Congenital infantile fibrosarcoma: a case study

Congenital infantile fibrosarcoma (CIFS) is a rare soft-tissue malignancy most commonly involving the extremities. Metastases are rare; however, local recurrence is common. Because the tumor is so rare and clinical experience is lacking, both diagnosis and treatment are difficult. The complex planning and implementation of neonatal and oncologic care require ongoing collaboration between both nursing specialties. This case study discusses the pathophysiology, diagnosis, and management of an infant with CIFS, with emphasis on the nursing care of the patient and family.     

Primary fibrosarcoma of the right ventricle

Mexican literature has information of two fibrosarcomas in the atria. In the present work the first fibrosarcoma of the present work the first fibrosarcoma of the right ventricle found in Mexico is presented. This case behaved clinically, electrocardiographically, and phonomechanocardiographically like an Ebstein's disease, with the exception that in the phono a giant "a" wave was found. A review is mad of the clinical history, EKG, radiologic, and phono findings, as well as the laboratory analysis and data found in the autopsy.     

Consistent numerical chromosome aberrations in congenital fibrosarcoma

Since its introduction by Schirmer in 1903, the Schirmer test has been widely used for the assessment of the adequacy of tear production. Although the faults of the Schirmer test have been well documented, it is nevertheless still being used to assess tear production, especially in the diagnosis of keratoconjunctivitis sicca (KCS) and for screening potential contact lens wearers. We summarize the findings and conclusions of some of the previous studies.     

A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma

The purpose of this study was to investigate whether patients who developed alcoholic liver disease have more awareness of the link between their behaviour and subsequent health than patients with non-alcoholic liver disease and people with drink problems with no liver disease. This study included three groups of patients, alcoholic liver disease (ALD) (n=57), non-alcoholic liver disease (n=77), and problem drinkers with no liver disease attending a London community day treatment centre (ACCEPT) (n=115). Health locus of control differentiates people into two groups, health externals who are individuals who maintain that their health is largely determined by external factors, as opposed to health internals, who believe that their behaviour plays a major role in determining their subsequent health or illness. The results of the Health Locus of Control (HLC) scale administered to the above subjects suggested that the ALD group had limited insight into the relationship between their drinking and subsequent liver disease, compared to the ACCEPT group. It is suggested that alcoholic liver disease patients receive counselling as part of their total management.     

Extraskeletal osteosarcoma of the hand. A case report

T cell mediated autoimmunity may be important in inflammatory skin disease, but target autoantigens have not previously been described. In studies aimed at defining T cell epitopes, aqueous extracts of normal facial and plantar stratum corneum have consistently been found to induce potent proliferation of peripheral blood mononuclear cells from normal donors and patients with inflammatory skin disease, giving stimulation indices up to 80. Potent stimulation was seen with both autologous and allogeneic stratum corneum extracts. Because of the presence of inhibitory material, demonstration of the stimulatory activity was critically dependent on extract concentration, and was facilitated by short-term pulsing of cultures with extract. The proliferation of cells purified from peripheral blood mononuclear cells by immunomagnetic beads and immunophenotyping of cell lines generated from peripheral blood mononuclear cells, confirmed the T cell nature of the response to stratum corneum extracts. The activity was inhibited by HLA-DR monoclonal antibody, indicating the presence of antigen or superantigen. Tetanus toxoid reactive clones and a purified protein derivative reactive line failed to respond to the stratum corneum extracts, indicating that the active material is not a nonspecific T cell stimulant such as a cytokine or mitogen. This and the failure of recombinant interleukin-1alpha to stimulate peripheral blood mononuclear cells in concentrations up to 1000 U per ml, indicate that the activity is not due to interleukin-1. We propose the hypothesis that antigenic or superantigenic material is normally sequestered from the immune system in the epidermis, but induces T cell activation when released following wounding and in disease, and that this represents an important and previously unrecognized pathogenic mechanism.     

Toe transplantation in congenital malformations of the hand

Toe transfer is a well established procedure for thumb and finger reconstruction after mutilation. The indications in congenital malformations are a mater of controversy. Out of a personal series of 209 patients, 42 were children presenting a congenital malformation. Thirty six, with 46 transfers were available for review. There is only one failure at the beginning of our experience. The main indication was absence of pinch either due to absence of thumb (like in congenital band syndrome or some extreme cases of ulnar club hand or cleft hand) or absence of long finger (like in symbrachydactyly monodactylous type) or lack of both thumb and finger (like in peromelic type of symbrachydactyly). In this last type, we have been disappointed by the functional result of the distal implantation of two second toes taken from both feet; we have proposed a "stub" operation consisting in a second toe transfer on the anterior aspect of the radial epiphysis to take advantage of the mobility of the wrist and the availability of plenty tendon transfer (in this proximal situation). When planing to "built" an absent pincer, an early age is mandatory for operation (mean 12 months), to ensure a good cortical integration. A less frequent indication is a partial toe transfer with a vascularized epiphysis to provide growth and mobility in some cases of thumb hypoplasia (like in symbrachydactyly or Blauth and Manske type III b). Results are difficult to assess due to the early operation but if the mobility has been disappointing (mean 32 degrees), sensibility (mean 2PD 5 mm) and growth were excellent.     

A case of congenital intraspinal neuroblastoma

A case of congenital intraspinal neuroblastoma with paraplegia in a newborn is described, and a review of previously reported cases is presented. Calcification within the spinal canal and abdominal mass was noted. At age 75 days, the patient underwent a resection of almost the entire retroperitonial tumor. A magnetic resonance scan showed hepatic metastasis, and chemotherapy was changed to a more intensive regimen. The liver metastasis disappeared; however, neurological signs persisted. A literature review of 38 cases showed 26 patients had a laminectomy and 12 patients did not. Treatment with or without laminectomy was associated with a poor prognosis for neurological recovery, suggesting cord compression must occur antenatally, and irreversible damage occurs before birth. Congenital intraspinal neuroblastoma has a relatively good prognosis (survival rate, 91.9%), although complete neurological recovery is highly unusual. Based on these findings, the authors conclude that a laminectomy is not indicated for those patients.     

A case of infantile digital fibromatosis with spontaneous regression

We reported a fourteen-month-old boy with infantile digital fibromatosis. At the age of seven months, a nodule appeared on the back of the left third toe, and developed into a slight red tumor divided into five hemispherical nodules. Histopathologically, spindle-shaped tumor cells with an eosinophilic inclusion body in the cytoplasm were seen in the dermis. Electron microscopy showed a dense body in the cytoplasm of the tumor cells. One year and two months after the first visit, the tumor regressed without any aggressive treatment. Japanese cases of infantile digital fibromatosis were reviewed. The literature review and our case suggest that the tumor should be observed without any aggressive treatment unless it causes mobile dysfunction of the affected finger or toe.     

Characteristics of infantile cases of congenital dilatation of the bile duct

At the end of the growout phase of broiler production essentially all broilers are caught and loaded into coops or cages entirely by hand. The catching of broilers remains one of the few aspects of broiler production not yet automated. Vacuum systems, permanent conveyors recessed in the floors of growout facilities, collecting mats, scoops, and self-propelled, combine-type devices have been tried with little success over the past 30 yr. Renewed efforts at developing mechanical broiler harvesters have been encouraging in recent years. Several equipment companies in North America and Europe have developed prototype mechanical harvesters that appear to have promise. The benefits of mechanical harvesting as compared to hand catching include lower costs and improved working conditions. In addition, studies conducted thus far indicate that mechanical harvesting will improve bird welfare both from a stress and injury standpoint.     

Primary bronchopulmonary fibrosarcoma: report of a case

We herein report a case of primary bronchopulmonary fibrosarcoma in a 70-year-old man. The patient was referred to our hospital for investigation of hemosputum and an abnormal shadow. On admission, chest radiograph and computed tomography scan showed a mass lesion in right S3 and an infiltrative shadow in the right upper lobe. Transbronchial biopsy specimens showed findings of malignancy, and adenocarcinoma was suspected. A right pneumonectomy was performed, and pathologic examination confirmed a diagnosis of fibrosarcoma. The patient had an uneventful recovery and no sign of recurrence has been found in the year since his operation, although strict follow-up is essential.     

A case of congenital quadricuspid pulmonary valve

The present report describes a case of congenital quadricuspid pulmonary valve, encountered during the dissection of an 84 year-old Japanese female cadaver at Kanazawa Medical University. In this case, no evidence of heart failure was found clinically or pathologically. The semilunar valve on the right posterior was the biggest and that of the left anterior was the smallest.     

Genetics of limb development and congenital hand malformations

Differences in breeding values between dominance and additive models were examined theoretically and with field data. Data included 5.2 million records on stature from 3.0 million US Holsteins. The largest full-sib family had 29 animals, and 7% of all animals had at least one full sib. The dominance model, which accounted for dominance covariances, included the following effects: management, age, stage of lactation, permanent environment, animal additive, and parental dominance (one-quarter of dominance variance) as well as a regression coefficient for inbreeding percentage. Two reduced models were also assumed; in the first, the parental dominance effect was removed, and, in the second, the inbreeding regression coefficient was also removed. The correlations between breeding values in the three models were > 0.999, but breeding values of some animals from full-sib families changed > 5 standard deviations of parental dominance. The largest changes were observed for parents with large numbers of full-sib progeny, with limited information from parents, and without individual performance records. On average, the differences were up to four times larger for cows than for bulls and up to five times larger for dams than for sires. The greatest differences in breeding values between the dominance and the additive models were observed for dams with full-sib progeny, female full sibs, and low reliability bulls with full sibs in the extended family. Animals with large amounts of additive information as progeny-tested bulls were influenced little by the inclusion of dominance. Animals with a large proportion of information coming from animals with dominance relationships, such as cows originating via embryo transfer changed the most.     

A case of contralateral pneumothorax after right pneumonectomy

A 46-year old man, who had a history of right pneumonectomy for pulmonary tuberculosis 28 years ago, was referred to our hospital for surgical treatment of contralateral pneumothorax. The patient underwent multiple excisions of bullae and parietal pleurectomy through the median sternotomy in order to save pulmonary function. The postoperative course was satisfactory as he could maintain preoperative pulmonary function. He returned to his work soon. We consider that the median sternotomy would be the most useful approach to save pulmonary function of patients who plan to undergo surgical treatment of contralateral pneumothorax after pneumonectomy.     

An unusual presentation of congenital infantile myofibromatosis arising from the interspinous ligament

The authors describe an extremely rare presentation of congenital infantile myofibromatosis. A full-term newborn boy presented with a thumb-sized subcutaneous mass on the mid-spinal line between the 2nd and 3rd lumbar spinous processes. A solid tumor arising from the interspinous ligament was resected. Microscopic and immunohistochemical studies revealed myofibromatosis.     

The position of symbrachydactyly in the classification of congenital hand anomalies

The clinical features of 53 cases of intercalated hypoplasia and 113 cases of distal aplasia are reviewed and compared with each other and with 129 cases of syndactyly. Tri-, di- and mono-phalangeal symbrachydactyly, and adactyly with nubbin digits are consecutive anomalies. Transverse deficiency may result if the mesenchyme is damaged severely, and if damage is mild and formation has continued, intercalated transverse deficiency may occur. Webbing in symbrachydactyly may result from failure of the apical ectodermal ridge under the influence of damaged mesenchyme.