Oncology

Strategy for treatment of isolated contralateral supraclavicular recurrence in patient with breast cancer after sentinel lymph node biopsy without axillary lymph node dissection
Isolated ipsilateral supraclavicular (SC) lymph node recurrences of breast cancer are rare. SC recurrence is generally predictive of significant distant recurrence and rather poor prognosis. However, a curative course of systemic chemotherapy and local irradiation can be effective for patients with isolated SC recurrence, since the outcomes of patients thus treated are better than those of hist...


Predicting Operative Bleeding in Elective Pediatric Surgeries Using the Pediatric Bleeding Questionnaire (PBQ)
To test the Pediatric Bleeding Questionnaire’s (PBQ) utility for predicting excessive bleeding during elective surgery, we retrospectively evaluated 60 pediatric patients who had been preoperatively investigated for von Willebrand disease. Of 58 patients with bleeding scores (BS) ≤2 (within normal range), 1 hemorrhaged during surgery. Two had a BS of >2 (positive BS); however, neither bled ...
Chediak-Higashi Syndrome: Novel Mutation of the CHS1/LYST Gene in 3 Omani Patients
Chediak-Higashi syndrome (CHS) is a rare, autosomal, recessive lysosomal disorder with hematological and immunologic abnormalities; however, stem-cell transplantation from a matched or related donor may be curative. Many mutations of the CHS1/LYST gene have been reported to date. We report a novel nonsense mutation of the CHS1/LYST gene in 3 Omani patients. Methods and Three patients from 2 dif...


Hemophagocytic Lymphohistiocytosis Associated With Parechovirus 3 Infection
Hemophagocytic lymphohistiocytosis (HLH) denotes the common final pathway of a potentially fatal hyperinflammatory condition of diverse etiologies. We describe the first case of documented HLH associated with human parechovirus 3. A monoallelic Ala91Val mutation was found in the PRF1 gene, but the contribution of this mutation to HLH remains controversial. The diagnosis, based on accepted crite...
High-grade Transitional Cell Carcinoma of the Bladder in a 5-Year-Old Boy Successfully Treated With Partial Cystectomy and Intravesical Bacillus Calmette-Guerin
Pediatric transitional cell carcinomas of the bladder are typically characterized by low-grade histology, adolescent and young adult age, and cure with surgical resection. Here, we report a high-grade transitional cell carcinoma of the bladder in a 5-year-old boy treated with a partial cystectomy and adjuvant intravesical Bacillus Calmette-Guerin.


Cisplatin-induced Hearing Loss: The Need for a Long-term Evaluating System
Cisplatin is an effective chemotherapeutic agent against pediatric cancers; however, ototoxicity is a concern. This study describes the frequency, severity, and clinical course of hearing loss in Japanese pediatric patients treated with cisplatin-based multimodal therapy. A total of 55 children who received cisplatin-based therapy from 1983 to 2012 underwent audiologic evaluations. Data were an...
Open-label Bendamustine Monotherapy for Pediatric Patients With Relapsed or Refractory Acute Leukemia: Efficacy and Tolerability
This open-label, single-arm, phase I/II, dose-escalation study was designed to determine the recommended phase II dose (RP2D), pharmacokinetics, tolerability, and efficacy of bendamustine in pediatric patients (age ranging from 1 to 20 y) with histologically proven relapsed/refractory acute lymphoblastic leukemia (ALL) or acute myeloid leukemia (AML). Patients (27 with ALL, 16 with AML) receive...

Taurolidine Specifically Inhibits Growth of Neuroblastoma Cell Lines In Vitro
Neuroblastoma is a common pediatric solid tumor with poor outcome for metastatic disease. Thus, novel therapeutic options are of main interest. The anti-neoplastic properties of taurolidine have been demonstrated on a variety of human cancer cells. However, data on neuroblastoma is lacking. Therefore, our aim was to evaluate the effect of taurolidine on growth of neuroblastoma cell lines. :Neur...
Both Hemophilia Health Care Providers and Hemophilia A Carriers Report That Carriers Have Excessive Bleeding
Hemophilia A, the result of reduced factor VIII activity, is an X-linked recessive bleeding disorder. Previous reports of hemophilia A carriers suggest an increased bleeding tendency. Our objective was to determine the attitudes and understanding of the hemophilia A carrier bleeding phenotype, and opinions regarding timing of carrier testing from the perspective of both medical providers and af...

Dermatopathic Lymphadenitis Associated With Human Papilloma Virus Infection and Verruca Vulgaris
Here we present a pediatric case of human papilloma virus associated with dermatopathic lymphadenitis (DL). A 5-year-old boy presented to the pediatric oncology clinic with swelling of the neck and warts on his lower jaw. His blood chemistry and complete blood count were normal, as was chest x-ray. HIV, EBV, CMV, and parvovirus serologies were negative. The patient was investigated for malignan...
A Pilot Study for the Detection of FLT3-ITD Mutation Frequency in 25 Iraqi Patients With Acute Lymphoid Leukemia in 2 Iraqi Hospitals
This study was conducted to evaluate the frequency of FLT3-ITD mutation in acute lymphoid leukemia (ALL) patients using conventional polymerase chain reaction (PCR) in Iraq, and to correlate this mutation with prognostic parameters of patients. A total of 25 individuals were enrolled (16 children with ALL and 9 adult with ALL), attending 2 teaching hospitals in Baghdad. Hematological investigat...

Liver Failure Due to Hepatic Angiosarcoma in an Adolescent With Dyskeratosis Congenita
Dyskeratosis congenita (DC) is a multisystem disease caused by genetic mutations that result in defective telomere maintenance. Herein, we describe a 17-year-old patient with severe DC, manifested by bone marrow failure, severe immunodeficiency, and enterocolitis requiring prolonged infliximab therapy, who developed fatal hepatic failure caused by an aggressive, infiltrating hepatic angiosarcom...
Pancytopenia After Allogeneic Bone Marrow Transplant Due to Copper Deficiency
Pancytopenia occurring 1 year or later after allogeneic bone marrow transplantation typically prompts a primary consideration for relapse. We present the case of a 15-year old-girl who underwent transplantation for therapy-related myelodysplasia secondary to Ewing sarcoma treatment who developed pancytopenia with myelodysplasia 1 year after transplant due to copper deficiency. Copper deficiency...
Myelofibrosis, Hepatic Extramedullary Hematopoiesis and Ascites Associated With Vitamin D Deficiency in Early Infancy
Primary myelofibrosis is rare in children. Many causes have been described for secondary myelofibrosis including vitamin D deficiency. Here, we describe a patient with myelofibrosis secondary to vitamin D deficiency, as diagnosed by laboratory evidence. The patient also developed resultant extramedullary hematopoiesis with secondary development of ascites as a result of myelofibrosis. These are...
Dyskeratosis Congenita Complicated by Hepatic Fibrosis With Hepatic Vein Thrombosis
We describe the case of 2 male siblings with dyskeratosis congenita (DC). Extensive genetic analysis failed to identify a causative genetic abnormality. The elder brother developed hepatic fibrosis accompanied with hepatic vein thrombosis at the age of 9 years. Recent studies have found that patients with DC sometimes develop hepatic complications, including cirrhosis. However, little is known ...