Pediatric high grade gliomas: Clinico-pathological profile, therapeutic approaches and factors affecting overall survival.

INTRODUCTION: Pediatric high grade gliomas are rare tumors of the central nervous system. Treatment is multidisciplinary, comprising surgical excision followed by radiotherapy and/or chemotherapy. OBJECTIVES: describe these tumors' characteristics as seen in our institution, and identify factors associated with better overall survival. PATIENTS AND METHODS: We conducted a retrospective study of 30 cases of pediatric high grade glioma treated consecutively in our institution over a 20-year period. Brainstem tumors and patients aged more than 22years were excluded. Univariate analysis was conducted to determine factors associated with better overall survival. RESULTS: The series comprised 30 pediatric high grade gliomas: 27 glioblastomas and 3 anaplastic astrocytomas. The sex ratio was 1.7. Mean age was 13years. Tumors were mainly located in the cerebral hemispheres (63.3%). Median tumor size was 5cm. Glioblastomas were subdivided into 26 cases of classical subtype (96.3%) and 1 case of epithelioid subtype (3.7%). Surgical strategy consisted in tumor resection in 24 cases (80%). Twenty-one patients (70%) received postoperative radiotherapy. Therapeutic response at end of treatment was complete in 7 cases (23.3%). Postoperative radiation therapy and complete treatment response were significantly associated with improved overall survival in all high grade gliomas and also specifically in glioblastomas (P<0.001 and P=0.005, respectively). CONCLUSION: Our results suggest that postoperative radiotherapy and complete treatment response are predictive factors for better overall survival in pediatric high grade glioma.

Beneficial role of vanillin, a polyphenolic flavoring agent, on maneb-induced oxidative stress, DNA damage, and liver histological changes in Swiss albino mice.

Vanillin, a widely used flavoring agent, has antimutagenic and antioxidant properties. The current study was performed to evaluate its beneficial role against hepatotoxicity induced by maneb, a dithiocarbamate fungicide. Mice were divided into four groups of six each: group 1, serving as negative controls which received by intraperitoneal way only distilled water, a solvent of maneb; group 2, received daily, by intraperitoneal way, maneb (30 mg kg-1 body weight (BW)); group 3, received maneb at the same dose of group 2 and 50 mg kg-1 BW of vanillin by intraperitoneal way; and group 4, serving as positive controls, received daily only vanillin. After 10 days of treatment, mice of all groups were killed. Our results showed that vanillin significantly reduced the elevated hepatic levels of malondialdehyde, hydrogen peroxide, and advanced oxidation protein product and attenuated DNA fragmentation induced by maneb. In addition, vanillin modulated the alterations of antioxidant status: enzymatic (superoxide dismutase, catalase, and glutathione peroxidase) and nonenzymatic (reduced glutathione, nonprotein thiol, and vitamin C) antioxidants in the liver of maneb-treated mice. This natural compound was also able to ameliorate plasma biochemical parameters (aspartate aminotransferase, alanine aminotransferase, gamma glutamyl transpeptidase, alkaline phosphatase, total bilirubin, and total protein). The protective effect of vanillin was further evident through the histopathological changes produced by maneb in the liver tissue. Thus, we concluded that vanillin might be beneficial against maneb-induced hepatic damage in mice.

Microcystic transitional cell carcinoma: a rare tumor of the urinary bladder.

Microcystic urothelial carcinoma is a rare variant of invasive transitional cell carcinoma recognized by the WHO classification. It is characterized by its deceptively benign appearance. The clinical course of this uncommon variety of carcinoma is not well known and their histological and immunohistological features are not well defined. We report a case of a 37-year-old man with a microcystic transitional cell carcinoma of the urinary bladder. He was diagnosed 4 years ago with cystitis glandularis lesions and nephrogenic adenoma. Through this observation we will try to define the clinical and pathological features of this uncommon tumor which must be differentiated from a number of proliferative lesions of the urothelium. The poor prognosis and aggressiveness of this tumor seems to be related to a higher stage and grade at diagnosis.

[TAFRO syndrome and monoclonal gammapathy: Uncommon association!]. / Syndrome de TAFRO et gammapathie monoclonale : association inhabituelle !

Castleman's disease is a lymphoproliferative disorder characterized by angiofollicular lymph node hyperplasia. Recently, a new variant of multicentric Castleman's disease has been identified in Japan called TAFRO syndrome. It is characterized by a constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction and organomegaly (TAFRO). It is usually associated with polyclonal hyperimmunoglobulinemia. Here, we report the first and unique case of TAFRO syndrome with monoclonal gammapathy.

Telangiectatic osteosarcoma of the rib: a rare entity and a potential diagnostic pitfall.

Osteosarcoma (OS) is a common primary malignant tumor of bones that produces osteoid matrix. Telangiectatic osteosarcoma (TOS) is a rare variant of OS. It affects the long bones especially the lower end of femur and the upper ends of tibia and humerus, a distribution similar to the conventional osteosarcoma. The rib involvement is very infrequent. We present a case of TOS of the rib that posed a diagnostic difficulty owing to its unusual location and to its resemblance to giant cell tumor and aneurysmal bone cyst. Correspondence.

[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.

[Disseminated juvenile xanthogranuloma expressing protein S100]. / Xanthogranulomes juvéniles disséminés exprimant la protéine S100.

BACKGROUND: Juvenile xanthogranuloma (JXG) is a form of self-healing non-Langerhans histiocytosis. We report a new case of disseminated JXG without visceral involvement but with positive immunostaining of the majority of histiocytes for protein S100. PATIENTS AND METHODS: A 6-month-old male infant was admitted to our department in January 2010 for congenital nodular lesions of varying size, shape and appearance. The remainder of the dermatological and physical examination was unremarkable. The diagnosis of disseminated JXG, strongly suggested clinically, was confirmed by a skin biopsy. However, immunohistochemistry was confusing, with positivity for protein S100. The lesions regressed spontaneously. DISCUSSION: The aim of this case report is to highlight the lack of any forced association between alarming skin lesions of eruptive JXG and systemic involvement. The distinguishing feature of our case is the positive immunostaining for protein S100 in the histological section, which although rare in XJG, does not cast doubt on the diagnosis of non-Langerhans histiocytosis providing the clinical presentation is typical. Therapeutic abstention is the rule.

[Primary choriocarcinoma of the maxillary gingival]. / Choriocarcinome gingivo-mandibulaire primitif.

INTRODUCTION: Primary non-gestational extragonadal choriocarcinomas are uncommon and their head and neck localization more exceptional. OBSERVATION: We report on a primary choriocarcinoma case of the mandibular gingivae in a 26-year-old woman who presented with pulmonary and renal metastasis. Complete response (clinical, biological and radiological) was achieved with combined chemotherapy according to APE regimen associating actinomycin, cisplatin and etoposid. The patient was free of disease 4 years after therapy completion. DISCUSSION: Primary gingival mandibular choriocarcinoma is very rare. Clinical presentation is atypical; diagnosis is based on histopathological examination and positivity for HCG. Our case report showed high chemo-sensitivity and comparable outcome to the other localizations.

[Desmoplastic fibroma of the skull in an infant]. / Fibrome desmoplastique du crâne chez un enfant.

Desmoplastic fibromas are uncommon osseous tumors that seldom involve the skull. These tumors are histologically benign but locally aggressive with a propensity for recurrence if resection is partial. To our knowledge, only 16 cases have been reported in the literature, seven of which concerned children. We report a further case of a desmoplastic fibroma of the skull in a 3-year-old boy who presented with a right parietal mass. The CT scan showed a lytic mass with brain compression and cortical destruction. The patient underwent a craniectomy and complete mass resection. Histological diagnosis was desmoplastic fibroma. Postoperative progress was normal without recurrence 6 months later.

[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.

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.

[Sacral chordoma: a rare tumor of the buttocks]. / Chordome sacré : une tumeur fessière rare.

INTRODUCTION: Described and recognized for the first time as a pathological entity by Virchow in 1857, chordoma is a tumour of embryonic origin secondary to an attack of the notochord. In most cases it is asymptomatic, resulting in fairly late diagnosis. PATIENTS AND METHODS: We report the case of a 62-year-old patient presenting a bulky tumefaction, nodular in places, not very painful, and extending towards the anal area, scrotum and the posterior aspect of the upper left thigh. Histopathological examination of a macrobiopsy sample of this tumefaction pointed to chordoma. On magnetic resonance imaging (MRI), the tumour presented multiple ramifications extending towards the scrotal area, the sciatic area and the posterior aspect of the left thigh. Palliative tumorectomy was performed. Given the very slow progression of the tumour and the risk of adverse effects in such a large tumoral exposure field, radiotherapy was ruled out. DISCUSSION: This is a typical observation of a rare tumour that dermatologists may encounter.

[Adenoid cystic carcinoma of the breast]. / Carcinome adénoïde kystique du sein.

Adenoid cystic carcinoma of the breast is a rare neoplasm, accounting for only 0.1% of all malignant breast tumours. It is more common in women in the sixth decade of their lives and often in the subareolar area. The clinical criteria is not specific and the radiographic examination showed a benign-appearing tumour. The preoperative diagnosis is possible with fine-needle aspiration cytology. The diagnosis is made by histological examination, presented a difficult differential diagnosis with cribriform carcinoma; so it is necessary to use histochemical or immunohistochemical techniques. The treatment is not well established. It consists of lumpectomy with radiation or mastectomy. Compared to other locations, adenoid cystic carcinoma of the breast has a favorable prognosis. Lymph node involvement or distant metastases seldom occur. The aim of our study is to describe the epidemiological, clinicopathological characteristics, the treatment and the prognosis of this rare type of breast tumour.

[Isolated extramedullary adnexal relapse of acute lymphoblastic leukemia: a case report]. / Rechute extramédullaire annexielle isolée d'une leucémie aiguë lymphoblastique : à propos d'un cas.

The occurrence of an isolated ovarian or pelvic relapse of acute lymphoblastic leukemia (ALL) in complete remission after chemotherapy has rarely been described. We report the case of a 12-year-old girl, treated for ALL, who developed an isolated left ovarian and fallopian tube localization without medullary or blood relapse 4 years after the end of the initial treatment. She presented with an isolated pelvic mass. The diagnosis was established by a CT-guided biopsy. The treatment consisted of a second course of chemotherapy and complementary surgery; a second complete remission was obtained.

[Pure colloid carcinoma of the breast: anatomoclinical study of seven cases]. / Carcinome colloïde pur du sein : étude anatomoclinique de sept cas.

PURPOSE: Breast mucinous carcinoma is a particular histological form characterized by the extracellular production of mucus. The pure form is rarely reported and its prognostic is better than the other types of the breast cancer. The aim of the present study was to discuss the various anatomoclinical, therapeutic aspects and the prognostic factors of the pure mucinous carcinoma of the breast. PATIENTS AND METHODS: We report seven cases diagnosed over a period of 11 years (1993-2003) in the laboratory of anatomy and pathological cytology of the university hospital of Sfax. A review of the clinical files with immunohistochemistry study (hormonal receptor, synaptophysine, chromogranine, Bcl2, Ki67, P53 and Her-2/neu (C-erbB-2)) were carried out for all the cases. RESULTS: pure colloid carcinoma of the breast accounted for 0,5% of the whole of the breast cancers. The average age of patients was 69 years. At the time of the diagnosis, four tumours were classified T4, one T3, two T2, two N1 and one N3; no patient had presented metastasis (M0) according to pTNM classification. The echomammography showed regular contours mass in four cases. The anatomopathological study showed that the neuroendocrine differentiation was found in two cases, all the tumours had presented positivity for the hormonal receptors (oestrogen and progesterone) and negativity for the other antibodies, two cases exhibited the Bcl2 positivity and negativity for the other antibodies. An adjuvant radiotherapy was carried out for all the patients. The average duration of follow-up was of 33 months; the evolution was marked by the occurrence of metastases in two cases. CONCLUSION: The pure colloid carcinoma of the breast constitutes a particular morphological entity which deserves to be individualized; neuroendocrine differentiation of these tumours must be required. Their forecast is relatively favourable.

[Esophageal leiomyomatosis revealing an Alport syndrome]. / Léiomyomatose oesophagienne révélatrice d'un syndrome d'Alport.

Alport syndrome is a rare progressive hematuric nephropathy associated with sensorineural deafness. Leiomyomatosis associated with Alport syndrome is quite rare. We report a particular case of Alport syndrome which was diagnosed in the setting of an oesophageal leiomyomatosis. Alport syndrome and leiomyomatosis are caused by mutation of the genes encoding for the alpha chain of type IV collagen. In view of the important clinical and genetic implications, renal function and urinary status should be controlled in any patient with oesophageal leiomyomatosis.

[Mucinous cystadenoma of the kidney]. / Le cystadénome mucineux du rein.

Mucinous tumors of the kidney are rare. The pathogenesis of these lesions is controversial. We report the observation of a 31-year-old woman presented with right-flank pain and hematuria. Abdominal ultrasonography and computed tomography revealed a multicystic mass of the right kidney. A total nephrectomy was performed. The pathological exam concluded on a mucinous cystadenoma of the right kidney. Mucinous cystadenoma of the kidney with a multicystic presentation has not been previously reported. In this work, we present the different pathogenic hypothesis and the means of treatment of this rare entity.