An extendable modular endoprosthetic system for bone tumour management in the leg

A modular endoprosthetic system has been developed at the Groningen University Hospital and the University of Twente. The system can bridge a defect resulting from the resection of a malignant bone tumour which has developed around the knee joint of a child. Since the other healthy leg continues to grow, the system includes an element whose length can be adjusted non-invasively by using an external magnetic field. In addition to this lengthening element, there are one hip and two knee components, connectors of various lengths, and fixation elements. The paper describes the elements of the modular endoprosthetic system. Tables are created by means of which the elemental composition of such an endoprosthesis can be determined for each individual patient

The treatment of primary tumors of the femur with chemotherapy (if indicated), resection and reconstruction with an endoprosthesis

The treatment protocol of 15 patients with a primary tumor of the femur, including osteosarcoma, malignant fibrous histiocytoma and chondrosar-coma is presented. All patients had been selected for resection and reconstruction with an endoprosthesis. An endoprothesis was implanted in 12 patients. \ud The results of this type of treatment appear to be satisfactory. In eight osteosarcoma cases resection and reconstruction with an endoprosthesis combined with preoperative and postoperative chemotherapy, according to Rosen, were performed. Follow-up in all 15 patients, varying from 1.4 to 6.0 years, showed no evidence of disease in 12 patients. Three patients had died. Function of the involved leg was satisfactory in most cases. \ud The advantage and disadvantages of the use of an endoprosthesis are discussed as well as complications in this series of patients

The use of hydroxychloroquine as a systemic treatment in erosive lichen planus of the vulva and vagina

Erosive lichen planus affecting the vulva and vagina (ELPV) is a rare inflammatory skin disease, presenting with painful erosions and severe scarring.1 The disease course is persistent and often refractory to treatment: up to 45% patients do not experience remission with topical treatments, while evidence for systemic treatments remains scarce.2 Hydroxychloroquine (HCQ) is frequently used in daily practice as a first choice systemic therapy.3 However, little evidence is available on HCQ for ELPV.2 The aim of this study was to analyse the effectiveness and safety of HCQ in ELPV.</p

Skin-coloured vulvar papules

We report a 43-year-old woman with asymptomatic vulvar papules. Histopathology showed ducts with luminal differentiation and eosinophil debris with a tadpole shape. Based on these findings, the diagnosis vulvar syringoma was made. Syringoma are benign eccrine sweat gland tumour mostly located on lower eyelids and cheeks. Vulvar syringoma are rare.</p

Case report of a clinically indolent but morphologically high-grade cutaneous mast cell tumor in an adult:Atypical cutaneous mastocytoma or mast cell sarcoma?

We present a case of an adult male with a solitary mast cell tumor of the skin with unusual nuclear pleomorphism and mitotic activity. The tumor was excised, recurred within 2 years, was reexcised after 4 years and did not recur >6 years after diagnosis. The tumor showed progressive cytonuclear atypia and a high mitotic and proliferation rate by Ki67‐staining from the onset. No KIT mutations were identified in the tumor and bone marrow. Serum tryptase levels and a bone marrow aspirate and trephine biopsy were normal. Although the histomorphology of the skin tumor was consistent with mast cell sarcoma, the clinical behavior without systemic progression argued against this diagnosis. The tumor was finally considered as atypical mastocytoma, borderline to mast cell sarcoma. Currently, the patient is in close follow‐up and still in complete remission