[A clinical observation of an extensive chondroma of oro- and laryngopharynx]. / Klinicheskoe nabliudenie obshirnoi khondromy roto- i gortanoglotki.

This article was designed to report a rare case of oropharyngeal chondroma in a 31 year-old male patient. Computed tomography revealed an encapsulated cystic-solid tissue mass having the clear-cut contours with multiple diffuse foci of calcification in the tissue thickness. The neoplasm was morphologically characterized as a chondroma. The tumour was removed with the use of the modified Lauers-Balon procedure with the dissection of the lower jaw.

Radiologic follow-up of untreated enchondroma and atypical cartilaginous tumors in the long bones.

BACKGROUND AND OBJECTIVES: Both enchondroma and atypical cartilaginous tumors (ACT) are not considered malignant, so inactive and asymptomatic tumors might not need surgery. To the best of our knowledge, this is the first study that has been done to evaluate the natural course of conservative-treated enchondroma and ACT in the long bones. METHODS: For this retrospective study, we analyzed the results of patients in whom we refrained from surgery and only regularly performed radiological follow-up of the tumor. Minimal follow-up after initial diagnosis was 24 months. RESULTS: Forty-nine patients were included in this study. Eight out of forty-nine cases received surgical treatment during follow-up of the tumor. The reasons for this surgery were radiologic growth of the tumor in two cases, pain in one case, patient request in three cases, another indication for surgery in the same limb in two cases. CONCLUSION: In this small series of conservatively treated enchondroma and ACT, only 6% of the patients had a medical indication for surgery. This study shows that indication for surgery should be discussed more thoroughly. Based on our results, we would recommend annual radiologic follow-up for asymptomatic enchondroma or ACT in the long bones, irrespective of tumor size. J. Surg. Oncol. 2016;114:987-991. © 2016 Wiley Periodicals, Inc.

Sellar Chondroma with Endocrine Dysfunction that Resolved after Surgery: Case Report.

Chondromas originating from the sella turcica are rare, and the most common initial symptoms are headache and visual disturbance. We describe a case of sellar chondroma with endocrine impairment as an initial manifestation that completely resolved after surgery. A 40-year-old Japanese woman with amenorrhea and galactorrhea for the last 2 years was referred to our department of neurosurgery for the evaluation of high prolactin levels and a tumor in the sella turcica. A biochemical assessment indicated endocrine dysfunction. Magnetic resonance imaging and computed tomography indicated a tumor in the sella turcica. The patient's presentation favored the preoperative diagnosis of pituitary adenoma or Rathke's cleft cyst. However, because calcification was detected, other types of tumors, such as craniopharyngioma, meningioma, and chordoma, were also considered. Endoscopic transsphenoidal surgery was performed, and the possibility of a bony tumor was recognized. Finally, the tumor was completely removed, and the histopathological findings confirmed chondroma. The postoperative course was uneventful, and endocrine function improved. Five years after surgery, the patient is doing well without pituitary insufficiency, pituitary hormone medications, or signs of tumor recurrence. In cases of sellar chondroma, endocrine dysfunction sometimes precedes other symptoms, such as headache and visual disturbance. When examining a patient with an intrasellar tumor harboring calcification, clinicians must consider the possibility of sellar chondroma. Furthermore, to the best of our knowledge, this case is the first of sellar chondroma treated with endoscopic surgery to be reported.

Images of a case of Carney triad by combined F-18 FDG PET/CT.

Carney triad is a very rare condition with gastrointestinal stromal tumor, pulmonary chondroma, and extra-adrenal paragangliomas. We present the images of a patient with the complete triad, including total gastrectomy for gastrointestinal stromal tumor at the age of 13, and a left pneumonectomy for pulmonary chondroma at the age of 15. Results of F-18 FDG PET/CT that was performed at age of 22 for recurrent pulmonary chondroma also demonstrated new lesions in the neck, mediastinum, and abdomen representing extra-adrenal paragangliomas.

Multifocal periosteal chondromas in the ring finger of an adolescent boy: case report.

We describe an unusual case of a 12-year-old boy who presented with a loss of motion in the ring finger caused by 2 separate periosteal chondromas involving the proximal and middle phalanges. Range of motion improved and recurrence did not occur at the 5-year follow-up after marginal excision of both lesions.

Curettage and cryosurgery for low-grade cartilage tumors is associated with low recurrence and high function.

BACKGROUND: Chondrosarcomas of bone traditionally have been treated by wide or radical excision, procedures that may result in considerable lifelong disability. Grade 1 chondrosarcomas have little or no metastatic potential and are often difficult to distinguish from painful benign enchondromas. Curettage with adjuvant cryosurgery has been proposed as an alternative therapy for Grade 1 chondrosarcomas given the generally better function after the procedure. However, because it is an intralesional procedure, curettage and cryosurgery may be associated with higher rates of recurrence. QUESTIONS/PURPOSES: We asked whether Grade 1 chondrosarcomas and enchondromas of uncertain malignant potential treated by curettage and cryosurgery are associated with low recurrence rates and high functional scores. PATIENTS AND METHODS: We retrospectively reviewed the records of 46 patients with Grade 1 chondrosarcomas and enchondromas of uncertain malignant potential treated by curettage and cryosurgery. Forty-one patients had tumors of the long bones. Patients were followed a minimum of 18 months (average, 47.2. months; range, 18-134 months) for evidence of recurrence and for assessment of Musculoskeletal Tumor Society (MSTS) functional score. RESULTS: Two of the 46 patients had recurrences in the original tumor site (4.3% recurrence rate), which subsequently were removed by wide excision, and both patients were confirmed to be disease-free 36 and 30 months, respectively, after the second surgery. The mean MSTS score was 27.2 of 30 points (median, 29 points). CONCLUSIONS: Our observations show curettage with cryosurgery is associated with low recurrence of Grade 1 chondrosarcoma and high functional scores. Curettage with cryosurgery is a reasonable alternative to wide or radical excision as the treatment for Grade 1 chondrosarcomas, and allows for more radical surgery in the event of local recurrence. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

PTHR1 mutations associated with Ollier disease result in receptor loss of function.

PTHR1-signaling pathway is critical for the regulation of endochondral ossification. Thus, abnormalities in genes belonging to this pathway could potentially participate in the pathogenesis of Ollier disease/Maffucci syndrome, two developmental disorders defined by the presence of multiple enchondromas. In agreement, a functionally deleterious mutation in PTHR1 (p.R150C) was identified in enchondromas from two of six unrelated patients with enchondromatosis. However, neither the p.R150C mutation (26 tumors) nor any other mutation in the PTHR1 gene (11 patients) could be identified in another study. To further define the role of PTHR1-signaling pathway in Ollier disease and Maffucci syndrome, we analyzed the coding sequences of four genes (PTHR1, IHH, PTHrP and GNAS1) in leucocyte and/or tumor DNA from 61 and 23 patients affected with Ollier disease or Maffucci syndrome, respectively. We identified three previously undescribed missense mutations in PTHR1 in patients with Ollier disease at the heterozygous state. Two mutations (p.G121E, p.A122T) were present only in enchondromas, and one (p.R255H) in both enchondroma and leukocyte DNA. Assessment of receptor function demonstrated that these three mutations impair PTHR1 function by reducing either the affinity of the receptor for PTH or the receptor expression at the cell surface. These mutations were not found in DNA from 222 controls. Including our data, PTHR1 functionally deleterious mutations have now been identified in five out 31 enchondromas from Ollier patients. These findings provide further support for the idea that heterozygous mutations in PTHR1 that impair receptor function participate in the pathogenesis of Ollier disease in some patients.

Chondroma/Chondrosarcoma of the spine.

Chondromas and chondrosarcomas are cartilage-forming tumors that occur rarely in the spine. These neoplasms exist on opposite ends of the pathologic spectrum, ranging from the benign chondroma to the malignant, high-grade chondrosarcoma. Unlike other sarcomas, a patient's long-term prognosis is influenced by the grade of the tumor. A complete en bloc resection is the ideal method of surgical management. This method holds especially true for chondrosarcomas, and can result in prolonged survival. These tumors are resistant to conventional chemotherapy and radiation therapy. Hypofractionated stereotactic radiation therapy may slow tumor progression, although the long-term effect of this modality is unknown.

Respiratory symptoms in a boy revealing Carney triad.

The association of gastrointestinal stromal cell tumor (GIST), paraganglioma, and pulmonary chondroma is known as the Carney triad, occurring predominantly in young adult females. We present the case of a 14-year-old male with respiratory symptoms resulting in the diagnosis Carney triad.

Tenosynovial (extraarticular) chondromatosis: an analysis of 37 cases of an underrecognized clinicopathologic entity with a strong predilection for the hands and feet and a high local recurrence rate.

Tenosynovial chondromatosis is a multinodular cartilaginous proliferation that arises from the tenosynovial membranes. This report describes the clinical, radiologic, and histopathologic findings in 37 cases of this uncommon entity. There were 17 males and 20 females, ranging in age from 20 to 86 years (mean and median age, 46 years). The process involved tenosynovium of the fingers (n = 19), feet (n = 8), wrists (n = 4), ankles (n = 2), hand, not otherwise specified, or palm (n = 2), knee (n = 1), and forearm (n = 1). Signs of disease or symptoms were present for 5 weeks to 18 years (median duration, approximately 2 years) before surgical excision. The two most common complaints were a painless mass and a mass that was mildly tender with pressure. None of the tumors had clinical, radiologic, or histopathologic evidence of articular or bone involvement. Histologically, all tumors consisted of a multinodular cartilaginous proliferation involving tenosynovium and/or subsynovial connective tissue. Mild or moderate atypia, as encountered in chondroma of soft parts and synovial chondromatosis, was a frequent finding. Follow-up information was available for 16 patients (43%). Only two patients with follow-up information remained disease free after their initial surgical procedure. Seven patients had one recurrence and seven patients had two or more recurrences. Tenosynovial chondromatosis appears to be an extraarticular counterpart of synovial (intraarticular) chondromatosis. Our review indicates this process is often confused with chondroma of soft parts, in part, because both entities have a predilection for the hands and feet. Diagnosis of this underrecognized entity is of clinical importance because of the high local recurrence rate.

Use of the Ilizarov external fixator in the treatment of patients with Ollier's disease.

The authors present the results of treatment of 10 patients with Ollier's disease using the Ilizarov technique. The Ilizarov device was used to treat leg length discrepancy and to enhance the conversion of chondroma cartilage into normal mature bone, with no curettage and bone grafting. The mean duration of treatment was 9.4 months. This technique was highly efficient in treating the disease. It led to conversion of the abnormal cartilage into histologically mature bone in all patients. Some complications were seen, such as decreased knee mobility, which required prolonged use of the device. The Ilizarov technique is successful in treating patients with Ollier's disease despite some complications and the difficulty in using the technique.

[Vascularized peroneal reconstruction after bloc resection of tumors or congenital malformations of the upper limb in children]. / Reconstruction par péroné vascularisé après résection en bloc pour lésions tumorales ou malformation congénitale du membre supérieur chez l'enfant.

Limb salvage surgery is the standard care for most malignant tumor affecting the extremities in the child, and a vascularized fibula transfer is probably the most popular microsurgical option to reconstruct long-bone defects. Between 1994 and 1999, nine children with intractable diseases of the upper limb were treated using free vascularized fibula grafts (one patient had resection in 1983 and initially prosthetic reconstruction, then fibula transplant in 1996). There were 6 boys and 3 girls. Mean age was 10 years (between 6 and 16). Eight patients had defects after sarcoma resection, one had an aggressive enchondroma. The reconstructed sites were the humerus (= 6), the radius (n = 3). The length of the bone defect ranged from 8 to 19 cm (mean: 14.4 cm). The fibula head with the cartilage and the growth plate was used in 3 children. One girl, 4.5 years old with congenital pseudoarthrosis of radius and cubitus had a resection and reconstruction with a U shaped fibula transplant. One patient died from lung and brain metastasis, two years after the reconstruction. There were no local recurrences. The complications were numerous but usually benign; fracture of the grafted fibula n = 7, necessity of additional bone grafts (n = 4) malunion (n = 1) needed reoperation, pseudoarthrosis (n = 2) with reoperation, ankle valgus (n = 1) required reoperation, necrosis of the fibula head (n = 1), radial inclination (n = 1). The ten patients had bone union. The mean period required to obtain radiographic bone union was 5 months. The functional results of the remaining patients were evaluated according to the scale of ENNEKING. The results ranged from 21 to 30 points. Our results were satisfactory with regard to pain, emotional acceptance, manual dexterity. The vascularized fibula graft is indicated in children with large bone defects, more than 8 cm in the humerus, radius and ulna.

Condromalacia patelar. Correlación clínico-artroscópica de un nuevo signo clínico / Patellar chondromalatia. Clinical-arthroscopical correlation of a new sign

Se elaboro el presente trabajo, prospectivo longitudinal, tomando 200 artroscopias efectuadas en el servicio, con el diagnóstico clínico preoperatorio de condromalacia patelar. A todos los pacientes se les realizó anamnesis dirigida así como exploración física que incluyó la búsqueda de los signos clásicos: palpación de facetas rotulianas, cepillo rotuliano y escape rotuliano, además, se realizó la maniobra de Carriedo, variante del escape rotuliano. Al establecer la correlación clínico-artroscópica se encontró que los signos clásicos sólo son confiables en cuanto al diagnóstico y no así en cuanto a la clasificación del grado de lesión. El signo de Carreido, en forma opuesta, mostró una alta confiabilidad para fines diagnósticos y sobre todo, para determinar el grado de lesión particularmente en las condromalacias grados III y IV. Lo anterior es de relevante importancia debido al elevado número de pacientes sin condromalacia o con ella en grados I y II a quienes se les efectúa una intervención artroscópica cuando, preferentemente, deberían ser manejados en forma conservadora. Se propone que en los servicios de ortopedia sea investigado este nuevo signo en forma sistemática en los pacientes en quienes se sospecha condromalacia patelar

Tratamiento de la condromalacia patelar con perforaciones tipo Pridie con la nueva guía en "C" de Carriedo / Treatment of chondromalacia patella with Pridie type drills with the new guide in \"C\" of Carriedo

La condromalacia patelar tiene una alta incidencia en México. Debido a que el tratamiento de sus grados avanzados es quirúrgico, se sugiere su realización a través de artroscopía, para lo que se propone una guía en forma de "C" para realizar perforaciones tipo Pridie con un amplio margen de exactitud y seguridad. En esta comunicación se presenta la experiencia obtenida con el uso de la guía en el tratamiento de 55 pacientes con condromalacia patelar

Análisis clínico-radiográfico de la condromalacia patelofemoral: estudio comparativo / Clinical and radiographic analysis of patellofemoral chondromalacia: comparative study

Se realizó un estudio prospectivo en la consulta externa del Departamento de Ortopedia del Hospital de Especialidades núm. 1, Centro Médico Nacional León. Se incluyeron 16 pacientes de uno y otro sexo de 16 a 40 años de edad, sin patología de rodilla. En ambos grupos, se realizó medición clínica del ángulo Q, así como mediciones radiográficas de la altura patelar eje femorotibial, ángulo sulcus y ángulo de congruencia patelofemoral; determinándose además el tipo de patela. Se encontró una relación estadísticamente significativa ente condromalacia y ángulo Q elevado, patela alta, incremento del ángulo sulcus y del ángulo de congruencia. No se encontró relación entre condromalacia y eje femorotibial ni con el tipo de patela.

Synovial chondromatosis.

Two patients were evaluated for a complaint of long-standing hip pain. No etiology of their progressively disabling symptoms was found by routine diagnostic studies. Hip arthrography and arthrotomography in each case, however, demonstrated multiple intraarticular filling defects consistent with nonradiopaque loose bodies. These were subsequently confirmed at arthrotomy. Synovial chondromatosis is a rare disease in which intrasynovial cartilage metaplasia results in multiple intracapsular loose bodies. Its presence in plain radiographs is uncommon, but has been previously documented. This can account for frequent long delays in accurate diagnosis. Arthrography and/or arthrotomography, however, can be key diagnostic tools. They are recommended in the evaluation of patients whose relatively normal initial clinical examination, laboratory, and roentgenographic studies fail to adequately explain the presence of genuinely disabling symptoms.