Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study

Background: Extracranial Germ cell tumors [GCT] are a rare and a heterogeneous group of pediatric cancers but highly curable

Aim: We aimed to review management, outcome and prognostic factors that influence overall survival [OS] in a pediatric Tunisian oncologic unit

Methods: We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit

Results: Patients had a mean age of 57 months [ranges: 1 day-13 years]. There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months [ranges: 0-96 months], OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage [p=0.04], the completeness of surgery [p<0.001] and the relapse [p = 0, 0001]. A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors [p=0,021] for 5-year OS

Conclusion: Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments

[Childhood soft tissue chondroma. two cases report]

Soft tissue chondroma is a rare benign tumour, which is generally seen in adult. It consists of islands of heterotopic cartilaginous tissue and most localised on the hands and the feet. The hypothesis that microtrauma is involved in the aetiology of this condition has yet to find any factual support. To report two paediatric cases of soft tissue chondroma. The first is a soft tissue chondroma of the posterior aspect of the left axilla in a 3-year-old boy. CT scans showed a spherical fatty density soft tissue mass without evident calcifications, attaching the infraspinous muscle. The second patient is a 9-year-old girl presented with a right auricular finger soft tissue chondroma. Radiographs showed several punctuated calcifications with adjacent bone scalloping. MRI revealed a lobulated soft tissue mass attaching the flexor tendons. The tumours were entirely removed. Histological examination showed cartilaginous tissue in both cases. At follow-up, the patients had good functions without evidence of recurrence. Simple excision should suffice to treat soft tissue chondroma but care should be taken to make the excision complete if recurrence are to be avoided

[Granulocytic sarcoma of the maxillary sinus: a pediatric case report]

Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvment of the head and neck region is rare, generally concerning the orbit. To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. We report a case of maxillary sinus granulocytic sarcoma in a 13-month old boy revealed by facial nerve palsy, ptosis and jugal swelling, without any evidence of haematological disorders. The patient underwent computed tomography and magnetic resonance imaging exam which demonstrated a non specific maxillary sinus mass with destruction of the orbit floor and the alveolar bone. Diagnosis was obtained after bone biopsy allowing histological and immunohistochemical studies. Granulocytic sarcoma is a serious condition because of the associated hematologic disorders. In a sinus or orbit location, imaging features are non specific. Unless hematologic history is present, diagnosis is difficult and an immuno histo chemical study is required

[Management of blunt duodenal and pancreatic injuries in children [about A series of 8 cases]]

The aim of this retrospective study is to report eight cases of blunt duodeno-pancreatic trauma in infants, emphasizing on the role of imaging in acute assessment of the lesions and in further management. We reported eight cases of duodeno-pancreatic injuries between 2006 and 2008, 5 boys and 3 girls with an age ranging from 3 to 12 years [median age:7 years]. Trauma circumstances were: car accident [n=2], domestic injury [n=5] and bicycle's fall injury [n=1]. All patients underwent abdominal ultrasonography and CT scan in the initial evaluation and during the follow-up. Imaging showed the following pancreatic lesions: 3 corporeal fractures, 2 caudal fractures and one between the corporeal and the caudal portions. Four pancreatic haematomas were found. Two isolated duodenal haematomas were found. Two patients improved spontaneously, the six others developed complications: 4 retroperitoneal collection. Management was chirurgical in one case medical in two cases, endoscopic in 2 cases and three percutaneaous drainages were performed. Blunt duodeno-pancreatic injuries in children have to be evaluated by an early imaging modality, in order to perform acute assessment of the lesions. Primary conservative treatment is advocated while clinic, biologic and imaging follow-up is required to detect complications, which management can be endoscopic percutaneous or surgical

Imaging of benign lipomatous tumours of the limbs in children

Lipomatous tumours of the limbs in children are rare, and lipoblastoma is the most common soft tissue tumour. Most of them have typical imaging features, but their clinical presentation and their management may vary, depending on the exact histological subtype.The aim of our study is to illustrate the main clinical, radiological and histological features of the different benign lipomatous tumours in children. review of the literature. it is about a descriptive study of paediatric cases of benign lipomatous tumours of limb in children. The imaging findings are helpful and can provide essential components for the diagnosis

[Pyelonephritis xanthogranulomatous in childhood: case report and litterature review]

Xanthogranulomatous pyelonephritis [XGP] is a specific form of chronic inflammatory kidney disease rarely seen in children. The Symptoms are often vague and non-specific the aim of this paper is to return the particularities of imaging features in xanthogranulomatous pyelonephritis. insisting on differential diagnosis with renal tumors, especially in case of no renal stone or tract obstruction evidence, We report a case of xanthogranulomatous pyelonephritis in a 2-year-old boy involving the lower renal pole which demonstrates the diagnostic difficulties encountered in this disease Xanthogranulomatous pyelonephritis is a rare condition in children and should be included in the differential diagnosis of a child presenting a renal mass

Extra pulmonary tuberculosis in children: a study of 41 cases

Extrapulmonary tuberculosis accounts for up o one third of all cases of tuberculosis and children show a higher predisposition to the development of extra-pulmonary tuberculosis. To review the clinical features of the extrapulmonary tuberculosis in children. Forty one children with extrapulmonary tuberculosis followed in the Children Hospital of Tunis between January 1995 and December 2007 were reviewed. Extrapulmonary tuberculosis constitutes 57.9%of all cases of tuberculosis. Male to female ratio was 0.7 and the mean age was 75 years. The most commonly involved sites were the peripheral lymphadenitis [14 cases] followed by abdominal [11 cases], central nervous system [7 cases], osteoarticular [5 cases] and multifocal [4 cases]. A positive family history of active tuberculosis was detected in 22.5%of the cases. Diagnosis delay was 4.7 months. Sequelae observed during the follow up were: neurosensory in 5 cases, and vertebral deformation in 1 case. Extrapulmonary tuberculosis represents an important fraction of tuberculosis in our study. The most common form is lymph nodes localization followed by abdominal and central system nervous forms. Neurosensory sequelae were frequent in central system nervous tuberculosis

[Fanconi anemia complicated by neutropenic enterocolitis]

Neutropenic enterocolitis [NEC] also known as typhlitis is an acute, life-threatening inflammation of the small and large bowel often seen in patients with leukaemia undergoing cytotoxic chemotherapy; occasionally this syndrome could present in other immunocompromised patients. To report an unusual case of fanconi anemia complicated by neutropenic enterocolitis. We report a case of a 13-year-old boy affected by Fanconi anemia with severe neutropenia and who presented Neutropenic enterocolitis. Abdominal ultrasonography showed thickening of cecum and ascending colon of 1.18cm. The outcome was favourable with medical management. Neutropenic enterocolitis in Fanconi anemia is a rare and a severe illness; however the outcome is improved with early management

[Imaging of neuro-behcet]

Behcet's disease is a vasculitis affecting predominantly the venous system. It's characterized by a classical triad of recurrent uveitis, oral and genital ulceration. Behcet's disease is more frequent in Japan. the Middle East and some Mediterranean countries. Its pathogenesis is still uncertain. Neurological manifestations in Behcet's disease are not rare and are associated with poor prognosis. These manifestations can be inaugural in 5% of cases and they are polymorphous. We report 15 new cases of neuro Behcet's disease explored by magnetic resonance imaging [MRI] and magnetic resonance angiography [MRA]

cavernome cerebral radio-induit. A propos d'un cas

Recently, there have been several case reports of cavernous angiomas appearing after radiation to the brain, typically in children. The time interval between irradiation and the detection of the cavernous angioma varied from three to nine years and the doses from 24 to 60 grays. We report the appearance of a cavernous malformation in a young girl 3 years after radiation therapy for a brain tumor