Condroma pulmonar y paraganglioma carotídeo como manifestaciones de la tríada de Carney: reporte de un caso / Pulmonary chondroma and carotid paraganglioma as expressions of Carney's triad: report of a case

Resumen Objetivos: Presentar caso clínico y revisión de la literatura sobre asociación de tumores poco frecuentes compatibles con diagnóstico de tríada de Carney. Paciente y Métodos: Revisión de ficha clínica de paciente de sexo femenino de 39 años de edad con antecedentes de asma, quien acude a servicio de urgencias por síntomas respiratorios. En estudio con imágenes se evidencia masa pulmonar en lóbulo superior derecho probablemente hamartoma y masa en la bifurcación carotídea izquierda compatible con posible paraganglioma. Se completó el estudio con endoscopia digestiva alta sin evidencia de tumor gástrico y PET-CT (tomografía de emisión de positrones-tomografía computarizada) que descartó otras lesiones. Resultados: La paciente fue sometida a resección quirúrgica de ambos tumores (pulmonar y carotídeo). En estudio histopatológico diferido, se plantean los diagnósticos de paraganglioma carotideo y hamartoma pulmonar, el cual, luego de una segunda revisión histopatológica, es corregido a condroma pulmonar. Discusión: La tríada de Carney se compone por la asociación de al menos 2 de 3 tumores: tumor estromal gastrointestinal (GIST), paraganglioma extra-adrenal y condroma pulmonar. Su expresión es variable, coexistiendo en forma completa en solo el 22% de los casos. Conclusión: Los pacientes con sospecha de tríada de Carney deben recibir evaluación multidisciplinaria, estudio completo en búsqueda de tumores asociados y seguimiento a largo plazo por posibles recurrencias o metástasis.

Objective: To present a clinical case and review of the literature on the infrequent association of pulmonary and extra thoracic tumors compatible with Carney's triad. Patient and Methods: Review of clinical records of a 39 years-old female patient with history of asthma who presented in the emergency department with respiratory symptoms. An imaging study showed a pulmonary mass in the right upper lobe with the aspect of hamartoma and a mass in the left carotid artery bifurcation compatible with a possible paraganglioma. Upper gastrointestinal endoscopy showed no evidence of gastric tumor and a PET-CT (Positron Emission Tomography - Computed Tomography) excluded other lesions. Results: Patient underwent surgical resection of both tumors (pulmonary and carotid). Diagnosis of carotid paraganglioma and pulmonary hamartoma were stated by histopathology. However, lung tumor after a second pathological analysis was confirmed to be a pulmonary chondroma. Discussion: Carney's triad is defined by the association of at least 2 of 3 tumors: Gastrointestinal Stromal Tumor (GIST), extraadrenal paraganglioma and pulmonary chondroma. Its expression is variable, coexisting completely in only 22% of cases. Conclusion: Patients with suspected Carney's triad should receive a multidisciplinary assessment, a complete study searching associated tumors and long-term follow-up for recurrences or metastases.

Expression of amphiregulin in enchondromas and central chondrosarcomas

OBJECTIVES: The aim of this study was to evaluate the role of amphiregulin protein, an epidermal growth factor receptor ligand, in cartilaginous tumors. METHODS: Amphiregulin expression was examined in 31 enchondromas and 67 chondrosarcomas using immunohistochemistry analysis. RESULTS: Overall, 15 enchondromas (48.40%) and 24 chondrosarcomas (35.82%) were positive for amphiregulin. According to the receiver operating characteristic curve test, no difference in amphiregulin expression was observed between enchondromas and low-grade chondrosarcomas (p=0.0880). Additionally, 39 lesions (16 in short bones, 13 in long bones, and 10 in flat bones) were positive for amphiregulin, exhibiting a higher percentage of positive cells (p=0.0030) and intensity of immunohistochemical expression (p=0.0055) in short bone lesions than in others. Among 25 enchondromas localized in short bones, 15 expressed amphiregulin; however, all 6 cases localized in long bones were negative for this marker (p=0.0177). CONCLUSIONS: Amphiregulin did not help in distinguishing enchondromas from low-grade chondrosarcomas. The present study is the first to document the expression of this immunohistochemical marker in enchondromas. Furthermore, amphiregulin expression in enchondromas was localized in short bones, indicating a phenotypic distinction from that in long bones. This distinction may contribute to an improved understanding of the pathogenesis of these lesions.

¿Qué debe saber el neurocirujano sobre condrosarcoma espinal primario? Condrosarcoma espinal primario / What should the neurosurgeon know about primary spinal chondrosarcoma? Primary spinal chondrosarcoma

El condrosarcoma ocupa el tercer puesto dentro de las neoplasias óseas primarias, siendo la columna vertebral una localización inusual. Según su etiología se clasifican en condrosarcoma primario o secundario a lesiones subyacentes de tipo cartilaginosa como el encondroma u osteocondroma; siendo entre el 80-90% de bajo grado. Pueden presentarse en cualquier nivel de la columna vertebral, siendo más frecuente en la región torácica y cervical, comprometiendo los elementos posteriores de la vértebra, los cuerpos vertebrales o ambos, en un 40%, 15% y 45% respectivamente. El síntoma más común es el dolor localizado asociado a manifestaciones neurológicas. El método diagnóstico de elección es la biopsia por punción y el tratamiento se basa fundamentalmente en la resección quirúrgica

Chondrosarcoma occupies the third position within the primary bony neoplasia's, with an unusual location at the level of the spine. According to their etiology, they are classified as primary chondrosarcoma or secondary to underlying cartilaginous lesions such as the enchondroma or osteochondroma, being between 80-90% of low grade. They can occur at any level of the spine, being more frequent in the thoracic and cervical region, compromising the posterior elements of the vertebra, the vertebral body or both, by 40%, 15% and 45% respectively. The most common symptom is localized pain associated with neurological manifestations. The diagnostic method of choice is biopsy and treatment is based primarily on surgical resection.

Enchondromas of the Hand: A 20-year Experience / Encondromas da mão: Uma experiência de 20 anos

Abstract Objective Enchondromas are the commonest tumors of the bones of the hand. Treatment approaches vary. The present article presents the characteristics of the tumors, diagnostic methods, and treatments. Methods We discuss the approach used in our institution, where we have treated 48 patients with enchondromas of the hand between 1996 and 2016. Our technique of treatment, which has remained the same over 2 decades, comprises the use of curettage, high-speed burr, and autologous bone graft (harvested with a minimally invasive technique, using a Craig biopsy needle). Results Pain and fractures were the most common symptoms leading the patients to consultation, at frequencies of 33.3% and 31.3%, respectively. A total of 27.1% of the cases were asymptomatic, and their lesions were discovered incidentally. The mean age was 34.4 years (SD = 12.9 years). Tumors were more frequently presented in the ulnar side of the hand, in the fifth ray (41.5%) and in the proximal bones (in the proximal phalanges [43.8%], and in the metacarpal [33.3%]). The size of the tumors ranged from 0.2 cm2 to 5.7 cm2, with a mean of 1.7 cm2 (standard deviation [SD] = 1.0 cm2) and were not associated with fracture (p = 0.291). Fracture was also not associated with any of the symptoms, neither with the age of the patients (p = 0.964). After the treatment, most patients achieved full range of motion (91.7%), with good integration of the bone graft. Three patients presented deficit in range of motion (6.3%) and the incidence of complications was also 6.3% (3 patients). At the end, after the needed surgical revisions, these three patients also recovered full function. They achieved full bone graft integration, regained full range of motion and returned to work. There was no tumor recurrence case during the follow-up period evaluated. For all cases, no donor site complications occurred. Conclusion Our method of treatment has consistently provided good outcomes, with only a few minor complications. Therapeutic level of evidence: IV.

Resumo Objetivo Os encondromas são os tumores mais comuns dos ossos da mão, com uma grande variedade de abordagens terapêuticas. O presente artigo apresenta as características dos tumores, métodos diagnósticos e tratamentos. Métodos Discutimos a abordagem da nossa instituição, onde tratamos 48 pacientes com encondromas da mão, entre 1996 e 2016. Nossa técnica de tratamento, que permanece a mesma ao longo de duas décadas, compreende o uso de curetagem, esmeril de velocidade e enxerto ósseo autólogo (retirado com uma técnica minimamente invasiva, usando uma agulha de Craig). Resultados A dor e as fraturas foram os sintomas mais comuns, levando os pacientes à consulta, nas frequências de 33,3% e 31,3%, respectivamente. Um total de 27,1% dos casos era assintomático, e suas lesões foram descobertas incidentalmente. A média de idade foi de 34,4 anos (desvio padrão [DP] = 12,9 anos). Os tumores foram mais frequentemente encontrados no lado ulnar da mão, no quinto raio (41,5%), e nos ossos proximais (nas falanges proximais [43,8%] e no metacarpo [33,3%]). O tamanho do tumor variou de 0,2 cm2 a 5,7 cm2, com média de 1,7 cm2 (DP = 1,0 cm2) e não foi associado à fratura (p = 0,291). A fratura também não foi associada a nenhum dos sintomas, e nem à idade dos pacientes (p = 0,964). Após o tratamento, a maioria dos pacientes alcançou amplitude completa de movimento (91,7%), com boa integração do enxerto ósseo. Três pacientes apresentaram déficit no arco de movimento (6,3%) e a incidência de complicações também foi de 6,3% (3 pacientes). No final, após as revisões cirúrgicas necessárias, esses três pacientes também recuperaram a função completa. Eles tiveram a integração total do enxerto ósseo, recuperaram toda a amplitude de movimento e retornaram ao trabalho. Não houve nenhum caso de recorrência do tumor durante o período de acompanhamento avaliado. Em nenhum dos casos ocorreram complicações no local doador. Conclusão O nosso método de tratamento forneceu consistentemente bons resultados, com apenas algumas complicações menores. Nível terapêutico de evidência: IV.

Tumores óseos benignos osteolíticos / Benign osteolytic bone tumors

INTRODUCCIÓN: Los tumores óseos primarios son raros. La localización, extensión y el carácter lítico dificultan el tratamiento aunque el comportamiento biológico sea benigno. OBJETIVO: Valorar las características y tratamientos de los tumores óseos benignos primarios líticos activos o agresivos tratados en el Hospital El Cruce. Comparar la resección realizada con la sugerida por los criterios de Enneking. MATERIAL Y MÉTODOS: se evaluaron historias clínicas y ateneos postquirúrgicos de pacientes con diagnóstico de tumores músculoesqueléticos atendidos en el Servicio de Ortopedia y Traumatología del Hospital El Cruce desde agosto de 2008 hasta junio de 2017. Se utilizaron los criterios de Ennekingde comportamiento biológico y resección. RESULTADOS: Se evaluaron 26 pacientes con diagnóstico de tumor benigno lítico activo o agresivo sobre un total de 124 tumores musculoesqueléticos primarios operados. Un paciente se reclasificó como maligno en la resección completa, por lo que se consideraron 25. Se trató de 16 mujeres, 9 varones, edad media 28,4.La localización fue: 13 miembro inferior (1coxal, 7 fémur, 3 tibia y 2 calcáneo), 9 miembro superior (1 húmero, 2 radio, 2 cúbito, 3 metacarpianos y 2 falanges) y 3 columna (1 cervical, 1 torácica y 1 sacra). Un paciente tuvo localización múltiple en la mano. Según Enneking 10 eran activos y 13 agresivos. Histología: 12 Tumores de células gigantes (TCG), 5 condromas, 4 quiste óseo simple, 3 quiste óseos aneurismáticos y 1 hemangioma. La resección fue intralesional en 16, en 3 marginal y en 6 amplia (1 amputación). La media de días de estada fue 7 días (0 a 33), 5 internación en UTI. La reconstrucción se hizo con: Injerto de Banco 7, Injerto de cresta 7, Injerto M.sup 3, Prótesis 2, Peroné vascularizado 2, Sustituto óseo y cemento en 1. Sin reconstrucción en 2. Dos tuvieron adyuvancia con denosumab. Once tuvieron complicaciones. Hubo un óbito. En 18/25 la resección realizada coincidió con los criterios de resección (Enneking). CONCLUSIONES: A pesar de la benignidad histológica, los tumores líticos primarios requirieron procedimientos complejos, con un 40 % de complicaciones. En el 72% la resección realizada concordó con los criterios de resección de Enneking.

INTRODUCTION: Primary bone tumors are rare. The localization, extension and lytic character make the treatment difficult even though the biological behavior is benign. OBJECTIVES: Assess the characteristics and treatments of active or aggressive benign primary lytic bone tumors treated in the El Cruce hospital. Compare the resection made with the one suggested by Enneking criteria. MATERIALS AND METHODS: Clinical records and postsurgical grand rounds were evaluated on patients diagnosed with musculoskeletical tumors treated in the Orthopedics and Traumatology Service of Hospital El Crucefrom August 2008 to June 2017. Enneking's criteria based on biologic behavior and resection were used. RESULTS:Twenty-six patients diagnosed with active or aggressive lytic bone tumor were evaluated out of a total of 124 operated primary musculoskeletal tumors. A patient was reclassified as malignant on the complete resection, therefore 25 were considered. There were 16 women, 9 men; average age was 28.4.The tumor localization was: 13 in lower extremity (1 coxal, 7 femoral, 3 tibial and 2 in calcaneus), 9 in upper extremity (1 humeral, 2 radial, 2 ulnar, 3 metacarpal y 2 in phalanges) and 3 in spine (1 cervical, 1 thoracic y 1 sacral). A patient had multiple localizations in his hand. Based on Enneking, 10 were active and 13 were aggressive. Histology: 12 giant-cell tumors (GCT), 5 chondromas, 4 simple bone cysts, 3 aneurysmal bone cysts and 1 hemangioma. The resection was intralesional in 16, marginal in 3 and ample in 6 (1 amputation). Mean hospitalization stay was 7 days (0 to 33), 5 days in ICU. The reconstruction was made with: draft from banks in 7, drafts from crests in 7, draft from upper extremity in 3, prosthesis in 2, vascularized fibula in 2, bone substitute and cement in 1. No reconstruction in 2.Two had adjuvant therapy with denosumab. Eleven had complications. There was one death. In 18 out of 25, the resection matched the resection criteria (Enneking). CONCLUSIONS: Despite the histological benignity, primary lytic tumors required complex procedures and there were 40% complications. In72%, the resection matched the resection criteria (Enneking).

Condroma del espacio parafaríngeo, reporte de un caso / Chondroma of the parapharyngeal space, report of one case

Introducción. El condroma es una neoplasia benigna de origen mesenquimal y de etiología desconocida, muy infrecuente en los tejidos blandos debido a su constitución de células de cartílago maduro sin hueso; sin embargo, son frecuentes las calcificaciones focales. Al presentarse en cabeza y cuello, suele hacerlo en el maxilar o el paladar duro, y son pocos los reportes de este tumor en el espacio parafaríngeo. Se reporta el caso de una paciente de 68 años de edad, que acudió a consulta por dolor en el paladar blando, cefalea y dolor paratiroideo izquierdo asociado a disfagia de cuatro años de evolución. Se practicaron los estudios pertinentes y, finalmente, se confirmó el diagnóstico de condroma mediante biopsia. Discusión. Los condromas muy rara vez se encuentran en los tejidos blandos, por lo cual se sugiere que el diagnóstico sea cuidadosamente orientado para descartar una posible malignidad de la lesión y que los controles posquirúrgicos sean frecuentes

Introduction: Chondroma is a benign neoplasm of mesenchymal origin and of unknown etiology, very infrequently appearing in the soft tissues due to its constitution of mature cartilage cells without bone; however, they usually present focal calcifications. When occurring in the head and neck, they usually appear in the jaw or hard palate. Just a few cases of this pathology are reported in the pharynx. Materials and Methods: We present a case of a female 68 years old, who came to the clinic due to pain in the soft palate, headache and left parathyroid pain associated with dysphagia, of four years of evolution. Corresponding studies were carried out and finally the diagnosis of chondroma was confirmed by biopsy. Discusion: Chondromas are rarely found in the soft tissues, suggesting that the diagnosis should be carefully oriented to rule out possible malignancy of the lesion and that post-surgical controls should be frequent

Tríada de Carney. Una rara asociación de tumores infrecuentes: caso clínico / Carney triad: report of one case

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Tumor condroide del cartílago tiroides: reporte de un caso clínico y revisión de la literatura / Chondroid tumor of the thyroid cartilage: case report and literature review

Los tumores condroides de la laringe son poco frecuentes, y generalmente se ubican en el cartílago cricoides. El tratamiento de elección es quirúrgico, con buen pronóstico general. Se presenta el caso de un hombre de 60 años con historia de disfonía. El estudio nasofaringolaringofibroscópico revela una masa laríngea supraglótica. Las imágenes son compatibles con una lesión del cartílago tiroides. La biopsia obtenida por microlaringoscopía directa informa tumor compatible con condroma. Se realiza una resección completa de la masa por abordaje externo, la biopsia corrobora el diagnóstico de tumor condroide de bajo grado. Se describe esta patología mediante revisión bibliográfica.

Chondroid tumors of the larynx are uncommon, and usually located in the cricoid cartilage. Surgery is the treatment of choice, with good prognosis in general. We report the case of a 60-year-old man consulting for dysphonia. The nasopharyngolaryngoscopy showed a supraglottic laryngeal mass. The images were compatible with a thyroid cartilage lesion. The biopsy sample obtained by direct microlaryngoscopy was consistent with a condroma. A complete excision of the lesion was performed by external approach and the biopsy confirmed the diagnosis of a low grade chondroid tumor. We present a review of chondroid tumors of the larynx based on available literature.

Encondroma solitario de la mano: seguimiento a largo plazo después del tratamiento quirúrgico. serie de casos / Isolated enchondroma of the hand. long-term follow-up after surgical treatment. case series

Introducción: los encondromas solitarios son los tumores óseos más comunes de la mano. Aproximadamente el 40% compromete esta extremidad, con predilección por los rayos cubitales y las falanges proximales. El riesgo de transformación maligna a condrosarcoma es del 1% y el de recurrencia, del 2-15%. Se describen la experiencia y los resultados de un importante número de pacientes con un seguimiento a largo plazo. Materiales y Métodos: Estudio descriptivo, retrospectivo, de tipo serie de casos. Se seleccionaron pacientes con diagnóstico confirmado de encondroma solitario de la mano, que fueron manejados mediante resección con curetaje del tumor más aplicación de injertos autólogos de cresta ilíaca o radio. Después del tratamiento quirúrgico, se evaluaron la función, mediante la clasificación de Takigawa, los resultados radiográficos y la frecuencia de complicaciones o recidivas. Resultados: Se incluyeron 19 pacientes con un seguimiento posoperatorio promedio de 11 años. De acuerdo con la clasificación de Takigawa, la función fue excelente en 16 pacientes (84,2%) y buena en 3 (15,7%). Un paciente presentó una infección del sitio operatorio incisional superficial, con resolución completa. No hubo recidivas. Conclusiones: En los pacientes evaluados, los resultados funcionales y radiológicos fueron buenos después de la cirugía. Los encondromas plantean el riesgo de fracturas patológicas, recidiva y, en menor proporción, de transformación maligna; sin embargo, esto no ocurrió en ninguno de los casos. Se describe una opción de manejo quirúrgico, sencilla con buenos y excelentes resultados a largo plazo.

Introduction: Isolated enchondromas are the most common tumors of the hand. Approximately 40% affect this upper extremity, with preference for cubital rays and proximal phalanges. Risk of malignant transformation to chondrosarcoma is 1% and the risk of recurrence ranges from 2 to 15%. Our experience and results with a large number of patients with a long-term follow-up are described. Methods: Descriptive, retrospective, case series study. Patients with diagnosis of isolated enchondroma of the hand that were treated with tumor resection, curettage and iliac crest or radius autograft placement were included. After surgical treatment, hand function was assessed using the Takigawa classification, radiological results and frequency of complications or recurrence were evaluated. Results: Nineteen patients with a mean follow-up of 11 years were included. According to the Takigawa classification, function was excellent in 16 patients (84.2%) and good in 3 (15.7%). One patient had an incisional superficial surgical site infection that resolved completely. No cases of recurrence were identified. Conclusions: Good functional and radiological results were obtained after surgical treatment. Enchondromas can pose the risk of pathological fractures, recurrence and, in a lesser degree, malignant transformation; however, none of these complications were identified. The results of a simple surgical treatment option with good and excellent long-term results are described.

Síndrome incompleto de Carney / Incomplete Carney's triad

El síndrome de Carney es una enfermedad rara, con diferentes formas de presentación, que afecta principalmente al sexo femenino y es asintomática en la mayoría de los casos. Se caracteriza por la aparición de tumores, generalmente benignos, principalmente en el pulmón, la glándula suprarrenal y el estómago; sin embargo, se pueden afectar otros órganos como el esófago. Su tratamiento es principalmente quirúrgico. Se presenta el caso de una paciente con síndrome incompleto de Carney, manejada quirúrgicamente, con excelente resultado posoperatorio. Se hace una revisión de la literatura científica actual.

Carney's triad is a rare condition with multimodal presentations, which affects mainly females and remains asymptomatic in most cases. It is characterized by the appearance of tumors, usually benign, in lung, adrenal, and stomach, but it can also affect other organs like the esophagus. Treatment of these lesions is primarily surgical. The objective of this paper is to review the current literature and to report a case of an incomplete Carney's triad managed surgically with excellent postoperative results.

Tumor condrolipomatoso (condrolipoma) de mama / Chondrolipoma of the breast

Introduction: Cartilage is an uncommon component in breast lesions, most of cases it correspond to metaplasia of malignant neoplasm and its occurrence in benign tumors is exceptional. Cartilage- containing breast benign tumors has been subclassified by their majors features accord to each author, but their main composition remains to be adipose tissue, fibrous stroma and cartilage, with or without breast epithelium. Chondrolipoma of the breast was reported for the first time by Kaplan in 1977, and since then reports of this entity has been anecdotal with less than 20 cases published. Clinical case: We expose the first case diagnosed of chondrolipoma of the breast in Colombia, performed on the Pathology Department of Universidad Industrial de Santander (Bucaramanga-Colombia) along with a literature review, due to the low frequency of this diagnosis and the few information about it.

Introducción: El cartílago es un componente poco común en las lesiones de mama, la mayoría de casos corresponden a metaplasias de lesiones malignas siendo excepcional el hallazgo en lesiones benignas; estas últimas se han subclasificado de acuerdo a las características histológicas destacables para cada autor, pero globalmente se componen de tejido adiposo, estroma fibroso y cartílago, mientras que el componente epitelial mamario es de presentación variable. El condrolipoma de mama fue reportado por primera vez en 1977 por Kaplan y desde esa fecha los reportes han sido anecdóticos con menos de 20 casos publicados. Caso clínico: Se expone el primer caso diagnosticado en Colombia de condrolipoma de mama, diagnóstico efectuado en el Departamento de Patología de la Universidad Industrial de Santander (Bucaramanga-Colombia) y se hace una revisión de la literatura de esta entidad, dada la baja frecuencia de su diagnóstico y a la escasa información al respecto.

Chondroma of the Cerebral Falx: Case Report of a Very Rare Intracranial Tumor / Condroma da foice do cérebro: relato de caso de um tumor intracraniano muito raro

Chondromas are a benign cartilaginous tumor that account for approximately 0.5% of primary intracranial tumors. They usually arise from the synchondrosis of the skull base, being rarely reported elsewhere (dura convexity, falx, or even intraparenchymal). Because they are very rare tumors sharing clinical behavior and imagiological similarities with the much more common meningioma, the clinical diagnosis is frequently mistaken after an initial imaging workup. The authors present a case of a 48-year-old woman, with frequent headaches and an extra-axial tumor arising from the anterior falx, initially diagnosed as a meningioma.

Condromas são tumores cartilaginosos benignos que correspondem por aproximadamente 0,5% dos tumores primários intracranianos. Eles geralmente surgem da sincondrose da base do crânio, sendo raramente registrados em outros locais como a convexidade dural, a foice, até mesmo no parênquima cerebral. Por serem tumores muito raros que compartilham comportamento clínico e imagiológico com meningiomas, o diagnóstico é frequentemente confundido após exame de imagem inicial. Os autores apresentam o caso de uma mulher de 48 anos, com cefaleia frequente e um tumor extra-axial ascendendo da foice anterior, inicialmente diagnosticado como um meningioma.

Condroma de partes moles relato de caso e revisão da literatura / Soft tissue chondroma case report and review of the literature

O presente estudo tem o objetivo de apresentar um relato de caso sobre condroma extra-esquelético ou de partes moles, uma lesão rara e benigna diagnosticada através da clínica, radiologia e exame histopatológico. O paciente (24 anos) compareceu ao Setor de Ortopedia do Hospital Unimed Betim com queixa de aumento de volume no polegar esquerdo, indolor há aproximadamente um ano. Durante o exame físico verificou-se presença de uma lesão nodular, de consistência dura, móvel, indolor que não acarretava em alteração funcional do dedo. O paciente foi encaminhado para setor de radiologia do hospital onde foi realizado RX e posteriormente ultrasonografia e ressonância magnética do polegar esquerdo. Diante dos achados dos estudos de imagem a equipe optou pela biópsia excisional da lesão. O exame histopatológico confirmou o diagnóstico de condroma extra-esquelético. Os elementos definidores do condroma de partes moles são: 1) curso clinico benigno e sintomatologia frusta; 2) ausência de conexão entre o tumor e estruturas adjacentes como periosteo, capsula articular ou osso; 3) crescimento lento; 4) ausência de prevalência por sexo; 5) características radiológicas e histológicas do tumor. O diagnóstico raramente é aventado quando confrontamos tumores de partes moles das mãos. A excisão é simples e deve ser completa para se evitar recorrências. A avaliação histopatológica deve ser realizada para confirmação diagnóstica.

The present study presents a case report of extraskeletal chondroma or soft tissue chondroma, a rare and benign lesion, diagnosed by clinical findings, imaging studies and histopathological exam. The 24-year-old patient presented at the Orthopedics Section of Unimed Betim Hospital with a slow painless growth on the left thumb about 1 year ago. During the physical examination, a nodular lesion was noted; it had a hard consistency, was mobile and painless, and didn't cause functional impairment for the finger. The patient was referred to the imaging unit of the hospital, where an X-ray was performed; later on, ultrasound and magnetic resonance imaging of the left thumb was done. After the imaging studies, the medical team chose excisional biopsy of the lesion. The histopathological examination confirmed the diagnosis of extra-skeletal chondroma or soft-tissue chondroma. Soft tissue chondroma is usually not considered for a soft-tissue tumor of the hand. Excision is easy and must be complete if recurrences are to be avoided. Histopathological evaluation is required for the final diagnosis.

Condroma de la glándula parótida / Parotid gland chondroma

El condroma es una neoplasia benigna, de origen mesenquimatoso, constituida por tejido cartilaginoso, que en el área maxilofacial es rara. Su sitio más frecuente en el complejo craneofacial es la estructura ósea maxilar o mandibular, aunque también se han descrito condromas de los tejidos blandos. Clínicamente se presenta como una lesión de crecimiento lento, asintomático, no dolorosa, de consistencia duro elástica, con una gradual expansión. El tratamiento de elección es la resección quirúrgica. Se tuvo como objetivo publicar esta infrecuente ubicación de la neoplasia por la dificultad que se presenta al diagnóstico diferencial. Presentamos un caso clínico de un tumor localizado en el lóbulo profundo de la glándula parótida, cuyo diagnóstico inicial, tras la citología por aspiración con aguja fina, fue de adenoma pleomorfo, lo que se comprobó luego por examen histopatológico de la pieza quirúrgica que se trataba de un condroma. Se concluye que ante una tumoración de parótida, siempre debe tenerse en cuenta el condroma en el diagnóstico diferencial(AU)

The chondroma is a benign tumor of mesenchymal origin, consisted of a cartilaginous tissue, and occurs rarely in the maxillofacial area. Its most frequent site in the craniofacial complex is the maxillary bony structure, although chondromas of the soft tissues have also been described in the literature. From the clinical viewpoint, it appears as a painless, hard elastic lesion of slow asymptomatic growth, with gradual expansion. The treatment of choice is surgery. This paper presented a clinical case of a tumor located in the deep lobe of the parotid gland. The initial diagnosis, after the fine needle aspiration test, was pleomorphic adenoma, which was then subjected to histopathologic testing to obtain the final diagnosis of chondroma. Concluding that although uncommon in this localization, it should always be kept in mind as possible diagnosis. It was concluded that when facing an uncommon parotid tumor, one must take into account the possibility of a chondroma in the differential diagnosis(AU)

Analysis of angiogenic factors and cyclooxygenase-2 expression in cartilaginous tumors: clinical and histological correlation

OBJECTIVES: To study the role of angiogenesis and cyclooxygenase-2 expression in cartilaginous tumors and correlate these factors with prognosis. INTRODUCTION: For chondrosarcoma, the histological grade is the current standard for predicting tumor outcome. However, a low-grade chondrosarcoma can follow an aggressive course-as monitored by sequential imaging techniques-even when it is histologically indistinguishable from an enchondroma. Therefore, additional tools are needed to help identify the biological potential of these tumors. The degree of angiogenesis that is induced by the tumor could assist in this task. Angiogenesis can be quantified by measuring the expression of vascular endothelial growth factor and CD34, and cyclooxygenase-2 can induce angiogenesis by stimulating the production of proangiogenic factors. METHODS: In total, 21 enchondromas and 58 conventional chondrosarcomas were studied by examining the clinical and histopathological findings in conjunction with the immunostaining markers of angiogenesis and cyclooxygenase- 2 expression. RESULTS: The significant variables that were associated with poor outcome were 1) higher-grade chondrosarcomas, 2) tumors that developed in flat bones, and 3) over-expression of CD34 (with a median count that was higher than 5.9 vessels in 5 high power fields). Moreover, CD34 expression (measured using the Chalkley method) revealed significantly higher microvessel density in flat bone chondrosarcomas. DISCUSSION: Previous studies have shown a positive correlation between Chalkley microvessel density and histological grade; however, in our sample, we found that the former is predictive of the outcome. Chondrosarcomas in flat bones have been shown to correlate with a poor prognosis. We also found that CD34 microvessel density values were significantly higher in flat-bone chondrosarcomas. This could explain-at least in part-the more aggressive biological course that is taken by these tumors. CONCLUSIONS: These results provide evidence that CD34 microvessel density in chondrosarcomas can be helpful in predicting patient outcome and may add to our understanding of chondrosarcoma pathogenesis.

Encondroma solitario de fémur / Femoral solitary enchondroma

El encondroma solitario es un tumor cartilaginoso de naturaleza benigna, habitualmente asintomático, que se presenta entre la tercera y cuarta década de la vida. Se reporta a continuación un caso de una mujer de 60 años con encondroma solitario en fémur izquierdo, confirmando su diagnóstico mediante una resonancia nuclear magnética.

Solitary enchondroma is a benign cartilaginous tumor, usually asymptomatic, presents between the 3 and 4 decade. We report a 60 years old woman with solitary enchondroma in left femur, confirming the diagnosis with magnetic resonance imaging.

Condroma de cartilagem cricóide / Chondroma of the cricoid cartilage

Introdução: Os tumores cartilaginosos da laringe são raros, compreendendo 1% de todos os tumores cartilaginosos. O condroma é tumor benigno mais comum acometendo a cartilagem cricóide da laringe (75%), manifestando-se comumente no gênero masculino, com disfonia, dispnéia progressiva e disfagia em alguns casos. Objetivo: O presente estudo tem como objetivo relatar um caso de condroma de cartilagem cricóide, em paciente com sintoma de lesão nodular em região cervical anterior, de crescimento lento e progressivo. Relato do Caso: O tratamento foi a laringectomia parcial modificada, com ressecção do hemisegmento inferior da cartilagem tireóide, hemicartilagem cricóide e primeiro anel traqueal com margens livres e reconstrução com de retalho de pericôndrio e muscular pré-tireoideano. O exame anátomo-patológico demonstrou condroma de 1,1 cm, de baixa celularidade e baixas figuras de mitose atipicamente na região anterior da cartilagem cricóide. Conclusão: Neste relato concordamos com a literatura para o tratamento primariamente cirúrgico, de extensão dependendo da localização e do tamanho do condroma de cricóide, porém outras modalidades de tratamento podem ser adotadas nos casos onde a extensão tumoral indique a laringectomia total ou quando esta não é passível de realização, visando à preservação da laringe. Para o tratamento adequado de condromas de cricóide e entendimento da evolução natural da doença mais relatos de casos ainda são necessários.

Introduction: The larynx cartilaginous tumors are uncommon and comprise 1% of all cartilaginous tumors. The chondroma is the most common benign tumor affecting the larynx cricoid cartilage (75%), and manifests normally in the male gender with dysphonia, progressive dyspnea and dysphagy in some cases. Objective: The objective of this study is to report a case of cricoid cartilage chondroma, in a patient with the symptom of a nodular lesion in the frontal cervical region of slow and progressive growth. Case Report: The treatment was the modified partial laryngectomy with resection of the lower hemisegment of the thyroid cartilage, cricoid hemicartilage and the first tracheal ring with free margins and reconstruction with a pericondrium and muscular prethyroidean piece. The anatomopathological exam showed a chondroma of 1.1 cm, of atypical low cellularity and low figures of mitosis in the frontal region of the cricoid cartilage. Conclusion: In this report we agreed with the literature for the primarily extensive surgical treatment depending on the location and the size of the cricoid chondroma; however, other modalities of treatment may be adopted in cases where the tumor extension appoints a total laryngectomy or when this is not possible to carry out, aiming at the preservation of the larynx. For the suitable treatment of cricoid chondromas, the understanding of the disease natural evolution and more case reports are still necessary.

Condroma de partes blandas / Condroma of soft parts

Se presenta un caso de condroma extra esquelético de pabellón auricular, en un paciente de sexo masculino de 11 años de edad, cuyo inicio fue dos años antes de la consulta en 1999, permaneciendo estable hasta la actualidad. Se consideran los diferentes diagnósticos diferenciales clínicos e histológicos, características evolutivas y el tratamiento del tumor.