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Defects in the control of Wnt signaling have emerged as a recurrent mechanism involved in cancer pathogenesis and acute myeloid leukaemia (AML), including the hematopoietic regeneration-associated WNT10B in AC133 bright leukaemia cells, although the existence of a specific mechanism remains unproven. We have obtained evidences for a recurrent rearrangement, which involved the WNT10B locus (WNT10B R ) within intron 1 (IVS1) and flanked at the 5' by non-human sequences whose origin remains to be elucidated; it also expressed a transcript variant (WNT10B IVS1 ) which was mainly detected in a cohort of patients with intermediate/unfavorable risk AML. We also identified in two separate cases, affected by AML and breast cancer respectively, a genomic transposable short form of human WNT10B (ht-WNT10B). The intronless ht-WNT10B resembles a long non-coding RNA (lncRNA), which suggests its involvement in a non-random microhomology-mediated recombination generating the rearranged WNT10B R . Furthermore, our studies supports an autocrine activation primed by the formation of WNT10B-FZD4/5 complexes in the breast cancer MCF7 cells that express the WNT10B IVS1 . Chemical interference of WNT-ligands production by the porcupine inhibitor IWP-2 achieved a dose-dependent suppression of the WNT10B-FZD4/5 interactions. These results present the first evidence for a recurrent rearrangement promoted by a mobile ht-WNT10B oncogene, as a relevant mechanism for Wnt involvement in human cancer.

Osseous hemangiomas are rare skeletal tumors that constitute 0.7% of all osseous neoplasms. The most common site is the vertebral column while involvement of the calvarium is extremely rare accounting for 0.2% of all bone neoplasm. The authors present a case of a 35-years-old man who was admitted with right parietal swelling and review the gross appearance, pathogenesis, histopathology, radiological features and treatment of this neoplasm.

Herein we report a case of a renal cell carcinoma with synchronous metastases to the lung and gallbladder. This appears to be the first case of renal cell carcinoma with metastasis to the gallbladder detected prior to autopsy.

The authors report a case of intramedullary spinal cord tumor in a 52-years old woman operated, 5 years before the beginning of medullary syndrome, for mammary carcinoma. Intramedullary metastasis was associated to another secondary localization in the pituitary stalk. The importance of magnetic resonance image and aggressive treatment is stressed.

A case of meningioma with inflammatory reaction within the fourth ventricle in a 37 year-old man is described. The differential diagnosis between inflammatory meningioma, plasma cell granuloma and a possible collision of a meningioma with a plasmacytoma is discussed. The immunohistochemical examination of plasma cell population is emphasized. This is the first case reported of inflammatory meningioma located in the fourth ventricle.

Mucoepidermoid carcinomas (MEC) make up 32% of the heterogeneous cases of malignant epithelial neoplasm of salivary glands observed in our Department. From 1980 to 1990, 270 patients with salivary gland tumefaction, which had previously been treated surgically, were studies. Thirty-four proved to be cases of malignant epithelial neoplasms while 12 oh these 34 were MEC. The biological behaviour of MEC appears to be closely linked to histological grading: a tumor with a low grading has good prognosis while in the case of tumors with a high grading there is a rapid development of local as well as distant metastases. At appears that, with regard to malignancy, localization is an important factors as well submandibular neoplasms are more aggressive the other cancers, probably because they attack periglandular tissue so promptly.

Clinical, radiological and histological aspects of a case of primary bone haemangiopericytoma, treated by wide surgical resection and pre- and postoperative radiotherapy, are described. The patient was disease-free 20 months from the operation. From a literature review of 52 cases of this very rare tumour it emerges that the tumour is aggressive but of variable malignancy. Prognosis seems to depend on histology, size at diagnosis and treatment. The best available treatment is probably wide exeresis plus radiotherapy.

In 34 patients operated on the morphological and morphometric patterns of the pulmonary vascular bed on lung biopsies, performed during the operation for ventricular septal defect or complete atrio-ventricular septal defect were studied. These patterns were related to the preoperative hemodynamic data. Ages ranged from 2 to 20 months. There were 16 ventricular septal defect (mean age 10 +/- 4 months) and 18 complete atrio-ventricular septal defect (mean age 9.8 +/- 1.2 months). Furthermore, of the latter 15/18 (83%) patients had Down's syndrome. The following hemodynamic data were considered: a) ratio between the pulmonary systolic artery pressure and the systemic systolic artery pressure; b) diastolic pulmonary artery pressure; c) pulmonary vascular resistance; d) ratio between pulmonary and systemic flow. The lung specimens obtained at surgery were routinely processed and embedded in paraffin. Sections measuring 4 microns were stained with hematoxylin-eosin, Masson's thricrome and Miller's elastin. The status of the small pulmonary arteries was assessed according to the Heath-Edwards classification and to the morphometric parameters proposed by Rabinovitch and coll. Ratio between the pulmonary systolic artery pressure and the systemic systolic artery pressure ranged from 0.48 to 1 (mean 0.8 +/- 0.23) in patients with ventricular septal defect, whereas in complete atrio-ventricular septal defects it ranged from 0.42 to 1 (mean 0.86 +/- 0.21). The mean diastolic pulmonary artery pressure was 20 +/- 10 mmHg (range 8-40 mmHg) in ventricular septal defects and 19.5 +/- 2.3 mmHg (range 10-35 mmHg) in complete atrio-ventricular septal defects.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors report a pathological study of functional neck dissection performed on 145 N0 laryngeal carcinoma patients from 1982 to 1988. The incidence of occult latero-cervical metastases in N0 was evaluated in relation to site and extension of the primitive tumor. A total of 108 supraglottic, 25 glottic and 12 transglottic tumors were observed. Among the cases treated the incidence of occult metastases was 28.3% while capsular rupture accounted for 3.4%. Diagnostic error, calculated on the number of neck dissections, was 20.2%. A greater incidence of occult metastases was found in supraglottic tumors (29.6%) than in either glottic (24%) or transglottic (25%) tumors. Furthermore, incidence of occult metastases proved proportional to the extension of the primitive tumor. There was a single metastases in 35 cases while only 6 cases proved multiple. Some final considerations are given on the importance of performing precautionary functional neck dissection in N0 laryngeal cancer.