[Hamartoma of the palate: case report and review of literature]. / Hamartome du palais: à propos d'un cas avec revue de la littérature.

INTRODUCTION: A hamartoma is a benign tumor-like malformation, often taking the form of vascular malformation such as hemangiomas and lymphangiomas; hamartoma composed of other tissue types are much rarer in the oral cavity, exceptionally reported in the palate. We report herein a new case of palatal hamartoma. Our aim is to describe the epidemiological, clinico-pathologic and therapeutic features of this lesion. OBSERVATION: A 53-years-old woman had a painless mass located on the right hard palate, measuring 2×1.5 cm of diameter, the mass was elastic with a smooth surface. On imaging, this mass was well-circumscribed, homogenous with moderate enhancement and thinning of the bone. A surgical excision was performed. Histological examination showed an ill-defined mass composed of hyperplasic salivary gland, nerve fibers, blood vessels and adipose tissue. According to these findings, the palatal lesion was interpreted as hamartoma. CONCLUSION: The palatal hamartoma can mimic clinically a malign neoplasm; its diagnosis is histological. Its treatment is essentially surgical. The prognosis is usually favorable without recurrence.

[Squamous cell carcinoma on a pleomorphic adenoma with amyloid stroma of the palate]. / Carcinome épidermoïde du palais sur adénome pléomorphe à stroma amyloïde.

INTRODUCTION: Squamous cell carcinoma ex-pleomorphic adenoma (CXAP) is a malignant and rare mixed tumor. We report a new case. OBSERVATION: A seventy-year-old woman consulted for a mass in the left hemi-face having evolved over the last 20years. The physical examination revealed a hard and large tumor invading all the palate. Computed tomography revealed a heterogeneous 8.5cm long maxillary mass. The diagnosis of CXAP was made on a biopsy. A histological study confirmed the diagnosis after surgical resection of the tumor, specifying its noninvasive character. DISCUSSION: CXAP is generally located in the parotid gland; it is very rarely located in the palate. The degenerated epithelial component generally corresponds to an adenocarcinoma or an undifferentiated carcinoma; squamous-cell carcinoma is more rarely reported. The prognosis is excellent for the micro and noninvasive types. Surgery remains the treatment of choice.

[Pediatric mandibular myofibromatosis]. / Myofibromatose infantile de la mandibule.

INTRODUCTION: Pediatric myofibromatosis is a rare tumor in neonates and children. Two forms are described, solitary and multicentric, the solitary type is more common and is localized mainly on the head and the neck, mandible involvement is rare. The aim of this article was to report the anatomoclinical and therapeutic features of this pediatric tumor in a case as well as its follow-up. CASE REPORT: A 10-year-old girl was brought to consultation for a lower left gingival swelling 5 cm in diameter, forming a unit with the mandibular bone. The volume had gradually increased over the last 12 months. Imagery revealed the presence of an osteolytic tumor benign in aspect, but locally aggressive. Conservative surgery was performed. The diagnosis of pediatric myofibromatosis was confirmed. Evolution was excellent and after three years of follow-up, there was no evidence of relapse. DISCUSSION: Pediatric myofibromatosis often presents as a painless, well-circumscribed, solid nodule. Imagery is very useful to assess lesion extension and for the therapeutic follow-up. The diagnosis is made on anatomopathological findings and immunohistochemical assessment. The treatment of the solitary myofibromatosis is primarily surgical and its prognosis is excellent contrary to the multicentric form.

[Metastatic signet ring cell carcinoma to the breast from stomach]. / Métastase mammaire d'un adénocarcinome à cellules en bague à chaton gastrique.

Metastatic tumors in the breast are quite rare and constitute 0.5 to 6% of all breast malignancies. They often occur in a polymetastatic context. The most frequent primitive tumors are lymphoma, leukaemia and malignant melanoma. The gastric origin is seldom reported. We report here the observation of a 40-years woman operated in urgency for an acute abdominal syndrome. A gastric tumor was discovered intraoperatively with ovarian metastasis and peritoneal carcinosis. The pathological examination revealed a gastric signet ring cell carcinoma with an infiltration of the right ovary. Four months later, the patient presented with a lump of the right breast. The histologic examination corresponded to a mammary metastasis by a signet ring cell carcinoma from stomach. The objective of our work is to discuss through this observation the anatomoclinical and evolutionary characteristics of breast metastasis.

[Granulosa-cell tumor of the ovary: report of 16 cases]. / Tumeur de la granulosa de l'ovaire: à propos de 16 cas.

OBJECTIVE: The aim of this work was to describe the epidemiological, pathological and clinical features of granulosa cell tumors and to study the different prognostic factors in order to determine an appropriate therapeutic attitude. PATIENTS AND METHODS: We proceeded with a retrospective study of 16 cases of granulosa cell tumors of the ovary diagnosed over a period of 10 years (1994-2003). These cases included one case of juvenile type and 15 adult types. RESULTS: Mean patient age was 46 years for the adult type (range 20-70 years) and 35 years for the juvenile type; 19% of the patients were nulliparous, 31% were menopausals. The predominant symptom was abdomino-pelvic pain with frequent hormonal manifestations. Mean tumor size was 10.5 cm with a solido-cystic aspect in 50% of cases. The treatment was surgical in all cases. Among the 16 patients, 14 (87.5%) were diagnosed at stage I and one (6.2%) at stage II. The juvenile tumor was at stage IV at time of diagnosis and only adjuvant chemotherapy was given. No relapse nor recurrence were noted for the adult type after a mean follow up of 2 years 2 months. For the juvenile form, locoregional recurrence with liver metastasis developed after 9 months. CONCLUSION: Granulosa cell tumor of the ovary is an uncommon neoplasm. The adult form progresses slowly and often is diagnosed in an early stage of disease. Surgery is indicated. The juvenile forms are more exceptional and more aggressive. A prolonged post therapeutic follow-up is necessary because of the risk of recurrences, late and exceptional for the adult form but frequent and early for the juvenile form.

[Pelvic actinomycosis: two cases]. / Actinomycose pelvienne: à propos de deux cas.

INTRODUCTION: Actinomycosis is a chronic suppurative granulomatous disease caused by different Actinomyces species, mostly bacillus: the Actinomyces israeli. The pelvis location of this infection is rare. OBSERVATIONS: We report two cases of actinomycosis that were diagnosed after the surgical treatment of a suspected ovarian tumor and a suspected acute peritonitis. Diagnosis in both cases was based on the histopathologic findings. CONCLUSION: The incidence of pelvic actinomycosis is increasing since 1960 related to the frequent use of intra uterine device. The clinical symptomatology is not specific, simulating a neoplastic or an inflammatory process. The treatment combines operative and antibiotic therapy.

[Rhabdoid tumours of the central nervous system: five case studies]. / Les tumeurs rhabdoïdes du système nerveux central: à propos de cinq observations.

PURPOSE: The rhabdoid cerebral tumors were first identified by Briner et al. in 1985. Their frequency was estimated to be 2.1% of those affecting children under 18 months. Such tumors are equally characterized by a critically and speedly mortal development. Their historical genesis is still of a controversial issue. The aim of the present study was to discuss the various anatomicoclinical and therapeutic aspects of these rare tumors. PATIENTS AND METHODS: We report five cases diagnosed over a period of eight years (1997-2004) in the Laboratory of Anatomy and Pathological Cytology of the University Hospital of Sfax. RESULTS: The average age of patients was 20 years old, there were children under 14 years and 4 patients were male. Clinical symptomatology showed the predominance of intra-cranial hypertension syndrome. Radiography through magnetic resonance revealed a heterogeneous tumor process localized respectively on the spine (one case), the insula (one case), the temporofrontal lobes (two cases) and the medulla (one case). Histological examination of the tumors also showed a proliferation of giant cells with a hyaline-based cytoplasmic inclusion. These inclusions were positive for vimentin and keratin. An adjuvant radiotherapy treatment was prescribed for two patients. A recurrence of rhabdoid tumors occurred in two cases. All five patients died within eighteen months. CONCLUSION: The cerebral rhabdoid malignant tumor constitutes one of the most aggressive and life-threatening intracranial tumors. The optimal management of such tumors remains unknown.

[Renal medullary carcinoma: a case report]. / Le carcinome médullaire du rein: une observation.

Renal medullary carcinoma is an aggressive malignant tumour, recently reported in the literature. It is usually reported in the relatively young patients with drepanocytic trai. Histologically, the tumour is constituted by a tumoral proliferation with diffuse or glandular architecture and inflammatory stroma. The carcinomatous cells have plasmocytoid or rhabdoid aspect. We report a case of 40 years old man who presented macroscopic hematuria. Through this observation and the review of the literature we discuss the anatomoclinical and the prognostic aspects of this exceptional tumour.

[Feminizing testicular syndrome with multiple hamartomas and bilateral paratesticular leiomyomas]. / Syndrome du testicule féminisant associé à des hamartomes multiples et à des léiomyomes paratesticulaires bilatéraux.

INTRODUCTION: Complete androgen insensitivity syndrome or testicular feminization syndrome (TF) is the most common form of male pseudohermaphrodism, caused by a failure of androgen receptor binding. Patient with male genotype 46 XY, has a female morphotype with well developed external sexual organs. EXEGESIS: - We report the case of a 29 year-old girl with a TF syndrome discovered during the exploration of a primary amenorrhoea. Bilateral orchidectomy was performed. The testis were immature; they showed bilateral leiomyoma of the tunica albuginea and multiple hamartomas on the right side. CONCLUSION: Benign tumors are developped in 80% of cases of TF and they are generally hamartomatous nodules of testis. Association of paratesticular leiomyoma to synchronous hamartoma has never been described, its histogenesis is discussed.

[Primary signet ring cell carcinoma of urinary bladder. A case report]. / Adénocarcinome à cellules en bague à chaton primitif de la vessie: à propos d'un cas.

Primary signet ring cell carcinoma of urinary bladder is a rare tumor; accounting for approximately 0.24% of all bladder malignancies. In this study, we report a case of a 58-year old man who consulted for complaints of gross hematuria, the bladder biopsy showed signet ring cell carcinoma; the exploration of gastrointestinal tract did not reveal any other tumor localizations. A total cystoprostatectomy was performed followed by radiotherapy. The aim of this study is to determine the anatomoclinical, therapy and evolution characteristics of this rare tumor.

[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.