Carmen Montero Martínez

Sarcoma de Ewing pulmonar/tumor neuroectodérmico primitivo (PNET): aportación de un caso y revisión de la bibliografía

Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung. We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.

Pulmonary Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Case Report and a Review of the Literature

Abstract Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung. We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.

Neoplasia in Lung Transplants

* Corresponding author. E-mail address: mba@mundo-r.com (M. Blanco Aparicio) The number of immunocompromised patients has increased in recent decades, as a result of the AIDS epidemic, advances in cancer chemotherapy and organ transplants. The incidence of neoplasia is increased when there are changes in the immune system, basically in cases of AIDS and solid organ transplantation, owing to the intensity of immunodepressive treatment and the reactivation of viruses implicated in oncogenesis, for example the Epstein-Barr virus (EBV), and the human herpes type 8 and papilloma viruses. The incidence of neoplasias in lung transplantation (LT) is not really known because survival rates are low and follow-up times short. We might expect the incidence to be high, if we take into account the fact that immunodepression is more intense than in the case of transplantation of other solid organs and, furthermore, the recipient often has risk factors for the development of solid neoplasias, chiefl y lung cancer. The registry of the International Society for Heart and Lung Transplantation (ISHLT) has reported that the incidence of neoplasias during the fi rst year after LT is 3.6%, 50% of this fi gure corresponding to lymphoproliferative diseases, and that 27.8% of patients who survive for 10 years after a transplant develop tumours, mainly affecting the skin. Apart from the ISHLT data, there are few studies that evaluate the risk factors, prognosis, and incidence of this complication. Many studies have been conducted entirely in 1 centre and the incidence cannot be evaluated because only cases with neoplasias were recorded. The Spanish Heart Transplantation Tumour Registry, which covers a period of 20 years (average posttransplant follow-up period of 5.2 years), found the frequency of neoplasias to be 14.4%. The principal risk factors for developing tumours include immunodepressants. In particular OKT3 induction has been implicated in the increase in post-transplant lymphoproliferative disorders, although there are studies which fail to corroborate this link. These differences may be attributed to the fact that the way OKT3 was used–the dose and duration of treatment–was different. However, the individual role of each immunodepressant is very diffi cult to evaluate because treatment regimes include various drug combinations and controlled trials specifi cally designed to investigate this aspect have not been carried out. It might also be the case that the intensity of immunodepression is more important than the actual agent used. Recently new immunodepressants, such as interleukin-2 receptor antagonists, which, according to the data that is currently available, do not increase the risk of any type of neoplasia and m-TOR (mammalian target of rapamycin) inhibitors, which could have a role in protecting patients against the development of posttransplant neoplasias, have been introduced. Other factors implicated in the increase in neoplasias are viruses which are potentially carcinogenic. The risk of lymphoproliferative disorders after transplantation is especially high in EBV-negative recipients who receive an organ from an EBV-positive donor, reaching an incidence of up to 33% compared to 1.7% in EBV-positive recipients. This would explain the higher frequency in children and patients with cystic fi brosis which has been described in some series. The introduction of anti-viral prophylaxis using aciclovir or ganciclovir to lower the risk of infection with herpes viruses, especially cytomegaloviruses, probably reduces neoplasias linked to viruses, such as Kaposi’s sarcoma, lymphoproliferative disease, or squamous-cell cancers. The lower incidence of lymphomas in series that include prophylaxis supports this hypothesis. Thus, the administration of OKT3 and antithymocytic globulin in patients who have not received aciclovir is associated with an increased risk of all types of neoplasias, whilst, in patients who received prophylaxis, OKT3 induction only increased the risk of tumours that were neither cutaneous nor lymphoproliferative. This is why, when the carcinogenic risk of immunodepressant agents is considered, we A R T I C L E I N F O

Organized Pneumonia Secondary to Increasing Doses of Temozolomide

Surgery, radiotherapy (RT), and chemotherapy have a role in the control of tumor growth, progression, and recurrence in high-grade gliomas. Temozolomide has been incorporated as the main chemotherapy agent for managing these tumors. Here, we present a case of a patient who developed a severe organizing pneumonia after increasing doses of temozolomide for a high-grade glioma.