[National network of paediatric central nervous system tumours reviewing by the Groupe d'Étude de Neuropathologie Oncologique Pediatrique (GENOP)]. / Réseau de relecture des tumeurs pédiatriques du système nerveux central par le Groupe d'étude de neuropathologie oncologique pédiatrique (GENOP).

Diagnosis of paediatric tumours of the central nervous system is often difficult because WHO classification criteria are mainly defined for adults tumours and do not always apply to their paediatric counterparts. These tumours are rare (400 cases/year among more than 50 pathological subtypes per year in France). Pathological diagnosis may be a challenge for a general pathologist with a too low number of paediatric cases in his recruitment. Hence, a reference group of paediatric neuropathologists was formed (GENOP) on the behalf of the comité "Tumeurs Cérébrales" de la Société Française de lutte contre les Cancers de l'Enfant. This network is supported by the Institut National du Cancer (INCa). GENOP aim is to structure a centralised review of paediatric central nervous system tumours in order to harmonise neuropathological diagnosis at the national level and enhance patients care. Cases assessed during the last 3 years led GENOP to better identify tumours subtypes for which there is a diagnostic challenge. A set of immunohistochemical or molecular specialised techniques was developed, leading to an increased diagnostic accuracy. It allowed a better distinction between diffuse and circumscribed glioma, a better recognition of glioneuronal differentiation and a better subtyping of embryonal tumours such as medulloblastomas. Inter-observer agreement varied according to the tumour subtypes.

Clinical Outcomes in Patients With Metastatic Papillary Renal-Cell Carcinoma: A Multi-Institutional Study in Japan.

BACKGROUND: Standard treatments have not been established in metastatic papillary renal-cell carcinoma (PRCC). We aimed to investigate treatment outcomes in patients with mPRCC. PATIENTS AND METHODS: This study included 51 patients who were diagnosed with PRCC at 14 institutions. Pathologic slides were reviewed by pathologists. The associations between clinical factors and overall survival (OS) were analyzed. RESULTS: Final pathologic diagnoses could be determined in 50 patients. Thirty-five tumors were diagnosed as PRCC (type 2 PRCC, 91.4%), and 15 were diagnosed as other histologic types. Targeted therapies (TTs) were provided to 25 mPRCC patients. Patients treated with TT survived significantly longer than those treated before the era of TT (median OS, 22.5 vs. 6.3 months; P = .0035). Median OS of patients who experienced stable disease for ≥ 9 months using single TT was 43.1 months. Patients treated with a tyrosine kinase inhibitor (TKI) as first-line TT survived longer after TT initiation than those treated with an mTOR inhibitor (median, 22.4 vs. 11.7 months; P = .2684). Patients treated with TKIs in both first- and second-line settings had significantly better survival after TT initiation than those treated with a TKI in one therapy line and an mTOR inhibitor in the other (31.4 vs. 12.9 months, P = .0172). Patients treated with a TKI as second-line TT survived significantly longer after second-line TT initiation than did those treated with an mTOR inhibitor (16.2 vs. 7.4 months, P = .0016). CONCLUSION: Prognoses of patients with mPRCC were improved by TT, and TKIs appeared to be the treatment of choice in both the first- and second-line settings.

Cystic ameloblastoma: a clinico-pathologic review.

OBJECTIVE: Cystic ameloblastoma represent 10-15% of all intra osseous ameloblastomas and appear to be less aggressive than the solid ameloblastomas. The aim of this study was to examine the clinico-pathologic characteristics of cystic ameloblastomas seen at a tertiary health care centre. MATERIALS: All cases diagnosed as cystic ameloblastoma in the Oral Pathology Department of University College Hospital, Ibadan over a 10 year period were investigated for age, gender, location of lesion, treatment, and follow-up. The cases were classified as luminal, intraluminal or mural, based on Ackermann classification. The data was entered into the statistical package for the social sciences version 18 (SPSS 18) and results expressed as percentages. RESULTS: Fifteen cystic ameloblastomas, representing 14.3% of a total of 105 ameloblastoma cases were seen. The mean age was 28.9(±14.5) years with 73.4% occurring in the second and third decades. The male:female ratio was 2:3. Fourteen (93.3%) of the lesions were in the mandible while only one (6.7%) was in the maxilla. The mural variant was the most common histological variant with 6(40%) cases while the luminal and intra-luminal had 4(26.7%) and 5(33.3%) respectively. The multilocular radiologic appearance was more common than the unilocular in this study (ratio 8:4). Cystic ameloblastoma with multilocular appearance occurred in a higher age group (mean age 31yrs) when compared with the unilocular type which had a mean age of 16.3years. CONCLUSION: This study shows similar findings with previous studies but shows a higher multilocular radiological appearance as compared to unilocular variant and no case of recurrence.

Pathologic findings of Whipple pancreaticoduodenectomy: a 5-year review on 51 cases at Taleghani general hospital.

AIM: The aim of this study was to comprehensively analyze histopathologic parameters of Whipple pancreaticoduodenectomy specimens at Taleghani general hospital pathology department. BACKGROUND: The Whipple procedure is performed for variety of tumors involving the head of the pancreas, ampulla of Vater, common bile duct, or duodenum. PATIENTS AND METHODS: Records of all cases of Whipple pancreaticoduodenectomy between 2007 and 2011were retrospectively reviewed and pathological details of diagnosis and staging were extracted. RESULTS: A total of 51 patients underwent Whipple procedure during a 5-year period, including 37 males and 14 females. The average age was 57 years (18-82 years). The most frequent presenting symptoms were jaundice and weight loss. Forty-four patients (86.3%) had malignant and 7 (13.7%) had benign lesions. Among malignant lesions, 27 (61.4%) were ampullary carcinomas, 12 (27.3%) were pancreatic carcinomas and 5 (11.4%) were cholangiocarcinomas. The pathological stage of most of the tumors was T3 (50%); followed by T2 (29.5%), and T1 (15.9%); only 4.5% were T4. Mean tumor size was 2.8 cm (0.2-7 cm). Duodenal and common bile duct margins were tumor-free in most cases (95.5 %). The pancreatic margin was free in 81.8% of patients; this margin had not been evaluated in 5 patients. Nearly 38.6% of all tumors showed vascular invasion while 68.2% showed perineural invasion. The average number of dissected lymph nodes was 4 (range 1-15); although in 25% of specimens, no lymph nodes had been found. Twelve specimens (35.3%) had lymph node metastases. CONCLUSION: The present study demonstrates that most of our patients are diagnosed with malignancy, at advanced stage, and further research is needed to develop practical methods for earlier diagnosis. The fact that 25% of specimens had no lymph nodes needs more consideration.