Imatinib mesylate in chordoma

BACKGROUND: To the authors' knowledge, no effective medical therapy currently is available for advanced chordoma. Imatinib mesylate is a tyrosine kinase inhibitor targeting platelet-derived growth factor receptor-beta (PDGFRB), BCR-ABL, and KIT. METHODS: Six patients with advanced chordoma were treated with imatinib mesylate at a dose of 800 mg daily. In all patients, the tumor was found to be positive for PDGFRB, and in four patients PDGFRB was shown to be phosphorylated/expressed. RESULTS: After a treatment period of > or = 1 year, overt tumor liquefaction was evident on computed tomography (CT) scan in the first patient. In previous months, a decrease in contrast enhancement on magnetic resonance imaging (MRI) and a decrease in glucose uptake on positron emission tomography (PET) were detected. Similar signs on MRI and PET were observed in subsequent patients, who had a shorter treatment period. One of these patients initially was removed from therapy and then was readmitted to therapy because of difficulties with regard to tumor response assessment; 1 month after the reinitiation of therapy, an overt decrease in tumor density was visible on CT scan in this patient. In four of five symptomatic patients, a subjective improvement was observed early in the course of treatment. The first patient died after 17 months, with a sizeable, mostly liquefied mass. Another patient died early, apparently of unrelated causes. The remaining patients were on therapy at the time of last follow-up. CONCLUSIONS: Imatinib mesylate has been found to have antitumor activity in patients with chordoma. This activity might be mediated by inactivation of PDGFRB. Tumor response manifests through patterns that are similar to those observed in patients with gastrointestinal stromal tumors who respond to molecular-targeted therapy, but evolves more slowly. The benefit to the patient entailed by this pattern of tumor response in chordoma needs to be elucidated, but may be limited in the presence of significant local disease.     

Unusual breast tumors: primary lymphoma. A case report

An 82-year-old woman presented with a right breast lump with erythematous reaction of the overlying skin and corresponding lymph nodes. Cytological examination of the breast lesion and lymph nodes suggested a lymphoid proliferation. Frozen section revealed carcinoma with lymphoid stroma. Simple mastectomy was performed because of the extent of the lesion. Histological diagnosis was non-Hodgkin type B large-cell lymphoma. Primary breast lymphomas behave similarly to lymphomas of similar histologic types occurring in other sites. The authors illustrate the diagnostic difficulties and the usefulness of complementary techniques in the diagnosis of an unusual breast mass.     

Early rehabilitation of facial nerve deficit after acoustic neuroma surgery

OBJECTIVE: To determine whether an early physical rehabilitative program could improve and/or accelerate recovery from a postoperative deficit of facial nerve (FN) function. MATERIAL AND METHODS: A retrospective study of the charts of patients who presented a postoperative FN deficit after surgery for acoustic neuroma (AN) was carried out. Twenty-nine patients were enrolled and divided into 2 groups: 18 who underwent early physical rehabilitation and 11 who did not undergo rehabilitation. All the AN patients underwent translabyrinthine removal and were classified preoperatively according to the House-Brackmann staging system. Physical rehabilitation was performed according to Kabat (i.e. neuromuscular facilitation). FN function was assessed postoperatively and classified according to the House-Brackmann grading system. RESULTS: In Grade IV and V patients, early rehabilitation allowed a faster and better recovery with respect to AN patients for whom rehabilitation was not carried out. CONCLUSION: Early physical rehabilitation has proved to be effective as a helpful tool for recovery from FN deficit and it is therefore advisable to use it soon after surgery, especially for FN deficits worse than Grade IV.