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Apoptosis, bcl-2 expression, and p53 expression in gastrointestinal stromal/smooth muscle tumors.

The authors investigated the relations between outcome and apoptosis, immunohistochemical demonstration of bcl-2 protein, and immunohistochemical staining for p53 protein in patients with gastrointestinal stromal/smooth muscle tumors (GIST). Patients whose tumors demonstrated cellular apoptosis using the terminal deoxynucleotidyl transferase-mediated 2'-deoxyuridine 5'-triphosphate-biotin nick end labeling (TUNEL) assay had an improved survival over those whose tumors did not improve. In contrast, patients whose tumors demonstrated staining for bcl-2 protein had a decreased survival compared with those whose tumors did not demonstrate bcl-2. There was no relation between p53 immunoreactivity and survival. These results suggest that inhibition of apoptosis may be associated with malignant behavior in patients with gastrointestinal stromal/smooth muscle tumors.

Apoptosis↗

Locally metastasizing vascular tumor. Spindle cell, epithelioid, or unclassified hemangioendothelioma?

The authors report the case of a 2-year-old boy with a recurrent and locally metastasizing, spindle cell, vascular tumor with histiocytoid cells involving the skin, subcutaneous tissue, and muscle of the right forearm, the right distal radius and ulna, and multiple lymph nodes of the right axilla. Diagnoses of hemangioma, hemangiopericytoma, angiomatosis, spindle cell hemangioendothelioma, and malignant hemangioendothelioma were made on successive excision specimens. The soft tissue of the right arm became diffusely enlarged, and a severe syndrome developed that was similar to that described by Kasabach and Merritt. The limb was amputated above the elbow, the axillary lymph nodes were cleared, and a total dose of 6,000 centigrays axillary radiation was given. After operation, the Kasabach-Merritt syndrome resolved. Despite the lymph node metastases and multiple tissues involved, the patient has remained well 6 years after surgery. Although the tumor exhibited some of the histologic features of a spindle cell hemangioendothelioma, the low-grade aggressive behavior resembled that of an epithelioid hemangioendothelioma. The reported case cannot be classified into any of the recognized categories or subdivisions of vascular tumors.

Bone Neoplasms↗

Gastrointestinal stromal tumours: correlation of immunophenotype with clinicopathological features.

Stromal tumours of the gastrointestinal tract remain a persistent source of controversy with regard to both their proposed lines of differentiation and the difficulty in predicting their biological behaviour. We have examined 60 cases with a panel of seven antibodies directed at the identification of smooth muscular or neural differentiation. In our hands, 36 per cent of cases showed neural differentiation (although only 6.6 per cent expressed S-100 protein); 31 per cent appeared smooth muscular; 20 per cent manifested bidirectional differentiation; and 13 per cent were negative for all the markers used. Histological appearances do not reliably reflect immunophenotype. We have attempted to correlate immunophenotype with the site of the lesions and with their clinical behaviour. Mean follow-up of 5 years was obtained in 42 cases. Tumours with a neural phenotype have the best prognosis. Gastric tumours expressing both desmin and smooth muscle actin (in the absence of other markers) with up to 4 mitoses per 30 HPF behave in a benign fashion. Larger studies are required to substantiate the value of immunophenotyping this complex group of tumours.

Adult↗

The spectrum of pediatric fibroblastic and myofibroblastic tumors.

Fibroblastic and myofibroblastic tumors in neonates, infants, and children provide a diagnostic dilemma in surgical pathology due to their relative rarity and similarity in appearances. These tumors may be congenital or occur early during the first years of life or later during the first and second decades of life. The morphologic, immunocytochemical, ultrastructural, cytogenetic, and molecular features of the more "common" pediatric fibroblastic and myofibroblastic tumors are reviewed. In addition, the importance of a multimodal approach to tumor diagnosis is emphasized, with correlation with treatment and outcome differences among these unique fibroblastic and myofibroblastic tumors. The importance of providing an accurate diagnosis with pediatric fibroblastic and myofibroblastic tumors cannot be overstated, because treatment, prognosis, follow-up, and outcome are based on the initial assessment of these fascinating, but oftentimes, perplexing tumors.

Adolescent↗

Granular cell myoblastoma of the esophagus after irradiation for carcinoma.

Granular cell myoblastoma is an uncommon, usually benign tumor. Only 20 cases are reported in the esophagus. We describe a 65-year-old woman who developed a granular cell myoblastoma of the postericoid esophagus in the area of a squamous cell carcinoma successfully treated with irradiation. To our knowledge, this is the 21st reported case, and the only case occurring in the esophagus after irradiation for primary squamous cell carcinoma.

Aged↗

Gastrointestinal stromal tumours: an immunohistochemical study of 165 cases.

The phenotype of 165 gastrointestinal stromal tumours was studied by immunohistochemistry. In each case the phenotype was compared to the histological diagnosis. The phenotype was muscle in 49 tumours (30%), neural in 18 (11%), histiocytic in 20 (12%) and mixed in five (3%); 68 tumours (41%) were positive for vimentin only, four tumours had no markers and one tumour was positive for keratin only. Histologically, the tumours were classified as smooth muscle, probably smooth muscle, probably nerve sheath tumours or tumours of undetermined differentiation. In 30 histologically unequivocal muscle tumours, the phenotype was muscle in 28. Half of them, all benign, arose in the oesophagus or gastric cardia. Apart from this group, there was no correlation between phenotype, site of tumour and histological differentiation. Actin was a more sensitive muscle marker than desmin. With the exception of oesophageal tumours, the histological appearances alone could not establish a diagnosis of malignancy and were inadequate in evaluating differentiation. Immunohistochemical examination determined differentiation in 54% of the tumours, but this finding should be interpreted with caution in terms of histogenesis. It allowed us, however, to specify the differential diagnosis in 57 tumours in which the histological diagnosis was uncertain.

Biomarkers↗

CD34 immunoexpression in stromal tumours of the gastrointestinal tract and in mesenteric fibromatoses.

The aim of this study was to explore whether CD34 immunoreactivity can distinguish between different types of gastrointestinal stromal tumour, i.e. smooth muscle and neurogenic. We studied 87 stromal tumours from different sites in the gastrointestinal tract, as well as the omentum and mesentery, using a monoclonal antibody to CD34 (QBEND10). We also determined the immunoexpression of smooth muscle and muscle specific actins, S-100 protein, cytokeratin, desmin and vimentin. In addition, 15 cases of mesenteric fibromatosis were tested for CD34. Immunoexpression of CD34 was observed in 40 of the 87 stromal tumours and correlated with evidence of differentiation towards a smooth muscle phenotype. Large intestinal stromal tumours were less likely than gastric lesions to be CD34 positive. None of 15 cases of mesenteric fibromatosis was positive for CD34. We conclude that CD34 immunoexpression is seen in a proportion of stromal tumors of the gastrointestinal tract, mesentery and omentum, particularly those of smooth muscle type, and it may be useful as part of an immunohistochemical panel in the differential diagnosis of these neoplasms.

Antigens, CD↗