Tumeur d’Askin : à propos d’un cas avec revue de la littérature

Tazi, Illias; Zafad, S.; Madani, Abdellah; Harif, Mhamed; Quessar, A. El; Benchekroun, Saïd

doi:10.1016/j.canrad.2009.05.016

Cited by 11 publications

(5 citation statements)

References 16 publications

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“…Most clinical centers performing intensive chemotherapy are reporting long-term survival rates between 60 and 70%, suggesting that ES is sensitive to anticancer agents. Current protocols of chemotherapy are based on a combination of two to six anticancer drugs, which are continued after locoregional treatment for up to twelve months [11]. These drugs include doxorubicin, actinomycin D, cyclophosphamide, ifosfamide, vincristine, etoposide, busulfan, melphalan and carboplatin.…”

Section: Discussionmentioning

confidence: 99%

Askin’s tumor: a case report and literature review

et al. 2013

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Askin’s tumor is a primitive neuroectodermal tumor developing from the soft tissues of the chest wall. Its diagnosis approach is complex and requires a multidisciplinary team. Given the rarity of this entity, no regimen has been validated in the literature. We report two cases of Askin’s tumor with a major response to polychemotherapy and surgical resection in one case. These cases show that treatment of Askin’s tumor should be multimodal, requiring discussion in multidisciplinary tumor working groups.

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Section: Discussionmentioning

confidence: 99%

Askin’s tumor: a case report and literature review

et al. 2013

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“…Neoadjuvant chemotherapy provides better local control of the disease, a less extensive surgery and treats microscopic distant metastasis [ 9 ]. Current protocols of chemotherapy are based on a combination of two to six anticancer drugs from doxorubicin, actinomycin D, cyclophosphamide, ifosfamide, vincristine, etoposide, busulfan, melphalan and carboplatin [ 2 ]. In our case, Surgery was recused due to the anatomical complexities.…”

Section: Discussionmentioning

confidence: 99%

“…Since, this tumor is known as Askin tumor, and very rare cases are reported worldwide. This tumor is at present classified as primitive neuroectodermal tumor (PNET) of the thoracopulmonary region and strikes by its histological, immunohistochemical and cytogenetic similarities with Ewing′s sarcoma [ 2 ]. To date, no treatment is codified and prognosis remains dark.…”

Section: Introductionmentioning

confidence: 99%

Rapidly fatal Askin’s tumor: a case report and literature review

Laine¹,

Ghorfi²,

Lambatten³

et al. 2014

Pan Afr Med J

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An 18-year-old male presented with a mass in the right anterior chest wall. Chest Computed tomography revealed a heterogenous mass of 19X13 cm in the right hemithorax with areas of necrosis. There was associated pleural effusion and infiltration of the soft parts of the chest wall. Bronchoscopy showed a tumor in middle lobe bronchus. CT guided biopsy of the mass was performed. Histological examination showed small round tumor cells with scanty cytoplasm, the nuclei are large and hyperchromatic. The tumor cells were positive for CD99 and neuron specific enolase, negative for cytokeratin, leukocyte common antigen and myogenin. Based on these histologic and immunohistochemical findings, the diagnosis of askin's tumor was made. The extension assessment was negative and the patient was given chemotherapy. Two months later, our patient died. Askin's tumor is a rare, highly malignant tumor affecting children and young adults. It is classified as primitive neuroectodermal tumor of the thoracopulmonary region. Prognosis remains poor. In our case, several prognostic factors may explain the shirt ‘term survival, despite no distant metastasis were found: important tumor size, impossibility of surgical treatment and pleural effusion.

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“…Furthermore, it is associated with a variety of organ dysfunctions including cardiovascular, endocrine, neural, gastrointestinal, skin, and musculoskeletal. Ectopic secretion of ACTH accounts for ∼10% of Cushing's syndrome etiologies [[5], [6], [7], [8]]. The following case is that of an 18 months-old Iranian boy presenting with Cushing's syndrome due to ACTH-secreting peripheral PNET.…”

Section: Introductionmentioning

confidence: 99%

Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case

Rahbar

Rahbar²,

Bahoush

2019

Annals of Medicine and Surgery

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IntroductionPrimitive neuroectodermal tumors (PNET) form a group of tumors defined by their appearance that are thought to develop from primitive (undifferentiated) nerve cells in the brain. They are rare tumors and their incidence is not well defined.Case presentationAn 18-month-old male presenting with typical Cushingoid appearance (moon face, central obesity, hirsutism and growth arrest) was admitted for evaluation of endocrine problems. Subsequent laboratory studies revealed markedly elevated adrenocorticotropic (ACTH) and cortisol levels, as well as a hypokalemic metabolic alkalosis, these data are consistent with the diagnosis of Cushing's disease. He was treated with metyrapone to control hypercortisolemia. One month and a half later, a mass was detected in the abdomen by ultrasonography. An abdominal Computed tomography confirmed a large heterogeneous retroperitoneal mass with a significant amount of extension into surrounding structures which was removed by laparoscopic abdominal surgery. The patient's symptoms completely resolved and the ACTH and cortisol levels were also greatly reduced. Histologically, the tumor tissue consistent with the diagnosis of the retroperitoneal primitive neuroectodermal tumor which was confirmed immunohistochemically. This case demonstrates the successful diagnosis and treatment of a rare neoplasm.ConclusionThis is the first rare case with ectopic ACTH syndrome caused by the peripheral primitive neuroectodermal tumor.

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Tumeur d’Askin : à propos d’un cas avec revue de la littérature

Cited by 11 publications

References 16 publications

Askin’s tumor: a case report and literature review

Askin’s tumor: a case report and literature review

Rapidly fatal Askin’s tumor: a case report and literature review

Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case

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