[Neutrophilic dermatosis associated with anti-neutrophilic cytoplasmic antibodies (ANCA) after benzylthiouracil therapy]. / Dermatose neutrophilique associée à des anticorps anticytoplasme des polynucléaires neutrophiles (ANCA) après prise de benzylthiouracile.

BACKGROUND: We report the case of a female patient who developed polymorphic expressions of neutrophilic dermatosis associated with p-ANCA while receiving benzylthiouracil for hyperthyroidism. CASE REPORT: A 41-year-old-woman was treated with benzylthiouracil for Basedow's disease. After 21 months of therapy, she developed fever with different expressions of neutrophilic dermatosis: pyoderma gangrenosum of feet, Sweet's syndrome of the forearms and the face. Biopsies confirmed the diagnosis of neutrophilic dermatosis. The histological examination of a skin specimen taken from the developing border of a foot lesion showed polynuclear neutrophilic infiltration with leucocytoclastic vasculitis and the presence of anti-myeloperoxydase p-ANCA. Abdominal ultrasound showed multiple splenic microabscesses. The myelogram, gastroscopy and colonoscopy findings were normal. Benzylthiouracil was stopped and systemic corticosteroid therapy resulted in regression of the skin lesions and splenic microabscesses. DISCUSSION: Different types of neutrophilic dermatosis were described in our case, confirming the notion of neutrophilic dermatosis continuum. The occurrence of neutrophilic dermatosis and p-ANCA after benzylthiouracil therapy suggests the involvement of polynuclear neutrophils in a common pathogenic mechanism. However, to date there have been no other reports analogous to ours, and inclusion of neutrophilic dermatosis as a benzylthiouracil-induced adverse effect would require confirmation by other instances of such associations.

[Kikuchi-Fujimoto's disease and connective tissue disease: a report of three cases]. / Maladie de Kikuchi-Fujimoto et connectivite: à propos de trois observations.

INTRODUCTION: Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis, clinicopathological entity of unknown aetiology, is a rare and benign cause of cervical lymphadenopathies. It can be associated with various auto-immune diseases especially systemic lupus erythematous (SLE) or with some infectious agents. EXEGESIS: This report describes a survey of three patients who developed Kikuchi's lymphadenitis occurring concomitantly with connective tissue disease: LES in two cases and non determined connective tissue disease in the other case. Comparing the clinical, histopathological and evolutionary findings to the literature allows to identify the main features of this self-limiting disorder: occurrence in young women; clinical presentation with cervical lymphadenopathy in a context of fever and asthenia. The definite diagnosis is usually made through histopathological examination of a lymph node biopsy. Disease course is generally favourable with spontaneous resolution within few weeks. It may be improved with corticosteroid treatment in patients with systemic involvement. Prognosis is related to the associated disease. CONCLUSION: Kikuchi-Fujimoto's disease is a rare and benign cause of cervical lymphadenopathy that could resemble lymphoma, tuberculosis and may be associated with a characterized systemic disease.

[Multifocal Langerhans cell histiocytosis of bone: late revelation in a 76-year-old woman]. / Histiocytose langerhansienne osseuse multifocale: révélation tardive chez une femme de 76 ans.

Langerhans cell histiocytosis or histiocytosis X has a variable course from a self-limited eosinophilic granuloma to an aggressive disseminated disease. It mainly affects children. We report a 76-year-old woman with multifocal bone histiocytosis X, involving the rachis, an iliac bone and the skull. The diagnosis has been established by histological exam. Outcome was favourable after chemotherapy.

Prognostic Value of BCL2 in Women Patients with Invasive Breast Cancer

Background: Breast cancers are heterogeneous, making it essential to recognize several biomarkers for cancer outcome predictions especially in young women where the classical prediction parameters are not suitable. The goal from this study is to evaluate the impact of B cell lymphoma 2 (BCL2), P53 and Ki-67 proteins expression on survival in young women patients with invasive ductal carcinoma. Patients and methods: Samples and clinical data from 238 patients were collected between 2003 and 2017. They were selected according to 2 criteria: age ≤40 years old and most of them are affected by an Invasive Ductal Carcinoma. We evaluated BCL2, P53 and ki-67 expression by immunochemistry test, and then we assessed correlations of these biomarkers expression with patient's clinicopathological characteristics and survival. Results: Triple negative breast cancer group showed a high frequency among our cohort but we emphasize an almost equitable distribution among all molecular groups. Contrary to other studies which reported that luminal A was correlated with better prognosis, our analysis demonstrated that luminal A is correlated with the Scarff, Bloom and Richardson (SBR) grading 2 or SBR grading 3. To better investigate the prognosis, we analyze three biomarkers known by their impact on physiopathology behavior on breast cancer BCL2, ki-67and P53. BCL2 is the more relevant one, it was correlated with molecular subtypes (p=0.0012) and SBR grading (p=0.0016). BCL2 seems to be the good prognostic biomarker related to survival (p=0.004) with a protective role among patients when endocrine therapy is not provided and Lymph Node (LN) involvement is positive (p=0.021, p=0.000 respectively). Conclusions: The classical prognostic parameters based mainly on the molecular classification in breast cancer seem insufficient in the case of young women. BCL2 protein expression analysis provides a better prognostic value. BCL2 should be clinically associated in current practice when young women specimens are diagnosticated.

[Expression of c-kit in North African nasopharyngeal carcinomas: correlation with age and LMP1]. / Expression de c-kit dans les carcinomes nasopharyngés nord africains, corrélations avec l'âge et LMP1.

PURPOSE: To determine the level and prognostic significance of c-kit expression in the two age groups of North African nasopharyngeal carcinomas. PATIENTS AND METHODS: A retrospective study of 99 NPC specimens from Tunisian patients was investigated by immunohistochemistry. Immunohistochemical data were correlated with Epstein-Barr virus LMP1 expression and pathological, clinical and survival parameters. RESULTS: c-kit was detected in 79% of the cases for patients under 30 years of age (juvenile form) but in only 56% of specimens in patients over 30 years (P=0.039) and was significantly over-expressed for patients with lymph node involvement (P=0.015). LMP1 score was 5.78 (+/-1.84) for c-kit negative tumors compared to 8,23 (+/-2.39) for c-kit positive tumors (P=0.002). Multivariate analysis including age, lymph nodes involvement and LMP1 expression as co-variables, showed that only age (P=0.027) and LMP1 expression (P=0.005) were significantly correlated to the c-kit expression. CONCLUSION: c-kit is highly expressed in the juvenile form of North African nasopharyngeal carcinomas. There is a significant association between LMP1 and c-kit expression. The contrasted levels of C-kit expression in the two age groups strengthen the hypothesis that these clinical forms result from distinct oncogenic mechanisms.

[A rare primary tumor of the mediastinum: pleomorphic liposarcoma]. / Une tumeur primitive rare du médiastin: le liposarcome pléomorphe.

Liposarcoma of the mediastinum is a rare tumor with various histologic features. We report a case of mediatinal pleomorphic liposarcoma in a 37-year-old man who complained of chest pain. Computed tomography showed an anterior expansive process within the mediastinum. Histological diagnosis was established by a trans-thoracic computed tomography guided core-needle biopsy. Despite a first cure of chemotherapy with gemcitabin/cisplatin, disease progression led to death 3 months after diagnosis. Mediastinal pleomorphic liposarcoma is an exceptional invading tumor affecting the middle-aged adult. This tumor, usually giant, becomes symptomatic by compression of mediastinal structures. Surgery is the best treatment when possible. The role of radiotherapy and chemotherapy are discussed. Prognosis depends both on the quality of resection and the grade malignancy.

Conventional and array-based comparative genomic hybridization analysis of nasopharyngeal carcinomas from the Mediterranean area.

Nasopharyngeal carcinoma (NPC) occurs with a high incidence in Southeast Asia and to a lesser extent in the Mediterranean area, especially in Tunisia, Algeria, and Morocco. Cellular gene alterations combined with latent Epstein-Barr virus infection are thought to be essential for NPC oncogenesis. To date, chromosome analysis with comparative genomic hybridization (CGH) has been reported exclusively for NPCs from Southeast Asia. Although NPCs from the Mediterranean area have several distinct clinical and epidemiological features, CGH investigations have been lacking. Chromosome analysis was therefore undertaken on a series of NPC xenografts and biopsies derived from patients of Mediterranean origin. Four xenografts were investigated with a combination of conventional CGH, array-based CGH, and comparative expressed sequence hybridization. In addition, 23 fresh NPC biopsies were analyzed with conventional CGH. Data obtained from xenografts and fresh biopsies were consistent, except that amplification of genes at 18p was observed only in xenografts derived from metastatic tissues. Frequent gains associated with gene overexpression were detected at 1q25 approximately qter (64%) and 12p13 (50%). Losses were noticed mainly at 11q14 approximately q23 (50%), 13q12 approximately q31 (50%), 14q24 approximately q31 (43%), and 3p13 approximately p23 (43%). Comparison with previous reports suggests that Mediterranean NPCs have higher frequencies of gains at 1q and losses at 13q than their Asian counterparts.

Contrasted frequencies of p53 accumulation in the two age groups of North African nasopharyngeal carcinomas.

EBV-associated nasopharyngeal carcinomas (NPCs) from Southeast Asia and North Africa have many common clinical and biological characteristics. However, they differ with regard to their age distribution. In Asia, NPC mainly affects patients in the 4th or 5th decade of their life, whereas in North Africa an additional peak of incidence is found between the ages of 10 and 20. The p53 gene is rarely mutated in NPC. However, several groups have reported a consistent accumulation of p53 in Asian NPCs. To determine whether p53 was also accumulated in North African NPCs, we investigated its expression, by immunohistochemistry, in a series of 90 Tunisian biopsies. Bc12 and CD95, two proteins involved in the regulation of cell survival and apoptosis, were investigated in the same study. We found accumulation of p53 in 81% of the cases for patients over 30 years of age, but in only 38% of specimens for younger patients (P = 0.00013). There was a trend toward a higher frequency of Bc12 detection in patients over 30, but it was not statistically significant. CD95 expression was detected in all biopsies, generally at a high level, even at advanced stages of the disease. The changing frequency of p53 accumulation, below and over 30, suggests that NPC cells often achieve malignant transformation through different pathways in both age groups.

[Immunohistochemistry contribution in Alport syndrome diagnosis]. / Apport de l'immunohistochimie dans le diagnostic du syndrome d'Alport.

UNLABELLED: Alport syndrome (AS) is an hereditary disease characterised by the association of progressive hematuria nephritis. The diagnosis is based on clinical genetic and ultrastructural findings. Nowadays, immunohistochemical technique is of great interest. It enables us to analyze the distribution of the different chains of the type IV collagen in renal basement membrane (RBM) and epidermal basement membrane (EBM) which appeared to be abnormal in 70% of cases. METHODS: We report a prospective study of five families affected with AS. Six patients were investigated by immunohistochemical studies of kidney (3 cases) and skin (6 cases) frozen specimens. Monoclonal antibodies recognizing the collagenous domain of alpha1 (MAB1), alpha3 (MAB3) and alpha5 (MAB5) chains of type IV collagen were used. Two methods were performed: direct immunofluorescence and immunohistochemical (ultravision) analysis. RESULTS: The different chains distribution of type IV collagen in the EBM and RBM was normal in four cases (4 men), abnormal in two patients (1 man and woman). Based on the clinical, genetical and immunohistochemical findings we established three transmission modes: autosomal recessive in two families, dominant X linked in two other familiales, and autosomal dominant in one family. CONCLUSION: Immunohistochemical studies is a simple technique of an easy interpretation accomplished on kidney frozen specimen, or even on a simple cutaneous biopsy. It could be very useful for the diagnosis and enables us in addition to determine the mode of transmission of AS.

High androgen receptor immunoexpression in human "Sertoli cell only" testis.

Our purpose was to evaluate cellular androgen receptor (AR) distribution and intensity of immunostaining in the human azoospermic testis. Thirty six biopsy specimens from azoospermic men were immunostained, using a monoclonal antibody of human AR. The localization and the intensity of AR immunostaining was evaluated in Sertoli Cell Only (SCO) testis (G1, n = 21), in spermatogenesis arrest testis (G2, n = 11) and in histologically normal testis (G3, n = 4). We found an AR immunostaining in Sertoli, peritubular myoid and Leydig cells, but not in germ cells. The intensity of the immunostaining varied substantially between biopsy specimens of different patients. Sertoli and Leydig cells AR immunostaining (score and intensity) in SCO group was higher than in the other groups. For Sertoli cells, the score means of AR immunoreactivity were 20 +/- 2.36, 10.18 +/- 1.0 and 1 +/- 1, for G1, G2 and G3 groups, respectively. For Leydig cells, the score means were 10.24 +/- 1.37, 6 +/- 0.71 and 0, for G1, G2 and G3 groups, respectively. We found significant differences between G1 and G2 (p = 0.0008), between G1 and G3 (p = 1.54 10-7) and G2 and G3 (p = 0.00032). These results suggest that in the testis AR is located exclusively in somatic cells and its expression is higher in SCO syndrome than in normal and in arrest spermatogenesis testes.

Nasopharyngeal carcinoma in childhood and adolescence: analysis of a series of 32 patients treated with combined chemotherapy and radiotherapy.

Standard therapy for nasopharyngeal carcinoma (NPC) in children has generally followed the guidelines established for adults. We report here, the treatment outcomes in 32 children and adolescents with NPC and we discuss treatment approaches. Between 1993 and 1997, 32 NPC patients aged

Extranodal Rosai-Dorfman disease in childhood.

We describe two children with Rosai-Dorfman disease who presented with exophthalmos, leukocytosis, an elevated sedimentation rate and hypergammaglobulinemia. Both became blind as a result of this condition. One child had associated bilateral cervical lymphadenopathy. Investigation revealed involvement of the nasal fossae and retro-orbital spaces by tumoural masses histologically consistent with the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML). Immunohistochemical studies suggest that these histiocytes are activated macrophages derived from a phenotype hybrid between "professional" phagocytic cells and immune accessory cells, expressing lysosomal antigens, S-100 protein but rarely, CDla. An underlying immune dysfunction may be central to the pathogenesis of this disease.

[Evaluation of tumoral and lymph node response to neoadjuvant chemotherapy in undifferentiated nasopharyngeal carcinoma]. / Evaluation de la réponse tumorale et ganglionnaire à la chimiothérapie néoadjuvante dans les carcinomes indifférenciés du nasopharynx.

Between January 1994 and June 1995, 19 cases of nasopharyngeal carcinoma, classified N2-N3 (AJC/UICC 1987) were treated by neoadjuvant chemotherapy including cisplatin 100 mg/m2 on day 1 and epirubicin 80 mg/m2 on day 1. Following course started on day 21. A clinical and scannographic evaluation was made after 3 courses of chemotherapy. Fifty-eight percent of the patients were N3. Seventy-four percent were T3-T4. Tolerance to chemotherapy was good in 100% of the cases. A functional improvement was obtained in 14 among 16 patients who were initially symptomatic. For lymph nodes, an objective response (OR) was observed in all patients. The response was complete (CR) in 53%. A regression of primary tumor was obtained in 68% of the patients, but it was complete in 16% only. Sixty-nine percent of the patients has have a tumoral OR have a lymph node CR. Ninety percent of the patients with a complete lymph node response have a tumoral regression more than 50%. A correlation seems likely between the importance of the tumoral and the lymph node responses.

[Radiation-induced glioblastoma and myxoma after treatment for undifferentiated carcinoma of the naspharynx]. / Glioblastome et myxome radio-induits après traitement d'un carcinome indiffencié du nasopharynx.

Radio-induced tumor have been known for a long time to occur after treatment of cancer during childhood. This entity is exceptional following radiotherapy of the cavum. Skull and facial osteosarcoma were described after treatment of UCNT. We report two observations of radio-induced tumors arising, respectively three and seven years after treatment of UCNT. The first one is a temporoparietal glioblastoma and the second is a rhino- and pharyngeal myxoma. The two patients are alive after treatment of the second tumor. The delay of appearance of these tumors, their situation in the field's irradiated and dose received suggests their radio-induced nature. However, the cytogenetic study is necessary to confirm the implication of radiotherapy in the genesis of these cancers.

[Lupic spinal cord diseases and antiphospholipid antibodies]. / Myélopathies lupiques et anticorps anti-phospholipides: 2 cas.

Central nervous system involvement in systemic lupus erythematosus (SLE) is frequent and severe, however, myelopathy is rarely reported (0.8 p. 100 of SLE cases). We describe two SLE-young women. They were 21 and 37 year old, presented with acute paraplegia. CSF study showed pleocytosis and moderately elevated protein. Antinuclear, anti-DNA and anticardiolipin were positive. High dose steroids therapy induced marked improvement. The pathogenesis of myelopathy in SLE remains unclear. Anti-phospholipid antibodies may cause thrombosis and ischemic myelopathy. However, auto-antibodies directed against central nervous system constituents may be involved.

[Hodgkin's disease with esophageal involvement]. / Localisation oesophagienne révélatrice d'une maladie de Hodgkin.

INTRODUCTION: Esophageal involvement in Hodgkin's disease, commonly known as a belated localization of the advanced forms, has been seldom reported (3 to 5% in post-mortem series and 0.7% in clinical series). EXEGESIS: We report the case of a 61-year-old man who had an esophagus localization revealing Hodgkin's disease stage IV EBb of Ann Arbor classification. The originality of this case was represented by: the revelation mode of the esophageal involvement such as dysphagia and upper gastrointestinal bleeding; the localization at the distal third of the esophagus with contiguous involvement of the gastric fundus; the absence of mediastinal nodes showing the primitive character of the esophageal injury. CONCLUSION: This observation incites us to consider Hodgkin's disease in the list of differential diagnoses of tumoral dysphagia, even if there was no ganglionic and/or visceral localization of the disease.

[Histological study of a case of chondrocostal aspergillosis]. / Etude histologique d'un cas d'aspergillose chondrocostale.

Histological study of biopsy specimens successively taken from the 7th, 5th and 6th right chondrocostal regions in a 33 year-old man; the patient was under chemotherapy for a diffuse lymphoma and has been operated for an aspergilloma of the right lung superior lobe. Non-specific chronic osteomyelitis was observed as well as Aspergillus fumigatus perichondritis (acute and chronic) which had lead to cartilage fragmentation. Cartilage fragmentation could be attributed to an enzymatic chondrolysis induced by polymorphonuclears inflammation. Such matrix degradation seems to favour the penetration of aspergillus into the cartilage surface. This case report recalls that fungi (including Aspergillus despite it appears exceptional) must be considered as a possible cause of osteomyelitis, particularly in immunosuppressed patients.

[Carcinosarcoma of the urinary bladder and renal metastasis]. / Carcinosarcome de la vessie et métastase rénale.

A 74 year-old woman developed a polypoid tumor of the bladder which was discovered by hematuria. Upon histological examination, the tumor was shown to be a carcinosarcoma with a weak epithelial composition confirmed by immunolabelling with keratin and was composed essentially of chondrosarcomatous material. Six months later, the patient developed metastases in the kidney, in the paravertebral muscles, and in the right para-ureteral lymph nodes. There are 55 cases of carcinosarcoma of the bladder described in the literature [3, 20, 22]. It is a tumor found more frequently in men than in women, between the ages of 33 to 83. The prognosis is very gloomy .70% death rate within 2 years), but it seems to be improved by radical cystectomy and adjuvant therapy.

[Malignant mesothelioma of the vulva. About one case]. / Mésothéliome malin de la vulve. A propos d'un cas.

Most malignant mesotheliomas are pleural; they rarely occur within the peritoneum and their location in the vulva is exceptional. A case of a malignant mesothelioma of the vulva is reported in a 47-year-old woman. The patient was operated for a tumour of the vulva. The preoperative diagnosis was that of a cyst of the Bartholin's gland or a fibroma. The microscopic and immunohistochemical exam confirmed the diagnosis of a biphasic malignant mesothelioma of the vulva. The computed tomography scan of the chest and the abdomen was normal. A local recurrence of the tumour occurred in spite of the postoperative radiotherapy. Our objective about this particular case is to discuss the clinical and pathologic aspects and the diagnostic problems of this exceptional tumour of the vulva.

[Bone hydatidosis. 12 cases]. / L'hydatidose osseuse. 12 cas.

The hydatic cyst is a parasitic disease caused by the development in man of the larval form of echinococcus granulosus. All parts of the body may be involved, but the liver and lungs are the main locations of the disease. The bone site is rare, accounting for 0.9 to 2% of all hydatic lesions. Between 1982 and 1988, we have compiled 12 cases of hydatic disease of bone. They were located in the skull (4 cases), the iliac bone (4 cases), the omoplate (1 case), the ribs (1 case), the femur (1 case) and the fibula (1 case). These lesions are generally considered as primary, but association with visceral locations was noted in 3 cases in our series.