Synovial sarcoma presenting as an intra-articular mass in a pediatric patient: a case report.

BACKGROUND: Synovial sarcoma (SS) is one of the reported sarcomas in the pediatric and adult populations. Delay in diagnosis and treatment is common in SS cases. SS may be excised before the correct diagnosis is made. CASE PRESENTATION: we present a case involving a 4-year-old boy who visited our service with complaints of left knee pain and limited knee flexion. Initially, the child was diagnosed with osteochondromatosis. Surgical excision was opted, and initial histopathological examination revealed a fibrous histiocytoma. The slide and blocks were then brought to the King Faisal Specialist Hospital Research Center (KFSH&RC) and histopathologic analysis has shown a well-circumscribed nodule in the synovium with a sub-synovial monomorphic spindle cell sarcoma, confirmed by fluorescence in situ hybridization (FISH). CONCLUSIONS: Therefore, we strongly recommend considering all differential diagnoses for soft-tissue masses when planning surgical management.

Nora's Lesion: Bizarre Parosteal Osteochondromatous Proliferation Causing Splay Foot Deformity: A Case Report.

Nora's lesion, or bizarre parosteal osteochondromatous proliferation (BPOP), is a rare benign lesion that is made up of varying degrees of cartilage, bone, and spindle cells. Most notably, calcification of the cartilage or "blue bone," is a feature of the disorder. The condition principally affects long tubular bones of the hands and feet, and is generally seen in patients in their second and third decades of life. We present a case of BPOP occurring in the second interspace with symptoms that would be consistent with a more common diagnosis of predislocation syndrome, or a second interspace neuroma. This case study may help the clinician in considering a more subtle cause of a splay deformity in the second interspace, and walk through the diagnostic and treatment course for BPOP.

The stiff shoulder; A case study.

Clinicians working in outpatient departments and advanced practitioner clinics frequently encounter patients presenting with multidirectional stiffness of the glenohumeral joint. This case report describes the assessment and treatment of a patient presenting with glenohumeral joint stiffness and describes the possible differential diagnoses. The evidence base used to inform the decision-making process is presented and the use of radiology that helped to ultimately establish the diagnosis is discussed. The clinical reasoning process of applying knowledge and experience to identify patient problems and to make appropriate decisions that result in positive patient outcomes is discussed. The case report highlights the importance of early diagnosis.

[Nora's Lesion: A Rare Differential Diagnosis for Benign Peripheral Bone Tumours]. / Die Nora-Läsion: Eine seltene Differenzialdiagnose bei benignen peripheren Knochentumoren.

We report on a bizarre parosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, in a 43-year-old female patient.

[Relapse of a parosteal osteochondromateous proliferation (Nora's lesion) of the distal thumb]. / Rezidiv einer bizarren paraossären osteochondromatösen Proliferation (Nora-Läsion) des Daumenendgliedes.

We report the case of a 29-year-old female patient with a relapse of a Nora lesion in an atypic location. The literature for this rare benign lesion is reviewed and etiology, diagnostics and therapy are discussed.

Haga su diagnóstico / Make your diagnosis

El diagnóstico es Osteocondromatosis Multiple Hereditaria

Osteocondromatosis múltiple familiar: a propósito de un caso / Multiple familial osteochondromatosis: presentation of a case

Los encondromas son tumores benignos de cartílago que crecen de forma lenta en las metáfisis de los huesos. Pueden ser lesiones solitarias o múltiples. Las encondromatosis comprenden un grupo heterogéneo de síndromes, difíciles de diferenciar, caracterizados por la presencia de múltiples encondromas que pueden llegar a producir malformaciones musculoesqueléticas (secundarias al acortamiento de extremidades), escoliosis, fracturas patológicas o seudoartrosis. La complicación más temida, el osteocondrosarcoma, puede acontecer hasta en el 25% de los pacientes. Exponemos el caso de un varón de 67 años, sin diagnósticos previos conocidos, que consulta por la aparición en cadera izquierda de una tumoración dolorosa y rápidamente deformante en el último año. Los antecedentes familiares y los datos clínico-radiológicos confirmaron el diagnóstico de osteocondromatosis múltiple familiar. Aun cuando la evolución clínica y los estudios de imagen hicieron sospechar una degeneración maligna (osteocondrosarcoma), esta no se confirmó en el estudio histopatológico de la pieza quirúrgica (AU)

Enchondromas are benign cartilage tumours that grow slowly in the bone metaphysis. They may involve solitary or multiple lesions. Enchondromatoses include a heterogeneous group of hardly distinguishable syndromes characterised by the presence of multiple enchondromas that may cause musculoskeletal malformations (secondary to limb shortening), scoliosis, pathological fractures, or pseudoarthrosis. The most dreaded complication, osteochondrosarcoma, occurs in up to 25% of patients. We present the case of a 67-year-old male with no previous diagnosis, requiring attention due to the appearance of a painful tumour in his left hip which degenerated rapidly over the past year. Family history and clinical-radiological data confirmed the diagnosis of Multiple Familial Osteochondromatosis. Although clinical evolution and imaging led to suspect a malignant degeneration (osteochondrosarcoma), this was not confirmed by the histopathological study of the surgical sample (AU)

Clinical, radiological and ultrasonographic features, treatment and outcome in 22 horses with caudal distal radial osteochondromata.

REASONS FOR PERFORMING STUDY: Although a well recognised clinical entity, only small numbers of osteochondromata on the caudal distal radius have previously been published and its occurrence in young racing Thoroughbreds has not previously been reported. Identification and management of associated lesions in the deep digital flexor tendon have received scant attention in the literature. HYPOTHESIS: Osteochondromata of the caudal distal radius occur commonly in young racing Thoroughbreds. They vary in size and location, sagittally and proximodistally, but the majority cause impingement damage to the deep digital flexor tendon. METHODS: Case records and diagnostic images of horses with osteochondromata of the caudal distal radius were reviewed retrospectively and follow-up information obtained. RESULTS: Twenty-five osteochondromata were identified in 22 horses, 19 of which were Thoroughbreds. All osteochondromata were metaphyseal. Twenty-two were in the middle one-third of the bone and laceration of the adjacent deep digital flexor tendon was identified in 21 limbs. Treatment in all cases consisted of removal of the osteochondroma with debridement of the deep digital flexor tendon when this was affected. All horses returned to work and none exhibited any evidence of recurrence. CONCLUSION: Osteochondromata of the caudal distal radius occur in young racing Thoroughbreds but are also identified in other horses. They have a consistent metaphyseal location and most are found in the middle one-third of the radius. Size varies, but most cause laceration of the adjacent deep digital flexor tendon. Treatment by removal of the mass and debridement of the tendon is associated with a good prognosis. POTENTIAL RELEVANCE: Osteochondromata of the caudal distal radius are an important cause of tenosynovitis of the carpal sheath of the digital flexor tendons in young racing Thoroughbreds. When present in the most common location of the middle one-third of the bone, they are likely to cause impingement damage to the deep digital flexor tendon. Tenoscopic surgery offers a good prognosis.

Cambios metafisiarios de los miembros inferiores originados por osteocondromatosis múltiple hereditaria / Mataphyseal changes of the lower limb caused by multiple hereditary osteochondromatosis

Se realizó un estudio de población, de tipo prospectivo, descriptivo y observacional en pacientes que acudieron a la Unidad de Tumores Óseos y Partes Blandas (UTOPB) del Hospital Universitario “Dr. Manuel Núñez Tovar” entre enero de 2008 y julio de 2010, con el diagnóstico de Osteocondromatosis Múltiple Hereditaria (OMH) que afectaba las articulaciones de las extremidades inferiores; se estudiaron 26 pacientes, equivalentes a 52 extremidades inferiores, con predominio del sexo masculino 1,2:1, las edades de los pacientes comprendían entre los 6 y 15 años, con mayor registro de casos a los 12 años (19,2%). Se cuantificó el número de osteocondromas periarticulares en las articulaciones de cadera, rodilla y tobillo, observando un predominio en la rodilla (48,7%) a expensas de la metáfisis distal del fémur. Se clasificó la afectación funcional de las articulaciones en tres grados (leve, moderada y severa), tomando como referencia el movimiento de flexoextensión, siendo el tobillo la articulación que presento el mayor grado de limitación y la cadera la menos afectada a la presencia de osteocondromas

We conducted a prospective, descriptive and observational population-based study, in patients attending the Unit of Bone and Soft Tissue Tumors (UTOPB) at the Hospital "Dr. Manuel Núñez Tovar" between January 2008 and July 2010, with the diagnosis of Multiple Hereditary Osteochondromatosis (MHO), which affected the joints of the lower extremities, were studied 26 patients, equivalent to 52 lower extremities; with a male predominance 1,2:1, the age of the patients ranged between 6 and 15 years, with highest number of cases at 12 years (19.2%). We quantified the number of periarticular osteochondromas at the hip, knee and ankle, having predominance in the knee (48.7%) at the expense of the distal femur metaphysis. We scored the functional range of the joints in three grades (mild, moderate and severe), with reference to the movement of flexion-extension, the ankle joint had the highest degree of limitation and, the hip was less affected to the presence of osteochondromas

Bizarre parosteal osteochondromatous proliferation of the metatarsal: a pediatric case report and archival review.

UNLABELLED: Bizarre parosteal osteochondromatous proliferations (BPOP), also known as Nora's lesions, are rare tumors occurring most commonly in the hands and feet. They are benign and rarely exhibit radiological evidence of cortical invasion. We report a case of BPOP showing atypical magnetic resonance imaging features that are inconsistent with BPOP and having a novel chromosomal aberration. We also review the BPOP cases in our regional benign bone tumor database. LEVEL OF CLINICAL EVIDENCE: 4.

[Bizarre parosteal osteochondromatous proliferation or Nora's lesion]. / Prolifération ostéocartilagineuse parostéale bizarre ou lésion de Nora.

Bizarre parosteal osteochondromatous proliferation (BPOP) is a very rare benign lesion on face bones. The first line treatment is surgical exeresis. The frequency of recurrence after exeresis may suggest a malignancy.

Fracturas osteocondrales del astrágalo: posibilidades artroscópicas y técnicas de reparación condral / Osteochondral fractures of the talus: arthroscopic possibilities and chondral repair techniques

Introducción. La artroscopia de tobillo ha experimentadoen estos últimos años un avance importante como consecuencia de la mejora de la tecnología y la experiencia quirúrgica acumulada. Actualmente es posible acceder artroscópicamente al tobillo y a los huesos del retropié, como a las lesiones condrales del astrágalo. No obstante, la técnica exige una adecuada curva de aprendizaje y la suficiente experiencia en otras regiones más habituales. La colocación de una tracción estable, los conocimientos anatómicos, la sistemática de los abordajes, la vía de agua accesoria y elinstrumental adecuado vuelven a ser, como siempre, losprotagonistas de una historia que se repite en cada articulación.Astrágalo. Con respecto a las lesiones del astrágalo, lasprincipales indicaciones de la artroscopia son el control de la reducción de los fragmentos en las fracturas articulares y el tratamiento de lesiones condrales y osteocondrales, tanto para su extirpación como para su osteosíntesis. Así mismo, es útil en el tratamiento de secuelas, como sinovectomías,limpiezas articulares o artrodesis.Tratamiento. A nivel del tratamiento o reparación de las lesiones osteocondrales del astrágalo, actualmente disponemos de diversas técnicas, como el desbridamiento y estimulación de la médula ósea, las técnicas de fijación del fragmento y los métodos de regeneración de nuevo cartílago hialino, entre los que se encuentran el autoinjerto osteocondral o mosaicoplastia, el aloinjerto y la implantación de condrocitos autólogos cultivados

Introduction. In the last few years, significant advances have been made in ankle arthroscopy as a result of improvements in technology and the surgical experience accumulated. It is currently possible to arthroscopically approach the ankle and the hindfoot bones in an attempt to address chondral injuriesof the talus. However, the technique requires a considerable learning curve and enough experience of other more usual regions. The creation of stable traction, the anatomical knowledge, the mastery of the different surgical techniques, the use of accessory portals and of the appropriate instruments are allprotagonists of a story that repeats itself no matter which of the joints we may want to approach.Talus. As regards talar injuries, the main indications for arthroscopy are controlling the reduction of fragments in joint fractures and the treatment of chondral and osteochondral lesions, with a view to both excision and osteosynthesis. Likewise, it is useful for the treatment of such sequelae as synovectomies, joint lavage or arthrodesis.Treatment. A series of techniques are currently availablefor the treatment or repair of osteochondral lesions of the talus, like debridement and bone marrow stimulation, as well as fragment fixation techniques and the new methods for new hyaline cartilage regeneration like osteochondral autografting or mosaicplasty, allografting and cultured autologous chondrocyte implantation

Bizarre parosteal osteochondromatous proliferation of the proximal humerus: case report.

Bizarre parosteal osteochondromatous proliferation (BPOP), or Nora's lesion, is a rare lesion of bone occurring predominantly in the long bones of the hands and feet. It exists as a puzzling clinical entity of uncertain origins and high recurrence rates after surgical resection. To our knowledge, this clinical entity has not been reported in the proximal aspect of the humerus. An interesting report of a lesion occurring in the proximal humerus, which initially was misinterpreted as a parosteal osteosarcoma, is discussed outlining the clinical, radiographic and pathologic features of the BPOP lesion.