Orbital amyloidosis and radiotherapy: A case report and review of literature.

An 80 year-old woman was referred for a one-eyed palpebral edema. She had ptosis and oblique diplopia resistant to corticotherapy. An orbit magnetic resonance imaging showed a mass infiltrating the optic nerve, with an enhancement of the whole orbital musculature. Biopsies of the lacrimal gland were positive for immunoglobulin light chain amyloidosis. Debulking surgery is the main treatment modality for symptomatic patients with localized orbital amyloidosis. Diffuse orbital involvement may make excision difficult. We managed to treat her through radiation therapy to allow a surgical procedure, delivering 20Gy in 10 fractions. The main aim of radiation therapy was to eradicate the B cells that produced the amyloid light chain deposition. After radiation therapy, the orbital inflammatory caused by the lymphoid infiltration diminished and corticotherapy could be stopped. The surgery could be performed with excellent clinical results. At 2-year follow-up, neither tumor regrowth nor late complications were detected.

Effect of culturing processes and copper addition on laccase production by the white-rot fungus Fomes fomentarius MUCL 35117.

AIM: To produce high laccase activities from the white-rot fungus Fomes fomentarius. METHODS AND RESULTS: Different culturing methods, viz, cell immobilization on stainless steel sponges and plastic material and solid-state fermentation (SSF) using wheat bran as substrate were used for laccase production by the white-rot fungus F. fomentarius. The SSF study expresses the highest laccase activities, nearly to 6400 U l(-1) after 13 days of laboratory flasks cultivation. When the wheat bran medium was supplemented with 2 mmol l(-1) copper sulfate, laccase activity increased by threefold in comparison to control cultures, reaching 27 864 U l(-1). With the medium thus optimized, further experiments were performed in a 3 l fixed-bed bioreactor (working volume 1.5 l) leading to a laccase activity of about 6230 U l(-1) on day 13. CONCLUSIONS: The results obtained clearly showed the superiority of wheat bran for laccase production over stainless steel sponges and plastic material. Supplementing the wheat bran solid medium with 2 mmol l(-1) copper sulfate allowed obtaining high activities at flask scale. The system was scaled to fixed-bed laboratory reactor. SIGNIFICANCE AND IMPACT OF THE STUDY: The high enzyme production along with the low-cost of the substrate, showed the suitability of the system F. fomentarius- SSF for industrial purposes.

[Primary rhabdomyosarcoma of the pleura presenting as recurrent pneumothorax]. / Pneumothorax récidivant révélateur d'un rhabdomyosarcome primitif de la plèvre.

INTRODUCTION: Rhabdomyosarcoma is the most common soft tissue sarcoma in the first two decades of life. Its most common location is the head and neck. It rarely arises in the thorax including the pleura. There are only 7 previously reported cases in the literature. We report a case of primary pleural rhabdomyosarcoma with an unusual clinical presentation and location. Our aim is to discuss the clinical presentation, treatment and prognosis of this uncommon location of rhabdomyosarcoma. CASE REPORT: We report a case of primary embryonal rhabdomyosarcoma of the pleura in a boy of 21 months. The presentation was characterized by recurrent spontaneous pneumothorax. A computed tomography scan showed only pleural detachment with no evidence of any pleural disease. The malignant cells incidentally found in routine pleural biopsy were the diagnostic clue. The rhabdomyoblastic nature of these cells was confirmed by positive immunostains for myoD1 and desmin. Investigation for metastases was negative. Despite chemotherapy, the tumour quickly increased in size and the infant died from acute respiratory failure. CONCLUSION: Thoracic rhabdomyosarcoma is rare and remains clinically silent for a long time. Its management is still controversial. Prognosis is generally poor when compared with other locations.

[Extrafollicular adenomatoid odontogenic tumour: report of two cases]. / Tumeur odontogène adénomatoïde extrafolliculaire: à propos de deux cas.

The purpose of this study was to analysis the clinicopathological features of adenomatoid odontogenic tumour extrafollicular and to discuss the diagnosis pitfull. We present two cases diagnosed and followed at the C.H.U. Habib Bourguiba, Sfax. It is about a 15 year-old boy and a 46 year-old woman who presented gingival swelling. The panoramic radiographs showed in both cases, a radiolucent area affecting teeth 23, 24 and 35 respectively. A computed tomography scan, realized at the second cas, demonstrated an expansile lesion with a sclerotic rim. An exploratory surgical approach was chosen and the final diagnosis was microscopically confirmed to be an extrafollicular variant of adenomatoid odontogenic tumour. The patients were healthy and have not shown any signs of recurrence at follow-up. The two cases described illustrate clinical and radiographic features of the extrafollicular variant of adenomatoid odontogenic tumour. Careful diagnostic procedure and adequate interpretation of radiographic findings may result in a correct diagnosis with otherwise may result in unnecessary endodontic treatment.

[Retroauricular dermatofibroma in a child simulating a malignant tumour]. / Dermatofibrome rétro-auriculaire chez un enfant simulant une tumeur maligne.

BACKGROUND: Dermatofibroma represents one of the commonest benign soft tissue tumours, and its diagnosis is usually straightforward if classical clinicopathologic features are evident. It occurs frequently on the extremities and trunk, often in young adults during the 3(rd) and 4(th) decades. We report a peculiar case of an aggressive cellular dermatofibroma in a child that simulated a malignant tumour. Our aim is to discuss the differential diagnosis. CASE REPORT: An 8-year-old boy presented with a retroauricular tumour. Clinically, the lesion, 2 cm in diameter, was located in the left retroauricular region. It was nodular, erythematous, infiltrating the underlying tissue with an anterior spread leading to an amputation of the auricular lobule and a retraction of the tragus. Computerized axial tomography revealed a local tissular thickening extending in soft tissues without any bone involvement. A malignant lesion was initially suspected. A biopsy was performed. It was followed by a surgical resection. The lesion had the full microscopic characteristics of a cellular dermatofibroma. CONCLUSION: Dermatofibroma is one of the major differential diagnosis of nodular cutaneous tumours in children that simulate malignancy. Histologic examination with immunohistochemical study is essential for diagnosis.

[Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation]. / Malformation adénomatoïde kystique congénitale du poumon: à propos de trois cas de révélation tardive.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.

Penconazole alters redox status, cholinergic function, and membrane-bound ATPases in the cerebrum and cerebellum of adult rats.

Pesticides exposure causes usually harmful effects to the environment and human health. The present study aimed to investigate the potential toxic effects of penconazole, a triazole fungicide, on the cerebrum and cerebellum of adult rats. Penconazole was administered intraperitoneally to male Wistar rats at a dose of 67 mg kg-1 body weight every 2 days during 9 days. Results showed that penconazole induced oxidative stress in rat cerebrum and cerebellum tissues. In fact, we have found a significant increase in malondialdehyde, hydrogen peroxide, and advanced oxidation protein product levels, as well as an alteration of the antioxidant status, enzymatic (superoxide dismutase and catalase) and nonenzymatic (glutathione), the cholinergic function, and membrane-bound ATPases (Na+/K+-ATPase and Mg2+-ATPase). Penconazole also provoked histological alterations marked by pyknotic and vacuolated neurons in the cerebrum and apoptosis and edema in the cerebellum Purkinje cells' layer. Therefore, the use of this neurotoxicant fungicide must be regularly monitored in the environment.

Biostoning of denims by Penicillium occitanis (Pol6) cellulases.

The Pol6 mutant of Penicillium occitanis, secreting a large quantity of cellulases, was cultivated in fermentor using a local paper pulp as an inducer substrate. A high titer of extracellular cellulase activity was reached after a fed batch process: 23 IU x ml(-1) filter paper activity, 21 IU x ml(-1) CMCases activity (endoglucanase units) and 25 mg x ml(-1) of proteins. Various tests were done to compare the action of the P. occitanis cellulases with those commercially available and with the traditional stonewashing process. This cellulase preparation was successfully applied in a biostoning process at an industrial scale. The abrasive effect of the P. occitanis cellulases was very uniform and with an efficiency comparable to that obtained by the commercial ones.

[Study of sex determination gene (SRY) in 46,XY gonadal dysgenesis]. / Etude du gène du déterminisme du sexe (SRY) dans les dysgénésies gonadiques 46,XY.

During mammalian embryogenesis, the presence of the SRY gene determines the bipotential gonad to develop as a testis. 46,XY sex reversal has been described in man. It is associated with an essentially female phenotype and a streak gonad. In a collaborative study, we analysed 36 patients with a 46,XY sex reversal. The testis determining region of the Y chromosome was analysed by Southern blotting and by DGGE analysis of the SRY open reading frame (orf). We found a total of 7 mutations in the testis determining region including the SRY gene. This brings to 19 the total number of mutations in SRY associated with sex reversal. No relationship was found between the SRY status and the presence or absence of gonadoblastoma. However, a correlation was observed between the SRY genotype and the histology of the gonad. A mutant in SRY is associated with a completely dysgenetic gonad. The presence of immature testicular tubules is usually observed when SRY is normal. These latter results suggest the existence of as yet unidentified testis determining genes.

[Non-Hodgkin lymphoma of maxillofacial soft tissue: a report of two cases and review of the literature]. / Lymphomes non-hodgkiniens des tissus mous maxillofaciaux : à propos de deux cas et revue de la littérature.

The extranodal non-Hodgkin lymphomas of maxillofacial soft tissues are rare. We report two cases of maxillofacial soft tissue non-Hodgkin lymphoma treated with chemotherapy followed by localized radiotherapy with complete remission after 3 and 6 months. We study the clinical, radiological and histopathological features as well as the treatment and the prognosis of extranodal non-Hodgkin lymphomas maxillofacial muscles.

[Rare cause of pulmonary embolism in a child]. / Embolie pulmonaire hydatique chez un enfant.

Cardiac hydaticyst is a rare condition and accounts for only 0.5 to 2% of all visceral locations of hydatid disease. The objective of this study was to point out the main clinical, radiological, and disease-course characteristics of this rare and serious pathology. We report a 13-year-old patient with a hydatid pulmonary embolism caused by a hydatid cyst of the interauricular septum. The diagnosis was established by transthoracic echocardiography, thoracic CT scan, and hydatid serology. Surgery was performed without delay and the outcome was good after 15 months of follow-up. This case underlines the need for rapid diagnosis and surgery before complications of cardiac hydatid cyst.

Unusual cause of intestinal obstruction: ileal endometriosis.

Endometriosis is a common condition affecting the female genital tract, but involvement of the ileum is very rare. Its symptoms are vague and are similar to other benign and malignant disorders, and radiographic findings lack specificity. We report the case of a 23-year-old woman presenting with acute intestinal obstruction for whom preoperative diagnosis favoured acute appendicitis. Laparotomy revealed ileal stenosis. A partial small bowel resection was performed. Pathological examination diagnosed ileal endometriosis. Endometriosis may be a cause acute abdominal pain in women, and should be considered in differential diagnosis. Difficulties in establishing its diagnosis are discussed.

Glomus tumour of uncertain malignant potential.

A 71-year-old man presented with a several year story of a slowly enlarging, painful mass of the knee. Surgical findings showed a subcutaneous, well-circumscribed mass fixed to the patella, and a wide resection was performed. Macroscopically, the tumour measured 6 x 5 x 5 cm and was well circumscribed. The tumour was characterised by superficial location, large size and focal marked atypia. In addition, areas of benign glomus tumour (GT) were identified. The findings appear to be consistent with the diagnosis of GT of uncertain malignant potential.

[Polymorphous low-grade adenocarcinoma: a palatine and a labial location]. / Adénocarcinome polymorphe de bas grade: deux localisations palatine et labiale.

INTRODUCTION: Polymorphous low-grade adenocarcinoma (PLGA) is a variant of malignant tumor of minor salivary glands. We had for aim to discuss morphology, evolution and differential diagnosis of this rare tumor. CASE REPORTS: The first case was a 65-year-old woman admitted for a two-month history of a right submaxillary swelling. The examination revealed a tumor of the right side of the palate. Biopsy was positive for a pleomorphic adenoma. The mass was enlarged and surgical resection was performed. The diagnosis was a partially resected PLGA. The patient also received adjuvant radiotherapy. There was a local recurrence 28 months after complete treatment. The second case was a 57-year-old woman who consulted for a 12-year history of lower lip swelling. The examination revealed a painless 2cm long located nodule in the mucosal side of the lower lip. An excisional biopsy was performed. The pathological examination concluded to a completely resected PLGA. The patient was free of disease at 54 months follow-up. DISCUSSION: The morphologic diversity and cytological uniformity of PLGA may make the differential diagnostic difficult especially with adenoid cystic carcinoma and pleomorphic adenoma. Its aggressiveness is proved by a local infiltrative growth pattern requiring a large surgical excision.

Mesenchymal hamartoma of the chest wall in an infant.

Mesenchymal hamartoma of the chest wall is a very rare, benign tumour with distinct clinical, radiological and histopathologic characteristics. The lesion develops during foetal life, and is present at or shortly after birth with an extrapleural mass arising from the rib cage with or without respiratory distress and marked rib deformity. Several imaging techniques have been used for diagnosis, but a definitive diagnosis is established only by histopathological examination. Such lesions are composed of a varying admixture of hyaline cartilage that has features resembling growth plate cartilage, along with fascicles of spindle cells, woven bone and hemorrhagic cysts. Accurate diagnosis of mesenchymal hamartoma is important since many chest wall masses in children are malignant. We report a case of mesenchymal hamartoma of the left posterior chest wall surgically resected in an infant who was found to have a palpable mass at birth. Two years after surgery, the patient is alive and well, with no evidence of recurrence.

[Recurrent toxoplasmic retinochoroiditis after clindamycin treatment]. / Récurrence de la rétinochoroïdite toxoplasmique après traitement par clindamycine.

PURPOSE: To evaluate the efficacy and safety of subconjunctival injections of clindamycin associated with oral corticotherapy in the treatment of toxoplasmic retinochoroiditis (TRC) and its effect on recurrence. PATIENTS AND METHODS: This study investigated 21 patients (10 males and 11 females) who presented to an ophthalmological department from 1995 to 2000, aged 14-55 years (mean, 28 years), with unilateral TRC. They were treated with subconjunctival injections of clindamycin and systemic corticotherapy at a dose of 1 mg/kg/day. Clinical follow-up consisted of assessment of visual acuity and biomicroscopic fundus examination, completed by retinal angiography if necessary. RESULTS: We observed clinical improvement in 68.75% of patients within 4 days and in all patients healing of the chorioretinal area within 1.6 months. During the follow-up period ranging from 6 to 52 months (mean, 22 months), there have been two recurrences (9%) over the first 18 months. Tolerance of the injections was good. Serious side effects were noted in two patients: one case of conjunctival necrosis and one case of corneal and conjunctival erosion. CONCLUSION: In treating ocular toxoplasmosis, clindamycin appears to be a safe and effective substitute for the classic protocol (pyrimethamine-sulfadiazine, pyrimethamine-azithromycin), for which a 50% recurrence rate has been reported. It provides relatively good protection against recurrence and warrants testing in further controlled studies.

Stabilization of Penicillium occitanis cellulases by spray drying in presence of Maltodextrin.

The stabilization of fungal cellulases by spray drying, the thermal stability of Penicillium occitanis cellulases and the effect of some additives were studied. We observed that CMCase activity presents a good stability at 50 degrees C, even after 60 h of incubation. On the other hand, beta-glucosidase activity was more sensitive (loss of 50%) and reacts on total cellulases activities (Filter paper activities). The addition of hydrophilic agents such as ethylene glycol, polyethylene glycol (PEG6000) enhanced enzyme activity. The effect of PEG and Maltodextrin, another water activity decreasing agent, were then tested during the spray drying of Pol6 cellulases. The presence of 1% PEG allowed the best recovery but had a negative effect on enzyme stability while 1% Maltodextrin showed a negative effect on enzyme recovery but a very positive effect on enzyme stabilization.

Clinical and anatomical spectrum in XX sex reversed patients. Relationship to the presence of Y specific DNA-sequences.

OBJECTIVE: Testicular differentiation can occur in the absence of the Y chromosome giving XX sex-reversed males. Although Y chromosomal sequences can be detected in the majority of male subjects with a 46,XX karyotype, several studies have shown that approximately 10% of patients lack Y material including the SRY gene. The aim of this study was to see if the classification of XX sex-reversed individuals into three groups, Y-DNA-positive phenotypically normal XX males, Y-DNA-negative XX males with genital ambiguities and Y-DNA-negative true hermaphrodites can be applied to our cases. DESIGN: Endocrinological and genetic studies were conducted in 20 XX sex-reversed patients. PATIENTS: Twenty patients with various phenotypes were studied. They were between 20 days and 35 years old. Ten presented ambiguous external genitalia (Prader's stages II to IV). After laparotomy or gonadal biopsy, the diagnosis was 46,XX true hermaphroditism in five, and XX male in 15. MEASUREMENTS: Blood samples were obtained from all patients for hormonal and molecular studies. Basal levels of testosterone, oestradiol and pituitary gonadotrophins were measured by RIA. In addition, two stimulation tests were performed: gonadotrophin stimulation with GnRH and testicular stimulation with hCG. Several Y-specific DNA sequences of the short arm of the Y chromosome were analysed by Southern blot and polymerase chain reaction methods. RESULTS: In this study, three categories of XX sex-reversed individuals were observed: phenotypically normal males with or without gynaecomastia, males with genital ambiguities, and true hermaphrodites. Endocrinological data were similar in XX males and in true hermaphrodites. Testosterone levels exhibited normal (n = 9) or decreased (n = 11) values. The hCG response was low. FSH and LH were elevated in 13 patients. Molecular analysis in ten patients showed varying amounts of Y material including the Y boundary and SRY. Ten patients with various phenotypes lacked Y chromosomal DNA. There was no relation between Leydig cell function (as indicated by testosterone levels before or after hCG stimulation) and the presence of Y chromosome material. CONCLUSION: Although the presence of Y-specific DNA generally results in a more masculinized phenotype, exceptions do occur. In the Y-DNA-negative group, complete or incomplete masculinization in the absence of SRY suggests a mutation of one or more downstream non-Y, testis-determining genes.