Benedikt Leidinger

High level of beta-hCG simulating pregnancy in recurrent osteosarcoma: case report and review of literature

PurposeA high serum level of beta human chorionic gonadotropin (hCG) normally indicates pregnancy in healthy women. We were confused by this finding in one of our patients. This 18-year-old girl presented with amenorrhoea of 1-month duration, a positive pregnancy test and a high beta-hCG serum level although taking contraceptives. Pregnancy was excluded by ultrasound. Three years previously, she had had an osteosarcoma of the humerus. The tumour initially had been wide resected and had shown a good response to neoadjuvant chemotherapy with COSS-96-protocol.MethodsWe reviewed the original histological result and the literature about possible similar findings. We analysed therapeutic options and the value of beta-hCG levels as a therapy monitor.ResultsDuring examination we detected a recurrent osteosarcoma of the left humerus. The local relapse evidently expressed beta-hCG which, retrospectively, could only sparsely be shown in the primary resectate. After intralesional surgery, chemotherapy and radiotherapy levels of beta-hCG normalised.ConclusionOsteosarcoma very rarely is able to produce a paraneoplastic syndrome by high levels of beta-hCG. This may well be of diagnostic value and offer an additional monitoring tool. It can indicate tumour recurrrence and dedifferentiation.

Correction of leg-length discrepancy after hip transposition.

Four patients were treated with limb lengthening to correct a leg-length discrepancy that developed after a hip transposition after pelvic resection for sacroma of the pelvis. Three patients had Ewing’s sacroma and one patient had osteosarcoma. All patients received chemotherapy; radiotherapy also was administered to the three patients with Ewing’s sacroma. Femur lengthening was started at an average of 5.7 years (range, 4.4–6.8 years) after tumor surgery. At the start of elongation, the average age of the patients was 17.3 years (range, 10.3–20.8 years). The average leg-length discrepancy was 10.3 cm (range, 6–12 cm). The average of achieved lengthening was 6.4 cm (range 6–7.5 cm). The average healing index was 32 days (range, 27–40 days) per 1-cm elongation. According to the classification of Paley, two problems and two minor complications were treated by additional interventions. At the final followup, the average functional score was 22 (73%) according to the system of the Musculoskeletal Tumor Society. Leg-length discrepancy after hip transposition can be corrected with distraction osteogenesis. All patients who wore a stiff ankle-foot orthosis before lengthening wore an ordinary shoe lift after lengthening. The problems of the lengthening procedure are similar to the general complication rate of bone lengthening. Because the 5-year survival after resection of a pelvic sarcoma is only 20% to 30%, leg lengthening after hip transposition should be offered only to long-term survivors with at least 5 years event-free survival.

Grice-Green Procedure for Severe Hindfoot Valgus in Ambulatory Patients with Cerebral Palsy

Grice-Green extra-articular subtalar arthrodesis is a treatment option for advanced valgus rearfoot deformity of neuromuscular origin in young patients. The purpose of this study was to evaluate long-term results of the procedure in ambulatory cerebral palsy patients. From January 1975 to December 1993, 57 operations were performed in 39 patients (22 males, 17 females) with a mean age of 7.8 ± 2.7 (range 3.9-14.4) years for excessive symptomatic hindfoot valgus. Thirty-five patients (51 feet) were followed for a mean 22.6 ± 4.6 (range 16.0-32.3) years. Preoperative GMFCS score, ambulation, hindfoot position, podoscopic view of the weight-bearing feet, and radiological examinations, along with complications and orthotic use, were compared. Based on a clinical rating scale, 39 results were excellent or good, 8 were fair, and 4 were poor. The poor results were attributed to hindfoot valgus recurrence or varus overcorrection. One case needed revisional surgery owing to slippage of the graft. Overall, the GMFCS score, level of ambulatory distance, and use of foot orthoses improved. Grice-Green subtalar arthrodesis did not result in early degenerative changes at the midterm follow-up. The procedure offers safe and long-lasting correction for severe and symptomatic hindfoot disorders in patients with cerebral palsy. Together with physiotherapy and orthotic use, it can improve ambulation. Whereas a slight hindfoot valgus in patients with cerebral palsy is tolerable without lack of function, overcorrection should be avoided.

Total tibial endoprosthesis including ankle joint and knee joint replacement in a patient with Ewing sarcoma

Copyright© Taylor & Francis 2005. ISSN 1745–3674. Printed in Sweden – all rights reserved. DOI 10.1080/17453670610046163 A 32-year-old man was treated for diaphyseal osteomyelitis of the tibia, proven by an open biopsy. A drilling of the whole tibia through a proximal approach and insertion of gentamycin chains was performed, with no effect on the pain. 8 weeks later, MRI showed pathological changes of the whole tibia and a small soft tissue extension in the mid-diaphyseal area which did not affect the vessels or the nerves. Again, an open biopsy was performed and Ewing sarcoma was diagnosed. After preoperative chemotherapy according to the EURO-Ewing protocol, the patient was referred to our department for knee disarticulation. Being aware of the contamination of the whole tibia and the anterior proximal skin and soft tissue, we performed a total resection of the tibia including a skin resection of 6 × 5 cm in the proximal tibia area. The flexor digitorum longus muscle, the posterior tibialis muscle and part of the tibialis anterior muscle were left on the tibia to allow a wide margin. The neurovascular bundles, the superficial flexors, the hallucis longus flexor muscle, the extensors and peroneal muscles could be preserved. The bony defect was reconstructed with a modular endoprosthesis (Figure 1) of the MUTARS System (Implantcast, Buxtehude, Germany). The knee joint was reconstructed with a rotating-hinge modular system with a connection to a titanium bone replacement system (MUTARS). The ankle joint was reconstructed with an unconstrained component with a talar surface replacement stabilized with a trans-talar and trans-calcanear stem. An arthrodesis of the talo-calcanear joint was performed. The endoprosthesis was enveloped with a Trevira tube (Gosheger et al. 2001) for attachment of the patellar tendon and attachment of a medial and lateral gastrocnemius flaps, which were transferred to the anterior side of the endoprosthesis for soft tissue coverage (Figure 2). The ventral capsule of the ankle joint was reconstructed with MITEK super anchors in combination with a Trevira Tube. A stable ankle joint could be achieved. A skin defect was covered with a skin-mesh graft on the gastrocnemius flap. The pathological analysis of the specimen showed that the surgical margin was wide and showed spread of the tumor in the whole tibia. There was a poor response to chemotherapy. The postoperative treatment consisted of 3 months of immobilization in an above-knee cast followed by stabilization with an orthosis, includ-

High incidence of metastatic disease in primary high grade and large extremity soft tissue sarcomas treated without chemotherapy

BackgroundThe risk of metastasis and the survival in patients with primary extremity soft tissue sarcomas is worse when tumour size is large and the grade of malignancy is high. Such tumours may receive chemotherapy and/or radiation therapy (RTX) for optimising local control. Irradiation can either be applied preoperatively or after tumour resection. The question arises if the kind of RTX in the absence of chemotherapy influences the outcome concerning local control, metastatic disease, survival and complications.MethodsWe retrospectively reviewed the clinical outcome of 233 patients with a primary extremity soft tissue sarcoma treated between 1990 – 2000 with a mean follow-up of 35.8 (4–120) months in our institute. 41 patients had high grade, deep and large tumours (>8 cm), an AJCC stage III (no evidence of metastasis prior to treatment) and were treated with limb salvage surgery and irradiation but stayed without additional chemotherapy. Two groups of patients were compared: the first group received postoperative RTX after tumour resection (n = 33); the second group was treated with preoperative RTX (n = 8). Both groups did not differ concerning clinical parameters. We analysed primary and secondary outcomes.Results56% (23/41) of the population developed metastatic disease, 24% (10/41) local recurrence. The risk of metastasis was higher in the group with preoperative irradiation (p = 0.046). The overall (p = 0.0248) and relapse free survival (p = 0.104) were worse in this group. The delay to tumour resection amounted 8 weeks on average in the preoperative group. Local control was not different (p = 0.38) in both study groups. Wound infections and other combined therapy related complications were equally distributed (p = 0.22).ConclusionWithout chemotherapy there remains a high risk of metastasis in AJCC grade 3 patients. In high risk patients treated without chemotherapy the elapsed time to tumour resection after preoperative radiation might contribute to the development of metastasis. This outcome may support the thesis that a combination of RTX and offensive multimodal treatment protocols is advantageous in such a subset of patients