Strangled by His Nerves-Cervical Plexiform Neurofibroma With Infantile Spinal Neurofibromatosis: Case Report in a 14 Years Old Child.

Background: Neurofibromatoses are a rare group of autosomal dominant tumor suppressor phacomatoses syndromes. Neurofibromatosis type 1 (NF1 or Von Recklinghausen's disease) is the most commonly found type of neurofibromatosis, and constitutes the most commonly found autosomal dominant disease of the nervous system. Case presentation: We report a case of a 14-year-old boy who reported a 3-year-history of a slowly enlarging right lateral cervical mass. He has a medical history of a progressive limping gait disorder with scoliotic attitude. MRI identified a dumb-bell shaped intradural right cervical process through right paravertebral gutter on C2 to C4, a second intradural dorsal mass with the same characteristics through left paravertebral gutter on D4 and D5 and a large tissue-like mass infiltrating the lumbosacral subcutaneous soft tissues. A Surgical excision of the cervical and lumbar masses was performed with a good outcome after surgical excision. Conclusions: This case illustrates the need for a collaboration of both neurological and head and neck surgeons in terms of managing difficulties related to a cervical neurofibroma. Benign plexiform neurofibromas are rapidly growing tumors, particularly in children and adolescents, which makes all the importance of early detection and appropriate treatment. Repeated interventions are usually needed in order to adapt and stabilize the tumors extension.

Glioma dataset from Rabat: Clinicopathological, immunohistochemical and disease progression features of 32 Moroccan patients with diffuse Glioma.

The Moroccan Glioma Dataset contains the clinical data of 32 patients with glioma. The clinical data including demographic data (age, sex), tumor characteristics (tumor location, Glioma type, Karnofsky performance score, mitotic activity, cell density, necrosis, endotheliocapillary vascular proliferation, MRI contrast pick-up, corpus collosum infiltration and Oedema), treatment strategy (subtotal resection, gross resection, biopsy, radiotherapy, chemotherapy), expression pattern of tumor biomarkers (IDH1, HIF-1alpha, P53, Ki-67), and survival data (Kaplan-Meier curves for disease progression). The dataset can be used to relate tumor characteristics to tumor biomarkers and to predict disease progression for a better treatment management. The data were presented, analyzed, and described in the article "Immunohistochemical expression of HIF-1α, IDH1 and TP53: prognostic profile of Moroccan patients with diffuse glioma" published in Journal of Chemical Neuroanatomy [1].

Immunohistochemical expression of HIF-1α, IDH1 and TP53: Prognostic profile of Moroccan patients with diffuse glioma.

Diffuse gliomas are growing brain tumors that occur in adult life. This study was designed to determine whether the immunohistochemical analysis of IDH1, HIF-1alpha, or TP53 can provide useful biomarkers of clinical severity and progression of diffuse gliomas. Also, it is hypothesized that the expression of IDH1 mutant induces HIF-1alpha. Immunohistochemical staining for HIF-1alpha, IDH1, and TP 53 was performed in biopsy or resection (sub-total or gross-total) tissue from diffuse gliomas in a clinical series of 32 patients. Associations of the HIF-1alpha, IDH1, and TP53 with clinical characteristics were evaluated and the co-expression of two biomarkers (HIF-1alpha and IDH1) was tested. Our data revealed that each biomarker is expressed in a subset of gliomas (IDH1 was positive in 56% cases, HIF-1alpha was positive in 50% cases and TP53 was positive in 44% cases). While no associations were found between clinical characteristics and the expression of HIF-1alpha, and TP53, IDH1 expression was associated with less severe clinical presentation (Karnofsky Performance Status) and disease progression and was more often expressed in females than males. In addition, there was no clear association between IDH1 and HIF-1alpha expression (21.9% of patients co-expressed IDH1 and HIF-1alpha). The current series provides clinical and immunohistochemical findings that can be useful for the clinical management of patients with diffused gliomas.

Primary intrapulmonary malignant peripheral nerve sheath tumor: A rare case.

A rare case of malignant peripheral nerve sheath tumor (MPNST) was found in the lungs of a 68-year-old male patient. He was referred to our hospital because of visual disturbances, confusion, and headaches. Upon admission, a chest-Abdomen-Pelvis CT scan (CT scan of CAP) showed a right posterobasal pulmonary lesion process associated with liver metastases and a lytic lesion of the vertebral body of D3. Brain MRI (Magnetic Resonance Imaging) revealed secondary brain lesions above and below the tentorium. Image guided liver biopsy showed a suspicious site of malignancy. In the histopathological examination, a diagnosis of MPNST was established. A biological agent, Imatinib, was administered and the patient underwent cerebral radiotherapy. CT scan of CAP showed a partial tumor response according to Chun's criteria. Pre-existing literature indicates that MPNSTs with metastases to the brain are very rare and have a poor prognosis - survival after brain metastases would be about 10 months. Our case report agrees with the literature, as the disease stabilized with treatment up to 7 months, after which the patient was unreachable and our team lost track of him. Our case report appears to be one of the first cases of primary MPNST in the lungs with brain metastases.

Metastasis to the pancreas: a rare site for secondary malignancy of breast cancer (a case report).

Breast cancer is the most frequent invasive cancer in women and the second cause of death by cancer in women after lung cancer. It causes metastases especially to bones, liver and lungs. Pancreatic metastases from a primary breast neoplasm are rare and unusual, occurring in less than 3% of the cases. There have been only 28 cases described in the literature. This paper adds one more case to the published literature. We present a case of pancreatic metastasis of the breast in a 64-year-old female and a discussion based on a review of the literature.

[Chondrosarcoma arising in solitary osteochondroma: a case study]. / Chondrosarcome né sur un ostéochondrome solitaire: à propos d'un cas.

Chondrosarcoma is a rare malignant bone tumor. It can arise de novo or secondary to a malignant transformation of a benign underlying cartilage tumor. Secondary chondrosarcoma arising from solitary benign osteochondroma is extremely rare and data show that the reported incidence of osteochondroma of the pelvis is very low. We here report the case of a 20-year old patient with chondrosarcoma secondary to malignant transformation of an osteochondroma of the right wing of ilium, adjacent to the sacroiliac joint.