ABSTRACT
Nevoid basal cell carcinoma (NBCC) syndrome is an autosomal dominant disorder characterized by distinctive congenital malformations and a variety of benign and malignant neoplasms, including ovarian fibromas. We describe pathologic and cytogenetic findings in a large unilateral ovarian fibroma from a 12-year-old female with NBCC syndrome. The pathologic findings were characteristic for ovarian fibroma, but were unusual for the ovarian fibromas associated with NBCC syndrome because of the absence of calcification, the lack of bilaterality, and the presence of focal hypercellularity. The karyotype of tumor tissue showed complex numerical and structural abnormalities. Although there is frequent loss of heterozygosity of 9q22.3 and mutations in the PTCHgene in Gorlin syndrome, the ovarian fibroma in this case did not have cytogenetically detectable abnormalities of chromosome 9.
Subject(s)
Basal Cell Nevus Syndrome/genetics , Fibroma/genetics , Karyotyping , Ovarian Neoplasms/genetics , Child , Female , Fibroma/complications , Fibroma/pathology , Humans , Ovarian Neoplasms/complications , Ovarian Neoplasms/pathologyABSTRACT
BACKGROUND: Second-look surgery after therapy for rhabdomyosarcoma (RMS) may yield prognostic information regarding tumor responsiveness to treatment. Favorable outcome is suggested by tumor cells which have undergone maturation (cytodifferentiation). PROCEDURE: Specimens from patients treated on Intergroup RMS Study-IV (IRSG-IV) were studied before and after treatment. All patients received chemotherapy and most received radiation therapy. Post-treatment specimens were graded according to the quantity of tumor showing cytodifferentiation (0 = absent, 1 = mild, 2 = moderate, 3 = extensive). Proliferative activity by MIB-1, topoisomerase II-alpha, and p53 protein expression were measured. RESULTS: 19/31 cases from IRSG-IV were adequate for analysis. Six out of nineteen patients failed therapy within 1.3 years of treatment. Grade 3 cytodifferentiation was present in 10 cases (2 BRMS, 8 ERMS); none failed therapy. Grade 2 cytodifferentiation was present in 5 cases (1 ERMS, 2BRMS, 2ARMS); 2 patients with ARMS failed therapy. Grade 0-1 cytodifferentiation was present in 4 cases (1 ERMS and 3 ARMS); all failed therapy. Proliferative activity by MIB-1 and topoisomerase II-alpha immunohistochemistry decreased or was unchanged after treatment for all ERMS/BRMS, and 4/5 cases of ARMS. p53 immunohistochemistry showed no consistent pattern of reactivity. Sparse persistent tumor cells were present in 9/10 ERMS, 3/4 BRMS, 5/5 ARMS. CONCLUSIONS: Extensive cytodifferentiation is more commonly seen in ERMS/BRMS compared with less evidence for cytodifferentiation in ARMS suggesting fundamentally different mechanisms of cellular response to therapy in RMS. Sparse persistent tumor cells in post treatment ERMS/BRMS specimens does not appear to affect outcome.
Subject(s)
Cell Transformation, Neoplastic , Rhabdomyosarcoma/pathology , Rhabdomyosarcoma/therapy , Adolescent , Antigens, Neoplasm/analysis , Antigens, Nuclear , Biomarkers, Tumor/analysis , Cell Division , Child , Child, Preschool , Combined Modality Therapy , DNA Topoisomerases, Type II/analysis , DNA-Binding Proteins , Female , Humans , Immunohistochemistry , Infant , Ki-67 Antigen , Male , Nuclear Proteins/analysis , Prognosis , Rhabdomyosarcoma/chemistry , Rhabdomyosarcoma, Alveolar/chemistry , Rhabdomyosarcoma, Alveolar/pathology , Rhabdomyosarcoma, Alveolar/therapy , Rhabdomyosarcoma, Embryonal/chemistry , Rhabdomyosarcoma, Embryonal/pathology , Rhabdomyosarcoma, Embryonal/therapy , Treatment Failure , Tumor Suppressor Protein p53/analysisABSTRACT
Radiation therapy to the cranial-spinal axis is typically targeted to the spinal cord and to the cerebrospinal fluid (CSF) in the subarachnoid space adjacent to the spinal cord and brain. Standard techniques employed in the treatment of the whole central nervous system do little to compensate for the varying depths of spinal cord along the length of the spinal field. Lateral simulation films, sagittal magnetic resonance imaging (MRI), or computerized tomography (CT) are used to estimate an average prescription depth for treatment along the spine field. However, due to the varying depth of the target along the spinal axis, even with the use of physical compensators, there can be considerable dose inhomogeneity along the spine field. With the advent of treatment machines that have full dynamic capabilities, a technique has been devised that will allow for more conformal dose distribution along the full length of the spinal field. This project simulates this technique utilizing computer-controlled couch motion to deliver multiple small electron beams of differing energies and intensities. CT planning determines target depth along the entire spine volume. The ability to conform dose along the complete length of the treatment field is investigated through the application of superpositioning of the fields as energies and intensities change. The positioning of each beam is registered with the treatment couch dynamic motion. This allows for I setup in the treatment room rather than multiple setups for each treatment position, which would have been previously required. Dose-volume histograms are utilized to evaluate the dose delivered to structures in the beam exit region. This technique will allow for precise localization and delivery of a homogeneous dose to the entire CSF space.
