Le rhabdomyosarcome de l'oreille chez l'enfant. Etude d'une observation

Nous rapportons un cas de rhabdomyosarcome de l'oreille localement étendu diagnostiqué chez un enfant de 5 ans. Le retard diagnostique observé peut s'expliquer par le caractère aspécifique de la symptomatologie avec une localisation qui simule au début une otite moyenne chronique. A la lumière de cette observation et au regard d'une revue de la littérature, nous soulignons l'intérêt du diagnostic précoce afin d'améliorer le pronostic de cette pathologie

Malignant tumours of childhood in Zaria

Background: The increased prevalence of hitherto uncommon tumours in children in our geographic setting formed the basis for this study. This study aimed to determine the current histopathologic distribution pattern of paediatric malignancies in Zaria. Materials and Methods : An eight year (2000-2007) consecutive analysis of malignant tumours in children ages 0 to 15 years in a referral University laboratory. All tissue biopsies were fixed in 10formalin and processed in wax. Tumours were characterised histologically into tissues of origin and categorised into three age groups; 1 year; 1-5 years and 6-15 years. Result : 189 children with malignant tumours were analysed. They showed a male preponderance (M: F; 1.2: 1.0) and their ages ranged from 5 days to 15 years. Tumours of mesenchymal origin were the commonest (115: 60.8) while epithelial tumours including germ cell tumours accounted for 74 (39.2) cases. The age group 1-5 years had the highest epithelial tumours while age group 6-15 years had the most tumours with 102 (54) cases overall. The five commonest tumours over-all were rhabdomyosarcoma; Burkitt lymphoma; retinoblastoma; non-Hodgkin's lymphoma and nephroblastoma. Germ cell tumours affected the ovary predominantly and two of the endodermal sinus tumour cases were seen in the testis of an eighteen month child and sacrococcygeum of a 5 year old girl; respectively. Of the six immature teratoma cases; four were cutaneous in distribution. The vascular tumours included epithelioid haemangioendothelioma; haemangioblastoma and Dabska tumour and they accounted for (5.8) of all tumours seen. The commonest sites of occurrence of these tumours were the oculo-orbital; jaw; head and neck regions with 82 cases (43.4) while lymph nodes were involved in 31 (16.4) cases. Conclusion : The distribution and occurrence of malignant tumours in children is age related. Lymphomas were the commonest tumours overall while retinoblastoma and Burkitt lymphoma were the commonest tumours affecting children below 5 years and 6-10 years old; respectively; in our centre. The head region and lymph nodes were the sites of predilection for majority of these tumours

Clinical characteristics and outcome of surgical treatment of childhood rhabdomyosarcoma: a 7-year experience

Background: The aim of this study was to describe the outcome and determine the prognostic factors of outcome of childhood rhabdomyosarcoma in a tertiary hospital in a developing country. Patients and Methods: This was a retrospective review of the clinical presentation; investigation; intervention; and treatment outcomes of children with rhabdomyosarcoma in our hospital over a 7-year period. Statistical analysis was performed using Chi-square test. Results: A total of 18 patients were identified with two-thirds being males (n = 12) with median age of 7 years. Most of the children were below 10 years of age. Lower limbs tumour predominated (n = 6) followed by the upper limbs and head and neck (n = 4 each). Other sites included perianal/perineal (n = 3) and the orbit (n = 1). Two patients were Intergroup Rabdomyosarcoma Study (IRS) group I; four group II; five group III; and seven group IV. Lymph node involvement was the commonest site of metastasis. Clinical group and stage was significantly more advanced in patients older than 10 years compared to younger than 10 years (P = 0.010; P = 0.008; respectively). There were 12 patients with alveolar disease while six had embryonal type of rhabdomyosarcoma. Treatment was by combination chemotherapy; and surgical excision which was done primarily in 11; after chemotherapy in four; and after radiotherapy in one. Two had biopsy only. Five patients are alive; two of them without evidence of disease at average follow-up period of 2 years. Conclusion: Mortality from rhadomyosarcoma in our setting is still unacceptably high. Late presentation may be the major contributor to high mortality. A more aggressive multimodality treatment approach may improve the outcome

Les cancers de l'orbite et l'etude retrospective : a propos de 31 cas

Introduction : Les cancers de l'orbite sont rares. Ils sont caracterises par leur diversite histologique et leur agressivite locoregionale rendant le traitement difficile et mutilant. Dans ce travail; nous rapportons notre experience dans la prise en charge diagnostique et therapeutique de ces tumeurs. Materiels et methodes : Notre etude retrospective a concerne 31 cas de cancers de l'orbite colliges sur 13 ans (1993- 2005). Tous les patients ont beneficie d'un examen clinique complet; d'une imagerie du massif facial (TDM et/ou IRM) et d'une biopsie de la tumeur. Le traitement a ete base sur la chirurgie; la radiotherapie et/ou la chimiotherapie. Resultats : La symptomatologie clinique etait dominee par les signes ophtalmologiques et les algies faciales. L'imagerie a montre dans tous les cas un processus expansif tissulaire a point de depart orbitaire; avec lyse osseuse orbitaire chez 16 patients (51;6); une extension au massif facial chez 7 patients (22;6); endocraniennes chez 6 patients (19;4) et des formes bilaterales atteignant les deux orbites dans 3 cas (9;7) L'anatomopathologie montrait une predominance des lymphomes malins non hodgkiniens (32;3) et des carcinomes epidermoides (32;3); suivis des rhabdomyosarcomes embryonnaires (19;4). Douze patients ont ete traites par chirurgie et radiotherapie postoperatoire; dix patients par une association radio-chimiotherapie; et neuf autres par une chimiotherapie neo-adjuvante. La survie globale etait de 67;8 a 3 ans; 48;4 a 5 ans et 22;6a 10 ans

Les tumeurs de la sphere otorhinolaryngologique de l'enfant

Les auteurs; dans une etude retrospective de 26 ans; presentent les tumeurs otorhinolaryngologiques observees chez les enfants de moins de 15 ans. Ils etudient la distribution de ces tumeurs selon l'age et le sexe; la presentation clinique; la repartition histologique et topographique et les procedes therapeutiques disponibles dans leur milieu. Ils estiment que la pathologie tumorale infantile est rare. Ils trouvent que les rhabdomyosarcomes des cavites naso-sunisiennes sont plus retrouves parmi les tumeurs malignes tandis que les papillomes larynges le sont dans les tumeurs benignes. Le traitement des cancers ORL a ete decevant; celui des tumeurs benignes juge satisfaisant