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.

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.

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.

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)

Enchondroma of the hand: the role of biopsy in the course of diagnosis and treatment

Enchondroma is the most frequent bone tumor of the hand, but chondrosarcoma is rare at this location. There is a high possibility of correct diagnosis of enchondroma and differentiating from its malignant counterpart by precise clinical and radiologic assessment without biopsy, a subject of debate in the literature. At the present study we substantially investigate this problem, in our patients. Case records, radiographs, and histology of 52 solitary enchondroma patients who underwent operation in our hospital between 1998 and 2010, were reviewed. Special attention paid to pre and post -op diagnoses, and compared with each other. Eighty-six percent of our patients were between the second to fourth decades of life, with a slight female predominance. In all, the primary diagnosis of enchondroma according to clinical presentation and radi-ographic appearance, supported by intraoperative gross appearance of tumor, and confirmed histologically by permanent section analysis. There was no mismatch between radiologic and histologic diagnosis. we concluded that correct diagnosis of enchondroma is almost always possible by precise clinical and radiographic assessment with no need for histologic confirmation before definitive treatment

[Childhood soft tissue chondroma. two cases report]

Soft tissue chondroma is a rare benign tumour, which is generally seen in adult. It consists of islands of heterotopic cartilaginous tissue and most localised on the hands and the feet. The hypothesis that microtrauma is involved in the aetiology of this condition has yet to find any factual support. To report two paediatric cases of soft tissue chondroma. The first is a soft tissue chondroma of the posterior aspect of the left axilla in a 3-year-old boy. CT scans showed a spherical fatty density soft tissue mass without evident calcifications, attaching the infraspinous muscle. The second patient is a 9-year-old girl presented with a right auricular finger soft tissue chondroma. Radiographs showed several punctuated calcifications with adjacent bone scalloping. MRI revealed a lobulated soft tissue mass attaching the flexor tendons. The tumours were entirely removed. Histological examination showed cartilaginous tissue in both cases. At follow-up, the patients had good functions without evidence of recurrence. Simple excision should suffice to treat soft tissue chondroma but care should be taken to make the excision complete if recurrence are to be avoided

Soft tissue chondroma: a case report and literature review

Extra skeletal soft tissue chondroma is a very rare, slow progressing benign tumor. It has a specific tendency for hand and feet. It can sometimes be misdiagnosed as a synovial cyst or a more serious condition such as synovial sarcoma. Moreover, it can exhibit worrying radiologic and histological features mimicking chondrosarcomas. This study presents a very rare case of soft tissue chondroma of the foot. A 42 year old Egyptian male patient was presented with a slow developing swelling on the left foot for 7 years duration. The patient was examined thoroughly and proved to have a hard, fixed, non-tender swelling of approximately 4x 2.5 cm in size with well defined borders involving the dorsal aspect of the proximal left foot. The patient was investigated using the following techniques; plain X-ray, MRI, and FNAC of the swelling which gave an impression of a benign chondroma arising from the soft tissues of the dorsum of the left foot. The patient felt pain while walking and wearing shoes, and was therefore scheduled for excision of the mass under general anesthesia and limb exsanguinations at Ghayathi hospital. Postoperative histopathological assessment identified a soft tissue chondroma. There is limited literature and limited cases reported of soft tissue chondroma. So far, this is the second case of its kind to ever be reported at Ghayathi hospital

Giant chondroma of falx: a case report

Chondromas are benign tumors that may be found in many parts of the body. Among intracranial neoplasms, tumors of cartilaginous origin are rare. It has been shown that most of these tumors arise from the skull base. Their occurrence in other parts of the intracranial cavity is unusual. Chondroma of falx is a rare neoplasm. In this report we introduce a case of falcine chondroma arose from frontal midline area in a 17-year-old boy

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

Ressecçäo artroscópica de tumor na articulaçäo coxofemoral: relato de um caso / Arthroscopic resection of tumor in the coxofemoral joint: report of a case

Os autores apresentam uma indicaçäo para artroscopia do quadril com diagnóstico de condroma, confirmado por exame anatomopatológico. A indicaçäo do procedimento artroscópico se deu após realizaçäo de diversos exames e constataçäo, à ressonância magnética, de imagem anormal intra-articular na regiäo da fóvea. O objetivo era a biópsia e, se possível, ressecçäo artroscópica, para evitar-se, assim, artrotomia ampla e possibilitar reabilitaçäo mais rápida e menos traumática. Após seguimento aproximado de um ano, o paciente encontra-se assintomático

Osteocondromas del cóndilo mandibular: presentación de tres casos clínicos / Osteochondromas of the mandibular condyle: presentation of three cases

El osteocondroma es un tumor de naturaleza benigna que afecta, en el área maxilofacial, el cóndilo del maxilar, por ser este un centro de crecimiento con presencia de tejido cartilaginoso. No se conocen transformaciones malignas y las alteraciones que produce están relacionadas con disfunciones articulares. El tratamiento consiste en la resección de la masa tumoral y remodelado de la cabeza del cóndilo

Chondromyxoid fibroma: a study based on 18 cases

The authors report on 18 cases of chondromyxoid fibroma seen at the Institute of Orthopedics and Traumatology, University of Säo Paulo, from 1953 to 1990. A survery of the literature on chondromyxoid fibromas was conducted and radiographic analysis of all cases is presented, with a mean follow-up of 64 months. The surgical treatment employed in each case is discussed, with emphasis on the use of cement as an adjuvant. This procedure was used in 10 patients, who have been free of recurrence up to the present time

Tumores de la pared torácica / Tumors of the thoracic wall

Se presentan 16 pacientes portadores de tumores primitivos del esquelto torácico, de los cuales 5 fueron benignos y 11 malignos. Resultaron más frecuentes aquellos derivados del sector cartilaginoso: condromas y condrosarcomas. Se describe la evolución de un condroma que, pese a su benignidad histológica, provocó paraplejía irreversible. Es conveniente la biopsia preoperatoria con aguja o a cielo abierto, pero la negatividad no debe hacer variar el plan terapéutico. El tratamiento quirúrgico consiste en: 1) resección en bloque del tumor y costillas o cartílagos en forma parostal, con amplio margen de seguridad, y 2) cierre del defecto parietal Si los elementos propios de la pared torácica, escápula, costillas y músculos adyacentes, no confieren en la caja torácica estabilidad suficiente como para evitar la respiración paradójica, utilizamos como elemento protésico la malla de Marlex. La fascia de bovino empleada a tal efecto en un tumor de células gigantes, fue eliminada con una gran infección pleural. Consideramos una maniobra de gran utilidad la movilización de una costilla adyacente al defecto, siviendo de apoyo a las partes blandas que han de cubrirlo. En todos los casos se debe conseguir hemeticidad en el cierre de la cavidad pleural, a fin de mantener la presión negativa