Pneumoblastoma. Report of a case. Immunohistochemistry and discussion of histogenesis

A case of pulmonary blastoma in a 50-year old man is reported. The initial diagnosis was one of pulmonary hydatid cyst, but at surgery a solid tumour of the left upper lobe was discovered. The histological study showed the characteristic features of pulmonary blastoma with its two components: epithelial and mesenchymatous. Immunohistochemistry yielded a double cellular response to keratin and vimentin--a finding which supports the hypothesis of a tumour arisen from a multipotent cell.     

Pulmonary inflammatory pseudotumor: difficulties in diagnosis and prognosis

Inflammatory pseudotumors are uncommon benign lesions of the lung of unknown origin, that may show aggressive behavior. Although pseudotumors constitute less than 1% of all lung tumors, they are reported to be the most common cause of solitary lung masses in children. Complete resection, when possible, is safe and leads to excellent survival. The case presented here highlights difficulties in clinical and histologic diagnosis.     

A deletion of proximal 20p inherited from a normal mosaic carrier mother in a newborn with panhypopituitarism and craniofacial dysmorphism

We describe a newborn male with a constitutional deletion of proximal chromosome 20p involving band p11.2. The phenotype included panhypopituitarism, craniofacial dysmorphism, a small phallus with a semi bifid scrotum, and bilateral widely separated first and second toes. The deletion was inherited from his mother, a mosaic carrier of the same deletion in peripheral lymphocytes. The only other similar case with a deletion of 20p11.22-p11.23 exhibited a phenotype that also included abnormal neural development (autism, craniofacial dysmorphism, and Hirschsprung disease). Our patient expands the spectrum of neurodevelopmental abnormalities associated with haploinsufficiency of band 20p11.2, and is the second deletion of 20p inherited from a normal mosaic carrier mother.     

Modulation of plasma cholesteryl ester transfer protein activity by unsaturated fatty acids in Tunisian type 2 diabetic women

BACKGROUND AND AIM: Type 2 diabetes mellitus is associated with atherosclerosis, which has been, in part, ascribed to abnormalities in the reverse cholesterol transport system. Among the key actors involved in this pathway is cholesteryl ester transfer protein (CETP) which mediates the transfer of cholesteryl esters (CE) from HDL to apoB-containing lipoproteins. METHODS AND RESULTS: The purpose of this study was to examine CETP activity in 220 patients with type 2 diabetes mellitus (type 2 DM) treated with diet alone or diet and sulphonylurea drugs and to identify the factors that may regulate it in the diabetic state. We also examined the effect of diet on the activity of plasma CETP in a subgroup of type 2 DM women. CETP activity was assessed by measuring plasma-mediated cholesteryl ester transfer (CET) between pooled exogenous HDL and apoB-containing lipoproteins. In 220 patients with type 2 DM, CET was significantly higher in conjunction with higher plasma triglycerides and lower HDL-cholesterol compared to 100 matched healthy controls. Correlation analysis showed that CETP activity was significantly correlated with the HDL-C to apoA1 ratio (r = -0.205, P = 0.003) and to LDL-C to HDL-C ratio in diabetic women (P = 0.010). Furthermore, CETP activity was correlated marginally with total energy intake (P = 0.052) but to a statistically significant extent with the amount of fat consumed daily (P = 0.008). A significant negative correlation was found between plasma CETP activity and MUFA of plasma phospholipids or free PUFA (P = 0.032), especially with omega3-fatty acids (P = 0.001). CONCLUSION: Our findings indicate that CET is accelerated in patients with type 2 DM and that this may be regulated by dietary fatty acids in the diabetic state.     

Monoclonal gammopathy and primary colonic mantle cell lymphoma

The association of a monoclonal gammopathy (MG) with a B cell non-Hodgkin's lymphoma (NHL) is a well-known phenomenon. It has been recognized in many subtypes of primary gastrointestinal lymphoma but its association with primary colonic mantle cell lymphoma has never been yet described. We report a 65-year-old man who presented with an exudative ascites and constipation. Serum electrophoresis showed a monoclonal peak in the gamma region of 45g/L and immunoelectrophoresis confirmed the presence of monoclonal gammopathy of IgM kappa type. Bone marrow aspirate was normal. Radiologic and endoscopic investigations evidenced a primary colonic mantle cell lymphoma. Although the association of an MG with an NHL and, in particular, to a primitive digestive location appears a rare phenomenon, endoscopic investigations in patients with MG appears legitimate in the presence of any digestive sign.     

Multiple myeloma presenting as bilateral breast lumps in pregnant woman

Background

Multiple myeloma (MM) is a systemic disease in the elderly. Its incidence in patients younger than 40 years old and especially in pregnant women is extremely rare. MM may involve extraosseous sites, and only in rare cases it is observed in the breast.

Case report

We describe the case of a 39-year-old woman diagnosed with an IgG lambda light-chain MM (Durie-Salmon stage IIIA, International Staging System II) in the 26th week of pregnancy, presenting with bilateral breast lumps. Dexamethasone (20 mg/m²/day on days 1–4, 9–12, and 17–20) was given as an induction agent to decrease tumor mass during pregnancy. Adverse response to dexamethasone was minor with excellent tolerance. A healthy baby was delivered at week 34 of gestation.

Conclusion

Breast nodules revealing MM are extremely rare. Clinical and radiological features are atypical. Presentation during pregnancy is extremely rare, making anti-MM treatment potentially challenging.