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ABSTRACT: The combination of cyclophosphamide and topotecan (cyclo/topo) has shown objective responses in relapsed Ewing sarcoma, but the response duration is not well documented. We reviewed characteristics and outcome of 14 patients with Ewing sarcoma, treated uniformly at a single institution and offered cyclo/topo at first relapse. Six patients (43%) had relapse at distant sites. All patients received first-line salvage therapy with cyclophosphamide 250 mg/m and topotecan 0.75 mg/m, daily for 5 days repeated every 21 days. The median number of cycles was 4 (range 1 to 10). All toxicities were manageable, the most common being transient cytopenias. There were also 4 episodes of febrile neutropenia, and 3 episodes of gross hematuria. Response was assessable in 13 patients and showed progressive disease in 6 (46%), stable disease in 4 (31%), and partial response in 3 (23%). Nine patients had local control, consisting of radical surgery in 2, radiation in 3, and a combination in 4 patients. Response, when it occurred, was maintained for a median of 8 months (range, 4 to 28 mo). Four patients (29%) are alive at 3, 7, 9, and 110 months after relapse; 1 is receiving cyclo/topo, 1 is on third-line therapy, and 2 are in second and fourth remission. The low toxicity of this combination, and the lack of sustained responses, warrant its investigation in combination with targeted or novel therapeutic agents in relapsed disease.
Journal of Pediatric Hematology/Oncology 10/2012; · 1.16 Impact Factor
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Maysaa Salman,
Hani Tamim,
Fouad Medlej,
Tarek El-Ariss,
Fatima Saad,
Fouad Boulos,
Toufic Eid,
Samar Muwakkit,
Nabil Khoury, Miguel Abboud,
Raya Saab
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ABSTRACT: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Outcome of patients treated on standard protocols, in a multidisciplinary cancer center setting outside of clinical trials, is not well reported. We reviewed characteristics and outcome of 23 pediatric patients treated at a single, multidisciplinary cancer center in Lebanon, between April 2002 and December 2010. Median follow-up was 41 months. The most commonly affected primary site was the head and neck (48%, n = 11). Nineteen tumors (82.6%) were of embryonal histology. Tumor size was ≥5 cm in eight (34.8%) patients. Sixteen patients (69.6%) had localized disease, and one (4.4%) had metastatic disease. Fifteen (65.2%) had Group III tumors. All patients received chemotherapy, for a duration ranging 21-51 weeks. Upfront surgical resection was performed in 10 patients (43.5%). Eighteen patients (78.3%) received radiation therapy. The 5-year overall and disease-free survival rates were 83% and 64%, respectively. Relapse correlated with absence of surgery. Treatment of childhood RMS in a multidisciplinary cancer center in Lebanon results in similar survival to that in developed countries when similar protocols are applied. There was a higher incidence of local relapse, but those were salvageable with further therapy and surgical local control.
Pediatric Hematology and Oncology 05/2012; 29(4):322-34. · 0.89 Impact Factor
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ABSTRACT: Pediatric ischemic stroke still represents a burden, and more than half of the survivors will experience cognitive or motor disabilities. The objective of this study was to investigate the role of thrombophilia in a cohort of children with arterial ischemic stroke. The records of infants and children with clinically and radiologically confirmed stroke were reviewed. Patients with venous or perinatal stroke were not included. Thirty-three patients were diagnosed with arterial ischemic stroke. The male/female ratio was 1.75:1. The median age was 4 years. The most frequent clinical manifestations were hemiparesis (54.5%) and seizures (33.3%). Genetic thrombophilia testing was available on 24 patients. Nine of the 24 patients (37.5%) were heterozygous for factor V Leiden. None of the patients carried the factor II G20210A variant. Ten patients (41.7%) were heterozygous and 3 (12.5%) were homozygous for methylenetetrahydrofolate reductase (MTHFR) C677T variant. Fifteen patients (62.5%) had one or more genetic polymorphism. Factor V Leiden was significantly associated with arterial ischemic stroke (P < 0.001). Stroke recurred in 2 children with multiple risk factors and MTHFR C677T mutation. Factor V Leiden is one of the major genetic risk factors for pediatric arterial ischemic stroke in Lebanon. MTHFR C677T was prevalent among patients with recurrent stroke.
Pediatric Neurology 09/2011; 45(3):155-8. · 1.52 Impact Factor
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ABSTRACT: We have sought to identify signals of assimilation of African male lines in Lebanon by exploring the association of sickle cell disease (SCD) in Lebanon with Y-chromosome haplogroups that are informative of the disease origin and its exclusivity to the Muslim community. A total of 732 samples were analyzed, including 33 SCD patients from Lebanon genotyped for 28 binary markers and 19 short tandem repeats on the non-recombinant segment of the Y chromosome. Genetic organization was identified using populations known to have influenced the genetic structure of the Lebanese population, in addition to African populations with high incidence of SCD. Y-chromosome haplogroup R-M343 sub-lineages distinguish between sub-Saharan African and Lebanese Y chromosomes. We detected a limited penetration of SCD into Lebanese R-M343 carriers, restricted to Lebanese Muslims. We suggest that this penetration brought the sickle cell gene along with the African R-M343, probably with the Saharan caravan slave trade.
Journal of Human Genetics 10/2010; 56(1):29-33. · 2.57 Impact Factor
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American Journal of Hematology 05/2010; 85(5):395-6. · 4.67 Impact Factor
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ABSTRACT: We evaluated the local recurrence rate (LRR) of bone sarcoma along the core-needle biopsy (CNB) tract in patients who underwent limb Salvage Surgery (LSS) following a diagnostic CNB performed irrespective of the planned surgical incision site and for which surgery did not involve any biopsy tract removal.
A retrospective review of 10 pediatric patients diagnosed with bone sarcoma using a computed tomography-guided core-needle biopsy, with evaluation of medical records, pathological specimens and radiological films from the date of diagnosis until the most recent follow-up.
None of the patients experienced local recurrence during their follow up, despite the lack of biopsy site resection. CT scans of the involved extremities were negative for any suspicious lesions in all patients up until the most recent follow-up.
Our study and review of the literature suggest that the incidence of tumor seeding the CNB tract in bone sarcoma patients is apparently low, and possibly negligible. CNB should be performed through the most direct approach to the tumor, and LSS can be performed safely through the standard approaches without excision of the biopsy tract.
The Iowa orthopaedic journal 01/2010; 30:80-3.
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ABSTRACT: Children with malignancies have low bone mass. Pathways for metabolic bone disease were investigated in children with cancer by concomitantly assessing lifestyle, clinical, and biochemical predictors of bone mass. Forty-one children who were receiving cancer therapy for 61 weeks and 39 controls were studied. Data on lifestyle factors, biochemical and hormonal parameters, dual-energy X-ray absorptiometry bone mass measurements, body composition, and bone age were obtained. Compared with controls, patients had higher weight percentile and fat mass, a 6-month delay in bone age, and lower estradiol levels. They also had higher parathyroid hormone levels and lower bone remodeling markers and bone mass. Age, height, lean mass, fat mass, and bone maturation correlated positively with several bone mass variables, so did serum estradiol, testosterone, and markers of bone remodeling. Conversely, corticosteroids, methotrexate (MTX), and intrathecal therapy negatively correlated with bone mass at the spine and hip (R = -0.33 to 0.40, p < 0.04). In the adjusted analyses, bone maturation, serum osteocalcin level, MTX, and intrathecal therapy were significant predictors of lumbar spine and total body Z-scores, bone maturation accounting for the largest proportion in Z-score variance. Chemotherapy-induced delay in bone maturation and suppression of bone modeling are major pathophysiologic pathways predicting bone mass in children with malignancies.
Journal of Clinical Densitometry 09/2009; 12(4):441-9. · 1.29 Impact Factor
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ABSTRACT: Renal venous thrombosis (RVT) is a rare but a well recognized entity in children and neonates. The clinical signs of neonatal RVT include hypertension, enlarged kidney(s), hematuria, renal insufficiency, proteinuria, thrombocytopenia, or all. Persisting impairment of kidney function and hypertension are serious and common complications in patients with RVT. Risk factors for the development of RVT include maternal diabetes mellitus, pathologic states associated with thrombosis (e.g., shock, dehydration, perinatal asphyxia, polycythemia), and sepsis. Inherited prothrombotic abnormalities have been described in some reports of RVT. We report the case of a male newborn with left RVT and associated homozygosity for both factor V Leiden (G1691A) and methylenetetrahydrofolate reductase C677T mutations in addition to elevated serum lipoprotein (a). The patient was treated with heparin. We believe our case to be the first reported case in the English medical literature of such an association between neonatal RVT and homozygosity for both factor V Leiden and methylenetetrahydrofolate reductase. This case and other studies clearly demonstrate that neonatal RVT should be evaluated for thrombophilia conditions.
Blood coagulation & fibrinolysis: an international journal in haemostasis and thrombosis 07/2009; 20(6):458-60. · 1.25 Impact Factor
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ABSTRACT: Background and Aim: Thyroid carcinomas are rare in childhood and adolescence. Management of this entity remains controversial. The aim of this study is to review our experience with multidisciplinary management of papillary thyroid cancer in the pediatric population at a tertiary care specialized medical center in Lebanon. Procedure: The medical records of all patients with thyroid cancer younger than 20 years who presented to our center between January 1991 and January 2006 were reviewed. Results: Thirteen patients with papillary thyroid carcinoma (PTC) were identified. No patient had previous exposure to ionizing radiation. The mean age at diagnosis was 14.5 years. There were 8 females; all patients presented with a cervical mass. Ten patients underwent total thyroidectomy and three subtotal thyroidectomy. Eight patients (61.5%) had regional lymph node metastases, only one of whom (7.6%) had lung metastases. All received radioactive "131I" ablation postsurgical excision. Five (38.5%) had recurrences and needed multiple surgeries and/or "131I" ablation. At a median follow-up time of 8.3 years all patients are disease free. Conclusion: Pediatric thyroid cancer in Lebanon is a rare tumor that presents mainly as a primary malignancy. The main clinical presentation is a cervical mass with locoregional lymph nodes metastasis. The mainstay of therapy is total thyroidectomy with lymph node dissection when indicated and radioactive "131I" ablation. Despite recurrences the prognosis is excellent.
Pediatric Hematology and Oncology 01/2009; 26(6):339-447. · 0.89 Impact Factor
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Pediatric Blood & Cancer 11/2008; 52(2):308. · 1.89 Impact Factor
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Journal of Pediatric Hematology/Oncology 05/2003; 25(4):344-5. · 1.16 Impact Factor
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ABSTRACT: Imatinib mesylate treatment is highly effective in chronic myeloid leukaemia and recent data have suggested that imatinib mesylate is also effective in the treatment of idiopathic hypereosinophilic syndrome (HES). Six patients with HES were treated daily with 100 mg imatinib mesylate. Five patients had normal karyotype and one showed trisomy 8. RT-PCR was negative for ETV6-PDGFRB and BCR-ABL fusion mRNAs. All patients rapidly achieved complete haematological remission. One patient remained in remission for more than 6 weeks after discontinuing treatment. No significant side effect was noted. Imatinib mesylate should be considered in the first-line therapy of idiopathic HES.
The Hematology Journal 02/2003; 4(6):410-2. · 1.86 Impact Factor
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ABSTRACT: To describe the imaging findings of abdominal complications caused by chemotherapy in pediatric cancer patients.
Radiology studies of 243 patients treated in our Children Cancer Center were reviewed, 164 of whom had abdominal studies. Medical records of 54 patients with abnormal imaging findings were studied.
Ultrasound and/or CT findings showed the following complications: (1) gallbladder sludge/crystals (n=13), gallstones (n=8), cholecystitis (n=3); (2) liver steatosis (n=23), siderosis (n=1), veno-occlusive disease (n=2); (3) pancreatitis (n=7); (4) typhlitis (n=12), esophagitis (n=2).
Awareness of these complications is essential for appropriate management, decreasing their mortality and morbidity.
Clinical imaging 33(4):253-60. · 0.73 Impact Factor
