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.

CT and Magnetic Resonance Imaging Findings of the Intracranial Extra-cerebral Chondroma:A Case Report / 中国医学科学院学报

Intracranial intradural chondroma is a rare disorder,the imaging findings of which have been rarely reported.The current study reported a case of intracranial extra-cerebral chondroma and described the detailed CT and magnetic resonance imaging findings,which would provide valuable imaging evidence for the diagnosis of intracranial extra-cerebral chondroma.

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.

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)