Rotationplasty after failed limb salvage: an alternative to amputation.

PURPOSE: Limb salvage procedures for musculoskeletal tumors have inherent complications. Though most complications can be managed with retention of the reconstructions, occasionally salvaging these reconstructions is not possible. We evaluated the outcomes of patients undergoing rotationplasty after multiple failed revisions of limb salvage surgery and document the success rate of this "salvage" technique and the subsequent functional outcome of these patients. METHOD: Between January 1, 1999, and December 31, 2018, 14 patients (12 male and 2 female) with a median age of 24 years (11-51 years) underwent rotationplasty after multiple failed revisions of limb salvage surgery. Indication for rotationplasty was infection (10 patients), failed megaprosthesis (2 patients), unstable biological reconstruction (1 patient) and local recurrence (1 patient). The mean number of surgeries done before the patient underwent rotationplasty was 5 (range 2-7). RESULTS: One patient developed a vascular complication (venous congestion) immediately after rotationplasty and underwent an early amputation. The remaining 13 patients had no surgical complications. Mean Musculo Skeletal Tumor Society score in 13 evaluable patients was 26 (23-30). CONCLUSION: Our study demonstrates the utility of rotationplasty as a "salvage" procedure after multiple failed lower limb salvage surgeries. It offers good success rates, low rates of complications and good functional outcomes in carefully selected cases.

Maffucci Syndrome. An Interesting Case and a Review of the Literature.

Maffucci syndrome, a rare sporadic form of enchondromatosis, is characterized by hemangiomas and multiple enchondromas, benign cartilaginous tumors that arise near growth plates. Previous studies demonstrate that individuals diagnosed with Maffucci syndrome have approximately 100% lifetime risk of malignant transformation. Identification of Maffucci syndrome by surgical excision and pathological diagnosis can be life-saving due to its high malignant potential relative to other subtypes of enchondromatosis such as Ollier's disease. We report a case of a 58-year-old man with enchondromatosis who experienced malignant transformation of the enchondroma in his distal femur into a chondrosarcoma. He underwent a right distal femoral replacement without complications. Two years later, new masses were identified in his left hand and excised following progressive growth, pain, and functional limitation. Pathology confirmed these to be hemangiomas, and he was diagnosed with Maffucci syndrome. At last follow-up, patient reported additional nodular tumor growths occurring unilaterally on the left side. For patients with Maffucci syndrome, continual follow-up and careful surveillance of these masses is crucial as these lesions can cause fractures, deformities, pain, and undergo malignant transformation. Our report reviews the literature and outlines the treatment and management plans for patients with this rare and potentially dangerous disorder.

Brown tumors of the femur and pelvis secondary to a parathyroid carcinoma: report of one case.

Brown tumors result from excess osteoclast activity and consist of collections of osteoclasts intermixed with fibrous tissue and poorly mineralized woven bone. They are secondary to hyperparathyroidism (HPT). Their incidence is higher in primary than in secondary hyperparathyroidism. We report a 69 years-old male, admitted in a state of confusion, lethargy and bedridden, with a pathological fracture of the femur caused by a brown tumor. The laboratory examination revealed a hypercalcemia (8.85 mEq/L), with high levels of ionized Ca (5.48 mEq/L), serum alkaline phosphatases (416 U/L) and serum parathormone (120 pg/mL). Ultrasound examination of the neck showed a large parathyroid tumor, probably corresponding to a carcinoma. A primary HPT was diagnosed. The patient was hydrated and high doses of diuretics and bisphosphonates were administered. After correction of serum calcium and neurologic symptoms, the patient was operated, performing an extensive resection of the tumor. The pathology report confirmed the diagnosis of parathyroid carcinoma.

Brown tumors of the femur and pelvis secondary to a parathyroid carcinoma: Report of one case / Tumores pardos de fémur y pelvis secundarios a carcinoma de paratiroides: Informe de un caso

Brown tumors result from excess osteoclast activity and consist of collections of osteoclasts intermixed with fibrous tissue and poorly mineralized woven bone. They are secondary to hyperparathyroidism (HPT). Their incidence is higher in primary than in secondary hyperparathyroidism. We report a 69 years-old male, admitted in a state of confusion, lethargy and bedridden, with a pathological fracture of the femur caused by a brown tumor. The laboratory examination revealed a hypercalcemia (8.85 mEq/L), with high levels of ionized Ca (5.48mEq/L), serum alkaline phosphatases (416 U/L) and serum parathormone (120 pg/mL). Ultrasound examination of the neck showed a large parathyroid tumor, probably corresponding to a carcinoma. A primary HPT was diagnosed. The patient was hydrated and high doses of diuretics and bisphosphonates were administered. After correction of serum calcium and neurologic symptoms, the patient was operated, performing an extensive resection of the tumor. The pathology report confirmed the diagnosis of parathyroid carcinoma.

Los tumores pardos son una consecuencia de una actividad osteoclástica excesiva y consisten en osteoclastos mezclados con tejido fibroso y tejido óseo mal mineralizado. Son secundarios a hiperparatiroidismo y más comunes en hiperparatiroidismo primario. Informamos de un hombre de 69 años que ingresa confuso y letárgico con una fractura patológica del fémur causada por un tumor pardo. El laboratorio mostró hipercalcemia de 8,85 mEq/L, fosfatasas alcalinas de 416 U/L y parathormona de 120 pg/mL. La ecografía del cuello mostró un tumor paratiroideo sospechoso de carcinoma. Se diagnosticó un hiperparatiroidismo primario. El paciente se hidrató y estabilizó con diuréticos y bifosfonatos. Una vez estabilizado, se operó efectuando una extensa resección del tumor. El estudio anatomopatológico confirmó el diagnóstico de cáncer de paratiroides.

Solitary amyloidoma related to THA.

Amyloidoma of soft tissues is rare, and no previously published reports describe it in relation to hip prostheses. This article presents the case of a 78-year-old woman with a medical history of myelodysplasia and mild renal failure who underwent a right-sided metal-on-polyethylene total hip arthroplasty in 2003. She presented to the outpatient clinic with a 1-year history of right anterior thigh pain. On examination, a large proximal anteromedial lump was palpable and was nontender, nonpulsatile, and firm. She had a positive Trendelenburg test but good hip range of motion. Plain radiographs showed signs of osteolysis around the hip prosthesis. Serology showed mildly raised liver function and renal function but a normal erythrocyte sedimentation rate and white blood cell count. Magnetic resonance imaging revealed a large mixed-signal soft tissue mass directly opposing the anterior cortex of the right femur, related to the hip prosthesis, measuring 16×10×7 cm. Associated destruction of the underlying cortex existed. Histological staining of a biopsy of the mass confirmed that the mass contained AL-type amyloid seen in primary amyloidosis. Mean patient age at diagnosis for amyloid tumors of soft tissues is 66 years. By definition, they start as solitary lesions. The types are important: AA type is related to infection and AL type is a primary process. Patients with AL amyloidomas have a poorer prognosis because they have a higher chance of malignancies. Early diagnosis can prevent long-term serious consequences of this condition.

The biomechanical effect of proximal tumor defect location on femur pathological fractures.

OBJECTIVES: The femur is the most common long bone affected by cancerous metastasis. Femoral tumor defects are known to induce pain and functional impairment in patients. Although prior studies exist evaluating the clinical and biomechanical effect of tumor defect size, no biomechanical studies have experimentally examined the risk of pathological fracture with respect to the anterior, posterior, medial, and lateral surfaces on which a proximal tumor defect is located on the femur. METHODS: Circular tumor-like defects of 40-mm diameter were created proximally in the subtrochanteric region on the Anterior (n = 5), Posterior (n = 5), Medial (n = 5), and Lateral (n = 5) sides of 20 synthetic femurs. Intact femurs served as a control group (n = 4). Femurs were tested for lateral, "offset" torsional, and axial stiffness, as well as axial strength. RESULTS: Lateral stiffnesses (range, 121-162 N/mm) yielded no differences between groups (P = 0.069). "Offset" torsional stiffnesses (range, 135-188 N/mm) demonstrated that the Medial group was less stiff than the Intact, Anterior, and Lateral groups (P ≤ 0.012). Axial stiffnesses (range, 1057-1993 N/mm) showed that the Medial group was less stiff than the Intact group (P = 0.006). Axial strengths (range, 3250-6590 N) for the Medial group were lower than Anterior (P = 0.001) and Posterior (P = 0.001) specimens, whereas the Lateral group had a lower strength than Anterior specimens (P = 0.019). No other statistical differences were noted. Axial failure of Medial and Lateral specimens involved the tumor-like defect in 100% of cases, whereas 100% of Intact femurs and 80% of Anterior and Posterior femur groups failed only through the neck. CONCLUSIONS: In 2 of 3 test modes, the Medial tumor-like defect group resulted in statistically lower stiffness values compared with Intact femurs and had lower strength than Anterior and Posterior groups in axial failure.

A case of osteosarcoma in a patient with pycnodysostosis.

Pycnodysostosis is a rare sclerosing bone dystrophy. The main clinical features are short stature and oral and maxillofacial abnormalities such as a large head, a small and underdeveloped face with prominent nose and eyes, irregular dentition, small hands and feet with dystrophic nails, and trunk deformities such as scoliosis. The differential diagnosis is established with other skeletal dysplasias such as osteopetrosis, cleidocranial dysplasia, and idiopathic acro-osteolysis. Since its first description in 1962 by Maroteaux and Lamy, about 100 cases have been published, some of these with uncommon features. We describe the case of a 22-year-old European man with pycnodysostosis who developed a chondroblastic osteosarcoma of the right femur. No case of bone cancer in this sclerosing bone disease had been described so far.

Malignant fibrous histiocytoma of the distal femur after an arthroscopic anterior cruciate ligament reconstruction: A case report and a review of the literature.

BACKGROUND: Malignant degeneration in association with orthopaedic implants is a known but rare complication. To our knowledge, no case of osseous malignant fibrous histiocytoma after anterior cruciate ligament reconstruction is reported in the literature. CASE PRESENTATION: We report a 29-year-old male Turkish patient who presented with severe pain in the operated knee joint 40 months after arthroscopic anterior cruciate ligament reconstruction. X-ray and MR imaging showed a large destructive tumor in the medial femoral condyle. Biopsy determined a malignant fibrous histiocytoma. After neoadjuvant chemotherapy, wide tumor resection and distal femur reconstruction with a silver-coated non-cemented tumor knee joint prosthesis was performed. Adjuvant chemotherapy was continued according to the EURAMOS 1 protocol. CONCLUSIONS: Though secondary malignant degeneration after orthopaedic implants or prostheses is not very likely, the attending physician should take this into consideration, especially if symptoms worsen severely over a short period of time.

Multiple granulocytic sarcomas in essential thrombocythemia.

A 59-year-old woman was diagnosed with essential thrombocythemia in 1988 and had been treated with hydroxyurea, mitobronitol, busulfan, and ranimustine, in that order. Hepatosplenomegaly, low-grade fever, and body weight loss manifested, and a few blasts were noted in the peripheral blood studied in March 2002. A biopsied specimen of the bone marrow showed myelofibrosis but not a leukemia in August 2004. An abnormal karyotype with der(1; 13) appeared for the first time. She was treated with low-dose prednisolone. In January 2005, she experienced left hip joint pain, and magnetic resonance scanning showed a tumoral lesion in the femoral head. Histological diagnosis of the biopsied mass revealed that it was a granulocytic sarcoma, and radiotherapy was performed. In April 2005, bone scintigraphy showed multiple lesions. She became febrile and red blood cell transfusion-dependent with hepatosplenomegaly and a small number of circulating blasts. Intravenous cytarabine (low dose) and etoposide relieved the fever and hepatosplenomegaly; however, she developed a pathologic fracture of the right humerus. An additional karyotypic abnormality (7q22 deletion) was noted. She subsequently died of infection. Granulocytic sarcoma is very rare in essential thrombocythemia, and this patient may be the first reported case of essential thrombocythemia that developed multiple lesions and a pathologic fracture without transformation to overt leukemia.

Malignant change secondary to fibrous dysplasia.

BACKGROUND: Malignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder. METHODS: Four cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases. RESULTS AND CONCLUSION: The sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.

Osteosarcoma and acromegaly: a case report and review of the literature.

A 72-yr-old acromegalic man, who presented with pain in the left femur, was found to have a metastatic osteosarcoma. Only three cases describing the coexistence of acromegaly and osteosarcoma have been reported by the literature. As the patient didn't have other risk factors for osteosarcoma, the hypothesis that accelerated rate of bone turnover caused by long-term exposure to high GH and IGF-I could act as a predisposing factor in the development of this malignant bone tumor is discussed.

[Osteoid osteoma after an old femoral shaft fracture]. / Ostéome ostéoïde sur le site d'une fracture diaphysaire ancienne du fémur.

An osteoid osteoma was discovered at the site of fusion of a mid-third femoral shaft fracture in an 18-year-old girl. This unusual localization was revealed by persistent pain after ablation of the material after fracture healing. Postoperatively, a discharge from the skin wound that persisted for a few days, raised the differential diagnosis between low-grade bone infection and an osteoid osteoma suggested by the patient's age and the clinical presentation. Computed tomography and leukocyte-labeled bone scintigraphy provided the diagnosis of osteoid osteoma that was confirmed at pathology examination after resection. Isotopic mapping of this very small intraosseous lesion that exhibited strong isotope uptake was particularly contributive.

[Malignant fibrous histiocytoma of bone 20 years after femoral fracture treated by plate-screw fixation: analysis of corrosion products and their role in malignancy]. / Histiocytofibrome malin de l'os 20 ans après ostéosynthèse par lame-plaque d'une fracture du fémur : analyse des produits de corrosion et de leur rôle dans la malignité

We report a case of malignant fibrous histiocytoma of the bone that developed 20 years after a femoral fracture treated by plate-screw fixation. Similar cases reported over the past fifteen years in the literature suggest the possible mechanisms of sarcomatous degeneration. The possible carcinogenic effect of corrosion products is emphasized. Dispersion energy spectrometry of intracellular particles on the periphery and at the center of the tumor demonstrated the presence of chromium, iron and nickel at different concentrations. The association with other elements clearly demonstrates that the corrosion products were metabolized. The presence of metallic components in tumoral cells suggests a possible relationship between metallic implants and malignancy. These observations emphasize the importance of creating a national, or even international, registry of malignant tumors that develop in contact with metallic implants in order to search for a possible cause and effect relationship.