[The cellular concept in internal medicine].
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Biomedical subjects
Publications and source records attributed to E Govoni.
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The authors report 38 cases of bone metastases of neuroblastoma. They discuss the problems of diagnosis and treatment from the point of view of the orthopaedic surgeon, who is frequently confronted with a patient with localised bone symptoms in whom the primary neuroblastoma has not previously been diagnosed. The authors propose a diagnostic protocol and discuss the situations in which operative intervention by the orthopaedic surgeon may be indicated.
In four patients, calf muscular hypertrophy developed after the onset of sciatica. Hypertrophic muscles were weak and showed electromyographic signs of denervation. In all cases, calf muscle biopsy showed striking hypertrophy of type 1 and, especially, type 2 muscle fibers. This hypertrophy was associated with other signs indicating a neurogenic lesion. Muscle hypertrophy is a rare finding in neurogenic lesions. Stretch and exercise of muscle are probably the causative factors.
A case of high-grade non-Hodgkin's malignant lymphoma with an immunologically proven B-cell origin is reported; 20 to 30 per cent of the neoplastic cells displayed electron-dense neurosecretory-like granules at the ultrastructural level. This observation confirms the existence of such a finding in lymphomatous growths and stresses its irrelevance in the differential diagnosis between non-Hodgkin's malignant lymphomas and apudomas.
Three cases of a heterofore undescribed neoplasm of major salivary glands morphologically similar to giant cell tumor of bone are presented. All tumors were located in the parotid gland of adult individuals, and all patients are alive and well following surgical excision. One of the three cases was associated intimately with a malignant mixed tumor (carcinoma in pleomorphic adenoma). Ultrastructural and immunohistochemical studies failed to provide conclusive evidence about the specific nature of the tumor cells. The major salivary glands should be added to the long list of organs in which extraskeletal giant cell tumors have been observed, whether alone or in association with an epithelial malignancy.
A man with Fabry's disease, who died at 52, suffered from lancinating limb pains between the age of 20 and 27, and from severe arthralgia between 20 and 40. The sural nerve showed a severe loss of fibers (1,614 myelinated fibers/mm2), chiefly affecting the small myelinated fibers. Inclusions of both homogeneous and lamellated appearance were present in the perineurial cells, fibroblasts and vessel walls. Teased fibers displayed moderate remyelination, regeneration and the presence of globular focal myelin thickening. The transient time course of painful manifestations in this case is stressed in view of a possible pathogenesis of pain in Fabry's disease. It appears that small-fiber neuropathy is not directly related to the occurrence of pain symptoms, since the prevalent loss of small myelinated fibers was seen in the absence of actual pain. The small sensory neurons in the dorsal root ganglia, that are especially affected by lipid accumulation, probably give rise to abnormal discharges producing pain via the prevalence of the small-fiber versus the large-fiber input to the dorsal horn of the spinal cord (gate control theory). Thus pain fits may spontaneously abate, as in our case, if degeneration and loss of the small sensory neurons overstep a critical level.
The large mesenteric lymph node taken from guinea pigs in a period of time ranging from the 10th day prepartum till the 26th day postpartum has been examined in order to study: the morphological features of the stromal stationary reticulum cells with particular regard to recognize their stages of development; the possible ontogenetic relationship between these cells during the maturation of the lymphoid tissue. Our data support the hypothesis that from local mesenchymal cells originates a pool of poorly differentiated reticulum cells that can give rise to stromal stationary reticulum cells (myofibroblast-like cells, fibroblast-like cells, pericyte-like cells and dendritic cells). These elements have a characteristic distribution pattern likely related to different local functional requirements.
A case of anaplastic type of medullary thyroid carcinoma is presented. Immunoperoxidase study revealed calcitonin within the tumor cells, which also showed argyrophilia. At ultrastructural examination the tumor also displayed mucoid and squamous differentiation. This multidirectional differentiation (endocrine and exocrine) underlies the difficulty of ascribing a precise histogenesis to this tumor.
Three new cases of sclerosing stromal tumor of the ovary have been studied by hormonal, immunohistochemical and electron microscopic analysis. The results confirm that this tumor may have hormonal activity. Ultrastructural study shows findings different from those previously reported. The authors propose an origin from the ovarian stroma with luteinization of stromal tumor cells.
Localized nodular myositis (LNM) is a rare variant of polymyositis beginning with inflammatory nodules within muscles. Only seven cases have so far been reported in the literature. We describe a probable further case of LNM in a 67-year-old man with ischemic claudication of the left leg for three years who presented with painful nodules in the left gastrocnemius muscle and signs of systemic disease; a complete follow-up was not possible, because the patient died after only two months and autopsy was not performed. Muscle biopsy showed localized areas of necrotic and inflammatory pleomorphic changes, in keeping with the features of the other known cases. The ultrastructural findings (not previously reported in this disease) were characterized by marked changes of endomysial capillaries, with fibroblastic metamorphosis of the endothelial cells, and by the presence of filamentous inclusions in the myonuclei. The differential diagnosis of LNM from other localized muscle masses, chiefly from muscle infarct, is discussed.
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The ultrastructure (US) of the skeletal muscle from 10 patients on conservative treatment (PCT) and 10 haemodialysed patients (HP) was studied. The two groups exhibited no qualitative differences but quantitative alterations of the mitochondrial apparatus and capillary vessels were more impressive in the HP group. Disarray and loss of the myofibrillar sarcoplasm was the most obvious change in the uraemic muscle. The US findings are consistent with a neurogenic atrophy of the uraemic skeletal muscle but other features indicate a primary myopathic damage probably caused by a deficit of muscle microcirculation and by changes in the mitochondrial respiratory chain.
Large multilobated nuclei lymphoma is a rare entity that is characterized morphologically by large neoplastic elements with prominent nuclear lobations and clinically by a predilection for extranodal sites and a favorable prognosis. According to the data collected to date, the neoplasia is thought to be of T-cell origin. The authors describe the clinical, morphologic and immunohistochemical findings of a case of non-Hodgkin's lymphoma with large multilobated nuclei that showed a definite B-lymphocyte origin.
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A primary malignant fibrous histiocytoma of the spleen was studied by light and electron microscopy and is believed to be the first reported. The neoplasm was predominantly fibroblastic, with a characteristic storiform pattern, and included histiocyte-like cells, giant and foam cells confirmed by ultrastructural studies. Additionally, undifferentiated cells, intermediate cells and myofibroblasts were seen. The differential diagnosis from other sarcomas and the histogenesis of this tumour are discussed.