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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., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Tubulo-papillary apocrine adenoma (TAA) is a very rare sweat gland tumor. TAA in association with syringocystadenoma papilliferum (SCP) is exceptional. A 2-year-old Tunisian child developed a mixed tumor on the scalp: TAA in association with SCP. Histologically, the tumor consisted of dilated duct-like areas with some apocrine gland-like areas. The superficial part of the tumor was connected to the epidermis and showed the characteristics of SCP. The characteristics and differences in histopathologic and immunohistochemical findings in this mixed tumor are described., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Wells' syndrome or "eosinophilic cellulitis" is characterized by clinical features of cellulitis and histological pictures of eosinophils infiltrate of the dermis with some « flame » figures. This is a very rare disease in the pediatric age. We report the case of a 14-month-old boy, presented with two farms painful nodular brownish lesions in the thigh and back of the foot as well as multiple erythematous papular and vesicular lesions on the forehead, cheeks, limbs and trunk. Biological analysis and histological examination confirmed the diagnosis of Wells' syndrome. The outcome was favorable with dermocorticoid., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Uterine leiomyoma with massive lymphoid infiltration is a rare and unusual pathological finding; only 20 cases have been reported in the literature. We report a case of this unusual lesion in a 35-year-old woman who underwent a myomectomy. On gross examination, the tumor was of white color and firm consistency. Histological sections showed interlacing bundles of spindle shaped cells of low density with moderate to severe lymphocytic infiltrate associated to lymphoid follicles and few plasma cells. Immunohistochemically, the diffusely infiltrating lymphoid cells were predominantly of T cell phenotype. The interspersed spindle shaped cells were positive with alpha smooth muscle actin, desmin and h-caldesmon. The cause of this unusual lesion is not clear, but the recognition of its distinct histological features is important to avoid possible confusion with differential diagnosis including malignant lymphoma, inflammatory pseudotumor and pyomyoma., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2010. Published by Elsevier Masson SAS.)

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.

Composite tumors of the adrenal medulla are rare and have been reported in both the presence and the absence of phacomatosis. Composite pheochromocytoma of the adrenal gland in multiple endocrine neoplasia 2B has not been reported so far. We report a case of a 27-year-old woman with marfanoid habitus and numerous mucosal neuromas of the oral cavity and the eyelids. Clinical investigations revealed a left adrenal medullary tumor and bilateral thyroid nodules. Histologic examination confirmed the presence of typical pheochromocytoma with large areas of ganglioneuroma and multifocal medullary carcinoma with cervical lymph nodes metastases. Our report is the first to describe composite pheochromocytoma with multiple endocrine neoplasia 2B; this report underlines the diversity of neoplasms that could be encountered in this disease and the complex mechanisms involved in its pathogenesis.

Primary aortic tumours are very rare. To our knowledge, only 120 cases had been reported in the world literature. Their clinical presentation is not specific. They are usually diagnosed at aortic surgery or by post-mortem examination. Intimal sarcoma is the most frequent histological type. We report a case of intimal sarcoma of the thoracic aorta diagnosed on autopsy of a 48-year-old man victim of sudden death. The main problem raised by intimal sarcoma lies in its poor differentiation or lack of differentiation. Thus, the pathologist must perform a phenotypic analysis as complete as possible of this tumor.

Epithelioid schwannomas mainly affect peripheral nerves and are frequently malignant. They are rarely encountered intracranially. Only five cases involving the eight nerve have been reported in the literature. We report the sixth case of epithelioid schwannoma of the acoustic nerve diagnosed in a 57 year-old woman operated for neurinoma of the right cerebellopontine angle. The patient was readmitted for local recurrence nine months later. Histologically, the tumour was composed of round or polygonal atypic cells associated with spindle cell areas. In our case, the local recurrence, nuclear pleomorphism and especially the high percentage of positive cells with p53 (80%) and Ki67 (30%) antibodies support the aggressive nature of the lesion.