Lipoprotein glomerulopathy associated with a mutation in apolipoprotein e

Lipoprotein glomerulopathy is a pathological condition characterized by lipid accumulation in the glomerular capillaries that has been associated with the presence of rare mutants of apolipoprotein E (ApoE). We describe a 51-year-old Italian patient presenting Type III hyperlipidemia and proteinuria in whom renal biopsy showed capillary ectasia and intraluminal lipid deposits, suggesting the diagnosis of lipoprotein glomerulopathy. The patient, who had elevated plasma ApoE level, was found to be heterozygous for a mutation in ApoE (Arg150Cys), designated apoEMODENA. This mutation induces the formation of ApoE dimers that are detectable under non-reducing conditions. Treatment with hypolipidemic drugs did not result in a complete remission of the proteinuria and was accompanied by a slow but progressive worsening of renal function with the persistence of intracapillary lipid thrombi. The introduction of low-density lipoprotein aphaeresis combined with a more aggressive lipid lowering and antihypertensive therapy resulted in the remission of proteinuria and a substantial improvement of renal function. Switching from low-density lipoprotein aphaeresis to plasma filtration did not result in an equivalent control of renal damage. The patient died of intracranial hemorrhage during an acute episode of malignant hypertension

Myxopapillary ependymoma of the sacrococcygeal region. Report of a case.

A case of subcutaneous myxopapillary ependymoma arising in the sacrococcygeal region of a 42-year-old woman is reported. Less than 60 cases of such tumors have so far been described in locations outside the central nervous system. Sacrococcygeal ependymomas are locally aggressive tumors that require a complete and wide surgical excision to prevent recurrences. They tend to metastasize in about 17% of cases

Malacoplachia del testicolo. Descrizione di un caso.

The histological and ultrastructural features of a case of malakoplakia affecting the testis and the epididymis of a 53-year-old man are described. Light microscopy shows a granulomatous inflammatory process, characterized by the accumulation of large mononuclear cells with abundant granular cytoplasm and PAS-positive calcific intracytoplasmic inclusions (so-called Michaelis-Gutman bodies). On E.M., such inclusions appear as concentric lamellated structures with a mineralized core. Although the pathogenesis of malakoplakia is still unclear, an abnormality of the bacteria-digesting function of tissue macrophages, leading to persistence of phagolysosomes, has been postulated. Malakoplakia of testis is a benign entity, which must be differentiated from neoplastic lesions, especially germ cell tumours, and granulomatous disease such as histoplasmosis, mycobacteriosis and idiopathic granulomatous orchitis

Sympathetic ganglia in diabetes. An ultrastructural and stereologic study.

An ultrastructural and stereologic study was performed on sympathetic ganglia collected at surgery from eight diabetic patients, seven age-matched non-diabetics and six subjects with glucose intolerance. All patients underwent surgery because of arteriosclerosis obliterans. Volume density (Vv) and mean thickness (tau) of the satellite cell layer were found to be significantly higher in diabetics (P less than .001 and P less than .002, respectively) than in the other two groups, between which no difference was found. Vv and tau of the endothelial basal lamina were found to be increased in the diabetic and glucose-intolerance groups at a low level of significance (P less than .05). Satellite cells are thought to exhibit a close similarity to Schwann cells. Ultrastructurally, cytoplasmic edema, increase of subcellular organelles and lipid inclusions were consistently observed in satellite cells from diabetic patients. The results suggest that there may be a metabolic impairment of these cells in diabetes

Primary solitary meningioma of the lung.

Light microscopic, ultrastructural and immunohistochemical features of a case of primary meningioma of the lung are described in this report. The lesion was incidentally discovered in the right lung of a 68-year-old man and treated by lobe resection. No evidence of recurrence or metastasis has been detected 2 years after surgery. The literature is reviewed and the differential diagnosis from other neoplasms presenting as coin lesions in the lung is stressed. Different histogenetical theories and the relation to so-called "minute pulmonary chemodectomas" are discussed

FATAL ASPERGILLUS FLAVUS INFECTION IN A NEONATE

Aspergillosis is an uncommon perinatal infection diagnosed with increasing frequency in recent years. We report a premature infant who required both nutrition and ventilation artificially assisted and developed a disseminated invasive nosocomial infection from Aspergillus flavus. Autopsy revealed marked hypotrophy of the thymus and ultisystem invasive aspergillosis chiefly involving the vascular and alimentary systems and also the respiratory tract, the central nervous system, and the skin. From what we know, this is the first case of the literature with a misleading initial clinical presentation involving the alimentary tract (hepatomegaly, ingravescent cholestatic icterus) and evolving in ntestinal occlusion

Extra-axial chordoma. Report of a case with immunohistochemical study.

A case of extra-axial chordoma arisen in the soft tissues of the posterior thoracic wall is reported. Immunohistochemistry showed coexpression of low molecular weight keratins, vimentin and S-100 protein in neoplastic cells. Although rare, examples of chordomas developing in extra-axial locations are reported in the literature and need be differentiated from other tumors that can mimic their histopathological features, such as myxoid chondrosarcomas

Expression of MHC class I and class II antigens in primary breast carcinomas and synchronous nodal metastases

Expression of the major histocompatibility (MHC) class I and class II antigens was studied by immunohistochemistry in a series of 70 primary breast carcinomas and in nodal metastases, In particular, the expression of class I (HLA A-B-C) and class II (DP, DQ and DR) molecules was compared in: a) primary breast cancers devoid of nodal metastases (n = 36) and tumors exhibiting metastatic deposits (n = 34) at the time of surgery, and b) primary breast carcinomas and their corresponding synchronous axillary nodal metastases, Reduced or absent HLA A-B-C antigen expression was seen in approximately 54.3% of primary breast carcinomas, whereas a partial or complete induction of class II products was observed in 18.5% (DQ), 30% (DP) or 48.5% (DR) of the same cases, An almost complete overlap of antigen expression was observed in breast tumors in which no metastases were found by histological examination of axillary nodes and in neoplasms showing histologically-diagnosed synchronous metastases, The reactivity for class I and class II antigens in nodal metastases roughly paralleled that exhibited by corresponding primary tumors, A discordant expression was seen in 11 cases (32%) stained for HLA A-B-C and in 8 (24%), 7 (21%) and 6 (18%) cases assayed for DP, DQ and DR products, respectively, When a discordant expression was detected, either decreased or increased staining patterns were observed in metastases, The finding of overlapping MHC antigenic profiles in the majority of primary breast tumors and nodal metastases casts doubts on the hypothesis that loss of MHC antigens can play an important role in the seeding and growth of metastatic breast carcinoma cells

Synchronous occurrence of epithelial and stromal tumors in the stomach - In reply to a letter to the editor.

The synchronous occurrence of epithelial and stromal tumors in the stomach is discussed

Algorithms for morphometric measurements on cancer cells in electron microscopy. Pilot tests.

Automated procedures were designed for handling quantitative data derived from a morphometric study of neoplastic cells on electron micrographs. Pilot studies were carried out on 3 breast carcinomas. Each tumor cell was coded by the sequence of 30 stereological parameters that described the general organization of subcellular constituents. Wide standard deviations, as large as 90% of the mean, were observed in some parameters. The practical application of correlation analysis between stereological parameters turned out to be of limited value, since only parameters related to the same subcellular structure showed correlation