ABSTRACT
PURPOSE: The authors report their experience with doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations (LMs) in children. METHODS: A retrospective chart review was used to collect data on 11 patients treated with doxycycline sclerotherapy for LMs of the head and neck at our institution since 2003. Radiographic imaging allowed classification of patient LM as macrocystic, microcystic, or mixed according to previously published guidelines. Only patients with macrocystic or mixed lesions were offered doxycycline sclerotherapy. Radiographic imaging and physical examination were used to determine efficacy of treatment. After each treatment, the clinical and radiographic response was characterized as excellent (> or = 95% decrease in lesion size), satisfactory (> or = 50% decrease in volume and asymptomatic), or poor (< 50% decrease in volume or symptomatic). RESULTS: Eleven patients underwent a total of 23 sclerotherapies with an average of 2 treatments per patient (range, 1-4). All 7 patients with macrocystic lesions achieved complete clinical resolution with an average radiographic resolution of 93%. The 4 patients with mixed lesions achieved only partial clinical resolution and an average of 73% radiographic resolution. No patient experienced any adverse effects related to the treatment. At a median follow-up of 8 months, 2 patients (18%) experienced lesion recurrence in the setting of concomitant infection. CONCLUSION: Doxycycline sclerotherapy is safe and effective as a primary treatment modality for macrocystic and mixed LMs of the head and neck in the pediatric population.
Subject(s)
Doxycycline/therapeutic use , Lymphatic Abnormalities/diagnosis , Lymphatic Abnormalities/therapy , Sclerotherapy/methods , Ambulatory Care , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Follow-Up Studies , Head , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging , Male , Neck , Physical Examination , Retrospective Studies , Treatment OutcomeABSTRACT
PURPOSE: To evaluate the potential influence of radiotherapy quality on survival in high-risk pediatric medulloblastoma patients. METHODS AND MATERIALS: Trial 9031 of the Pediatric Oncology Group (POG) aimed to study the relative benefit of cisplatin and etoposide randomization of high-risk patients with medulloblastoma to preradiotherapy vs. postradiotherapy treatment. Two-hundred and ten patients were treated according to protocol guidelines and were eligible for the present analysis. Treatment volume (whole brain, spine, posterior fossa, and primary tumor bed) and dose prescription deviations were assessed for each patient. An analysis of first site of failure was undertaken. Event-free and overall survival rates were calculated. A log-rank test was used to determine the significance of potential survival differences between patients with and without major deviations in the radiotherapy procedure. RESULTS: Of 160 patients who were fully evaluable for all treatment quality parameters, 91 (57%) had 1 or more major deviations in their treatment schedule. Major deviations by treatment site were brain (26%), spinal (7%), posterior fossa (40%), and primary tumor bed (17%). Major treatment volume or total dose deviations did not significantly influence overall and event-free survival. CONCLUSIONS: Despite major treatment deviations in more than half of fully evaluable patients, underdosage or treatment volume misses were not associated with a worse event-free or overall survival.
Subject(s)
Medulloblastoma/radiotherapy , Spinal Neoplasms/radiotherapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Child , Cisplatin/administration & dosage , Cranial Irradiation/methods , Etoposide/administration & dosage , Humans , Infratentorial Neoplasms/drug therapy , Infratentorial Neoplasms/mortality , Infratentorial Neoplasms/radiotherapy , Medulloblastoma/drug therapy , Medulloblastoma/mortality , Quality Assurance, Health Care , Radiotherapy/standards , Spinal Neoplasms/drug therapy , Spinal Neoplasms/mortality , Survival RateABSTRACT
B5 fixation achieves superior morphologic detail. However, environmental concerns have led to labor-intensive and costly requirements for disposal of mercury-containing fixatives. We performed a blinded prospective study to find a safe, mercury-free alternative to B5. Morphology was evaluated with 6 fixatives, including B5, in a blinded fashion. Acetic acid-zinc-formalin (AZF) was selected for further evaluation of immunohistochemistry, in situ hybridization, and molecular analysis. AZF fixation resulted in overall staining and morphologic detail comparable to B5 and achieved equivalent or superior antigen preservation for immunohistochemical studies. Strong signal intensity was achieved with in situ hybridization, and DNA amplification could be successfully performed. AZF allows greater flexibility in fixation times, decreases decalcification time, and eliminates labor-intensive steps required for B5 processing.
Subject(s)
Acetic Acid , Fixatives , Formaldehyde , Mercury Compounds , Tissue Fixation/methods , Zinc , Humans , Immunohistochemistry , In Situ HybridizationABSTRACT
Lateral meningoceles were first described by Lehman et al. [(1977); J Pediatr 90: 49-54] in a patient with other skeletal findings and distinctive craniofacial features. Subsequently, six more patients with the so-called lateral meningocele syndrome (LMS) have been reported. We describe the findings in three new cases and expand the phenotype. The existence of an affected mother and daughter supports the hypothesis that LMS is a dominant disorder affecting primarily the connective tissue.
