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.

Reparación artroscópica transósea sin anclajes del manguito rotador con encondroma del húmero proximal. Reporte de un caso / Arthroscopic transosseous repair without anchorage of the rotator cuff with enchondroma of the proximal humerus. Case report

A 55-year-old woman presented to our service with chronic pain in her right shoulder with no history of trauma. Shoulder x-ray and CT scan revealed an enchondroma of the proximal humerus with no associated fracture. The MRI also showed a complete rupture of the supraspinatus and infraspinatus tendons. An arthroscopic transosseous rotator cuff repair without anchors was performed. At 12 months of follow-up, the patient had recovered functionality and the enchondromatous lesion remained stable. Anchorless transosseous arthroscopic repair of a rotator cuff tear may represent a good surgical option for patients whose bone quality makes anchor placement inappropriate, such as in the presence of a benign bone tumor such as enchondroma. Level III of evidence: Level IV; case report.

Encondroma: a propósito de un caso / Enchondroma: a case report

RESUMEN Fundamento: los tumores óseos son lesiones frecuentes, cada tipo histológico tiene patrones típicos que los identifican. Sin embargo, en ocasiones se pueden presentar de forma atípica. Objetivo: conocer el caso de una paciente con encondroma en la región proximal de la tibia derecha. Presentación del caso: mujer de 74 años de edad, blanca con antecedentes de hipertensión arterial y asma bronquial, la cual acude a la consulta de Ortopedia y Traumatología por dolor a nivel de la rodilla derecha que aumenta con la actividad física y se acompaña de limitación funcional y crepitación articular. La tomografía axial computarizada mostró imagen osteoblástica a nivel de la meseta tibial derecha sin ruptura de la cortical. Además se evidenció múltiples quistes subcondrales de aspecto degenerativo, presencia de osteofitos tibiales, femorales ipsi y contralateral, espacio femoro-rotuliano muy disminuido asociado a esclerosis del cóndilo femoral. Con los elementos descritos se decidió llevar la paciente al quirófano, para tratamiento de tipo quirúrgico. Se realizó exéresis de la tumoración, la que fue enviada al departamento de anatomía patológica para estudio histológico que confirmó el diagnóstico. La zona de hueso extraída se llenó con cemento quirúrgico. Conclusiones: el encondroma es un tumor benigno de origen cartilaginoso, su presencia por encima de los 40 años de edad es ocasional y constituye el principal diagnóstico diferencial del condrosarcoma de bajo grado histológico. Debido a lo infrecuente de esta afección fuera de su rango de edades, es necesaria la biopsia para confirmar el diagnóstico.

ABSTRACT Background: bone tumors are common lesions, there are specific features related to each histological type, but unusual and atypical presentations do occur. Objetive: to show the case of a patient with an enchondroma in the proximal right tibial plateau. Case report: a 74 year old white woman, who had had previous personal history of hypertension and asthma is taken to the Orthopaedic and Traumatology outpatient department complaining of right knee pain which gets worse with physical activity associated to limited range of motion and articular crepitus. CT scan showed an osteoblastic lesion in the proximal upper right tibial plateau without cortical breaking. On the other hand, multiple subchondral degenerative cysts were found in the knee joint as well as osteophytes, sclerosis and joint space narrowing. Taken into account the entire previous patient' features, surgery was performed and consist of tumor resection with bone cement filled. The specimen was send to the pathology department showing enchondroma. Conclusions: enchondroma is a benign cartilaginous bone tumor unfrequently found over 40 years of age, the main differential diagnosis is low grade chondrosarcoma. Because of the infrequent presentation of this case in regards to age, surgery was warranted to confirm diagnosis.

¿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.

Hidrocefalia asociada a un condroma vertebral lumbar: presentación de un caso / Hydrocephalus associated with a lumbar vertebral chondroma: presentation of a case

RESUMEN Fundamento: los osteocondromas son tumores benignos del hueso que se localizan por lo general, en esqueleto axial y su vínculo con hidrocefalia es inusitado dadas las características fisiopatológicas de ambas enfermedades. Objetivo: presentar un caso operado de condroma vertebral lumbar que desarrolló una hidrocefalia. Presentación del caso: paciente de 43 años de edad, masculino, que acudió con manifestaciones neurológicas de compresión radicular lumbar que no aliviaban con tratamiento sintomático. Se operó por vía lumbar posterior y se realizó laminectomía estándar con resección quirúrgica de la lesión, se confirmó el diagnóstico de un condroma y un mes posterior a la excéresis apareció una hidrocefalia. Conclusiones: los condromas espinales de localización lumbar son infrecuentes y la aparición de hidrocefalia en el postoperatorio de esta lesión es más inusual.

ABSTRACT Background: osteochondromas are benign tumors of the bone that are located, generally, in axial skeleton and their association with hydrocephalus is infrequent due to physiopathologic characteristics of both diseases. Objective: to present an operated case of lumbar vertebral chondroma that developed a hydrocephalus. Case report: a 43-years-old male patient who came with neurological manifestations of lumbar radicular compression that was not relieved with symptomatic treatment. The surgical resection of the lesion is performed and the diagnosis of a chondroma is confirmed and hydrocephalus appears one month after the surgery. Conclusions: the spinal chondromas of lumbar location are infrequent and the appearance of hydrocephalus in the postoperative period of this lesion is more unusual.

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

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.

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.

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)

Maffucci’s Syndrome: report of a case with oral manifestation / Síndrome de Maffucci: relato de caso com manifestação oral

Introduction: Maffucci’s Syndrome is a rare nonhereditary mesodermal dysplasia consisting of multiple haemangioma of the soft tissue and enchondromas, mostly affecting phalanges and long bones. The syndrome can also be associated with a variety of other benign and malignant tumors. Case report: Here we report a case of Maffucci’s Syndrome and haemangioma of lip and palate which is rare in this syndrome. This case report describes an 18 year old boy with multiple nodular soft tissue swellings involving the anterior hard palate and lower labial mucosa and bony abnormalities (enchondromas) involving the lower limb.

Introdução: A Síndrome de Maffucci é uma displasia mesodermal não hereditária rara. Consiste de múltiploshemangiomas de tecido mole e encondromas, a maioria afetando as falanges e os ossos longos. A síndromepode também estar associada a uma variedade de tumores benignos e malignos. Relato de caso: O presente trabalho apresenta um caso de Síndrome de Maffucci e hemangioma de lábio e palato, o que é raro nessa síndrome, descrevendo o caso de um garoto de 18 anos de idade com inchaços nodulares múltiplos de tecido mole envolvendo o palato duro anterior e a mucosa labial inferior, além de anomalias ósseas (encondromas)acometendo um membro inferior.

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.

Condromalacia de rótula. Incidencia de algunos aspectos clínicos, terapéuticos y epidemiológicos / Patellar chondromalacia. Behavior of some clinical, therapeutical and epidemiological aspects

Se realizó un estudio explicativo en un número de trece pacientes diagnosticados y tratados por condromalacia de rótula mediante artroscopia en el Hospital Provincial Docente Manuel Ascunce Domenech de nuestra ciudad. Se encontró una mayor incidencia en el sexo femenino con un 61, 5 %. El grupo de edades con mayor afección fue el de más de 45 años para un 38, 4 %. Predominó la condromalacia grado II en el 46, 1 %. Las lesiones de menisco fueron las afecciones asociadas más frecuentes en un 38, 4 %. El índice de complicaciones en este proceder es muy bajo con solo un 15, 3 %.

An explicative study was carried out in a member of thirteen patients diagnosed and treated for patellar chondromalacia by arthroscopy at Manuel Ascunce Domenech Provincial Hospital of our city. It was found a higher incidence in the femenine sex with 61, 5 %. The age group most afeccted was over 45 years for 38, 4 %. Chondroolacia grade II prevailedin 46, 1 %. Meniscus injuries were most frequent associated pathologies in 38, 4 %. Complication index in this procedures is very low with only 15, 3 %.