Hypertrophic osteoarthropathy is characterized by clubbing of the digital tips and periosteal reaction of long bones. Most of the cases are associated with malignancy or other conditions such as congenital heart disease, liver cirrhosis, pulmonary fibrosis, biliary atresia, and gastrointestinal polyps. Hypertrophic osteoarthropathy associated with malignancy is rare in children. A few cases of hypertrophic osteoarthropathy in children with nasopharyngeal carcinoma have been reported, however, there has been no report of such case in Korea. We present a case of hypertrophic osteoarthropathy associated with nasopharyngeal carcinoma with lung metastasis in a 14-yr-old boy. In this case, hypertrophic osteoarthropathy regressed after intensive chemotherapy, but subsequently the patient died of progressive lung metastasis.
Adolescent ; Bone and Bones/radiography ; Carcinoma/*diagnosis/mortality ; Disease Progression ; Fatal Outcome ; Human ; Joint Diseases/pathology ; Lung Neoplasms/mortality/*secondary ; Male ; Nasopharyngeal Neoplasms/*diagnosis/mortality ; Neoplasm Metastasis ; Osteoarthropathy, Primary Hypertrophic/*diagnosis/mortality/radiography ; Prognosis

BACKGROUND: Nasopharyngeal carcinoma (NPC) is very rare in children and adolescents. The aim of this study was to evaluate clinical characteristics and treatment outcomes of pediatric NPC.METHODS: Medical records of 9 patients treated for NPC at the Seoul National University Children's Hospital between 1988 and 2012 were analyzed retrospectively.RESULTS: The median age at diagnosis was 11 years (range, 9-13 years). One patient had stage II disease, 3 had stage III disease, and 5 had stage IV disease. The histologic subtypes were undifferentiated carcinoma and squamous cell carcinoma in 7 and 2 patients, respectively. All patients were initially treated with cisplatin (100 mg/m2 intravenous [IV] every 4 weeks for 4-6 months), bleomycin (15 unit/m2 IV every 1 weekx7), and fluorouracil (1,000 mg/m2 IV every 4 weeks for 1 year). Eight patients received radiotherapy with doses of 45-59.4 Gy at the primary site and neck nodes. Seven patients (77.8%) achieved complete remission, 1 (11.1%) achieved partial remission, and 1 (11.1%) showed disease progression. Six patients developed fluorouracil-related neurotoxicity; the regimen was changed to cisplatin, epirubicin, and bleomycin in five of the 6 patients. One patient died of progressive disease without responding to treatment. Treatment-related mortality occurred in 1 patient owing to septic shock. Secondary osteosarcoma developed in 1 patient 6 years after treatment. The overall survival was 77.8%, with a median follow-up of 40.8 months (range, 4.5-287.6 months).CONCLUSION: Children and adolescents with advanced NPC treated with combined chemotherapy and radiotherapy have a good survival rate.
Adolescent ; Bleomycin ; Carcinoma ; Carcinoma, Squamous Cell ; Chemoradiotherapy ; Child ; Cisplatin ; Diagnosis ; Disease Progression ; Drug Therapy ; Epirubicin ; Fluorouracil ; Follow-Up Studies ; Humans ; Korea ; Medical Records ; Mortality ; Nasopharyngeal Neoplasms ; Neck ; Osteosarcoma ; Pediatrics ; Radiotherapy ; Retrospective Studies ; Seoul ; Shock, Septic ; Survival Rate

OBJECTIVETo evaluate the cost-effectiveness of different screening strategies for nasopharyngeal carcinoma (NPC) and recommend a preferable NPC screening strategy.

METHODSA Markov simulation model was constructed based on the natural history of NPC. Seven strategies (A. Annual screening; B. Annual screening for (Epstein-Barr virus, EBV) EBV-seropositive subjects, triennial screening for seronegative subjects; C. Biennial screening; D. Triennial screening; E. 4-year screening; F. 5-year screening; G. 6-year screening) were evaluated. The NPC-pickup rate, cost, quality-adjusted life-years (QALYs) and incremental cost-effectiveness ratio (ICER) were calculated.

RESULTSThe ICERs of the 7 strategies were 83 111.6, 47 768.9, 50 164.7, 40 016.2, 34 272.8, 32 215.6, and 32 248.0 Yuan/QALY, respectively. The discounted QALYs of the strategies were 23 079.9, 22 955.6, 22 810.4, 22 636.5, 22 522.7, 22 445.0, and 22 361.9 years, respectively. The ICERs of the strategies were less than three times of the average per capita gross domestic product (89 976 Yuan) in China in 2010. The strategy A achieved a highest NPC pick-up rate (81.7%), a highest discounted QALY and a smallest number of NPC death (681), but a highest discounted cost and a greatest ICER. Compared with the strategy A, the strategy B achieved a little smaller NPC pick-up rate (73.1%), a little smaller number of NPC death (707), however, the ICER of the strategy B decreased by 38.2%.

CONCLUSIONThe strategy B (annual screening for EB virus seropositive subjects and triennial screening for seronegative subjects) is a preferable option for NPC screening.

Adult ; Carcinoma ; China ; epidemiology ; Cost-Benefit Analysis ; Early Detection of Cancer ; economics ; methods ; Epstein-Barr Virus Infections ; diagnosis ; Female ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Male ; Markov Chains ; Mass Screening ; economics ; methods ; Middle Aged ; Nasopharyngeal Neoplasms ; diagnosis ; mortality ; Quality-Adjusted Life Years ; Survival Rate