Nora's lesion. Clinical and therapeutic considerations

In this article we report a case of bizarre paraosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, arising on the distal phalanx of the fifth finger of the hand in a young-adult subject. In this paper, we discuss the possible therapeutic programme related to the grade of severity of the clinical features and underline the rarity of this lesion and the difficulty of diagnosis, which is exclusively histological.     

Preservative surgery of the kidney for haemorrhagic angiomyolipoma: a report of two cases

Renal angiomyolipomas are very rare benign tumours (3% of renal tumours) that may present as isolated tumours or tumours associated with other pathologies, particularly tuberous sclerosis (40%), neurofibro-matosis, or Sturge-Webers disease. Clinically, renal angiomyolipoma is asymptomatic until the tumour becomes larger than 4 cm, causing urinary symptoms such as pain, infection, and microhaematuria. Rarely, in cases of large hypervascularised lesions, the clinical picture at onset may consist in spontaneous haemorrhage due to vessel rupture. The therapy consists in non-operative treatments for small tumours (< 4 cm) and surgical treatment, probably preservative, for larger tumours. In complicated haemorrhagic cases, an angiographic approach or surgical treatments, possibly conservative, are possible, offering the opportunity for further elective treatment. On the basis of case reports starting with haemorrhage and treated in urgency and of a review of the literature, we conclude that it is possible and mandatory to perform emergency preservative treatments of the kidney with an angiographic or surgical approach, and to ensure haemostasis. Treatment of the disease can be postponed when clinical and environmental conditions are better. Furthermore, we stress the need, once the clinical urgency/emergency is over, to submit the patient to suitable examinations to detect possible associated pathologies (tuberous sclerosis, neurofibromatosis, Sturge-Webers disease) in other parts of the body.     

Rotational Position of Femoral and Tibial Components in TKA Using the Femoral Transepicondylar Axis

Proper femoral and tibial component rotational positioning in TKA is critical for outcomes. Several rotational landmarks are frequently used with different advantages and limitations. We wondered whether coronal axes in the tibia and femur based on the transepicondylar axis in the femur would correlate with anteroposterior deformity. We obtained computed tomography scans of 100 patients with arthritis before they underwent TKA. We measured the posterior condylar angle on the femoral side and the angle between Akagi’s line and perpendicular to the projection of the femoral transepicondylar axis on the tibial side. On the femoral side, we found a linear relationship between the posterior condylar angle and coronal deformity with valgus knees having a larger angle than varus knees, ie, gradual external rotation increased with increased coronal deformity from varus to valgus. On the tibial side, the angle between Akagi’s line and the perpendicular line to the femoral transepicondylar axis was on average approximately 0°, but we observed substantial interindividual variability without any relationship to gender or deformity. A preoperative computed tomography scan was a useful, simple, and relatively inexpensive tool to identify relevant anatomy and to adjust rotational positioning. We do not, however, recommend routine use because on the femoral side, we found a relationship between rotational landmarks and coronal deformity. Each author certifies that he or she has no commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article. Each author certifies that his or her institution has approved the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research.     

Management of AIDS-related Kaposi's sarcoma

The advent of highly active antiretroviral therapy (HAART) has lead to a substantial reduction in the prevalence, morbidity, and mortality associated with AIDS-related Kaposi's sarcoma. Similarly, concomitant advances in chemotherapy and supportive-care protocols have allowed for Kaposi's sarcoma to be managed more effectively in comparison with the pre-HAART era. Furthermore, developments in our understanding of the pathogenesis of Kaposi's sarcoma have identified several molecular targets that can potentially provide new therapeutic strategies. This Review discusses the role of conventional chemotherapeutic and immunomodulatory agents in the treatment of Kaposi's sarcoma and summarises the current status and future prospects of novel molecularly targeted agents in the treatment of this disease.     

Thalidomide in combination with oral daily cyclophosphamide in patients with pretreated hormone refractory prostate cancer: a phase I clinical trial

AIM: This is a phase I study investigating the toxicity and the potential efficacy of thalidomide and oral cyclophosphamide in patients with hormone refractory prostate cancer (HRPC), previously treated with docetaxel-based regimens. METHODS: Two dose levels of thalidomide (100 and 200 mg every day) were studied. Patients were accrued to each dose level in cohorts of 3 starting from dose 1 level (100 mg). Thalidomide was started on day 1 at the assigned dose and continued for four consecutive weeks; oral cyclophosphamide (50 mg for day) was given for four consecutive weeks (1 cycle) starting on the same day initiating thalidomide. Toxicity was evaluated every two weeks; changes in prostate-specific antigen (PSA) levels were evaluated every cycle. Treatment was planned for four cycles. RESULTS: Sixteen men were treated. Ten patients in cohort 1, and 6 in cohort 2 were enrolled respectively. Grade 1-2 constipation, peripheral neuropathy and fatigue were the most common side effects, noted in 6 (37.5%), 5 (31.25%) and 3 (19%) patients, respectively. Three patients stopped the treatment at level 2, during the first cycle, for toxicity. Those three patients were evaluable only for toxicity. The MTD was 100 mg thalidomide. Thirteen patients completed two cycles. Two patients (15%) had a >50% decrease in PSA, while in one patient (8%) the PSA decrease was less of 50%. Overall PSA decrease was of 23%. CONCLUSIONS: The oral combination of thalidomide and cyclophosphamide is well tolerated and appears to be associated with biochemical response in this population. Future phase II trials, in pre-treated and untreated patients, are needed to evaluate clinical efficacy of this regimen in HRPC.