ABSTRACT
Stiff-person syndrome (formerly known as stiff-man syndrome) is a very rare autoimmune and neurogenic disorder, thought to present as a paraneoplastic variant in association with thymoma. Pure red blood cell aplasia is also a paraneoplastic disorder associated with thymoma. Although separate cases of stiff-person syndrome and pure red blood cell aplasia have been reported, we describe here what is to our knowledge the first case of recurrent thymoma with both stiff-person syndrome and pure red blood cell aplasia. We describe the successful treatment of the neurogenic symptoms of stiff-person syndrome and the progressive anemia associated with pure red blood cell aplasia by tumor excision.
Subject(s)
Neoplasm Recurrence, Local/surgery , Paraneoplastic Syndromes/surgery , Red-Cell Aplasia, Pure/diagnosis , Stiff-Person Syndrome/diagnosis , Thymoma/surgery , Thymus Neoplasms/surgery , Aged, 80 and over , Female , Follow-Up Studies , Humans , Neoplasm Recurrence, Local/diagnosis , Paraneoplastic Syndromes/diagnosis , Red-Cell Aplasia, Pure/complications , Red-Cell Aplasia, Pure/surgery , Reoperation/methods , Risk Assessment , Stiff-Person Syndrome/complications , Stiff-Person Syndrome/surgery , Thymectomy/methods , Thymoma/diagnosis , Thymus Neoplasms/diagnosis , Time Factors , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
Lung cancer accounts for the largest number of new cases of cancer deaths annually. The treatment of locally advanced non-small-cell lung cancer(NSCLC)will continue to be a problem for many years. In particular, the border-zone subset of stage III A(N2)patients, which lies between the generally resectable stage I and II tumors and the unresectable stage III B patients, has been the subject of a wide variety of clinical trials incorporating various combinations of chemotherapy, radiotherapy, and surgery.What is the ideal therapy for stage III A(N2)patients ? is a controversial question, and the role of surgery is not clearly defined because of its heterogeneous nature. Most importantly, treatment decisions for these patients should be dictated by the stage of the patients' disease and the patients' performance status, medical comorbidities, and preferences. At our hospital, therefore, all of these patients' data are discussed at our cancer-board conference, incorporating the options of thoracic surgeons, medical oncologists, and radiation oncologists to determine the optimal prospective treatment strategies for the patients. We focused on a treatment strategy for the patients with the so called marginally resectable' lung cancer in this article.
Subject(s)
Lung Neoplasms/surgery , Combined Modality Therapy , Humans , Lung Neoplasms/diagnosis , Lung Neoplasms/pathology , Lung Neoplasms/therapy , Lymphatic Metastasis , Neoplasm Staging , Survival RateABSTRACT
The Group-II/III WW domains bind Pro-rich sequences, the most frequent protein motif found in eucaryotic genomes. We have proposed that the Group-II and -III WW domains be merged into a larger group because the members of each group have relatively wide specificity and bind to the common ligands [Kato et al., J Biol Chem 2004;279:31833-31841]. We have also proposed that Group-II/III has a common surface patch, the XP2 groove, to bind the ligands. The first WW domain of FBP11/HYPA is one of the Group-II/III WW domains. The solution structure of the 26 residue-long converged region exhibits an antiparallel triple stranded beta-sheet with a small hydrophobic core. The WW domain of FBP11/HYPA has both XP and XP2 grooves on its surface. Ligand titration by 1H-15N HSQC NMR spectra revealed that the WW domain of FBP11/HYPA binds all the peptides with the PL, PP, and PR motifs. The profile patterns of chemical shift perturbation were quite similar among the spectra titrated with all three ligands. In addition, the titration significantly shifts the signals of the residues that compose the XP2 groove. All these findings suggest the functional importance of the XP2 groove and group definition of Group-II/III of the WW domains.
