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Squamous cell passage from the peritoneal cavity to lymph glands and pulmonary lymphatics in a malformed infant: a mechanism for endometriosis and pulmonary metastasis.
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Mechanisms of malignant ascites production.
The accumulation of malignant ascites is determined primarily by the obstruction of diaphragmatic lymphatics with tumor inhibiting the outflow of peritoneal fluid. An abnormal increase in peritoneal fluid production has been shown to contribute to ascites formation by a marked neovascularization of the parietal peritoneum. Cell-free malignant ascitic fluid obtained from rats with intra-abdominal Walker 256 carcinoma when infused into the peritoneal cavities of normal animals causes an increase in edema formation and an increase in the permeability of protein from normal omental vessels. Protamine sulfate, a known inhibitor of angiogenesis when infused into the peritoneal cavity along with cell free malignant ascitic fluid, significantly reduces the leak of protein from the intravascular space when compared to ascites alone. Persistent permeability changes continue to exist even after the inhibition of vessel proliferation. These results indicate that angiogenesis is responsible for a major portion of the increase in permeability caused by malignant ascitic fluids. Other tumor-induced factors may be present which alter vascular permeability by other mechanisms which remain to be elucidated.
Teratologic evaluation of hexabrominated naphthalenes in C57BL/6N mice.
Hexabrominated naphthalenes (HBNs) have been identified as contaminants in the commercial PBB mixture, Firemaster. Similarities between polyhalogenated naphthalenes and polyhalogenated biphenyls, dibenzofurans, and dibenzo-p-dioxins with regards to structure and capacity to elicit certain toxicological responses in laboratory animals have previously been evaluated. In this study, teratogenicity and embryo/fetal toxicity, which have been associated with maternal exposure to other toxic polyhalogenated aromatics, was characterized for a synthetic mixture of HBNs in C57BL/6N mice. Pregnant adult mice were treated on gestation Days 6 through 15 with 0.5, 1.0, 2.5, 5.0, 7.5, and 10.0 mg HBN/kg body wt/day and sacrificed on gestation Day 18. Maternal toxicity as evidenced by decreased body weight gain or actual weight loss was observed at 7.5 and 10.0 mg/kg/day. Maternal liver-to-body weight ratios were increased relative to controls at all exposure levels. Dose-related increases were recorded in fetal mortality at 5.0, 7.5, and 10.0 mg/kg/day, and in the incidence of various teratogenic effects at all dose levels. Kidney lesions, best described as apparent hydronephrosis, were the most sensitive fetal abnormality observed, followed by a reduction in the size of lymphatic organs (thymus and spleen), cleft palate, subcutaneous edema, sternebral anomalies, and delayed cranial ossification. HBNs are concluded to be potent fetotoxic and teratogenic agents producing similar effects to TCDD and other toxic polyhalogenated aromatic hydrocarbons.
Red cell hypoplasia and monoclonal gammopathy in a patient with lymphoproliferative disorder.
A 69-year-old male was observed to have red cell hypoplasia. Two years later monoclonal gammopathy IgG-I, K, Gma+ and InV (1-) was documented in this patient. Persistent lymphocytosis, abnormal response to phytohemagglutinin, and at autopsy multiple lymphoid nodules occurring in the bone marrow, suggestive of lymphoproliferative disorder, were observed. A review of the literature indicates that this clinical presentation is rare. The possibility that red cell aplasia may be associated with lymphoproliferative disorder in some instances must be considered inasmuch as this may have an important bearing in the management of such patients.
Scintilymphangiography with 99 mTc-antimony sulfide colloid in hereditary lymphedema (Nonne-Milroy diseases).
A family of four patients suffering from Nonne-Milroy disease has been examined by pedal scintilymphangiography. Lower limb edema was present in three patients and was bilateral in two instances. Scintilymphangiographic abnormality was invariably present, ranging from almost total obstruction of lymphatic flow to lymphatic pooling. The inguinal, iliac and para-aortic nodes on the affected sides were also noted to be small and few in number. The examination is safe and simple and may be used to distinguish Nonne-Milroy edema from edema due to other causes.
Lymphoscintigraphic manifestations of Hennekam syndrome--a case report.
Hennekam syndrome is a rare, recently described genetic disorder in which facial anomalies and mental retardation accompany congenital lymphedema and intestinal lymphangiectasia. Several other somatic abnormalities have variously been described, as have milder degrees of lymphatic dysfunction. The authors herein describe a case of Hennekam syndrome in which the diagnostic difficulties were partially overcome by the judicious use of radionuclide scintigraphy to verify the lymphedematous component of the patient's presentation.
Yellow-nail syndrome: report of three cases.
The yellow nail syndrome, a combination of yellow discolouration of and dystrophic changes in the nails, pleural effusions and lymphedema, is thought to be relatively rare; to date 44 cases have been reported. Of a further three patients with this syndrome, one had all three features, one had the yellow nails alone and the other had pleural effusions and lymphedema without classic nail changes. Each had recurrent lower respiratory tract infections; and of all 47, chronic pulmonary infections occurred in approximately one quarter and were frequently associated with chronic sinus infections. The underlying abnormality is presumed to be a congenital defect of the lymphatics, but so far this has not been demonstrated to be the cause of the nail changes, the pathogenesis of which remains obscure.
CLINICAL pathological conference.
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[Lymphangioleiomyomatosis].
Lymphangioleiomyomatosis (LAM) is a progressive and usually fatal interstitial lung disease characterized by an abnormal smooth-muscle proliferation in the lung and axial lymphatics. It affects almost exclusively young women of childbearing age. The presenting features most commonly include dyspnea, symptoms of pneumothorax and cough. Less commonly patients can present with chest pain, pleural or pericardial effusion and lymphedema. Our patient, a 41-year-old woman, complained mainly of fatigue that had lasted for 2 months and finally became febrile and dispneic, especially when lying down. Pulmonary diagnostic procedures revealed several multicystic destruction of lung parenchyma. There was also respiratory insufficiency with O2 saturation of 87% and lung diffusion capacity reduced to 48%. The retroperitoneum was filled with neoplastic mass as shown on an abdominal CT scan. Pathohistologic analysis of retroperitoneal mass together with the radiologic finding of the lungs correlated with the diagnosis of LAM. The patient was prescribed corticosteroid therapy, which led to rapid clinical improvement. After making a definite diagnosis, the patient was recommended further treatment with medroxyprogesterone. This case shows that LAM, although rare, can present a diagnostic problem to clinicians and should always be considered as one of the diagnostic possibilities in young women with nonspecific pulmonary symptoms.
[Regional hypoplasia of dermal lymphatic vessels--a new variant of congenital lymphedema].
The value of new investigative methods of the most peripheral lymphatics of the skin (fluorescence microlymphography, indirect lymphography) is demonstrated by the case report of an 18 year-old girl. Regional hypoplasia of dermal lymphatics was detected in the lymphoedematous proximal and middle phalanges of the fingers while the initial lymphatics of the normal-appearing distal phalanges showed no obvious change. The subcutaneous lymph-collectors were ectatic and increased in the oedematous parts as well as in the normal-looking ends of the fingers. Hence, this is a new variant of lymphoedema, which is not caused by hypoplasia of lymph-collectors, but by a regional lack of initial lymphatics of the skin.
Ultrastructural and electrophysiologic changes of experimental acute cardiac lymphostasis.
Experimental impairment of cardia lymph flow in dogs produced histologic and electrophysiologic changes in the heart. Interstitial edema, lipid swelling of myofibrils, dilatation of lymph vessels, and fibrinoid degeneration of small coronary arteries occurred in and near the sinus node and the atrioventricular conduction system. On electrical stimulation of the heart, significant shortening of the atrial and ventricular effective refractory periods, increases in the sinus node recovery time and in the atrioventricular conduction time, and ventricular extrasystoles and ventricular fibrillation were observed. Many of these EKG changes are similar to those observed in sick sinus syndrome in man. An attempt was made to create dynamic lymphatic insufficiency by rapid electrical stimulation of the heart. EKG abnormalities observed in these cases could be prevented by intravenous injection of calcium dobesilate.
[Microangiopathic hemolytic anemia and consumption coagulopathy with metastasizing gastric carcinoma (author's transl)].
Microangiopathic hemolytic anemias (MHA) are frequently at the root of metastasizing gastric carcinoma. If the characteristic fragmented erythrocytes ("schistocytes") are found to be increased in the blood smear, this can be taken as a pointer to gastric neoplasm. Consumption coagulopathy frequently intensifies the clinical symptoms. A report of a 51-year-old woman with gastric carcinoma, MHA and consumption coagulopathy is given in whom the determination of numerous schistocytes in the peripheral blood was of decisive significance for the diagnosis.
Lymphangioleiomyomatosis.
Lymphangiomyomatosis (LAM) is a progressive disease afflicting women of childbearing age. LAM remains a rare disease, unfamiliar to many clinicians. It usually poses a diagnostic dilemma for the primary physician, possibly resulting in a delayed or missed diagnosis, unnecessary operative intervention, and inappropriate therapy. LAM is characterized microscopically by abnormal smooth muscle proliferation causing gradual obstruction of small airways, lymphatics, and vasculature. The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea, recurrent pneumothorax, chylous effusion, and hemoptysis. Despite a variety of treatment regimens developed since the first description of LAM, patient survival has not improved appreciably. Most patients die within 10 years of the time of diagnosis. This report presents a patient with LAM and a review of the literature.
[Bilateral changes in Klippel-Trenaunay-Weber syndrome with malformations in the arterial, venous and lymphatic vascular system].
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Peribronchovascular interstitium of the pulmonary hilum: normal and abnormal findings on thin-section electron-beam CT.
The peribronchovascular interstitium, including bronchial vessels and lymphatic channels, is an important anatomic component of the lung, especially the pulmonary hilum [1-3] (Fig.1). Various pathologic processes involve and spread along this interstitium [4, 5]. Although it has been difficult to accurately evaluate the morphologic changes occurring in the peribronchovascular interstitium with conventional CT or MR imaging [6], spiral CT and electron-beam CT can show these changes clearly and consistently [7, 8]. A judgement as to whether the peribronchovascular interstitium is normal or abnormal is necessary for the CT diagnosis of pulmonary hilar lesions. In this assay, the normal peribronchovascular interstitium and various pathologic processes involving this interstitium are shown as they appear on thin-section electron-beam CT scans.
Ultrasonographic diagnosis of subclinical filariasis.
Asymptomatic persons with lymphatic filariasis may harbor microfiliariae in the circulation, and despite the lack of symptoms, these patients may have occult pathologic lesions and renal abnormalities. Earlier investigators have shown that it is possible to detect live adult filarial worms and dilation of lymphatic channels with ultrasonography. It is also possible to assess response to therapy. Using sonography, we detected twirling motions in dilated lymph channels and characteristic sonographic findings associated with presence of adult filariae. On follow-up examination we also found evidence of loss of worm activity after chemotherapy.
Retroperitoneal anomalies in men with testicular germ cell tumours.
OBJECTIVES: To assess whether vascular and other retroperitoneal anomalies are more frequent during retroperitoneal lymph node dissection (RPLND) for metastatic testicular tumours (when retroperitoneal masses persist after chemotherapy) than would be expected, based on the initial observations from one centre with a large experience of RPLND in the UK. PATIENTS AND METHODS: A prospective series of 278 consecutive patients treated with RPLND for testicular tumours comprised the sample population. For each patient the presence or absence of four factors from the history was recorded. Each patient then underwent RPLND. During surgery, a template was constructed of the anatomy of the retroperitoneum and the information stored. Eight different retroperitoneal anatomical anomalies were identified in the sample population; the incidence of each was then compared with the largest available study of a normal population, and differences analysed statistically. RESULTS: Of the 278 patients who had RPLND, 55 had 59 anomalies (21%), found by history and as retroperitoneal vascular and urological anomalies; cryptorchidism was present in 7.6%, 9.5 times the incidence in the control population (P < 0.01). A left-sided inferior vena cava was present in 3.6% of patients, 21 times the incidence in the control population (P < 0.001); a retro-aortic left renal vein in 3.2%, four times that in the control population (P < 0.05); and ipsilateral renal agenesis had an incidence of 1% in the test population, 11 times greater than in the control population (P < 0.01). CONCLUSIONS: This prospective study of 278 RPLNDs provides evidence that some retroperitoneal anatomical anomalies are associated with testicular germ cell tumours. The link between maldescent and testicular tumours, rather than an isolated association, should be considered as part of a spectrum of retroperitoneal anomalies that occur in these men.