Portal vein aneurysm: report of six cases and review of the literature

We studied the alpha-oxidation of phytanic acid in human fibroblasts of controls and patients affected with classical Refsum disease, rhizomelic chondrodysplasia punctata, generalized peroxisomal disorders and peroxisomal bifunctional protein deficiency. Cultured fibroblasts were incubated with phytanic acid, after which medium and cells were collected separately. 2-Hydroxyphytanic acid and pristanic acid were measured in the medium and cells by stable isotope dilution gas chromatography mass spectrometry. In controls, 2-hydroxyphytanic acid and pristanic acid could be detected in the medium after incubation with phytanic acid, proving that alpha-oxidation of phytanic acid via 2-hydroxyphytanoyl-CoA to pristanic acid was active and intermediates were excreted into the medium. In cells from patients with a defective alpha-oxidation (Refsum disease, rhizomelic chondrodysplasia punctata and generalized peroxisomal disorders) 2-hydroxyphytanic acid and pristanic acid were low or not detectable, showing that in these disorders the hydroxylation of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA is deficient. In cells with a peroxisomal beta-oxidation defect, 2-hydroxyphytanic acid and pristanic acid were formed in amounts comparable to those in the controls.     

Pentazocine fibrous myopathy: report of two cases and literature review

Two patients are described in whom the analgesic, pentazocine (Talwin), injected intramuscularly over long periods of time, resulted in a fibrous myopathy of the injected muscles. Although early testing of pentazocine indicated a low abuse potential and no skin or muscle complications, reports of such abuse and complications have since appeared in the literature, a review of which is included.     

Extradural myxopapillary ependymoma: report of two cases and review of the literature

The cauda equina is the most frequent location for ependymomas, particularly the myxopapillary variant, which generally arises from the filum terminale. These tumors have a characteristic histopathologic pattern and are usually easily recognized. The occurrence of these tumors in an extradural, sacrococcygeal, or subcutaneous location may prove challenging, particularly in the absence of any obvious central nervous system connection. We describe two such extradural cases, one with multiple regional and distant metastases and the other with multiple recurrences. The origin of these tumors from subcutaneous sacrococcygeal ependymal rests is postulated on the basis of earlier reports. Clinical and histopathological features are described and a review of the literature is presented.     

Cranial cystic epidermoid: report of two cases and review of the literature

Among non-Hodgkin's lymphomas occurring in childhood two major histologic subgroups can be identified: (1) Burkitt's lymphoma and (2) T-cell lymphoblastic lymphoma, an uncommon high-grade malignant non-Hodgkin's lymphoma. Although Burkitt's lymphoma with maxillofacial involvement is a well-documented disease, T-cell lymphoblastic lymphoma in the perioral region is rare. An unusual case of T-cell lymphoblastic lymphoma with initial oral manifestation in an 18-month-old child is presented.     

Calcifying odontogenic cyst: report of two cases and review of literature

The "Histological Typing of Odontogenic Tumours" (W.H.O., 1992) classified the Calcifying Odontogenic Cyst (C.O.C.) into two variants: the non-neoplastic cystic C.O.C. and the odontogenic ghost cell tumour, which is predominantly solid. We reported two cases of C.O.C.: a case with intraosseous development and another with extraosseous localisation, in the soft tissue of the alveolar area. The first case represents a cyst delimited by a squamous, non-keratinized epithelium, thickened in some areas through the accumulation of ghost cells (big pale-staining cells with a non-staining nuclear area). The connective tissue wall contains small ameloblastoma like islands. Dysplastic dentine islands, adjacente to the basal layer of the epithelium or in the connective tissue wall were also observed. The second case was a well-delimitated tumour consisting of ameloblastoma-like islands with numerous ghost cells inside. Islands of dysplastic dentine with psammomathous calcifications also exist. In certain histological sections microcystic aspects surrounded by ghost cells, dentinoid and ameloblastoma-like structures were noticed. The histochemical reaction for keratin and the immunohistochemical reaction for epithelial membrane antigen and for citokeratin were positive for ghost cells, suggesting their epithelial origin. Through this article we try to render pathologists sensitive with a particular and rare maxillary tumour.     

Ichthyosiform erythroderma and cardiomyopathy: report of two cases and review of the literature

We report two children with ichthyosiform erythroderma who at the ages of 9 weeks and 8 years, respectively, developed dilated cardiomyopathy, which was fatal in one and required heart transplantation in the other. A link between these conditions is considered likely, either as a primary genetic syndrome or secondary to micronutrient deficiency and/or infection. Owing to its insidious onset, cardiomyopathy may be overlooked, or symptoms attributed to the other conditions such as severe infections and failure to thrive that are common in these patients. We therefore recommend that children with congenital erythroderma are monitored closely, clinically and with echocardiography, for cardiomyopathy.     

Chondrosarcomas of the skull base: review of the literature and report of two cases

The use of intravital microscopy as a tool for studying the microcirculation has increased greatly over the last several decades. Early microscopes provided the first pictures of the microcirculation, but were cumbersome to use and subjected the tissue to a high light intensity, a problem which has recently become the subject of much discussion. The goal of this project was therefore to build a more ergodynamic microscope which minimizes the light exposure to the tissue. The automation of the microscope controls provides a platform on which other options can be built into the microscope, such as an autofocus feature. Furthermore, the use of the Optimas software also opens the possibility for on-line data processing.     

Blunt internal carotid artery trauma: report of two cases and review of the literature

Blunt injury of the internal carotid artery (ICA) is a rare entity that should be considered by Maxillofacial surgeons in patients with facial fractures. Its recognition is often delayed because of the common association with other severe multi-system injuries. Early diagnosis is the key to successful management; the arteriography plays a confirmatory role on the diagnosis and determines whether surgical management of the injury is feasible. Therapeutic alternatives vary from one center to another; they include observation, conservative treatment, anticoagulation, ligation of the carotid artery with or without extracranial-intracranial bypass, and arterial reconstruction.     

Choroid plexus carcinoma in infants: report of two cases and review of the literature

The new cytostatics titanocene dichloride and vinorelbine were compared to cisplatin and paclitaxel using a human ovarian cancer xenografts model. Biopsy material from a native human ovarian carcinoma was expanded and transplanted into 96 nude mice. The animals were divided into six treatment groups: cisplatin 3 x 4 mg/kg, paclitaxel 5 x 26 mg/kg, vinorelbine 1 x 20 mg/kg, titanocene dichloride 3 x 30 mg/kg, titanocene dichloride 3 x 40 mg/kg and a control group treated with 0.9% saline. Each experiment was repeated with eight mice in each treatment group. Treatment groups were evaluated in terms of average daily increase in tumor volume and average daily body weight increase of nude mice based on slopes of least-square regressions performed on individual animals. The slope factors alpha and beta of the body weight (alpha) and tumor volume changes (beta) within each group during the course of an experiment were calculated. Both a statistically significant decrease (p<0.05) in the body weight of the experimental animals (cisplatin: alpha = -0.5163, vinorelbine: alpha = -0.6598, paclitaxel: alpha = -0.6746, titanocene dichloride 3 x 30 mg/kg: alpha = -0.6259, titanocene dichloride 3 x 40 mg/kg: alpha = -0.7758) and a significant reduction (p<0.05) of the increase in tumor volume (cisplatin: beta = 12.049, vinorelbine: beta = 0.504, paclitaxel: beta = -1.636, titanocene dichloride 3 x 30 mg/kg: beta = 6.212, titanocene dichloride 3 x 40 mg/kg: beta= -0.685) was shown in all treated groups compared to the control group (alpha = -0.1398; beta = 23.056). No significant weight changes were observed between the individually treated groups. A statistically significant reduction of the tumor growth occured under paclitaxel (beta = -1.636), vinorelbine (beta = 0.504) and titanocene dichloride medication 3 x 40 mg/kg (beta = -0.685), as compared to the group treated with cisplatin (beta = 12.049). We found titanocene dichloride to be as effective as paclitaxel and more effective than cisplatin. Vinorelbine seems to be a very effective antineoplastic agent exhibiting a significant higher cytostatic effect than cisplatin. Both titanocene dichloride and vinorelbine provide new therapeutic options in women with ovarian carcinoma not responding to standard chemotherapy.     

Choledochal cysts in adults: a report of two cases and review of the literature

A choledocal cyst is a dilation of some component of the biliary tract that may include both intra- and extra-hepatic sites. They are classified into six types, all of which are relatively rare. Previously, choledochal cysts were treated with biliary-enteric bypass procedures. The current recommendation is to attempt complete excision to minimize the known risk of malignancy and the development of recurrent cholangitis or pancreatitis that may occur in patients with these cystic lesions. Two cases are discussed in which type I choledochal cysts presented. One was removed from a 31-yr-old man who presented with vague abdominal complaints the other from a 32-yr-old man who presented with pancreatitis. The epidemiology, diagnosis, surgical treatment, and risk of cancer in choledochal cysts is described.     

Neuroendocrine tumours of the digestive tract report of two cases and literature review

Neuroendocrine tumors (NET) of the digestive system are rare. They comprise only 1% of all the tumors of the gastrointestinal tract. The aim of this study is to report two cases of exceptional localization of non secretary neuroendocrine tumors. Their difficulty in diagnosis, treatment and controversy in chemotherapy merit their study in depth. These are two patients that were treated in the department of general surgery (Beau Séjour) in Charles Nicolle Hospital for non secretary (NET) of the pancreas and stomach. The first was presented as a painless epigastria mass with frequent diarrhea. The second was presented as a painful mass in the left hypochondriac area without any other symptoms. Biochemical evaluation confirmed their non secretary properties. Surgery remains the first line of treatment. It was possible for the gastric, but not for the pancreatic tumor. Both were very advanced and had metastasis. Histopathological evaluation and immunohistochemical study using the method of peroxydase antiperoxydase (PAP) complexes for some special monoclonal antibodies confirmed the diagnosis. Chemotherapy of both cases was mal tolerated and was discussed in details.     

Renal myxoma. A report of two cases and review of the literature

Renal myxomas are rare neoplasms. Seven cases have been reported, of which only two are convincingly diagnosed as myxoma; the remaining cases exhibit features of sarcoma, fibroepithelial polyp, or myxolipoma. We report two additional cases; one in a 52-year-old man and another in a 68-year-old woman. They were discovered incidentally by radiological examination. The resected kidney in both patients contained a well-demarcated gelatinous intraparenchymal tumor, which consisted of occasional slender spindle cells scattered in an abundant myxoid stroma, closely resembling myxomas of other sites. The tumor cells showed immunoreactivity for vimentin but not for S-100 protein, epithelial membrane antigen (EMA), CAM 5.2, HHF-35, or smooth muscle actin. Ultrastructural features were of fibroblast-like cells with an elaborate network of cytoplasmic processes. The differential diagnosis of myxoid tumors of the kidney includes myxoid variants of renal sarcomas and carcinomas, renomedullary interstitial cell tumors, and fibroepithelial polyps. It is important to recognize the existence of a renal myxoma, to avoid confusing this benign tumor with the malignant neoplasms with secondary myxoid features that may involve the kidney.     

Endobronchial lipoma: two cases and review of the literature

Endobronchial lipoma is a benign tumour of the large bronchi occurring in middle-aged men. To the 38 successfully treated cases in the English literature a further 2 are added. The symptoms are those of obstructive pneumonitis mimicking bronchogenic carcinoma, and the result of delayed therapy may be bronchiectasis. Treatment includes local resection through a bronchoscope or a bronchotomy incision, or removal, if necessary, of the obstructed lobe or lung at thoracotomy. Smoking may be important in the pathogenesis of this tumour.     

Bone marrow transplantation in Shwachman-Diamond syndrome: report of two cases and review of the literature

Patients with Shwachman-Diamond syndrome (SDS) have an increased frequency of myelodysplasia and leukemic transformation. We described two patients who received allogeneic stem cell transplantation and developed multiple complications, including seizure, hyperglycemia and renal tubular acidosis. A review of the literature showed that patients with SDS appeared to have an increased incidence of various transplant-associated problems. These patients frequently have underlying organ dysfunction and should be managed with extreme caution when treated with allogeneic stem cell transplantation.     

Focal myositis of the floor of mouth: report of two cases and review of the literature

Focal myositis is an uncommon inflammatory myopathy of unknown cause affecting skeletal muscle. It may be mistaken on clinical evaluation for a malignant neoplasm. We describe two cases, both involving the mylohyoid muscle of the floor of the mouth. In each case excisional biopsy of a firm indurated mass revealed a focal lymphocytic and histiocytic infiltrate associated with degenerating and regenerating skeletal muscle fibers. No clinical or biochemical evidence of generalized muscle disease was seen at presentation or at 1-year and 7-year follow-up examination. The literature on focal myositis involving the head and neck region is reviewed.     

Pigmented squamous cell carcinoma of the skin: report of two cases and review of the literature

Melanoma is the most common malignant tumor in which melanin synthesis occurs, although other nonmelanocytic tumors synthesize melanin or contain nonneoplastic melanocytes. We present two cases of infiltrating pigmented squamous cell carcinoma of the skin and review the clinical, morphologic, and ultrastructural features. Melanin was found in epithelial tumor cells as well as in macrophages and dendritic melanocytes. Interestingly, one of the neoplasms was associated with an adjacent melanocytic nevus and pigmented solar keratosis. Immunohistochemical analysis showed that neoplastic cells stained for keratin and melanin-filled dendritic cells were found to be S-100 protein and HMB45 positive. A careless examination of the immunohistochemical stains for S-100 protein and HMB45 could cause the misdiagnosis of melanoma, a neoplasm that has a more ominous outlook.     

Dystrophic kyphoscoliosis in neurofibromatosis type I: a report of two cases and review of the literature

As the Department of Defense initiates Tricare, its new system of integrated health delivery, outcome assessment will become increasingly important. Traditional outcomes have focused on disease and its more serious consequences, disability and death. Patient satisfaction is only recently emerging as an important measure of the quality of health care delivery. In this paper, we focus on patient satisfaction, exploring its historical roots, its evolution as an outcome measure, and its potential utilities and weaknesses in quality assessment.     

Primary gastric choriocarcinoma: case report with review of world literature

A case is herin reported of a primary gastric choriocarcinoma occurring in an elderly woman associated with very high titers of circulating chorionic gonadotropin. Unlike the majority of previously reported cases, in which adenocarcinomatous components were present, the tumor in this case was purely trophoblastic. The clinicopathologic findings of the previously reported cases are summarized, along with the various theories of histogenesis.     

Laryngeal melanosis: report of four cases and literature review

Tumors grafted into mice may modify the proliferation of normal cell populations. In this paper, we have studied the evolution of mitotic activity (MA) in duodenal-crypt enterocytes of ES12a hepatocarcinoma-bearing mice; a total of 87 28-day-old female animals of the C3H/S strain were used after standardization for circadian-periodicity analysis. The mice were distributed into two groups: those remaining intact and those receiving tumor grafts. Each group was then divided into six batches (n = 6-10), one of which was sacrificed every 4 h over a period of one day. A dose of colchicine (2 micrograms/g) was administered to each animal 4 h before killing. Samples of duodenum were fixed in 10% (v/v) buffered formalin and processed for assessment of mitotic activity. The number and topographic localization of the colchicine-arrested metaphases were recorded among the entero-cytes within 20 longitudinal sections of the duodenal crypts in each animal. From these data the mitotic indices over the total crypt-cell population as well as within each previously-established zone were determined along with mean +/- SEM for each experimental group. The statistical significance of the differences among the data were analyzed by Student t test. The results show that the presence of ES12a tumor inhibits the mitotic activity of the duodenal-crypt enterocytes and produces an apparent temporal shift in the peak and trough within the circadian curve for this growth parameter.