RESUMEN
Solitary fibrous tumor (SFT) is uncommon and known to affect in middle-aged adults, with rare reports of occurrences in children. Although frequently involving the pleura, recent reports show that SFT may also involve extrapleural sites. SFT is known as a benign tumor, but in some cases SFT recurs, invades locally, or shows malignant transformation. We experienced a case of SFT that developed in the axilla of a 4-year-old girl with multiple congenital anomalies with constitutional chromosomal abnormality of 46,XX[44]/47,XX,+mar[11]. Her tumor could not be surgically resected due to the patient's multiple anomalies including congenital heart disease, chronic lung problem and seizure disorder. She died of congestive heart failure with respiratory failure due to tumor growth. This report is meaningful not only because SFT affected a pediatric patient with a constitutional chromosomal abnormality, but also because the tumor originated from the axillary area, a site of origin rarely reported for SFTs.
Asunto(s)
Adulto , Niño , Preescolar , Femenino , Humanos , Axila , Aberraciones Cromosómicas , Epilepsia , Cardiopatías Congénitas , Insuficiencia Cardíaca , Pulmón , Pleura , Insuficiencia Respiratoria , Tumores Fibrosos SolitariosRESUMEN
Solitary fibrous tumor (SFT) is uncommon and known to affect in middle-aged adults, with rare reports of occurrences in children. Although frequently involving the pleura, recent reports show that SFT may also involve extrapleural sites. SFT is known as a benign tumor, but in some cases SFT recurs, invades locally, or shows malignant transformation. We experienced a case of SFT that developed in the axilla of a 4-year-old girl with multiple congenital anomalies with constitutional chromosomal abnormality of 46,XX[44]/47,XX,+mar[11]. Her tumor could not be surgically resected due to the patient's multiple anomalies including congenital heart disease, chronic lung problem and seizure disorder. She died of congestive heart failure with respiratory failure due to tumor growth. This report is meaningful not only because SFT affected a pediatric patient with a constitutional chromosomal abnormality, but also because the tumor originated from the axillary area, a site of origin rarely reported for SFTs.
Asunto(s)
Adulto , Niño , Preescolar , Femenino , Humanos , Axila , Aberraciones Cromosómicas , Epilepsia , Cardiopatías Congénitas , Insuficiencia Cardíaca , Pulmón , Pleura , Insuficiencia Respiratoria , Tumores Fibrosos SolitariosRESUMEN
We reviewed eight solitary fibrous tumors occurring at sites other than pleura (three orbit, two retroperitoneum, one each hard palate, thyroid, and tongue) which shared the histologic and immunohistochemical features of solitary fibrous tumors of pleura. Six patients were women, and two were men, aged from 26 to 74 years. The tumors ranging from 1.5 to 19 cm in diameter presented as well-circumscribed, unencapsulated, soft to rubbery tissue masses. Histologically they were characterized by a proliferation of spindle or ovoid cells intervened by a dense bundles of collagen. A variety of growth patterns was identified but the so-called patternless pattern was the predominant one. One tumor exhibited highly cellular sarcomatous areas with extensive necrosis, which was diagnosed as malignant solitary fibrous tumor. Immunohistochemical studies showed that all of the tumors were strongly positive for both CD34 and vimentin, but negative for cytokeratin, S-100 protein, EMA, and desmin. One case examined ultrastructurally showed features of fibroblast. All but one showed no evidence of recurrence or metastasis over follow-up period of 14 to 32 months. We conclude that extrapleural solitary fibrous tumors represent a distinct mesenchymal tumor with variable histologic features and should be differentiated from other spindle cell mesenchymal tumors.