Bone and [18F]fluorodeoxyglucose positron-emission tomography/computed tomography scanning for the assessment of osseous involvement in Hodgkin lymphoma in children and young adults.

To compare FDG PET/CT and bone scans for the management of young patients with Hodgkin lymphoma (HL). Eighteen patients, aged 7-24 years, with HL received bone and PET scans within 2 weeks--11 at diagnosis and 7 at known or suspected relapse (37 scan pairs). In six patients (nine scan pairs) both studies were positive. In four patients (five scan pairs), PET showed involvement not evident on bone scan. In four patients (four scan pairs), one of the studies was equivocal and the other negative. In one patient, bone scan showed abnormal uptake in a benign lesion. In nine patients (19 scan pairs), both studies were negative. All osseous metastases identified on bone scan were present on PET. Many additional sites of bony involvement were identified on PET. The bone scan may be safely eliminated.

Clinical and CT features of benign pneumatosis intestinalis in pediatric hematopoietic stem cell transplant and oncology patients.

BACKGROUND: Pneumatosis intestinalis in children is associated with a wide variety of underlying conditions and often has a benign course. The CT features of this condition have not been systematically investigated. OBJECTIVE: Defining benign pneumatosis intestinalis as pneumatosis intestinalis that resolved with medical management alone, we sought to: (1) determine whether the incidence of benign pneumatosis intestinalis had increased at our pediatric cancer hospital; (2) characterize CT features of benign pneumatosis intestinalis; and (3) determine the relationship between imaging features and clinical course of benign pneumatosis intestinalis in this cohort. MATERIALS AND METHODS: Radiology reports from November 1994 to December 2006 were searched for "pneumatosis intestinalis," "free intraperitoneal air," and "portal venous air or gas." Corresponding imaging was reviewed by two radiologists who confirmed pneumatosis intestinalis and recorded the presence of extraluminal free air, degree of intramural gaseous distension, number of involved bowel segments, and time to pneumatosis resolution. RESULTS: The search revealed 12 boys and 4 girls with pneumatosis intestinalis; 11 were hematopoietic stem cell transplant recipients. The annual incidences of benign pneumatosis have not changed at our institution. Increases in intramural distension marginally correlated with the number of bowel segments involved (P=0.08). Three patients had free air and longer times to resolution of pneumatosis (P=0.03). CONCLUSION: Male children may be at increased risk of benign pneumatosis intestinalis. The incidence of benign pneumatosis at our institution is proportional to the number of hematopoietic stem cell transplants. The degree of intramural distension may correlate with the number of bowel segments involved. Patients with free air have a longer time to resolution of benign pneumatosis.

Evaluation of a comprehensive transcranial doppler screening program for children with sickle cell anemia.

BACKGROUND: Although transcranial Doppler ultrasonography (TCD) screening is effective in identifying children with sickle cell anemia (SCA) who are at high risk of stroke, not all children are screened. In 2003, we instituted a comprehensive TCD screening program designed to screen all at-risk children treated at our sickle cell center. PROCEDURE: We evaluated the efficacy of our program by comparing the number of patients screened per year and incidence of first stroke across three periods defined by TCD usage: (1) pre-dating TCD screening, (2) sporadic TCD screening, and (3) comprehensive TCD screening. RESULTS: During Period 2, an average of 52 patients/year were screened. In Period 3, an average of 95 patients/year were screened representing 99% of the at-risk population. Eighteen strokes occurred in Period 1, 22 in Period 2 and three in Period 3. The first stroke incidence was significantly lower in Period 3 compared to Periods 1 and 2 (P = 0.047). Furthermore, of the 25 patients in Periods 2 and 3 who had stroke, only six had received TCD screening (four in Period 2, two in Period 3). These six either declined prophylactic transfusion therapy (n = 2), had co-existing stroke risk factors (n = 2), or did not return for appropriate TCD follow-up (n = 2). Therefore, strokes that occurred were not the result of a failure of TCD screening per se. CONCLUSIONS: It is possible to perform TCD screening of most children with SCA. TCD screening is effective in reducing first stroke incidence in these children.

Prolactinoma as the first manifestation of Gardner's syndrome.

Familial adenomatous polyposis (FAP) is an inherited condition causing numerous adenomatous colorectal polyps and a markedly elevated risk of colon cancer. FAP may be associated with various extracolonic manifestations such as desmoid fibromatosis and osteomas (termed Gardner's syndrome) and brain tumors, usually medulloblastoma or glioma [termed Brain Tumor Polyposis (BTP) syndrome type 2]. We describe a pediatric patient who initially presented with prolactinoma and later was found to have Gardner's syndrome. A germline mutation of the APC (adenomatous polyposis coli) gene was identified. Our case illustrates the association between prolactinoma and FAP, which may represent a rare subtype of Gardner's and BTP syndromes.

PET/CT characterization of fibroosseous defects in children: 18F-FDG uptake can mimic metastatic disease.

OBJECTIVE: The purpose of this study was to characterize the anatomic appearance and metabolic activity of nonossifying fibromas, fibrous cortical defects, and cortical desmoids on PET/CT images. CONCLUSION: Over a 14-month period, we identified eight nonossifying fibromas, four fibrous cortical defects, and two cortical desmoids in 330 children who underwent PET/CT for the evaluation of a known or suspected malignancy. CT, conventional radiography, MRI, or clinical follow-up was used to confirm the diagnoses of these fibroosseous lesions. Eleven of the 14 children underwent multiple PET/CT examinations; thus, 34 studies were included. The lesions showed variable metabolic activity as indicated by 18F-FDG uptake: 19 PET/CT examinations showed lesions with mild 18F-FDG uptake, eight showed moderate 18F-FDG uptake, and seven showed intense uptake. When PET reveals metabolically active osseous abnormalities in children who are at risk for bone metastases, benign fibroosseous lesions should be considered in the differential diagnosis before additional diagnostic procedures are undertaken. Benign fibroosseous lesions may be metabolically active and thus mimic metastatic osseous disease in children with underlying malignancies. Correlative CT or other anatomic imaging can confirm the benign nature of these lesions.