Ewing sarcoma of the adrenal gland: a case report and review of the literature.

BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy. CASE PRESENTATION: We report the case of a 23-year-old Moroccan woman presenting with flank pain. An abdominal computed tomography scan showed a large and enhancing left suprarenal mass. After radical nephrectomy, histologic examination revealed a small round cell proliferation. The diagnosis of Ewing sarcoma was confirmed by molecular analysis; time to final diagnosis was 5 months due to financial and coordination issues. Computed tomography (on an asymptomatic patient) revealed a locoregional recurrence, our patient received 12 cycles of the vincristine, doxorubicin and cyclophosphamide/ifosfamide and etoposide protocol used in an alternating schedule, with partial radiologic response (62%) and pathologic complete response, then underwent adjuvant radiotherapy of 45 Gy. The young women is still in remission after 36 months of follow-up. CONCLUSIONS: Our patient had an early recurrence due to absence of adjuvant treatment, but did respond well to neoadjuvant chemotherapy with a pathologic complete response. Management of adrenal Ewing sarcoma could be extrapolated from skeletal one with good outcomes even in locoregional recurrence.

Successful management of retinal metastasis from renal cancer with everolimus in a monophthalmic patient: a case report.

BACKGROUND: The retina is an uncommon site for metastases, in particular from solid tumors. Some authors have reported a recent increase in the incidence of metastases in infrequent sites, such as brain or bone, probably due to the expanded treatment options and the resulting improved survival. Choroidal metastasis is the most common type of intraocular malignancy. The most common primary sites associated with choroidal metastasis are breast cancer in women and lung cancer in men. Treatment options are limited, but they must be discussed and adapted to the patient profile. CASES PRESENTATION: We report a case of a 62-year-old Moroccan man with a history of monophthalmitis secondary to a war injury of 30 years' duration. He has been followed for 28 months for metastatic clear-cell renal carcinoma. The first-line treatment was effective for 24 months, before disease progression as retinal metastasis and accentuation of lung metastases. A second-line treatment with everolimus resulted in marked improvement of symptoms, complete recovery of visual function, and partial response in retinal localization. CONCLUSIONS: Choroidal metastasis of renal cancer is a rare situation that must be actively sought in order to arrive at a suitable therapeutic approach.

Posterior cerebral fossa medulloepithelioma: report of a case.

BACKGROUND: Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood. Due to its rarity, the optimal management is still unknown. The prognosis is poor, especially when resection is incomplete. Adjuvant radiochemotherapy is often indicated. CASE PRESENTATION: We report a rare case of infratentorial medulloepithelioma in a 3 year old girl. She presented symptoms of increased intracranial pressure. On examination, she had coordination problems, ptosis and exotropia of the right eye. Magnetic resonance imaging demonstrated a large cerebellar vermix tumor. Immuno-histochemistry revealed a diffuse positivity for Vimentin and focal positivity for the epithelial membrane antigen, but Glial Fibrillary Acidic Protein and Synaptophysin were negative, the MIB-1 antibody was very high. She received postoperative craniospinal irradiation and died 7 months later. CONCLUSION: We describe the features (epidemiological, clinical, histological, immunohistochemical and therapeutic outcomes) of our case and confront it to literature data.

[Neuroendocrine carcinoma of the breast: about a case and review of the literature]. / Carcinome neuroendocrine du sein: à propos d'un cas et revue de la literature.

Primitive neuroendocrine carcinomas of the breast are rare tumors. They are now included in the latest WHO classification of tumors of the breast. We report the case of a 39-year old patient with tumor located in the breast. It was a locally advanced tumor that required mastectomy and ipsilateral axillary node dissection. Adjuvant chemotherapy was indicated. The evolution was marked by local progression. The patient died in a state of febrile pancytopenia after a one-year survival.