Endodermal sinus tumor of the mediastinum. Ultrastructural study.

This is the eighth case report of an endodermal sinus tumor of the mediastinum (first case studied by electron microscopy). The ultrastructure of the tumor was found to mimic that of the normal yolk sac and was also similar to that of three previously reported cases of endodermal sinus tumor of the ovary, thus confirming the correctness of Teilum's original interpretation of the tumor as arising in germ cells and differentiating towards extraembryonic structures.     

Endodermal sinus (yolk sac) tumors in pediatric oncology. Report on 14 cases

Fourteen cases of the endodermal sinus tumors in children are reported. The most frequent localization was the vagina, next presacral region, ovary and testis. No ovarian case was observed under 3 years of age.     

Sinus nodal responses to atrial extrastimuli in patients without apparent sinus node disease.

In 36 patients without sinus node disease scanning with an atrial extrastimulus (A2) was performed during sinus rhythm with the sinus cycle length measured in milliseconds. Zones of nonreset due to interference, reset, interpolation and sinus echoes were defined by noting the timing of the first response after A2. Zones were defined in terms of their longest and shortest A1-A2 coupling intervals (in milliseconds). A zone of nonreset was found in 12 of 12 patients in whom A2 was delivered late. The mean cycle length in these 12 patients was 779 msec, with a mean zone of nonreset of 779 to 585 msec (25 percent of cycle length). All 36 patients (100 percent) had a zone of reset. The mean cycle length in these 36 patients was 803 msec with a zone of reset from 692 to 319 msec (46 percent of cycle length). Seven of 36 patients (19 percent) had a zone of interpolation. The mean cycle length in these seven patients was 754 msec, with a mean zone of interpolation of 344 to 279 (9 percent of cycle length). Four of 36 patients (11 percent) had a zone of sinus echoes. The mean cycle length in these four patients was 870 msec, with a mean zone of echoes from 350 to 313 msec (4 percent of cycle length). Calculated sinoatrial conduction time ranged from 40 to 153 msec (mean +/- standard deviation 92 +/- 30 msec). Shortening of the cycle length with atrial pacing increased the number of patients with zones of interpolation and echoes. In conclusion, zones of nonreset and reset are found in all patients with normal sinus nodal function, whereas zones of interpolation and echoes are much less common. Sinoatrial conduction time is surprisingly long in patients without apparent sinus node disease.     

Endodermal germ cell carcinoma (endodermal sinus tumor) of the vagina in infant girls

Summary An endodermal sinus tumor (endodermal germ cell carcinoma) was diagnosed in a 1-year-old girl in the vagina after vaginal hemorrhage; the tumor was completely removed by radical abdominal surgery. Postoperative polychemotherapy was performed for two years with Actinomycin D, Adriamycin, Vincristin, and Cyclophosphamide. The infant is now tumor-free for 26 months, showing almost normal somatic and psychic development.The characteristic histological patterns and clinical course of this strongly malignant tumor are demonstrated, based on 25 published casereports of endodermal sinus tumors in the vagina of little girls (aged 5–26 months). This neoplasm in early infancy has to be separated from the clear-cell adenocarcinoma of the vagina which occurs after puberty in adolescent girls and young women, and is induced by stilbestrol therapy to the mother during early pregnancy.     

Cytomegalovirus-associated acute pancreatic disease in patients with acquired immunodeficiency syndrome. Report of two patients

Pancreatic disease has been infrequently reported in patients with acquired immunodeficiency syndrome. Over the last 3 years at our hospital, two patients with the acquired immunodeficiency syndrome and acute pancreatic or biliary disease, demonstrated at autopsy to be secondary to cytomegalovirus infection of the pancreas, have been evaluated. However, pancreatic disease was not recognized antemortem in our two patients because of their underlying diseases and the atypical presentation. Cytomegalovirus infection of the pancreas may cause acute symptomatic disease in patients with acquired immunodeficiency syndrome.     

Late occlusive thrombosis of mitral prosthesis with sinus rhythm. Report of two surgically treated patients

Two cases are reported of late occlusive thrombosis of a mitral bioprosthesis with sinus rhythm. Two men were concerned (40 and 54 years of age), hospitalized for acute pulmonary oedema which was resistant to medical treatment, 3 years after replacement of a mitral valve (Carpentier Edwards No. 31 and Liotta No. 25). Catheterization showed that in both cases there was an average transmitral holodiastolic gradient greater than 25 mmHg. Emergency surgery revealed two anatomical forms of occlusive thrombosis: in one case, a localized red thrombus hindered the opening of a valve cusp; in the other, exuberant fibrin deposits lined the ventricular face of the valve cusps. There were no signs of degeneration of the bioprostheses and, in particular, there was no calcification. Both patients were asymptomatic 1.5 years and 3 years respectively after their operations. Late occlusive thromboses of mitral bioprostheses are exceptional (13 detailed cases collected from the literature).     

Traumatic lateral sinus thrombosis. Report of two cases.

Two cases with thrombosis of the transverse sinus caused by a fracture crossing the sinus are reported, and the roentgenological changes seen in cerebral angiography are discussed. In 1 case, a filling defect was seen which at subsequent autopsy was found to correspond with an intraluminal thrombosis. In the other case, not verified at autopsy, the angiographic changes were consistent with a thrombosis of a transverse sinus.     

Adrenal rest tumor of the liver causing Cushing's syndrome: treatment with ketoconazole preceding an apparent surgical cure

Ketoconazole, an imidazole derivative known to inhibit cytochrome P450-dependent adrenal enzymes was given to a patient with a functioning adrenal rest tumor of the liver in preparation for surgery. The drug was administered in a stepwise manner for 42 days starting with 400 mg and reaching 1 g the last 4 weeks of the trial. Clear clinical improvement was evident early in the trial and was associated with evidence of amelioration of her hypercortisolism and striking changes in serum and urinary levels of steroid hormones and metabolites. Sex steroids in serum and urine fell dramatically from the first day to the end of the trial. Urinary 17-ketosteroid excretion fell from a basal average of 139 mg/24 h to near normal levels within a week of therapy; serum testosterone fell from a basal level of 2.4 to 0.18 ng/ml; serum 17 beta-estradiol fell likewise from 1096 to 150 pg/ml. In contrast, cortisol levels in serum and urine increased in the first 2 weeks of the trial and subsequently fell to values below the basal levels. Similarly, serum 17 alpha-OH-progesterone levels increased 63% above the basal levels by day 6 of the trial and declined afterwards. Nine months after successful tumor resection the patient is apparently cured as judged by steroid hormone levels and physical appearance. We conclude that ketoconazole was effective in blocking tumoral steroidogenesis which resulted in clinical benefit.     

Assessment of sinus node function in asymptomatic subjects with sinus bradycardia and in symptomatic patients with sino-atrial disease.

Sinus node function was evaluated in 18 patients with sinus bradycardia without complaints (Group I), in 16 patients with sinus bradycardia and/or sinoatrial block with complaints (subgroup IIa) and in 14 patients with the bradycardia-tachycardia syndrome (subgroup IIb). Mean values of corrected sinus node recovery time (CSRT), atrial effective refractory period (AERP) and atrial functional refractory period (AFRP) differentiated significatively asymptomatic subjects of group I from the two subgroups of patients with sinoatrial disease, but failed to differentiate each subgroup from the other one. There was no significative difference in mean sinoatrial conduction time (SACT) between group I and each of the two subgroups. Three patients of subgroup IIa and 1 patient of subgroup IIb had a false negative response after both overdrive and premature programmed atrial pacing. Spontaneous cycle length was directly correlated with the sinus node recovery time and the atrial refractoriness in group I, and with the only sinus node recovery time in subgroup IIb. No direct correlations were observed in subgroup IIa. This suggests a less disturbed sinus node automaticity in bradycardia-tachycardia syndrome.     

mu-chain disease. Report of two new cases.

We report two cases of mu-heavy-chain disease. Both patients were affected with a lymphoproliferative disease that shared several suggestive features with the previously reported cases of mu-chain disease: the presence of vacuolated plasma cells in bone marrow, a small amount of alpha 2 moving abnormal mu-chain protein, and urinary kappa Bence Jones protein in one case.     

Granular cell tumor of the mediastinum

Mediastinal granular cell tumor is an exceptionally rare tumor; there are only a few previously reported cases. We report a 21-year-old man with a granular cell tumor in the right posterior mediastinum. Chest computed tomography scans and magnetic resonance imaging revealed a 15x20 mm well circumscribed, homogeneous mass, which was closely associated with the vertebral column. The T1-weighted magnetic resonance image showed low intensities, and moderate intensities were found on the T2-weighted magnetic resonance image. Gd-DTPA enhancement was remarkable on the tumor surface. The present case differed from ordinary neurogenic tumors in magnetic resonance imaging findings. Thoracoscopic resection of the tumor was performed. The tumor was associated with the sympathetic nerve. Histologic analysis revealed a benign granular cell tumor.     

Anomalous leukopoiesis in two patients with Crohn's disease.

Crohn's disease is a major form of chronic inflammatory bowel disease in the western world. The molecular genetic basis of Crohn's disease is unknown. In this study, we present evidence for anomalous leukopoiesis-namely, the generation of a leukocyte subset characterized by aberrant expression of gammadelta T cell receptor (gammadeltaTCR) with or without CD19 on a myeloid background-in two patients with Crohn's disease. The aberrant cells of patient 1 have the surface phenotype gammadeltaTCR + CD19 - CD14 + CD64 +. The aberrant cells of patient 2 have the surface phenotype gammadeltaTCR + CD19 + CD14 - CD64 + CD16 + CD13 + CD33 +. The results presented here are significant both in light of recent speculation that a critical defect in Crohn's disease may be at the level of hematopoiesis and because the CD19 gene lies within the region on chromosome 16 that corresponds with the Crohn's disease susceptibility locus IBD1.     

Endodermal sinus tumour of the ovary: a comparative light and electron microscopic study.

An endodermal sinus tumour of the ovary from a 12 year old girl is analysed light- and electron microscopically. The histological appearance is characterized by glandular-papillary structures and microcystic-reticular areas with inclusion of occasional endodermal sinuses. By electron microscopy immature and highly differentiated cells can be distinguished. In analogy to certain structures in the human yolk sac the most differentiated cells in the tumour are regarded as neoplastic endoderm. On the basis of transitional forms between immature and differentiated cells a development of the latter from the former is suggested. The cells in mesenchyme-like areas differ from those of solid and glandular parts merely in the degree of cytoplasmic differentiation but are otherwise believed to represent the same "cell line". In addition to other possible cell functions the cytoplasmic features of the differentiated cell indicates a protein synthesis and secretion. From our observations it is concluded that the endodermal sinus tumour originates from germ cells and differentiates into yolk sac endoderm. The ultrastructural differences between this tumour and clear cell carcinomas of the ovary are discussed.     

Sensitization to horse allergens without apparent exposure to horse. Report of two cases

It has been well established that occupational exposure to horses increases the risk of allergic sensitization to horse hair, on the contrary very few data have been published in individuals without direct contact with horses. We describe two cases of severe respiratory symptoms triggered by a single direct exposure in two patients with only 4-5 previous short contacts with horses during the entire their life, since they live in urban area of Naples. Cutaneous and serological diagnostic tests showed significant sensitization to horse dander other than some common inhalant allergens. Our report suggests that, in already (but unaware) horse-sensitized patients, the last direct contact with a horse has triggered severe respiratory symptoms. Passive carriage of horse allergen by the clothes of individuals in contact with horses/horse environments or a cross-reactivity between horse allergen (a lipocalin) and the major allergens (also lipocalins) of several furred animals are likely to explain our findings. Some other considerations can be derived by the clinical observation. First, it is likely that allergic sensitization to horse allergens could be more frequent than expected also in subjects without direct exposure to horse because of living in urban areas. Secondly, individuals suspected to be highly atopic or those already sensitized to some pet dander should be tested by SPTs/radio-allergo-sorbent test before the introduction of a pet indoors or, in the case of a big animal as the horse, a direct horse contact/entering into environments containing a horse.     

Methotrexate-associated lymphoma in patients with rheumatoid arthritis. Report of two cases

This report describes 2 patients with longstanding seropositive rheumatoid arthritis (RA) treated with oral methotrexate (MTX) who developed large cell lymphoma of B cell phenotype. In situ hybridization studies showed nuclear staining for Epstein-Barr virus (EBV) within the malignant lymphoid cells. In both cases, the lymphoma was undetectable several weeks after diagnostic biopsy followed by discontinuation of MTX. These observations suggest that, in patients with RA who develop an EBV-associated lymphoproliferative disorder, a trial of discontinuation of immunosuppressive agents may be warranted before chemotherapy is considered. In addition, there is a need for a heightened awareness of the development of lymphoma in this patient population.