Poland sequence in two siblings suggesting an autosomal inheritance transmission.

We report on Poland sequence observed in two siblings (a girl and a boy) in the same family. This suggests an inheritance pattern consistent with an autosomal recessive or dominant trait transmission with reduced penetrance.

Growth in patients after allogeneic bone marrow transplant for hematological diseases in childhood.

The growth of 66 long-term survivors, transplanted in two centres (Genoa S. Martino and Monza) is reported. Patients were all under age 15 at the time of bone marrow transplantation (mean 9.

Non-familial hemophagocytic lymphohistiocytosis.

A case of non-familial hemophagocytic lymphohistiocytosis (HLH) is described. The patient had a chronic course being alive and doing relatively well 3 years since onset. The specific pathological features of this disorder are discussed with emphasis on the fact that a remarkable loss of lymph node structure is sustained by a mature lymphohistiocytic infiltrate.

The xanthogranulomatous process. Xanthogranulomatous inflammation.

The precise histopathological features of the xanthogranulomatous process (XP) have been defined in an extensive review of all cases from various organs available in our files. The result was that the XP has the following aspecific but characteristic findings: the presence of granular, eosinophilic, PAS positive histiocytes in the initial stages; the mixture of foamy macrophages and activated plasma cells; the presence of suppurative foci and hemorrhages. Abscess, necrosis and most of all hemorrhages play a a role in eliciting this inflammatory response.

Pleomorphic (anaplastic) neuroblastoma.

Four very unusual cases of neuroblastoma in children are reported. Clinically, they had a similar presentation: advanced disease at onset, diffuse abdominal spread, poor treatment response, and rapid progression. Pathologically, the four cases were characterized by a striking degree of cellular and nuclear pleomorphism and anaplasia with bizarre and monstrous expressions.