ABSTRACT
CASE: A thirty-one-year-old woman presented with symptoms of worsening left knee and thigh pain. Radiographs and magnetic resonance imaging demonstrated extensive lytic and cystic changes throughout the femur. A biopsy demonstrated necrosis, chronic granulomatous inflammation, and laminations suggestive of an echinococcal cyst. Serology confirmed an Echinococcus granulosus infection. Treatment with anthelmintic agents was initiated, but reconstruction with a total femoral endoprosthesis was implemented as definitive management. The patient tolerated surgery well and returned for a one-year postoperative visit without evidence of recurrence of infection. CONCLUSION: Osseous hydatidosis is a rare disease, but it should be included in the differential diagnosis of patients with extensive destructive bone processes.
ABSTRACT
Segmental neurofibromatosis (NF-5) is an extremely rare variant of neurofibromatosis involving a single extremity without pathologic features beyond the midline. A case of segmental neurofibromatosis involving the sciatic nerve and its branches is presented with a detailed description of the patient's preoperative findings plus postoperative course through 1-year follow-up. Clinical, histologic, and genetic findings are given along with a brief review of the literature on segmental neurofibromatosis. Last, treatment options and postoperative care recommendations are provided.
Subject(s)
Neurofibromatoses/pathology , Peripheral Nervous System Neoplasms/pathology , Sciatic Nerve/pathology , Adult , Disease-Free Survival , Female , Humans , Neurofibromatoses/physiopathology , Neurofibromatoses/surgery , Peripheral Nervous System Neoplasms/physiopathology , Peripheral Nervous System Neoplasms/surgery , S100 Proteins/metabolism , Schwann Cells/metabolism , Sciatic Nerve/surgeryABSTRACT
Primary bone diffuse large B-cell lymphomas (PB-DLBCL) are uncommon extranodal lymphomas. Herein, we report the clinical, pathologic, immunohistochemical, and molecular features of 21 cases of PB-DLBCL. The mean age of the patients was 54 years (range: 13 to 85 y). The male and female ratio was 1.6:1. The tumors consisted of diffuse sheets of large atypical cells or a polymorphous mixture of small-to-large cells with large multilobated nuclei, fine chromatin, and inconspicuous to prominent nucleoli. Twelve (57%) cases were non-germinal center B (GCB) and 9 (43%) were GCB subtype based on immunohistochemical classification. B-cell lymphomas (BCL)-2 was positive in 17/21 (81%), TP53 in 11/21 (52%) positive and the mean MIB-1 index was 57%. Polymerase chain reaction showed 10 cases with immunoglobulin heavy-chain (IGH) and 4 cases with IGH/BCL-2 gene rearrangement. The fluorescence in-situ hybridization analyses showed 14% of cases with BCL-6, 19% of cases with BCL-2, and 9% of cases with C-MYC gene rearrangement. Age <60 years and complete response to initial treatment were significant predictors of survival outcome (P< or =0.05). Even though no association was observed between the subtype of PB-DLBCL (GCB vs. non-GCB), BCL2, TP53, MIB1 index and overall survival (P>0.05), due to small sample size, and variability in treatment received, this analysis may be interpreted with caution.
Subject(s)
Biomarkers, Tumor/analysis , Bone Neoplasms/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , Bone Neoplasms/genetics , Bone Neoplasms/mortality , DNA-Binding Proteins/genetics , Female , Genes, bcl-2 , Genes, myc , Humans , Immunohistochemistry , In Situ Hybridization, Fluorescence , Ki-67 Antigen/genetics , Lymphoma, Large B-Cell, Diffuse/genetics , Lymphoma, Large B-Cell, Diffuse/mortality , Male , Middle Aged , Polymerase Chain Reaction , Prognosis , Proto-Oncogene Proteins c-bcl-6 , Translocation, Genetic , Tumor Suppressor Protein p53/genetics , Young AdultABSTRACT
Primary bone lymphoma is an uncommon malignancy that accounts for less than 5% of all primary bone tumors. The radiographic appearances of primary bone lymphoma are variable, and, because the lesion can appear near normal on plain radiographs, a second modality such as bone scintigraphy or magnetic resonance (MR) imaging should be used. Despite this variability, the presence of a solitary, permeative, metadiaphyseal lesion with a layered periosteal reaction on plain radiographs and a soft-tissue mass on MR images, especially in a patient older than 30 years, is highly suggestive of lymphoma. The case for a diagnosis of primary bone lymphoma is further strengthened if the soft-tissue mass and marrow changes are associated with surprisingly little cortical destruction. Primary bone lymphoma has a better prognosis than many other malignant bone tumors; therefore, early identification allows for appropriate treatment. MR imaging not only permits early identification but also depicts the extent of soft-tissue involvement and can be used to assess the outcome of treatment.
