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

[Atypical presentations of internal mammary chain involvement in breast cancer]

Breast cancer is the first cancer in women. Lymphatic involvement in breast cancer is common, especially in our patients because of the frequency of locally advanced forms. This contrast with a weak rate of diagnosed internal mammary chain invasion. To clarify the atypical presentations of internal mammary chain involvement in breast cancer. We present observations of patients presenting atypical forms of internal mammary chain involvement. The invasion of internal mammary chain is often underestimated. Indeed, this site of lymphatic spread is not accessible to the clinical exam and its radiological exploration is not systematic. Otherwise, different clinical, pathological and radiological presentations have to attract our attention to a potential internal mammary chain invasion. Our misrecognition of this site of spread and its different presentations can partly explain the lack of diagnosis

[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

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

Are JLA DQB1 alleles correlated with breast cancer histoprognostic parameteres in Tunisia?

Tumor cells express surface structures different from normal cells. These structures may be recognized by the immune system, which ensure anti-tumoral surveillance. Antigenic presentation requires HLA molecules role. Since, these molecules are encoded by a high polymorphic system, immune response can be modulated according to HLA genotype. So, HLA polymorphism could be correlated with tumor escape from anti-tumor immunosurveillance. We have aimed to search for possible associations between HLA DQB 1 alleles and the histoprognostical parameters in breast cancer in the Tunisian population. DQB 1 alleles were determined by PCR-SSO molecular typing in 100 healthy matched and unrelated Tunisian female and 87 Tunisian women with breast cancer. Allelic distribution between the two studied groups showed no significant associations between this locus and the occurrence, the EE grade and the lymph node invasion of breast cancer in the Tunisian population. This result may be explained by the fact that cancer is a multifactoral disease due to several interacting factors that might change from one population to another

[Internal mammary lymph node invasion in breast cancer: myth or reality]

The internal mammary nodes are often underestimated as breast cancer lymphatic pathway spread. It is yet the first site of lymphatic invasion in central or internal tumors and the second site in external tumors. The intra-thoracic situation of internal mammary nodes explains partly, the difficulty of its exploration. To evaluate the risk of internal mammary node invasion, some predictive factors are established [tumor size, internal or central tumor location, axillary node invasion and young age]. Prognostic and therapeutic impact of invasion justifies its systematic research. Without exploring internal mammary nodes status, TNM classification remains, incomplete. CT scanner, magnetic resonnance imaging, positron emission tomography scanner and sentinel node exploration technique are helpful to explore this region and to adapt its irradiation

Primary leiomyosarcoma of broad ligament

Primary broad ligament leiomyosarcoma [LMS] is an extremely rare tumor with few well-documented cases. We are aware of 15 previously reported observations. Aim: Report of a new case. In this report we report a case of a right pelvic sidewall leiomyosarcoma with involvement of the appendix in a 49-year-old woman. A hysterectomy, bilateral salpingo-oophorectomy, omentectomy and appendicectomy were done, without complications. The patient died five months later. The case illustrates the difficulty of correct diagnosis before intervention. On this occasion, review of the literature confirms that this site remains unusual and the management is difficult

Synchronous association of ileal carcinoid and recto-colonic carcinoma. a case report

Multiple primary malignant neoplasm in a single patient have been well documented in the literature. The lesions can be limited to a single organ or involve multiple organ systems and can be synchronous or metachronous. Report of a new case. We report the case of a 63 year old women with a rectocolonic carcinoma; during surgery an ileal nodule was discovered also as liver metastasis. The final pathologic report on the specimen revealed an adenocarcinoma of the colon and a carcinoid tumor of the small bowel with liver metastasis. Tumoral association is relatively common for patients with small intetinal carcinoids. The association carcinoide of hail with primitive tumour not carcinoide is reported primarily with carcinomes of the digestive tract in particulary recto colics carcinoma