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Biomedical subjects

L B Kahn

Publications and source records attributed to L B Kahn.

At least 91 records · Page 5Linked to original sources

Chondrosarcoma with dedifferentiated foci. A comparative and ultrastructural study.

The light and electron microscopic features of a well-differentiated chondrosarcoma with dedifferentiated foci (CDF) are compared with those of a poorly differentiated chondrosarcoma with spindle cell elements. The differentiation of this lesion from mesenchymal chondrosarcoma and from primitive multipotential primary sarcoma of bone is discussed. Ultrastructurally, the cells of the cartilaginous region of the CDF resembled those of the poorly differentiated chondrosarcoma and of the cartilaginous zones of an extraskeletal mesenchymal chondrosarcoma reported by Fu and Kay, and were characterised by an abundance of dilated endoplasmic reticulum and a scalloped and microvillous cell membrane. The stroma was devoid of mature crossbanded collagen fibers. The dedifferentiated portion was composed of mesenchymal-type cells surrounded by a relatively sparse matrix containing scanty mature collagen fibers; these cells resembled those in the cellular regions of the two previously documented mesenchymal chondrosarcomas but differed from the cartilaginous type cells in the cellular regions of the poorly differentiated chondrosarcoma.

Adult↗

Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association: ultrastructural study and review.

A case of a vagal body tumor with a solitary cervical node metastasis is described. There was a remarkable familial association: a biopsy-proven paraganglioma occurred in two members of the patient's family and there was a strong clinical suspicion of a paraganglioma in two others. In a review of the literature, eight patients with metastasizing vagal body tumors and eight patients in whom the vagal body tumor was a component of multicentric presentation of paragangliomas were found. Only one other patient in whom there was a familial occurrence involving a vagal body tumor has been reported. Ultrastructural study of the cervical node metastasis revealed the presence of light and dark chief cells containing scanty, membrane-bound, densecore, neurosecretory-type granules. Atypical granules were noted in a few of the dark cells. Nerve fibers, synaptic vesicles, and sustentacular cells, such as occur in normal paraganglionic tissue, were not observed in this tumor.

Adult↗

Primary intestinal lymphoma of "Western" and "Mediterranean" type, alpha chain disease and massive plasma cell infiltration: a comparative study of 37 cases.

The clinical and pathological features of 17 "Western" type primary abdominal lymphomas (WTL) are compared with 14 of "Mediterranean" type (MTL). The MTL involved only young adult Mulatto and African patients in whom malabsorption and abdominal pain were the major clinical features. The WTL also predominantly affected Mulatto patients but four cases occurred in Caucasians, and the mean age at presentation was two decades later. An obstructive presentation was the most common; only one patient in this group had evidence of malabsorption. The WTLs were located mainly in the distal small bowel and were nearly all of monomorphic lymphocytic or histiocytic type. The MTLs were sited mainly in the duodenum and jejunum and were of an unusual pleomorphic histologic type. A spectrum of cells from those resembling atypical lymphocytes to large histiocytic types were seen, some of the latter resembling Reed-Sternberg cells. The pattern of mesenteric node infiltration in the MTLs was also unusual in that preservation of the medullary sinuses was a common finding. A notable feature of the MTLs was the presence of a heavy infiltration of mature-looking plasma cells associated with a fairly severe villous atrophy in the lamina propria of the small bowel. In the WTLs the adjacent small bowel did not show this feature. In addition to the above cases three patients with a similar heavy plasma cell infiltrate and villous atrophy but without evidence of a lymphoma are described. These cases may represent examples of MTL in a pre-malignant phase. Also included in this study are three patients with alpha-chain disease (alpha-CD), all with a heavy plasma cell infiltration and villous atrophy of the lamina propria and a pleomorphic type lymphoma involving the mesenteric nodes in all, and the small bowel in two. The lymphomas in alpha-CD have been interpreted as immunoblastic sarcoma by Lukes and Collins. Both genetic and environmental factors may be operative in the MTLs including the cases of alpha-CD.

Adult↗

Subepidermal bullous disease. A correlated clinico-pathologic study of 51 cases.

The histologic features of 51 cases of subepidermal bullous disease were evaluated and correlated with the clinical features including the course of the disease and the response to therapy. There was a good clinico-pathologic correlation, 46 out of 51 cases showing concordant clinical and histologic diagnoses. We conclude that although immunofluorescence is a confirmatory procedure histologic diagnosis is still of great value, particularly in the early stages of the disease when the clinical features may not yet have evolved sufficiently to allow for a confident diagnosis.

Child↗

Localised myeloma with osteogenesis and Russell body formation.

A case of an osteosclerotic myeloma of the mandible is described. Bone formation took the form of prominent sunray spiculation and radiologically mimicked an osteosarcoma. No other well-documented solitary lesion of this type could be found in the English literature. In addition, this tumour contained an abundance of intracytoplasmic Russell bodies and also produced a paraprotein. The paraprotein peak disappeared after resection.

Endoplasmic Reticulum↗

Familial haemophagocytic reticulosis. Report of 2 cases in sibs.

Two cases of familial haemophagocytic reticulosis occurring in sibs are described. The disease is characterised by a widespread proliferation of histocytes showing haemophagocytosis and it usually manifests clinically with haematological abnormalities. The initial presentation may be that of a meningo-encephalitic illness, the result of a lymphohistiocytic infiltration of the meninges and cerebral tissues. The clinical course and pathological features are described and the differential diagnosis is discussed.

Autopsy↗

Sclerosing haemangioma of the lung. A case report.

A case of pulmonary sclerosing haemangioma composed of blood-filled cavernous spaces separated by septa containing sheets of uniform, epithelioid-type cells and numerous mast cells is described. Although the histogenesis has not been conclusively established, the appearances in this case favour a primary vascular lesion. The variable histological patterns observed in this tumour may cause problems in diagnosis; the differentiation from haemangioma, haemangiopericytoma, pulmonary chemodectoma and benign clear cell tumour of the lung is discussed.

Aged↗

IgG monoclonal gammopathy associated with lymphoproliferative disorders.

The association of IgG monoclonal gammopathy with lymphocytic lymphoma and chronic lymphocytic leukaemia is reported. Although rare, such immunoglobulin synthesis may have prognostic importance and is of value in monitoring the effect of therapy. The morphological features which should draw attention to this pattern of protein abnormality are briefly reviewed. This finding emphasises the histogenesis of these tumours from the immune system and questions their classification on a purely morphological basis.

Aged↗

Malacoplakia in childhood. Case report with ultrastructural observations and review of the literature.

Malacoplakia involving the adrenal gland and colon was found in a 6-week-old infant. To our knowledge, this is the first reported instance of adrenal involvement and it occurred in the youngest patient described to have malacoplakia. The adrenal gland was the site of a previous hemorrhage. Ultrastructurally, the cytoplasm of the malacoplakia cells contained numerous granular inclusions within which were tetrad-shaped particles resembling ferritin. A thorough search failed to show the presence of any bacteria. Several recent papers have suggested that malacoplakia represents a morphologic manifestation of altered host macrophage response and that Gram-negative enteric bacilli are the usual specific etiologic agents. The pathologic findings in the present case raise the possibility that the lesion in this instance represents an altered macrophage response to extravasated erythrocytes alone or to erythrocytes and bacteria.

Adrenal Cortex↗