[A 29-year-old male with jaundice, abdominal pain and distension after a transplant of allogeneic bone marrow for acute leukemia].
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Biomedical subjects
Publications and source records attributed to J A Bombi.
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A Merkel cell tumor of the preauricular region in a seventy-seven years old woman is described. Histological, electronmicroscopic and tissular hormonal studies, were made. From the pathological point of view, a nodular and trabecular pattern of oval or elongated cells with poor basophilic cytoplasma is characteristic. Large nuclei with irregular, oval prominent nucleoli are also present. Electron microscopy shows oval and polygonal cells of irregular nuclei with one or multiple prominent nucleoli. In the cytoplasm of these cells, microfilaments and dense core granules with clear halo are found. The tissular hormonal determinations were normal. Two years after the surgical treatment, the patient remains asymptomatic.
A case of malignant polyposis coli, occurring in a 71-year-old man in association with a primary invasive adenocarcinoma of the gallbladder is presented. The eventuality that the carcinoma of the gallbladder could be a metastasis from the colonic neoplasia was excluded due to the observation in the mucosa of the gallbladder of changes corresponding to carcinoma in situ. This is the fourth case reported of polyposis coli coexisting with a carcinoma of the biliary tract.
Weekly SC administration of 2,2-dioxopropylnitrosamine to 88 Sprague-Dawley rats resulted in the induction of 76 separate neoplasms in the respiratory region of the nasal cavities. No tumors of the olfactory region were found. Histologically there were 37 squamous papillomas, 17 transitional papillomas, and 22 squamous cell carcinomas. These tumors were compared with a selected group of 53 related human tumors of the nasal and paranasal cavities, consisting of 11 squamous cell papillomas, 13 transitional papillomas, and 29 squamous cell carcinomas. While the experimental squamous cell papillomas showed a striking histological resemblance with the squamous papillomas observed in man, none of the experimental transitional papillomas had evidence of the characteristics inverted pattern seen in humans; nevertheless, the experimental tumors showed signs of incomplete squamous metaplasia and preservation of intraepithelial secretory glands, features commonly seen in transitional papillomas of man. The squamous cell carcinomas of this experiment were very similar to the corresponding human tumors in morphology and sex incidence. In both species a significant number of squamous cell carcinomas were keratinizing and of a high grade of histological malignancy. In addition, 69% of the human squamous cell carcinomas occurred in the male sex, and 72% of the experimental carcinomas developed in males. This comparative study suggests that further studies investigating the significance of nitrosamines as suspected etiologic factors of nasal carcinogenesis in man may be useful.
In a series of 500 autopsies cancer was found to be the first cause of death (24.6 por 100), followed by infectious diseases (23.8 per 100), and vascular diseases (22.2 per 100). The most frequent kind of malignant tumors found were lung neoplasms (17.8 per 100), followed by leukemias (17.0 per 100) and digestive tract tumors (11.3 per 100). Metastases were produced most frequently by tumors of the lungs (82.6 per 100), liver (81.8 per 100) and digestive tract (64.2 per 100). Metastases were found mainly in the lymph glands and liver.
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Tuberous sclerosis (TS) is a rare disease of the nervous system, being characterized by seizures, mental retardation and adenoma sebaceum. Concomitant pleuropulmonary lesions and spontaneous pneumothorax are extremely rare during the evolution of this disease. To date, only 19 cases of TS and spontaneous pneumothorax have been described in the literature. Here we present a case of TS and associated pleuropulmonary lesions with spontaneous pneumothorax in a 29-year-old female patient. Clinical, roentgenographic and histological aspects of this disease are commented upon.
Polypoid pseudosarcoma (PP) is a rare type of squamous-cell carcinoma of mouth, fauces and larynx with sarcoma-like reaction. Clinically PP is characterized by large pedunculated or sessile masses of rapid growing. This tumour recurs easily but rarely provokes metastasis. A case of polypoid pseudosarcoma of the tongue is reported. Differential diagnosis with spindle-cell carcinoma can be made following the criteria: a) the frequency and the sarcomatous appearance of the metastasis in the spindle-cell carcinoma, while PP rarely provokes metastasis which have a squamous-cell pattern; b) the gradual transition within the epithelial and the sarcomatous aspects in the spindle-cell carcinoma, not present in PP; and c) the presence of tonofilaments and desmosomes in the sarcomatous cells of the spindle-cell carcinoma, which lack in the sarcoma-like masses of PP.
Two cases of cutaneous self-healing histiocytosis X in a 6 and a 16 months-old children, are reported. Clinically, the lesions were characterized by few, small, translucent and confluent papules, sometimes purpuric. The scalp lesions were seborrheic eczema-like; the elements on the groins were erosive. Systemic examinations, laboratory data and general development were completely normal. The histological pattern, in both cases, were identical of that in histiocytosis X (atypic mononuclear cells with glassy eosinophilic cytoplasm and excentric kidney-shaped nucleus). Electron microscopy of the second case showed a proliferation of histiocytes containing Langerhans bodies. Ten per cent of these cells showed multivesicular, myelin and dense laminar bodies. The latest had been specially described in other selfhealing histiocytic syndromes: congenital reticulohistiocytosis (Hashimoto-Pritzker) and generalized eruptive histiocytoma. Since the clinical behaviour and prognosis of the histiocytic proliferations can not be assumed on clinical and histological data, an attempt, of classification and understanding of these processes is presented. The possibility that histiocytosis represent a proliferation of the mononuclear-phagocytic system at different levels of cellular maturation, is considered. So it would be possible to consider: 1) Acute malignant processes: xantholeukaemia, malignant histiocytosis syndromes (histiocytic medullary reticulosis . . .), and Letterer-Siwe disease; 2) Chronic and severe processus: xanthoma disseminatum, necrobiotic xanthogranuloma, disseminated plane xanthoma, multicentric reticulohistiocytosis, localized reticulohistiocytoma of Crosti and Hand-Schüller-Christian disease; and 3) Benign processus, some of them being involutive: juvenile xanthogranuloma, regressing atypical histiocytosis, eosinophilic granuloma of the bone, Illig-Fanconi disease, Hashimoto-Pritzker disease, benign cephalic histiocytosis and generalized eruptive histiocytoma. These groups include classic X-histiocytosis, self-healing X-histiocytosis, non-X malignant histiocytosis and non-X benign or auto involutive-histiocytosis (some with intracellular lipid storage: xanthohistiocytosis) (see table II). The ultrastructural cytoplasmic markers of histiocytosis (table I) are consistent features in the accuracy of diagnosis of these conditions.
Clinicopathologic correlation was analyzed in the primary diagnoses for a series of 500 clinical post mortem studies of 440 adults, 9 children aged 1-14 and 51 infants of perinatal age. The diagnoses were incorrect in 43% of all cases, of which 17% were important errors, 14% of relative importance and 12% of slight importance. There is an inverted relationship between the proportion of errors and duration of the hospital stay of the patients. This underlines the importance of clinical post mortem studies for control, improvement and better teaching methods in a hospital.
Heterotopic glial tissue was seen within the lungs of a newborn with a cranial malformation and anterior spina bifida. Histological examination of the lung showed solid islands of glial tissue intermingled with pulmonary structures. Well developed bronchioles that were in continuity with the main bronchial tree could be identified within the nodules. The central cystic cavity of the nodules is a bronchial lumen probably dilated by the respiratory movements. Several hypotheses have been elaborated to explain the heterotopic glial tissue in lung. The presence of an anterior spina bifida suggests a migratory mechanism to explain the embryogenesis of this case.
In 38 patients suffering from rickettsiosis caused by Rickettsia conorii (Mediterranean Exanthematous Fever), hepatic involvement was studied via laboratory tests and in 26 cases by means of liver biopsy. SGOT, SGPT and alkaline phosphatase were found to be elevated in more than half of the patients (SGOT 74.4 +/- 93 U.K., SGPT 82.2 +/- 93 U.K., a.p. 58 +/- 21 mU/ml). In 14 patients, liver biopsy showed the existence of inclusion corpuscles in Kupffer's cells. Electron microscopic study demonstrated the existence of phagosomes inside the epithelioid cells, which, however, were difficult to categorize. The frequent existence of granulomatous hepatitis in this rickettsiosis was confirmed, while the presence of the infecting agent in the liver could not be established.
For the purpose of describing a new case of the Woringer-Kolopp disease, we are up-dating the problem and its classification and contributing clinical, histopathological, and ultrastructural facts which show that this is a lymphoma of the T cells and therefore should be classified along with the mycosis fungoides and the Sézary syndrome. The identification test of the lymphocytes and the ultrastructural study bring to light new facts of this disease.
Electron microscopic aspects in ten cases of normolipidemic cutaneous xanthomatosis have been investigated. Two additional types IV and V hyperlipoproteinaemic xanthomatosis have also been included. Ultrastructural findings in all cases were similar. Abundant histiocytic cells with numerous intracytoplasmic lipid vacuoles, lysosomes, and myelin-figures, were the striking features. Moreover, in older lesions microfilaments and lipid vacuoles were found in some fibroblastic cells, as well as long space collagen around them. In some specimens we observed: giant multinucleated histiocytic cells, crystalline cleft-like spaces in histiocytes and some mastocytes with lipidic crystals in the extracellular space, as well as lipid vacuoles in Schwann cells, endothelial cells and pericytes. Rod-shaped tubulated bodies were found in some endothelial cells, with multiple basal vascular laminae. In xantelasma palpebrarum and in disseminate plane xanthoma the histiocytary foamy cells adopted a perivascular arrangement, as in hyperlipoproteinemic xanthomatosis. We concluded that ultrastructural aspects of different xanthomatosis are fairly similar as a consequence of the large amount of intracytoplasmic lipids accumulated in xanthomatosus cells. In xanthelasma palpebrarum and in disseminated plane xanthoma this cell phase is reached by similar pathways to those for hyperlipoproteinemic xanthomatosis, whilst in xanthoma disseminatum and juvenile xanthogranuloma the pathways seem to be different. A classification of normolipidemic xanthomatosis is also provided.
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Conley et al., in 1971, described a special type of melanoma characterized by a superficial melanic lesion at the onset; repeated local relapses as subcutaneous tumorations with an histological picture closely resembling an atypical fibroxantoma or fibrosarcoma. After a review of all the published material the autors presents a personal case with the clinical, histological and evolutive characteristics of this disease. The most interesting findings of the published case are the following: The special stains for the melanocytes (silver stain, Dopa, tyrosinase and cholinesterase) were all negative. There was an intense positivity for the lisosomal enzymes (non specific sterases, and acid phosphatases). The ultrastructural study of the tumoral tissues as well as the cells of cultures showed abundant cells with tumoral aspects, with prominent nucleoli somewhat dilated granular endoplasmic reticulum, myelin-like figures, lipidic vacuoles and abundant lisosomes. No melanosomes or premelanosomes were observed. Beside these tumoral cells abundant typical fibroblastic elements were found. There was a great amount of collagen fibers with periodicity superior to the normal. The conclusion is that the desmoplastic melanoma must be considered as a tumor of mesenchimatous origin intervening in its development multiple local and general factors.
One hundred and sixty-five coded liver biopsy specimens were studied by light microscopy to evaluate the occurrence and diagnostic significance of giant mitochondria, which have been identified as periodic acid-Schiff-negative globular hyaline cytoplasmic inclusions of regular outline, clearly distinguishable from Mallory bodies. In 4 cases, electron microscopy confirmed that these globules were in fact enlarged mitochondria. The incidence of giant mitochondria was significantly higher in patients with high alcohol consumption (72%) than in those with low or no alcohol intake (10%). Their presence was related to the amount of daily ethanol consumption and to the shortness of abstinence before the biopsy. It was independent of other changes in the liver, and was detected with similar frequency in biopsies showing different alcoholic liver diseases. Our study emphasizes that giant mitochondria may be detected by light microscopy in a high proportion of alcoholics, and rarely in nonalcoholic liver diseases. Although less specific, they are much more frequent than Mallory bodies. Consequently they should be considered as a diagnostic hint of recent and heavy alcoholsm.