Le dermatofibrosarcome protubérans de Darier et Ferrand est une tumeur cutanée rare. Le but de cette étude est de décrire les aspects épidémiologiques, cliniques, histologiques, thérapeutiques et évolutifs avec comparaison aux données de la littérature. Il s'agissait d'une étude rétrospective sur 10 ans à propos de 38 cas de dermatofibrosarcome de Darier et Ferrand pris en charge à l'Institut National d'Oncologie. Les données épidémiologiques, diagnostiques, thérapeutiques et évolutives ont été recueillies à partir des dossiers cliniques des patients et rapportées sur une fiche préétablie. L’âge médian des patients était de 41,5 ans. Tous les patients avaient une preuve histologique de dermatofibrosarcome. Seulement 30 patients ont été traités à l'Institut National d'Oncologie. L'exérèse chirurgicale de la tumeur était large, avec des marges de sécurité de 6-10 cm chez 6 patients (20%), 5 cm chez 16 patients (53%), 2-3 cm chez 4 patients (13%). En profondeur, l'exérèse emportait une barrière anatomique saine. 5 malades ont bénéficié d'une radiothérapie externe avec une dose médiane de 59,5 Gy. Le recul médian était de 64,4 mois (28- 138 mois). Le dermatofibrosarcome protubérans de Darier et Ferrand est une tumeur se distinguant par son évolution lente, son agressivité locale, son haut pouvoir de récidive et la rareté des métastases. Le traitement consiste en une exérèse large et profonde. Le pronostic dépend essentiellement de la qualité de l'exérèse initiale chirurgicale. La série étudiée présente des similitudes épidémiologiques, cliniques, et thérapeutiques avec les données de la littérature.
Background Ependymomas are rare adult tumors that originate from the ventricular system or the ependymal surface of the central canal. Extra-axial supratentorial ependymomas are extremely rare, and only five cases have been reported to date in the English literature. Case presentation A 46-year-old previously healthy male presented with a gradual painless loss of vision in the right eye. Cerebral MRI showed a right parietal-occipital lesion resembling an atypical meningioma. Surgical resection was performed, and immunohistochemical staining results concluded that it was a very uncommon location of a grade 3 ependymoma. Favorable outcome was observed one year after completion of postoperative radiotherapy. Conclusion Clinical and imaging aspects are misleading in rare brain tumors, and immunohistochemistry is essential to re-address diagnosis.
Malignant tumors of the kidney represent rare primary intestinal metastases. They occur in 4% of cases. These intestinal metastases are mainly the small intestine; colon is a rare achievement. We report a case of small intestine metastatic of a tubulo-pappilary carcinoma revealed by a multiple intussusception a year after nephrectomy. The cross-sectional imaging (ultrasound and CT) was the gold standard for positive diagnosis of intussusception and the nature of secondary underlying tumor. Intestinal metastases of renal cell carcinoma sometimes occur many years after nephrectomy. On imaging, metastases simulate all tumor aspects.
Background: Merkel cell carcinoma is a rare, very aggressive neuroectodermal tumor of the skin. It is typically located on sun-exposed skin and frequently found in white men aged between 70 and 80 years. Case presentation: We report a case of a 58-year-old black woman diagnosed with Merkel cell carcinoma of the posterior face of the right elbow. She had biopsy excision and was lost to follow-up. Four months later, she presented with recurrent disease on the inferior third of the right arm with three ipsilateral axillary lymph node metastases. Amputation of the right arm and ipsilateral axillary lymph node dissection were performed, followed by adjuvant radiotherapy. Six months later, the patient died as a result of respiratory failure caused by lung metastasis. To the best of our knowledge, no specific studies have been done comparing the course and the characteristics of Merkel cell carcinoma in white and black populations, and no similar case has been reported in the literature. Conclusions: The Merkel cell carcinoma is very rare in black people. As described elsewhere in the literature, our patient had a poor outcome despite radical management. To date, to the best of our knowledge, there has been no comparison of the prognosis of this tumor in white and black populations.
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