Two infants with Down syndrome, one 4 weeks old and the other stillborn, at necropsy showed hepatic and pancreatic fibrosis, which was very severe in the liver of the liveborn infant and in the pancreas of the stillbirth. The liveborn infant had typical hematological features of the transient congenital leukemoid reaction of Down syndrome, and the identification of a megakaryoblastic component was consistent with recent opinion that this is a spontaneously-remitting congenital megakaryoblastic leukemia. The hydropic stillborn infant had intense extramedullary megakaryocytosis. The visceral fibrosis may have had a pathogenesis similar to that postulated for the myelofibrosis of megakaryoblastic leukemia in older children.
We report a case of a pure seminomatous relapse in the retroperitoneum 6 years after orchiectomy for an apparent stage I mixed germ cell tumor of the testis. The 4 cm. metastatic mass was not imaged on computerized tomography, tumor markers were negative and confounding symptoms made diagnosis difficult. The propensity for seminomatous tumors to relapse later than nonseminomatous tumors has profound implications for intensive surveillance programs for apparent stage I disease in mixed germ cell tumors. These programs often involve routine computerized tomography only for the first 2 years and rely on physical examination, simple radiology and serum tumor markers thereafter. Such programs may fail to detect pure seminomatous relapse and delay the onset of curative treatment.
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