5 and 4 years, recently presented with gross chylous ascites and chylothorax respectively. In both cases the underlying pathology was found to be rhabdomyosarcoma. Offprint requests to: O. B. Eden     

A retrospective review of undifferentiated malignancy in childhood

We reviewed the experience of children with "undifferentiated" cancer at The Children's Hospital of Pittsburgh (CHP) from 1971 through 1987. Of 2,095 patients 0-18 years old with diagnoses during that time of any cancer, the initial CHP pathology report rendered a diagnosis in 22 children (1.1%) of "undifferentiated malignancy" or "malignant tumor" (15 cases), "undifferentiated carcinoma" (two cases) or "anaplastic tumor or carcinoma" not otherwise specified (five cases). A review of pathologic findings using current methods and immunostaining led to the assignment of a specific diagnosis in 15 of the 20 cases so studied. Thus, the incidence of undifferentiated cancer by current criteria was reduced to no more than 0.23%. Two of the five tumors for which an alternative diagnosis could not be established were described as "rhabdoid," but because it is not clear that these tumors fit into a single category they were still considered to be undifferentiated. Clinical features and management of the 22 cases including the five persistently diagnosed as undifferentiated malignancy were heterogeneous. In the face of reassigned diagnoses, a number of patients would likely have received different chemotherapy as well as radiation. Nonetheless, seven patients with malignancy show no evidence of disease (NED), including several whose therapy, given the current best diagnosis, would not be considered to have been optimal. We recommend that for patients undergoing biopsy of a tumor, sufficient material be obtained for extensive pathologic evaluation. In this way, the diagnosis of undifferentiated malignancy in children can be almost eliminated.(ABSTRACT TRUNCATED AT 250 WORDS)     

A rare primary pulmonary tumor of childhood

Pleuropulmonary blastoma (PPB) is known to be the pulmonary blastoma of childhood. It has a range of macroscopic and microscopic features which appear to correlate with eventual prognosis. Type 1, presenting as a multicystic lesion, occurs at an earlier age and has a more favorable prognosis than other types. The presented case of type 1 PPB had a microscopic focus of rhabdomyosarcoma. Although this patient was disease-free one year after the initial diagnosis without chemotherapy, he presented at 14 months with local dissemination and cardiac metastasis, revealing the inevitable chemo-radiotherapy need in PPB.     

Pneumonia and pneumonitis in childhood malignancy

We conducted a survey over a 21-year period of the incidence and risk of occurrence of episodes of pneumonia and pneumonitis in children treated for solid tumours and leukaemia. One hundred episodes occurred amongst 219 patients, seven of which were associated with death. Focal opacification on the chest radiograph was more common than diffuse opacification. Patients with leukaemia had a significantly higher rate of occurrence of pneumonia and pneumonitis during the periods of induction and maintenance compared with the off-treatment period, and during the relapse period compared with the period of maintenance. Patients with solid tumours had a significantly higher rate of occurrence during treatment compared to the off-treatment period. The rate of occurrence on treatment was the same in patients with solid tumours and acute leukaemia. Children with malignancy have a high incidence of pneumonia and pneumonitis and death is rare if the patient does not have terminal malignant disease.     

Breast feeding and childhood hematological malignancy

Breast milk is known to have anti-infective and immunomodulating effects on infants, but its association with childhood cancer has not been well studied. Artificial feeding may affect the immune response in carcinogenesis. In this communication the authors have reviewed different articles describing the association between breast feeding (BF) and subsequent development of childhood hematological malignancy. It appears that BF may have a protective effect on childhood cancer, both the duration of BF as well as the quantity of milk ingested is probably critical to the beneficial immunological effects of BF against childhood cancer if any.     

Intrahepatic cholangiocarcinoma as a rare secondary malignancy after allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia: A case report

Secondary malignancies are a significant cause of non‐relapse mortality in patients who undergo allogeneic HCT. However, secondary liver cancer is rare, and ICC following HCT has never been reported in the literature. Secondary solid cancers typically have a long latency period, and cholangiocarcinoma is classically a malignancy occurring in older individuals. Here, we report the first case of secondary ICC, which presented just 3 years after HCT in a young adult with a history of childhood ALL. A 26‐year‐old male with history of precursor B‐cell ALL presented with asymptomatic elevated liver function tests 3 years after HCT. Laboratories were indicative of biliary obstruction. ERCP showed focal biliary stricturing of the common and left hepatic ducts. MRCP revealed left intrahepatic duct dilatation, suggestive of intrahepatic obstructing mass. Additional workup lead to a clinical diagnosis of ICC. The patient underwent left hepatectomy with extrahepatic bile duct resection and portal lymphadenectomy. Surgical pathology was consistent with moderately differentiated cholangiocarcinoma. Our case illustrates a rare SMN following HCT for ALL. It is the first case report of ICC occurring as a secondary cancer in this patient population. Although cholangiocarcinoma is characteristically diagnosed in the older population, it must remain on the differential for biliary obstruction in post‐HCT patients.     

Adaptation of siblings to childhood malignancy.

To explore the impact of childhood cancer, school-aged patients and their healthy siblings from 71 families were given one or more of the following psychologic tests: the Piers-Harris Children's Self-Concept Scale, the Bene-Anthony Family Relations Test, and the Thematic Apperception Test. The siblings showed even more distress than the patients in the areas of perceived social isolation, perception of their parents as overindulgent and overprotective of the sick child, fear of confronting family members with negative feelings, and concern with failure (older siblings only). In other areas, such as anxiety and vulnerability to illness and injury, the patients' and siblings' experience appeared very similar. These results indicate that siblings, like patients, experience severe stress when confronted with a chronic, life-threatening illness such as cancer. In recognizing and attending to their needs, one can practice primary prevention in pediatrics by facilitating their healthy adaptation to a very difficult situation.     

Unusual secondary tumors after childhood lymphoid malignancy

Second malignant neoplasms (SMN) in individuals who survived childhood cancer have been reported with increasing frequency during the last decades. The overall probability of developing second malignancy for children treated for cancer was estimated at about 2–5% at 25 years. In children, the tumors most often associated with the development of SMN are retinoblastoma and Hodgkin's disease. We report two cases of unusual second tumors in two patients cured of lymphoid malignancy: one boy cured of acute lymphoblastic leukemia developed mediastinal ganglioneuroma nine years later and one girl had gastric carcinoma seven years after Hodgkin's disease. Both developed a tumor in nonirradiated areas. Gastric carcinoma and ganglioneuroma are not reported as recurrent SMN in survivors after childhood cancer, with one single case of gastric carcinoma and one of ganglioneuroblastoma having been reported as second tumor in survivors after childhood cancer. © 1995 Wi1ey-Liss Inc.     

Erythrocyte fatty acid profiles in childhood malignancy

The erythrocyte fatty acid composition was determined for 79 children using gas chromatography. The stearic:oleic acid ratio (SOR) was significantly lower in those children with newly diagnosed malignancies than in a reference group of healthy children. However, a control group of children with chronic, non-malignant conditions were also found to have a low SOR. These results suggest that the erythrocyte SOR cannot be used as a tumour marker in children.     

Growth of long term survivors of childhood malignancy

The growth of all children from one referral centre alive and well more than three years following the diagnosis of malignancy was analysed. Those treated with chemotherapy alone showed significant catch up growth. Children treated with chemotherapy and spinal irradiation showed no overall height loss, but manifested significant reduction in sitting height. Those treated by cranial irradiation had a significant height loss in the first year from which no catch up growth occurred and also had significantly reduced sitting height.     

Association of rib anomalies and malignancy in childhood

A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P<0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.     

The pattern of childhood malignancy in eastern Nigeria

The pattern of childhood malignancies in the developing countries of Africa is characterized by a high incidence of lymphoma and a low incidence of leukaemia. The converse is the case in the developed countries of Europe and North America. A high incidence of lymphoma with low incidence of leukaemia has been associated with poor living standards. The clinical records of 257 children admitted with malignant disease to the University of Nigeria Teaching Hospital, Enugu between January 1978 and December 1982 were studied retrospectively to determine the pattern of malignant diseases amongst these children. The lymphomas were the commonest tumours, constituting nearly 40% of the total, followed by Wilm's tumour (14.0%), leukaemia (12.9%) and CNS tumours (9.7%). However, the incidence of lymphoma was lower while the incidence of leukaemia was higher than in previous reports from other parts of Nigeria and Africa. The rarity of Ewing's tumour, histocytosis-X and testicular tumours in African children is confirmed in the present study.     

Antioxidant micronutrients and childhood malignancy during oncological treatment

Serum antioxidant vitamins A (retinol) and E (α-tocopherol), β-carotene, zinc, and selenium, and cholesterol and related proteins for 170 children with newly diagnosed malignancy were measured at diagnosis and 6 months after initiation of treatment, and compared with those of 632 cancer-free controls. Incident cancer cases and controls were 1–16 years old and recruited between 1986 and 1989. At diagnosis, age- and sex-adjusted serum concentrations of retinol, β-carotene, zinc, and α-tocopherol were significantly inversely associated with cancer. No significant decreases in mean values were observed at 6 months, except for the α-tocopherol–to–cholesterol ratio in patients with bone tumors and serum zinc in bone tumors and central nervous system malignancies. An increase during the period of treatment was found for retinol and selenium in leukemia patients. β-carotene was maintained at the initial concentrations determined prior to therapy. These findings provide further information about micronutrient requirements in children with cancer. Med. Pediatr. Oncol. 29:213–217, 1997. © 1997 Wiley-Liss, Inc.     

Viridans streptococcal bacteremia-related encephalopathy in childhood with malignancy

Viridans streptococcal bacteremia is a prognostic factor in pediatric patients with malignant disease accompanied by severe neutropenia. Here the authors describe 4 patients with viridans streptococcal bacteremia-related encephalopathy who showed serious complications, which included seizures and loss of consciousness. Therapy for relief of brain edema on seizures was started quickly, and included the administration of midazolam, dexamethasone, and mannitol with antimicrobial therapy. The treatment was successfully completed without sequelae. The authors registered 28 episodes of viridans streptococcal bacteremia in their hospital. The peak of serum C-reaction protein was higher in viridans streptococcal bacteremia patients with encephalopathy than in those without encephalopathy. The authors concluded that viridans streptococcal bacteremia can induce encephalopathy in pediatric patients with malignancy and that it is crucial to establish an accurate diagnosis and initiate therapy as soon as possible.     

Increased prevalence of minor anomalies in childhood malignancy

Body measurements were taken and the prevalence of major malformations and of 57 minor anomalies was determined in 106 children with malignant disease, in 81 of their sibs, and in 106 control subjects matched to the patients according to sex, age and ethnic origin.Leukaemic children had a significantly smaller head circumference than the corresponding control children, but no significant differences in height, anthropometric and syndromologic indices were found.No differences were observed in the frequency of associated major malformations including renal malformations detectable by sonography. The prevalence of minor anomalies was significantly higher in the patients with malignant disease and their sibs than in the control children: 69.2% of the patients, 63.0% of the sibs and 34.6% of the control subjects had at least one minor anomaly. When two and more minor anomalies were considered, the prevalence figures were 36.5%, 29.6% and 12.5%, respectively. Among the single minor anomalies only the Sydney line was significantly more frequent in patients with solid tumours. No specific association of an individual dysplasia or a pattern of minor anomalies with a given tumour could be established.