Le dermatofibrosarcome de Darier et Ferrand (DFS) est une tumeur cutanée rare, caractérisé par son agressivité locale et son important potentiel aux récidives, dont le pronostic dépend essentiellement de la qualité de sa prise en charge. Une réponse complète avec des marges saines d'un DFS qui a été jugé initialement inopérable, après 1 an d'imatinib en situation néoadjuvante est rarement décrite dans littérature. Nous rapportons dans notre article, le cas d'une jeune patiente prise en charge au service d'oncologie médicale de Fès pour une DFS localement avancé présentant une réponse complète, afin d'évaluer la place de l'imatinib et des thérapies ciblées dans le traitement de DFS.

Nuclear protein in testis (NUT) midline carcinoma (NMC) is a very rare and aggressive human cancer characterized by overexpression of the nuclear protein in testis (NUT) most commonly due to a chromosomal translocation that fuses the NUT gene on chromosome 15 with the BRD4 gene on chromo-some19. It has been described mainly in younger individuals in the mediastinum and head and neck regions and known to be highly aggressive with poor outcomes. We report the case of 23 years old male, diagnosed with locally advanced mediastinal malignancy metastatic to the lung with elevated serum alpha-fetoprotein (AFP) suggestive of germ cell tumor. However, pathology with immunohistochemistry excluded the dignosis of germ cell tumor and confirmed the diagnosis of poorly differentiated carcinoma. Despite aggressive treatment, evolution was marked by rapid clinical deterioration leading to death within 1 month of initial diagnosis. We report this case to underline the rarity of this disease, clinico-radiological and pathologic features, especially misleading presentation with germ cell tumors, treatment management and prognosis.

Background: The anorectal location of melanomas is extremely rare (1%-3% of all melanomas), and the prognosis remains poor because of the aggressiveness and the high metastatic potential of those tumors. The discovery that the KIT oncogene may be aberrantly activated in a subset of patients with anorectal melanoma creates a realm of possibility for the development of targeted molecular therapy. Aim: to show the epidemiologic, clinico-radiological, histological features and treatment management especially in patients with over-expression of C-KIT treated by Imatinib. Methods: It is a retrospective study conducted in the department of medical oncology at Hassan II University Hospital between January 2007 and January 2012, including all patients with histologically proven melanoma of the anorectal area. Results: 11 cases were collected, with slight female predominance. Nine patients were metastatic at the moment of diagnosis, and only two with local stage, but evolution was marked by local and distant recurrence less than 12 months after abdominoperineal resection. First line of chemotherapy was based mainly on paclitaxel, carboplatine and dacarbazine. Response was modest with only 3 partial responses, 4 patients with disease stability, and 4 patients with disease progression. Two patients, with over expression of C-KIT, received Imatinib as second line of treatment with significant improvement of symptoms and radiological response reaching 50%. Seven patients died with a median survival of 11 months from diagnosis to the date of death. Conclusion: Primary anorectal melanomas are very rare, with high aggressiveness and poor prognosis. Treatment management is still a big challenge given to the modest efficacy of conventional chemotherapy. Better understanding of carcinogenesis and signaling pathways will allow development of new targeted therapies.

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DiscussionRadiation-induced sarcomas are rare but a well-known late AbstractRadiation-induced osteosarcomas (RIOS) after nasopharyngeal carcinomas are rare complication of radiotherapy and are associated with poor prognosis. Few cases are reported in the literature. Case report:We report a case of radiation-induced osteosarcoma involving the proximal humerus in a patient treated with radiotherapy for nasopharyngeal carcinoma (NPC) nine years ago. Surgical treatment could not be performed, and the patient received induction chemotherapy. He died from disease spread after three cycles.Conclusion: RIOS after treatment of NPC is very aggressive complication. Only a regular follow up of treated patient allow early detection of these tumors and best chances of survival.