http://www.pedresearch.org/cgi/content/abstract/59/2/259

CLINICAL INVESTIGATIONS

Patterns of Growth and Body Proportions After Total-Body Irradiation and Hematopoietic Stem Cell Transplantation During Childhood

BOUDEWIJN BAKKER, WILMA OOSTDIJK, RONALD B. GESKUS, W. HENRIËTTE STOKVIS-BRANTSMA, JAAK M. VOSSEN and JAN M. WIT

Department of Pediatrics [B.B., W.O., W.H.S.-B., J.M.V., J.M.W.], Department of Medical Statistics [R.B.G.], Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands

Patterns of growth and body proportions were studied in 75 children receiving total-body irradiation (TBI) and hematopoietic stem cell transplantation (SCT) before onset of puberty. Of the 19 patients receiving GH, only data obtained before onset of GH were included. Thirty-two patients reached final height (FH). Median change in height SD score (SDS) between SCT and FH was –1.7 in boys and –1.1 in girls. Peak height velocity (PHV) was decreased in the majority of the patients (median PHV 5.7 cm/y in boys and 5.3 cm/y in girls), even though it occurred at appropriate ages. Changes in body proportions were analyzed by linear mixed-effects models. Decrease in sitting height SDS did not differ between boys and girls. In boys, decrease in leg length SDS was of comparable magnitude, whereas, in girls, decrease in leg length was less pronounced, leading to a significant decrease in SDS for sitting height/height ratio in girls only. The sex-specific effects of several variables on height SDS were analyzed by linear mixed-effects modeling, showing a slightly faster decrease in younger children and a more pronounced decrease during puberty in boys compared with girls. We conclude that 1) younger children are more susceptible to growth retardation after TBI and SCT, 2) pubertal growth is more compromised in boys, and 3) leg growth is relatively less affected in girls, possibly due to a high incidence of gonadal failure in girls.

Abbreviations:
FH, final height
PHV, peak height velocity
SCT, stem cell transplantation
SDS, standard deviation score
TBI, total-body irradiation

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16394892&dopt=Abstract

J Pediatr Hematol Oncol. 2006 Jan;28(1):40-2.
   
Long-term survival following a phase I/II trial with VETOPEC for solid tumors in childhood

Ziegler DS, McCowage G, Cohn RJ, White L.

Centre for Children's Cancer and Blood Disorders, Sydney Children's Hospital, Randwick, NSW, Australia.

The objective of this study was to evaluate long-term survival after treatment during a phase I/II trial with a specific regimen of vincristine, etoposide, and escalating cyclophosphamide (VETOPEC). Fifty-six children with poor-prognosis solid tumors were enrolled on study between May 1991 and May 1994. All had tumors that had relapsed on, or were refractory to, conventional treatment, or for whom existing treatment options were considered ineffective. The records of all surviving patients were reviewed to ascertain their disease and health status. Of the 56 patients, 10 patients (18%) remain alive with no further disease progression at a median follow-up of 11 years (range 7-13 years). Eight patients (14%) remain completely free of disease. None of the patients show long-term side effects directly attributable to the VETOPEC regimen, apart from one patient with ovarian failure. The VETOPEC regimen can offer not only good tumor responses but also the chance of cure for a surprisingly large number of children with very-poor-prognosis solid tumors. This regimen warrants continuing development and consideration for use in future trials.

PMID: 16394892 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16425266&dopt=Abstract

Pediatr Blood Cancer. 2006 Jan 19; [Epub ahead of print]
 
Analysis of biologic surrogate markers from a Children's Oncology Group Phase I trial of gefitinib in pediatric patients with solid tumors.


Jimeno A, Daw NC, Amador ML, Cusatis G, Kulesza P, Krailo M, Ingle AM, Blaney SM, Adamson P, Hidalgo M.

Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University, Baltimore, Maryland.

This trial evaluated the effect of gefitinib on the plasma circulating levels of epidermal growth factor receptor (EGFR), vascular endothelial growth factor (VEGF), matrix metalloproteinases (MMP)-2 and -9 of patients treated on a pediatric Phase I trial. Complete plasma correlative studies were obtained from 16 of the 25 enrolled patients. There was a trend for lower MMP-2 baseline levels in patients with partial response or stable disease. The Ewing sarcoma from the only patient with partial response lacked egfr mutations. Gefitinib did not induce any significant variation in the levels of the assessed parameters, and none of these determinations showed significant predictive or prognostic value. Pediatr Blood Cancer (c) 2006 Wiley-Liss, Inc.

PMID: 16425266 [PubMed - as supplied by publisher]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16435380&dopt=Abstract

Pediatr Blood Cancer. 2006 Jan 24; [Epub ahead of print]   

Topotecan by 21-day continuous infusion in children with relapsed or refractory solid tumors: A Children's Oncology Group study.

Hawkins DS, Bradfield S, Whitlock JA, Krailo M, Franklin J, Blaney SM, Adamson PC, Reaman G.

Children's Hospital and Regional Medical Center, Seattle, Washington.

PURPOSE: The Children's Oncology Group conducted a phase II trial of 21-day continuous infusion topotecan to determine the response rate in pediatric patients with recurrent or refractory malignant solid tumors. PROCEDURE: Patients with Ewing sarcoma family of tumors (ESFT), osteosarcoma (OS), soft tissue sarcomas (STS), medulloblastoma (MB)/primitive neuroectodermal tumor (PNET), astrocytoma, or neuroblastoma (NB) recurrent or refractory to conventional therapy, measurable disease, and adequate organ function were treated with topotecan 0.3 mg/m(2)/day by continuous intravenous infusion for 21 consecutive days, followed by 7 days without therapy prior to response assessment. RESULTS: Fifty-five patients were enrolled; two were ineligible, two were removed from protocol therapy prior to evaluation for response, and one was inevaluable for response, leaving 53 and 50 patients evaluable for toxicity and response, respectively. Objective responses were seen in 2/20 patients with ESFT (both partial responses, 4 and 19 courses), 0/10 OS patients, and 0/12 STS patients. There were insufficient patients enrolled to determine the response rate for the MB/PNET, astrocytoma, and NB strata. The most common Grade 3 or 4 toxicities during the first course of therapy were thrombocytopenia (12/53), neutropenia (8/53), and fatigue (7/53). CONCLUSION: Intravenous topotecan by 21-day continuous infusion is tolerable in pediatric patients with recurrent or refractory solid tumors. Limited activity was seen in ESFT and further development of this topotecan schedule as a single agent is not warranted. Pediatr Blood Cancer (c) 2006 Wiley-Liss, Inc.

PMID: 16435380 [PubMed - as supplied by publisher]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16421909&dopt=Abstract

Pediatr Blood Cancer. 2006 Jan 18; [Epub ahead of print]

An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma.

Paulino AC, Nguyen TX, Mai WY.

Department of Radiology, Division of Radiation Oncology, Baylor College of Medicine and Methodist Hospital, Houston, Texas.

PURPOSE: To examine prognostic factors for primary site control and analyze late effects according to local treatment modality in non-metastatic Ewing sarcoma (ES). MATERIALS AND METHODS: From 1976 to 2001, 76 patients with localized ES and a median age of 14.5 years were seen and treated at one institution. Tumors were located in the extremity in 38, pelvis in 13, spine in 11, trunk in 8, and head and neck in 6. Tumor size was </=8 cm in 44 and >8 cm in 32. Local therapy included radiotherapy (RT) alone in 40, surgery (S) alone in 27, and surgery followed by postoperative radiotherapy (S + RT) in 9. Chemotherapy (CT) was delivered to 65 patients (86%). Median follow-up for surviving patients was 9.7 years. RESULTS: The 5- and 10-year overall survival rates were 57.5% and 52.1% while the 5- and 10-year local control rate was 79.2%. The 5- and 10-year local control rates were 78.2% for RT, 77.6% for surgery, and 88.9% for S + RT (P = 0.68). Multivariate analysis showed that only the use of CT was found to be a prognostic factor for local control (P = 0.014). The 5- and 10-year local control rates were 83.7% for those receiving CT and 51.1% for those not receiving CT. For patients followed at least 5 years from diagnosis, late effects were seen in 10 of 19 (52.6%) receiving RT, 2 of 5 (40%) receiving S + RT, and 4 of 16 (25%) receiving surgery alone. The most common late effects with RT were muscular atrophy, limb length growth delay, and development of second malignancy. Scoliosis and decrease range of motion of an extremity were seen regardless of local treatment modality. Three patients who had surgery alone had an amputation whereas two who had RT had an amputation secondary to relapse or development of osteosarcoma. CONCLUSION: In this single institution study, the use of CT was the only factor found to impact on local control. Late effects were common regardless of local control strategy. Pediatric Blood Cancer (c) 2006 Wiley-Liss, Inc.
PMID: 16421909 [PubMed - as supplied by publisher]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16411221&dopt=Abstract


Pediatr Blood Cancer. 2006 Jan 12; [Epub ahead of print]

Second and third malignant solid tumor in a girl with ovarian Sertoli-Leydig tumor.

Panagiotou JP, Polychronopoulou S, Sofou K, Vanvliet-Constantinidou C, Papandreou E, Haidas S.

Department of Pediatric Hematology/Oncology, "Aghia Sophia" Children's Hospital, Athens, Greece.

We report a Sertoli-Leydig cell (SLC) tumor of the right ovary in a 10-year-old girl, which was dealt with surgical removal. Three months after resection, she presented with a new episode of acute abdomen because of an abdominal mass, which histologically was compatible with an undifferentiated embryonal rhabdomyosarcoma. Chemotherapy, according to SIOP-??? 89 protocol, was administered additionally to radiotherapy (3,960 cGy). Three years after completing treatment, the patient developed a painful swelling at her left upper arm. The diagnosis was Ewing sarcoma of the humerus, which was confirmed by identification of the typical 11; 22 translocation on cytogenetic and molecular analysis of the tumor tissue. The patient died 14 months from Ewing diagnosis due to progressive disease. Pediatr Blood Cancer (c) 2006 Wiley-Liss, Inc.

PMID: 16411221 [PubMed - as supplied by publisher]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16418250&dopt=Abstract

Radiographics. 2006 Jan-Feb;26(1):157-71.

Causes of facial swelling in pediatric patients: correlation of clinical and radiologic findings.

Khanna G, Sato Y, Smith RJ, Bauman NM, Nerad J.

Department of Radiology, University of Iowa College of Medicine, 200 Hawkins Dr, Iowa City, IA 52242, USA. geetika-khanna@uiowa.edu

Facial swelling is a common clinical problem in pediatric patients. The causes of swelling are diverse, and knowledge of the typical clinical and imaging manifestations and the most common sites of occurrence of these conditions is needed to formulate a differential diagnosis. The general clinical manifestations may be classified into the following four groups: (a) acute swelling with inflammation, (b) nonprogressive swelling, (c) slowly progressive swelling, and (d) rapidly progressive swelling. Conditions that may account for acute swelling accompanied by inflammation include lymphadenitis, sinusitis, odontogenic infection, and abscess. Contrast-enhanced computed tomography is the modality of choice for detection of abscesses requiring surgical drainage. Nonprogressive midfacial swelling is suggestive of a congenital anomaly (eg, a cephalocele, nasal glioma, or nasal dermoid or epidermoid cyst). Slowly progressive swelling may indicate the presence of a neurofibroma, hemangioma, lymphangioma, vascular malformation, or pseudocyst, or of fibrous dysplasia. The differential diagnosis for rapidly progressive facial swelling in association with cranial nerve deficits should include rhabdomyosarcoma, Langerhans cell histiocytosis, Ewing sarcoma, osteogenic sarcoma, and metastatic neuroblastoma. (c) RSNA, 2006.

PMID: 16418250 [PubMed - in process]