Esthesioneuroblastoma metastatic to the breast in a young woman.

Background: Metastases to the breast are rare and can be missed without knowledge of the clinical history. We report an unusual breast metastasis originating in an olfactory neuroblastoma.

Case: A breast metastasis from esthesioneuroblastoma occurred in a 20-year-old woman 2 years after the onset of the disease.

Homocysteine-lowering therapy and early progression of transplant vasculopathy: a prospective, randomized, IVUS-based study.

Although observational studies suggest that hyperhomocysteinemia may be a risk factor for coronary allograft vasculopathy (CAV), prospective data on homocysteine-lowering interventions and CAV development are lacking. We, therefore, randomized 44 de novo heart transplant (HT) recipients to 15 mg/day of 5-methyl-tetrahydrofolate (n=22), or standard therapy (control group, n=22) to investigate the effect of homocysteine lowering on the change in coronary intimal hyperplasia during the first 12 months after transplant, as detected by intra-vascular ultrasound (IVUS). Although 12 months after HT, homocysteinemia was lower in folate-treated patients (p<0.

[Outcome of isolated small bowel transplantation in adults: experience from a single Italian center].

Aim: Isolated small bowel transplantation is becoming the treatment of choice for adult patients with serious parenteral nutrition (PN) related complications: we report our three-year experience (December 2000-December 2003) from a single Italian center (Modena-Italy), with one of the larger European series.

Methods: We transplanted 14 patients, with a previous mean PN course of 27 months and a mean 21-month post-transplantation follow-up (range 3-36 months), obtaining a one-year actuarial survival rate of 92.3% with no intraoperative deaths.

Correlation between dietary polyunsaturated fatty acids and plasma homocysteine concentration in vitamin B6-deficient rats.

Background And Aim: Vitamin B6 as cofactor of Delta6 desaturase is involved in polyunsaturated fatty acid metabolism; moreover, it is a cofactor of the trans-sulfuration pathway of homocysteine. Some studies report that low concentrations of pyridoxine, by increasing homocysteine levels, are associated with coronary artery disease, and carotid and arterial lesions. The aim of this study was to verify whether different dietary amounts of polyunsaturated fatty acids associated with low content of vitamin B6 could modulate homocysteinemia.

Osteosarcoma with rhabdomyosarcomatous component or so-called malignant mesenchymoma of bone.

Background: Primary malignant mesenchymoma of the bone is a rare neoplasm consisting of two or more unrelated malignant mesenchymal components. The literature reports fewer than 20 cases, most of which were composed of osteosarcoma and liposarcoma.

Observation: We report an exceedingly rare case of primary malignant mesenchymoma of bone composed of rhabdomyosarcoma, osteosarcoma, and a minor chondrosarcoma component, arising in the right proximal humerus of a 15-year-old girl.

[Extra-nodal Rosai-Dorfman disease: a case report with thyroid involvement].

Extra nodal involvement by Rosaï-Dorfman disease (RDD) is not rare but remains poorly described clinically and microscopically. We report a case of RDD involving the thyroid and revealed by a 15 mm cold nodule developed on an ancient goiter. The patient was a 53 year old North African woman.

Primary broad ligament cystadenocarcinoma with mucinous component: a case report with immunohistochemical study.

Primary cystadenocarcinoma that arises in the broad ligament is extremely rare, especially when it is mucinous. We report the case of a 59-year-old woman with a cystic mass of the right broad ligament who underwent a complete excision of the mass (7 x 7 x 3 cm) with hysterectomy, right salpingo-oophorectomy, omentectomy, appendicectomy, and peritoneal biopsies. Pathologic examination showed a low-grade cystadenocarcinoma with a mucinous component limited to the broad ligament.

[The value of cytology in the diagnosis of extragonadal germ cell tumors].

Extragonadic germinal tumors are frequently mixed tumors. When a metastatic sacrococcygeal teratoma is clinically suspected in children, a yolk-sac tumor component could be judiciously demonstrated by either an elevated serum level of alphafoetoprotein (AFP), or fine needle aspiration cytology. We report the case of a 25 month aged girl presenting a metastatic sacrococcygeal tumor (lymph node and bone metastasis) with high level of AFP (34.

[Abscess of obturator internal muscle].

The abscess of obturator internal muscle is a rare disorder. The literature states only sporadic cases. Because of its rarity, the diagnosis is often late.

Cytoplasmic glycosylation of protein-hydroxyproline and its relationship to other glycosylation pathways.

The Skp1 protein, best known as a subunit of E3(SCF)-ubiquitin ligases, is subject to complex glycosylation in the cytoplasm of the cellular slime mold Dictyostelium. Pro143 of this protein is sequentially modified by a prolyl hydroxylase and five soluble glycosyltransferases (GT), to yield the structure Galalpha1,Galalpha1,3Fucalpha1,2Galbeta1,3GlcNAcalpha1-HyPro143. These enzymes are unusual in that they are expressed in the cytoplasmic compartment of the cell, rather than the secretory pathway where complex glycosylation of proteins usually occurs.

[Pseudo-angiomatous hyperplasia of mammary stroma: a case with gigantomastia].

Pseudo-angiomatous hyperplasia of mammary stroma (PASH) is a histopathological entity which is a microscopic fortuitous finding in mammary biopsies performed for different reasons. It may be symptomatic and appears then as a palpable lump. The term pseudo-angiomatous emphasizes the characteristic aspect of the stroma simulating a vascular tumor.

[True aneurysm of the common hepatic artery: a case report].

We report a case of hepatic artery aneurysm suggested by the appearance of epigastric mass. The diagnosis was confirmed by doppler sonography and helical CT. Surgical excision of the aneurysm without vascular reconstruction was carried out.

Outcome of isolated small bowel and pancreas transplants retrieved from multiorgan donor: the in vivo technique.

Even when considering the possibility of organ rejection and the complications of immunosuppression, the risks associated with total parenteral nutrition therapy are life-threatening. Therefore, for patients with end-stage bowel disease small bowel transplantation (SBTx) is the only therapeutic option. The preferred method to procure these organs is debated, especially when, graft retrieval is associated with concurrent abdominal organ procurement of the pancreas, which shares part of the vascular inflow and outflow with the small bowel.

Primary Hodgkin's disease of the nasopharynx: a rare but bona fide disease.

We report an uncommon case of Hodgkin's disease confined to the nasopharynx. An isolated polypoid mass of the nasopharynx was observed in a 41-year-old man presenting with increasing bilateral nasal obstruction. Histological study revealed a mixed cellularity type of Hodgkin's disease.

[Juxtacortical osteosarcoma of the radius in a child].

The authors report a rare case of juxtacortical osteosarcoma of the radius in a child. The diagnosis and treatment were delayed because of misleading and wrongly reassuring radiological and histological aspects. The 12-year-old boy was admitted for a tumefaction of the proximal extremity of the right radius noticed after trauma.

[Fibromatosis of the breast: clinical and pathology analysis of two cases].

Fibromatosis of the breast is an uncommon benign disease that mimics carcinoma clinically as well as radiographically. Its microscopic diagnosis among spindle cell tumors is fundamental, especially on frozen sections. We report two cases of mammary fibromatosis observed in young woman (26 and 31 years old).

Influence of age, sex and vitamin status on fasting and post-methionine load plasma homocysteine levels.

Background And Objectives: To investigate the effects of age, sex and vitamin status on total plasma homocysteine (tHCy), both after fasting (FtHCy) and two hours post-methionine load (PML-tHCy). The secondary aim was to determine the reference values for FtHCy and PML-tHCy.

Design And Methods: A cohort of apparently healthy volunteers underwent blood sampling for FtHCy, PML-tHCy, creatinine, serum folate, vitamin B12 and vitamin B6 (pyridoxal-5-phosphate, PLP).

Folate supplementation after heart transplantation: effects on homocysteine plasma levels and allograft vascular disease.

Background And Aims: After heart transplantation, the effects of folate supplementation on total homocysteine plasma levels (THcy) and heart allograft vascular disease (AVD) remain unclear.

Methods: Accordingly, we prospectively analyzed 48 heart transplant receipients referred for routine follow-up from July to September 1998 (age 54+/-11 years, 75% male, 35+/-27 months from transplant). Among these patients, 17 were treated with folate supplementation for 12 months (Group F), while 31 cross-matched for age, gender, serum creatinine and time from transplant (P>0.

Biological Determinants of Fasting and Postload Plasma Homocysteine Levels in Healthy Italian Subjects.

Genetic and nutritional factors are determinants of total plasma homocysteine (tHCy) whose increased levels play a pathogenic role in atherothrombosis and cardiovascular disease. This study investigated the influence of sex, age, creatinine, serum folate, vitamin B12 and vitamin B6 (pyridoxal- 5-phosphate, PLP) on fasting (FtHCy) and two hour postmethionine loading levels of tHCy in 147 apparently healthy subjects (M/F= 82/65, age range: 14-94 y). FtHCy was higher in men than women (9.