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Resumen
La asociación de síndrome de Peutz-Jeghers y neoplasias epiteliales múltiples es rara. Los tumores del tracto digestivo, aparato genital y mama son frecuentes en este síndrome, y los de riñón y de vías biliares, excepcionales. Se presenta el caso de una mujer de 68 años con síndrome de Peutz-Jeghers que presentó un carcinoma de riñón derecho y numerosos tumores bilaterales y pequeños de los cordones sexuales con túbulos anulares del ovario. Se practicó nefrectomía derecha, doble anexectomía e histerectomía total. A los 2 años de evolución falleció tras el desarrollo de un carcinoma rectal diagnosticado por sigmoidoscopia y biopsias, y de un episodio de ictericia obstructiva compatible con un colangiocarcinoma.
The association between Peutz-Jeghers syndrome and multiple epithelial neoplasias is infrequent. Tumors of the gastrointestinal tract, genitals and breast are common in this syndrome and tumors of the kidney and biliary tract are exceptional. We present the case of a 68-year-old woman with Peutz-Jeghers syndrome who presented with right kidney carcinoma and numerous small, bilateral tumors of the sex cords with annular tubules of the ovary. Right nephrectomy, double annexectomy and total hysterectomy were performed. The patient died two years later following the development of rectal carcinoma diagnosed by sigmoidoscopy and biopsy and an episode of obstructive jaundice compatible with cholangiocarcinoma.
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