Type 1 neurofibromatosis and adult extremity sarcoma. A report of two cases.

We report two cases of malignant soft-tissue tumours--one myxoid malignant fibrous histiocytoma and one pleomorphic rhabdomyosarcoma--which were diagnosed in two young adult patients with type 1 neurofibromatosis (NF 1). The patients were evaluated with criteria for Neurofibromatosis 1 and NF 1 gene analysis was performed. Four of seven criteria were found in both patients. The tumours were stage II and III respectively. Both patients were treated with radiotherapy or chemotherapy and surgical intervention. Diagnoses of myxoid malignant fibrous histiocytoma and pleomorphic rhabdomyosarcoma in adult NF 1 patients are exceedingly rare. Thus detection of subtypes of rhabdomyosarcoma and malignant fibrous histiocytoma with immunohistochemistry may be helpful for the management of these tumours among other pleomorphic sarcomas that may occur in type 1 Neurofibromatosis.

[Treatment of AO type C2-C3 fractures of the distal end of the radius with external fixation]. / Radius distal uç kiriklarinin (AO tip C2-C3) eksternal fiksatörle tedavisi.

OBJECTIVES: We evaluated the results of combined treatment with closed reduction, grafting, K-wires, and external fixation in patients with fractures of the distal end of the radius. METHODS: Of 24 patients (15 males, 9 females; mean age 34 years; range 21-73), 16 patients had C2, eight patients had C3 fractures according to the AO classification. Three patients had open fractures, and four patients had signs of median nerve compression. Treatment included closed reduction, autologous grafting (16 cases) followed by fixation with 2 to 5 K-wires, and an external fixator. Clinical and radiographic results were assessed according to the Gartland-Werley's and Stewart's criteria, respectively, and degenerative changes to the Knirk-Jupiter's criteria. The mean follow-up was 35 months (range 25 to 52 months). RESULTS: All of the 19 patients who were employed returned to preinjury working conditions in a mean of 3.5 months. Radial length was preserved to a great extent. The mean amount of collapse of the joint surface was 1.4 mm. The mean loss was 6.7% in radial inclination, and 17.6% in the volar tilt. According to the Stewart's criteria, the results were good, moderate, and poor in 12, nine, and three patients, respectively. Flexion-extension (75%), supination-pronation (70%), and radial-ulnar deviation (75%) were preserved to a great extent. Grip force amounted to 70% of the healthy side. According to the Gartland-Werley criteria, three patients had excellent, eight patients had good, 10 patients had moderate, and three patients had poor results. According to the Knirk-Jupiter's criteria, degenerative changes were slight in 11 patients, moderate in six patients, and severe in one patient. Six patients had no signs of degeneration. Complications included pin track infections (n=4), early transient reflex sympathetic dystrophy (n=2), and hypoesthesia along the superficial branch of the radial nerve (n=2). CONCLUSION: Treatment of the distal end fractures of the radius (AO type C2-C3) with a combination of external fixation, grafting, and percutaneous K-wires provides almost normal radiologic and clinical parameters.

Reconstruction of defects following bone tumor resections by distraction osteogenesis.

INTRODUCTION: The authors present the results of nine patients (two females, seven males) who had bone tumors that were treated with en bloc resection and distraction osteogenesis between 1991-2000. MATERIALS AND METHODS: The average age of the patients was 19.3 years (14-42 years). The histological diagnosis was osteosarcoma in four cases, Ewing's sarcoma in two, giant cell tumor in one, osteofibrous dysplasia in one and osteoblastoma in one. A uniplanar external fixator was applied to one case and circular external fixator to the other eight cases. RESULTS: The average follow-up period was 59.1 months (27-129 months). The external fixator was removed at an average of 18.1 months (range, 4-19 months). The average bone defect after resection was 14 cm (8-24 cm) and the average external fixation index, distraction index, and maturation index were 31.5 (18.7-40.0), 11.2 (10.9-11.2) and 17.8 (7.5-32.7), respectively. The function of the affected leg was excellent in four patients, good in three, fair in one and poor in one according to the Enneking scoring system. We did not observe any early consolidation or osseous binding in the defect area in any patient. Frequent complications were pin tract infection and non-union at the docking site. CONCLUSION: Our results indicate that the Ilizarov method allows effective restoration of bone defects in the treatment of bone tumors despite various disadvantages.

Treatment of proximal fibular tumors with en bloc resection.

Proximal fibular tumor resection has always been a challenge to an orthopedic surgeon due to the proximity of two major structures; the peroneal nerve and anterior tibial artery. Extra-articular resection of the proximal tibiofibular joint, sacrificing of peroneal nerve and split resection of lateral tibial wall are major points of debate. Malawer described two types of resection for aggressive benign and malignant tumors of the proximal fibula, type I for benign and type II for malignant tumors. Between 1992 and 2002, nine male patients with proximal fibula tumors were treated by en-bloc resection as described either by Malawer and or by one of two new resection techniques. Of the nine tumors, six were diagnosed as giant cell tumor (one of them recurrent), two as osteosarcoma and one as benign fibrous histiocytoma. The mean age of the patients was 23.6 (20-48) years. The mean follow up period was 42.8 months (15-117). There were no complications leading to a secondary surgical procedure+no local recurrence. Tumor volume was over 250 ml in two GCT cases, so the deep peroneal nerve was sacrificed to provide a wide margin. Iatrogenic peroneal nerve palsy developed in two patients. Late tendon transfers were performed for the management of drop foot. Our results indicate that if tumor is recurrent or has a large volume, wide resection (including deep peroneal nerve) should be done. Despite satisfactory tumor management, functional outcomes turned out to be variable, therefore precise ligament and muscle reconstruction is recommended.

Giant cell tumor of tendon sheath simulating giant cell tumor of bone: report of a case.

A 24-year-old male patient presented with a painful eccentric lytic lesion of the proximal tibial epiphysis with a soft tissue component. Clinical and radiological assessment led to the tentative diagnosis of aggressive giant cell tumor of bone. The patient was treated with curettage, high-speed burr, and cementation after intraoperative pathology consultation. The final pathological report indicated that the tumor was giant cell tumor of the tendon sheath with bone invasion. Although uncommon, GCTTS should be considered in the differential diagnosis of such lesions when there is a prominent soft tissue component. Although the resection was intralesional, the thermal effect of the cementation of the involved cavity and complete removal of the tendon sheath may allow successful local control conjecture of lesions that otherwise present with clinical and radiographic findings suggesting giant cell tumor of the bone.

[Successful results of total femoral resection and prosthetic replacement in two patients]. / Total femur rezeksiyonu ve prostetik replasmanin basarili sonuçlari: Iki olgu sunumu.

Resection of the whole femur, together with endoprosthetic replacement is a major limb-salvaging procedure. We performed total femoral resection and endoprosthetic replacement with TMTS (Turkish Musculoskeletal Tumor Society) prosthesis in two young (20 years old) male patients with stage-IIB osteosarcoma. Postoperative complications were only temporary sciatic nerve neuropraxia and minor skin necrosis around the surgical wound. After a four-staged individualized rehabilitation program, both patients were able to walk without any support or limitation. At the end of a follow-up period of 22 and 26 months, both patients achieved a nearly full range of motion of the knee and the hip, with Enneking functional scores being excellent. Endoprosthetic total femoral reconstruction results in satisfactory oncologic and functional outcome in selected patients.

Rupture of the pectoralis major muscle in a paratrooper.

Rupture of the pectoralis major muscle is a very rare injury. Excessive contraction of muscle fibers during certain forms of sports, such as weightlifting and bench pressing, is the most common cause. Among the 150 reported cases in the literature, in only 1 case did the injury happen during the landing phase of parachuting. Here we report a case of pectoralis major muscle rupture caused by a different mechanism than published previously. A paratrooper was injured during a tactical jump out of an aircraft after becoming entangled with the risers. The mechanism of injury was excessive traction and malpositioning of his shoulder when the parachute deployed. A three-phase conservative treatment regimen was performed and results were assessed by dynamometry. The patient was satisfied with the treatment and the dynamometric results were good at 9 mo after injury and at the end of a 20-mo follow-up period. We suggest that three-phase rehabilitation can be an effective treatment option for pectoralis major muscle rupture in selected patients. Prevention of this type of altitude injury would be possible by applying the fundamentals of parachuting.

Does false aneurysm behave like a sarcoma? Distal femoral arterial false aneurysm simulated a malign mesenchymal tumor. A case report and review of the literature.

We report a case of a pseudo-aneurysm of the distal femoral artery presenting as a malignant mesenchymal tumor with insidious onset, progressive symptoms, large volume mass, and atypical radiological findings.

Chondroblastoma of the distal femur. A case report.

The authors report a case of chondroblastoma which was localised in the distal femoral epiphysis in a 16-year-old boy. The lesion was large, rapidly expanding and extended into the knee joint. After diagnostic evaluation including tru-cut biopsy, the lesion was treated surgically with curettage and grafting with coralline hydroxyapatite. Four months after surgery the patient had no pain and had nearly full range of motion of the left knee. He was followed up for thirty-five months with routine radiographs and physical examination. He had no recurrence, no pain, and regained full range of motion of his knee. Most chondroblastomas involve the medullary cavity; they may rarely involve the cortex but to the best of our knowledge, no cases with soft tissue involvement have been reported in the literature.