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Biomedical subjects

L B Kahn

Publications and source records attributed to L B Kahn.

At least 55 records · Page 3Linked to original sources

Extraskeletal osteosarcoma arising in an ectopic hamartomatous thymus. Report of a case and review of the literature.

A unique case of an osteosarcoma arising within a hamartoma of the thymic gland located in the left pleural cavity of an 11-year-old girl is described. The presence of abundant mature lamellar bone within this thymic hamartoma provides an explanation for the osseous histogenesis of the sarcoma. The development of an osteosarcoma from extraskeletal osseous tissue is a rare occurrence and has been documented in association with myositis ossificans and most recently in a case of dermatomyositis with metaplastic ossification.

Angiography↗

Case report 235. Ewing-like adamantinoma of the left radial head and neck.

Skeletal adamantinoma is a rare malignant bone tumor with a poorly understood histogenesis. The lesion is easily recognized by a distinctive microscopic pattern of anastomosing trabeculae of epithelial-like cells in a fibrous stroma. Ultrastructural evidence has been presented to support an origin from two widely-divergent cell types: an epithelial cell with squamous characteristics [12, 15] and a mesenchymal cell with endothelial features [5, 9]. An addition to the histogenetic controversy has been the recent description of a bone neoplasm with an adamantinoma-like growth pattern which proved both by subsequent histological evolution and ultrastructure, to have features of Ewing tumor [10]. The authors report a further example of this entity and suggest that it may be sufficiently distinctive within the adamantinoma group to merit the term Ewing-like adamantinoma. Consonant with increasing evidence supporting the concept of tumor "metaplasia" that transcends traditional concepts of epithelial-mesenchymal immutability, the authors propose that all adamantinomas arise from a common stem cell.

Adult↗

Chondroid chordoma. Electron-microscopic study of two cases.

Chondroid chordoma is an unusual tumor composed of an admixture of chondromatous and chordomatous tissue usually located in the spheno-occipital region. This tumor shares many of the clinical and histologic features of classic chordoma and chondrosarcoma and has been shown to have a better prognosis than either of these lesions. To the best of our knowledge, no ultrastructural studies have been performed in the 26 cases of chondroid chordoma published previously. We document the ultrastructural features of two examples of chondroid chordoma. Certain features such as prominent and dilated rough endoplasmic reticulum, intracytoplasmic glycogen aggregates, and abundant fibrillogranular matrix are common to chordoma, chondrosarcoma, and chondroid chordoma. The presence of well-formed tonofilament desmosome complexes as well as complexes composed of alternating profiles of rough endoplasmic reticulum and mitochondria were seen only in chordoma and chondroid chordoma, but not in cartilaginous tumors. Of particular interest was the finding of crystalline, tubular structures within the rough endoplasmic reticulum of both cases of chondroid chordoma, a finding not described previously. The distinction of chondroid chordoma from classical chordoma is said to be a difficult one at the light-microscopic level, and we suggest that these intraergastoplasmic tubular structures might constitute an extremely helpful differential marker.

Adult↗

Angio-immunoblastic lymphadenopathy.

Two patients presented with a short history of constitutional symptoms including fatigue, weight loss, night sweats and pruritus. Both had hepatosplenomegaly and tender lymphadenopathy, and in each case lymph node biopsy revealed prominent vascularization of the interfollicular zones and the presence of an amorphous eosinophilic fibrillar material, together with many epithelioid histiocytes, immunoblasts and plasma cells. In other areas of the same lymph nodes unequivocal lymphocytic lymphoma was present. The first patient was treated with levamisole, to which there was dramatic response. She subsequently died of septicaemia, and at autopsy was shown to be free of lymphoma. The second patient responded completely to the epipodophyllotoxin VP16-213 and is back at work as a heavy labourer without any residual disease. These 2 cases illustrate that complete clinical remission can be obtained in patients with this disease even after lymphomatous transformation has occurred. Levamisole has the additional attraction of being less immunosuppressive in patients whose immune response is already impaired.

Adult↗

Chronic granulomatous disease of childhood presenting as gastric outlet obstruction.

A case of chronic granulomatous disease of childhood was first diagnosed when a 20-month-old infant developed gastric outlet obstruction. Because of the unusual clinical presentation and negative gastric mucosal biopsies, the diagnosis was delayed. A subsequent full thickness biopsy of the stomach wall which led to a correct diagnosis showed a granulomatous infiltrate in the muscularis propria. Characteristic yellow pigment-laden macrophages were seen in the gastric mucosa and pyloric lymph node. Early recognition of this chronic disorder is important not only for proper management but genetic counseling as well.

Granulomatous Disease, Chronic↗

Ultrastructural comparison of Hodgkin's and non-Hodgkin's lymphomas.

The ultrastructural features of Reed-Sternberg cells and reactive epithelioid histiocytes from patients with Hodgkin's disease were compared with those of large cell non-Hodgkin's lymphomas. The cytoplasmic organelles of Reed-Sternberg cells were dissimilar to those of neoplastic large lymphocytes from follicular centre cell lymphomas, immunoblastic lymphomas, lymphoblastic lymphomas, and mycosis fungoides both in type and distribution. Reed-Sternberg cells could be distinguished from reactive epithelioid histiocytes; however, the cytoplasmic organization of the Reed-Sternberg cells bore a closer resemblance to these histiocytes than to the cells of the various large cell non-Hodgkin's lymphomas.

Histiocytes↗

Hemangiopericytoma: a light microscopic and ultrastructural study.

An ultrastructural study of three cases of hemangiopericytoma showed cells partially or completely enveloped by well-formed basement membrane and basement membrane-like material. The cells exhibited prominent cytoplasmic filaments, some showing dense body formation, interdigitating cytoplasmic processes, and pinocytotic vesicles. A review of the literature revealed only 19 other cases of hemangiopericytoma studied by electron microscopy, and these included seven cases of meningeal origin ("angioblastic meningioma"). The most consistent feature seen in all but two cases was the presence of a basal lamina or basal lamina-like material either partially or completely surrounding tumor cells and separating endothelial cells from pericytes. The light microscopic diagnosis of hemangiopericytoma is difficult, and there is a tendency to append the diagnosis to a variety of other tumors with a prominent vascular pattern in which other specific diagnoses are not immediately evident. The presence, ultrastructurally, of well-developed basement membrane, myogenic type filaments, and pinocytotic vesicles in a tumor with light microscopic features suggestive of hemangiopericytoma would consolidate the diagnosis and usually eliminate diagnostic uncertainty.

Aged↗

Multiple juvenile polyposis. A study of the pathogenesis of juvenile polyps and their relationship to colonic adenomas.

Solitary juvenile polyps are common lesions whose pathogenesis is poorly understood. Multiple juvenile polyposis is characterized by large numbers of these lesions either confined to the colon or throughout the gastrointestinal tract. A study of two cases of multiple juvenile polyposis provided fresh insight into the pathogenesis of juvenile polyps and their relationship to colonic adenomas. Mucosal ulceration in very early lesions, together with glandular epithelial calcification, suggested that impaired cell renewal resulting from disturbed regenerative kinetics may predispose to surface epithelial erosion, setting in motion a cycle of ulceration, inflammation, and granulation tissue formation. We postulate that a dyskinetic continuum may link juvenile, "metaplastic," and adenomatous polyps. The finding in our second case of multiple adenomatous lesions, including a villoglandular polyp, emphasizes the neoplastic potential of juvenile polyposis.

Adenoma↗

Acute lymphocytic leukaemia terminating in malignant histiocytosis. A case report and literature review.

A 5-year-old Black girl with acute lymphatic leukaemia died after a short illness during which haematological remission was induced with standard chemotherapeutic agents. At autopsy an unexpected finding was involvement of the spleen, lymph nodes, bone marrow and liver by malignant histiocytosis (MH). There was also evidence of residual leukaemia. A review of the literature revealed 11 other cases with a similar association of leukaemia preceding MH; in 8 of these the leukaemia was of the acute lymphatic type, in 2 of the monocytic type and in 1 of an unusual atypical large lymphoid cell type. The clinico-pathological features of these cases are reviewed.

Bone Marrow↗

Nongranulomatous septic vasculitis due to miliary tuberculosis. A pitfall in diagnosis for the pathologist.

A 38-year-old patient with chronic alcoholism was on chemotherapy for acute myelomonocytic leukemia and developed purpuric and erythematous papules. Skin biopsy showed a vasculitis. Subsequent autopsy revealed disseminated tuberculosis with a poor cellular response. This case calls attention to the lack of tuberculoid granulomatous reaction is anergic patients and to the need of pathologists to suspect tuberculosis when necrotizing vasculitis in the skin is encountered in a patient with decreased immunological competence.

Adult↗

Ultrastructural organization of the Reed-Sternberg cell: its resemblance to cells of the monocyte-macrophage system.

A dichotomy exists in the characterizations of the histogenesis of Hodgkin's disease. The Reed-Sternberg cell has been shown to possess functional properties both of lymphocytes and of mononuclear phagocytes. The majority of ultrastructural studies support the lymphocytic origin although a conflicting report describes some features of "histiocytic" cells [9]. In our study of four cases of Hodgkin's disease, the Reed-Sternberg cells contained a well-developed Golgi, moderate numbers of free polyribosomes and smooth and rough endoplasmic reticulum, variable numbers of lysosomes, lipid droplets, glycogen, fibrillar bodies, and irregular cytoplasmic processes. The number, size, and organization of cytoplasmic organelles are more consistent with a mononuclear phagocyte than a transformed lymphocyte and are similar to those seen in monoblastic leukemias. These findings support the concept of the derivation of the Reed-Sternberg cell from a monocyte-macrophage.

Adult↗

Locally aggressive fibrous histiocytoma of bone. A case report.

A fibrohistiocytic tumour involving the distal diaphysis of the left femur in a 44-year-old woman is described. The lesion had a completely benign appearance cytologically but behaved in a locally aggressive manner in that it eroded the adjacent cortical bone and even extended into the soft tissue. To the best of our knowledge, no other similar case has been documented. A lesion of the humerus has been described as an atypical fibrous histiocytoma on the basis of prominent nuclear pleomorphism; the patient involved was alive 5 years after a disarticulation. The differential diagnosis of such a locally aggressive fibrous histiocytoma of bone would include a metaphyseal fibrous defect, benign fibroxanthoma, and malignant fibrous histiocytoma. We believe that the differentiation of a locally aggressive fibrous histiocytoma from the malignant variety may be important in view of the probable differing therapeutic and prognostic implications.

Adult↗

Benign lymphoepithelial lesion (Mikulicz's disease) of the salivary gland: an ultrastructural study.

An ultrastructural study of a benign lymphoepithelial lesion of the parotid gland demonstrated that the so-called epimyoepithelial cell islands were sharply demarcated from the surrounding parenchyma by a thick basement membrane containing collagen fibers. The hyaline material seen by light microscopy within the islands was ultrastructurally similar in appearance to this delimiting basement membrane. The epithelial cells within the islands were united by well formed desmosomes and many had prominent tonofilament bundles, but myogenic differentiation was not observed. Hydropic degeneration was not seen in these epithelial cells; cells with a perinuclear clear space seen by light microscopy corresponded to large lymphoid cells ultrastructurally.

Basement Membrane↗