Gastrointestinal stromal tumors (GISTs): SEAP-SEOM consensus on pathologic and molecular diagnosis

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the digestive tract, with an incidence of 1.1 cases/100,000 inhabitants/year. A group of experts from the Spanish Society of Pathology and the Spanish Society of Oncology met to discuss a brief update on GISTs and agree on aspects relating to the pathological and molecular diagnosis of these tumors. GISTs are generally solitary, well-circumscribed lesions of variable size (< 10 mm-35 cm) that may present with intra- or extra-luminal parietal growth or a mixed-type (hourglass) growth pattern. Histologically, they are unencapsulated neoplasms displaying expansive growth and spindle-shaped (70%), epithelioid (20%), or mixed cellularity (10%). Mitotic activity is generally moderate or low and should be evaluated only in areas with high cellularity or higher mitotic frequency. The great majority of GISTs harbour mutually exclusive activating mutations in genes coding for the type III receptor tyrosine kinases KIT and PDGFRA; less commonly, GISTs have also been reported to display mutations elsewhere, including BRAF and NF1 and SDH-complex genes. The method most widely used to detect KIT and PDGFRA mutations is amplification of the exons involved by polymerase chain reaction followed by direct sequencing (Sanger method) of these amplification products. Molecular analyses should always specify the type of analysis performed, the region or mutations evaluated, and the sensitivity of the detection method employed (AU)

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Gastrointestinal stromal tumors (GISTs): SEAP-SEOM consensus on pathologic and molecular diagnosis.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the digestive tract, with an incidence of 1.1 cases/100,000 inhabitants/year. A group of experts from the Spanish Society of Pathology and the Spanish Society of Oncology met to discuss a brief update on GISTs and agree on aspects relating to the pathological and molecular diagnosis of these tumors. GISTs are generally solitary, well-circumscribed lesions of variable size (<10 mm-35 cm) that may present with intra- or extra-luminal parietal growth or a mixed-type (hourglass) growth pattern. Histologically, they are unencapsulated neoplasms displaying expansive growth and spindle-shaped (70%), epithelioid (20%), or mixed cellularity (10%). Mitotic activity is generally moderate or low and should be evaluated only in areas with high cellularity or higher mitotic frequency. The great majority of GISTs harbour mutually exclusive activating mutations in genes coding for the type III receptor tyrosine kinases KIT and PDGFRA; less commonly, GISTs have also been reported to display mutations elsewhere, including BRAF and NF1 and SDH-complex genes. The method most widely used to detect KIT and PDGFRA mutations is amplification of the exons involved by polymerase chain reaction followed by direct sequencing (Sanger method) of these amplification products. Molecular analyses should always specify the type of analysis performed, the region or mutations evaluated, and the sensitivity of the detection method employed.

99mTc-HDP SPECT/MRI in isolated xanthoma of the temporal bone / 99mTc-HDP SPECT/RM en xantoma único del hueso temporal

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[Renal oncocytoma. Review of our 22 patients]. / Oncocitoma renal. Revisión de nuestra serie de 22 pacientes.

UNLABELLED: Renal oncocytoma (OR) is a benign tumor. It may represent up to 3-7% of solid kidney masses, and shows specifics cellular and evolutive characteristics. Metacronicity, multifocality and bilateralism has been reported. MATERIALS AND METHODS: Between 1986 and 2005, 478 kidney tumors have been surgically treated at our institution. We report the frequency and characteristics of OR in our patients, compared with renal cell carcinomas (RCC). We try to find out the rate of multifocality, bilateralism and other tumor association, and the number of neoplasms originally diagnosed as OR before surgery. Mean and median follow up: 36.86 and 13 months (1-193). Specific survival rate 100%. RESULTS: We found 24 OR in 10 men and 12 women with a mean age of 59 years (34-84). 12 in the left kidney and 12 in the right one, one patient presenting oncocytomatosis. Tumor mean size was 4.64 cm (1-12.5 cm). Tumors were discovered incidentally in 17 cases. Presentation symptoms in the rest of patients were gross hematuria. CONCLUSIONS: The rate of OR found in our sample population of renal tumors undergoing surgery matches other series already published. Two synchronic OR, but not metacronous, bilateral or metastatic tumors were found. All cases presented a benign evolution.

Oncocitoma renal. Revisión de nuestra serie de 22 pacientes / Renal oncocytoma. Review of our 22 patients

Introducción: El oncocitoma renal es un tumor benigno cuya frecuencia de presentación oscila entre el 3- 7% de las masas renales sólidas. Muestra especiales características celulares y evolutivas, con posibilidad de metacronicidad, bilateralidad y multifocalidad, además de un difícil diagnóstico diferencial con neoplasias malignas. Material y Método: Revisamos retrospectivamente en 428 tumores renales intervenidos desde Enero de 1986 hasta Abril de 05 la proporción de oncocitomas diagnosticados, analizando sus características anatomopatológicas y comportamiento clínico, comparándolas con las presentadas por los carcinomas de células renales. Observamos su posible bilateralidad, multifocalidad y asociación con otros tumores. Determinamos en cuántos casos los métodos diagnósticos de imagen permitieron sospechar la naturaleza tumoral benigna previamente a la intervención. Seguimiento medio y mediano 13 y 36,86 meses respectivamente (1-193). Resultados: Hemos encontrado 24 oncocitomas (5,67%), en 10 hombres y 12 mujeres con una edad media de 59 años (34-84). 12 de ellos en riñón izquierdo y 12 en derecho, además de una oncocitomatosis renal. Tamaño medio de 4,64 cm (1-12,5). En 17 pacientes el diagnóstico ha sido incidental. En los 5 restantes el síntoma de debut fue hematuria. Supervivencia específica 100%. Se sospechó la naturaleza tumoral benigna o específicamente oncocitomatosa previamente a la cirugía en 22,73% (5/22) y 9,9% (2/22) respectivamente. Conclusiones: En nuestra serie de tumores renales intervenidos el porcentaje de oncocitomas coincide con lo publicado en la literatura. Hemos encontrado dos casos de tumor sincrónico, pero ninguno metacrónico, bilateral ni metastásico. Todos han presentado un comportamiento benigno

Renal oncocytoma (OR) is a benign tumor. It may represent up to 3-7% of solid kidney masses, and shows specifics cellular and evolutive characteristics. Metacronicity, multifocality and bilateralism has been reported. Materials and methods: Between 1986 and 2005, 478 kidney tumors have been surgically treated at our institution. We report the frequency and characteristics of OR in our patients, compared with renal cell carcinomas (RCC). We try to find out the rate of multifocality, bilateralism and other tumor association, and the number of neoplasms originally diagnosed as OR before surgery. Mean and median follow up: 36.86 and 13 months (1-193). Specific survival rate 100%. Results: We found 24 OR in 10 men and 12 women with a mean age of 59 years (34-84). 12 in the left kidney and 12 in the right one, one patient presenting oncocytomatosis. Tumor mean size was 4.64cm (1-12.5 cm). Tumors were discovered incidentally in 17 cases. Presentation symptoms in the rest of patients were gross hematuria. Conclusions: The rate of OR found in our sample population of renal tumors undergoing surgery matches other series already published. Two synchronic OR, but not metacronous, bilateral or metastatic tumors were found. All cases presented a benign evolution

Histiocitosis de células de Langerhans de larga evolución / Langerhans-cell histiocytosis of 28-year evolution

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