ABSTRACT
La aparición de tumoraciones en Pediatría es una causa de gran angustia familiar. En concreto, las masas esternales son entidades poco frecuentes pero que se deben conocer. Presentamos el caso de un paciente con aparición súbita de una tumoración a nivel esternal. Tras realizar anamnesis y exploración física se llevaron a cabo las siguientes exploraciones complementarias: análisis sanguíneo, radiografía de tórax y ecografía de la lesión. Mediante estos procedimientos, se diagnosticó al paciente de una entidad denominada tumor esternal autolimitado de la infancia o SELSTOC (self limiting sternal tumors of childhood) por sus siglas en inglés. Es aconsejable reconocer esta entidad dado que se trata de un proceso benigno que no requiere de la realización de pruebas cruentas y que permite transmitir tranquilidad a los familiares por su buen pronóstico (AU)
The appearance of tumors in pediatric patients is a cause of great family distress. Specifically, sternal masses are infrequent entities, but they should be known. We present the case of a patient with sudden onset of a sternal tumor. After anamnesis and physical examination, the following complementary examinations were performed: blood analysis, chest X-ray and ultrasound of the lesion. By means of these procedures, the patient was diagnosed with an entity called self-limiting sternal tumor of childhood or SELSTOC (self-limiting sternal tumors of childhood). It is advisable to recognize this tumor since it is a benign process that does not require invasive tests and that allows us to reassure family members of its good prognosis. (AU)
Subject(s)
Humans , Male , Infant , Bone Neoplasms/diagnostic imaging , Sternum/diagnostic imaging , Neoplasm Regression, Spontaneous , Conservative TreatmentABSTRACT
Rhabdomyosarcoma is the most common soft-tissue sarcoma in children; it can appear in any part of the body. Its biological behavior varies widely, and despite the absence of specific clinical or radiological characteristics, rhabdomyosarcoma should be taken into account in the differential diagnosis of solid tumors in children. This review focuses primarily on the imaging findings and anatomical distribution of the histological subtypes of childhood rhabdomyosarcoma and secondarily on the differential findings in histological studies.
Subject(s)
Rhabdomyosarcoma, Embryonal , Child , Humans , Rhabdomyosarcoma, Embryonal/classification , Rhabdomyosarcoma, Embryonal/diagnostic imaging , Rhabdomyosarcoma, Embryonal/pathologyABSTRACT
BACKGROUND: Nail-patella syndrome (NPS) is an inherited disease produced by mutations in the LMX1B gene. It is characterized by fingernail dysplasia, hypoplastic or absent patella, dysplasia of the elbows and iliac horns on X-ray. It is useful to know this syndrome since some patients develop nephropathy and eye abnormalities. There are very few accurate descriptions related to this syndrome in the literature. OBJECTIVE: Describe the features of 11 patients with NPS in a paediatric hospital. METHODS: We retrospectively reviewed our clinical database of 11 patients with proven diagnosis of NPS from 1977 to 2014. Clinical and radiological features were assessed. RESULTS: Eleven children (seven male/four female) were included in the study. Mean age at the time of diagnosis was 6.54 years (range 0-11 years). Five patients had a family history of NPS. All patients had nail abnormalities (100%), the most frequent finding being hyponychia. Triangular lunulae were observed in four patients. The knee was the most commonly affected joint, aplasia or hypoplasia of the patella being the most usual findings. Only one patient presented renal involvement. The genetic study revealed three different LMX1B mutations. CONCLUSION: Nail-patella syndrome is a rare disorder. The aim of the present study is to highlight the importance of nail examination in children with skeletal dysplasias, in order to diagnose the NPS.
Subject(s)
Nail-Patella Syndrome/diagnosis , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Nail-Patella Syndrome/genetics , Nail-Patella Syndrome/pathology , Retrospective StudiesABSTRACT
OBJECTIVE: To determine the frequency of pancreatic metastasis from renal cell carcinoma in patients studied with MDCT during 2007 and to describe the patterns of presentation on MDCT. MATERIAL AND METHODS: We retrospectively studied 133 patients with renal cell carcinoma who underwent MDCT between January and December 2007. Forty-nine patients presented disseminated disease. We analyzed the frequency, location, and patterns of presentation of pancreatic metastases. RESULTS: Pancreatic involvement was identified in six patients. Four patients had isolated pancreatic nodules and two presented multiple nodules. A total of nine pancreatic lesions ranging between 8mm and 40 mm were detected. All nodules had increased uptake of contrast material in the arterial phase except for one in a patient with multiple nodules, due to necrosis. Two cases were associated with pancreatic duct dilation. Histology was obtained in only one patient. CONCLUSION: Pancreatic involvement of renal cell carcinoma was detected in 4.5% of patients, ranking fifth in frequency in patients with disseminated disease. The arterial phase is necessary to detect pancreatic involvement of renal cell carcinoma. The pattern of presentation is nearly constant, helping differentiate pancreatic metastasis from primary pancreatic adenocarcinoma.
