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

Laine M, Ghorfi IA, Lambatten D, Kettani F, Abid A - Pan Afr Med J (2014)

Bottom Line: There was associated pleural effusion and infiltration of the soft parts of the chest wall.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pneumology, Mohamed V Military Teaching Hospital, Faculty of Medicine and Pharmacy, Mohamed V University, Rabat, Morocco.

ABSTRACT
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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Histological examination (HES, X 400): small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic
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Figure 0004: Histological examination (HES, X 400): small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic

Mentions: CT guided biopsy of the mass was performed, microscopic examination (Figure 3, Figure 4) showed small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic. Mitotic activity is high. The immunohistochemistry study was strongly positive for CD99 (Figure 5) and NSE, but was negative for cytokeratin (CK), leukocyte common antigen (LCA) (Figure 6) and myogenin. Based on immunohistochemistry findings, the diagnosis of Askin tumor was made. Tumor staging procedure, including bone scintigraphy, abdominopelvic and brain CT, did not detect distant metastasis. The patient was given carboplatin-etoposide chemotherapy and has complete 2 cycles of treatment. His physical status worsened. The patient died 2 months after diagnosis, mainly attributed to tumor progression.


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

Laine M, Ghorfi IA, Lambatten D, Kettani F, Abid A - Pan Afr Med J (2014)

Histological examination (HES, X 400): small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4232025&req=5

Figure 0004: Histological examination (HES, X 400): small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic
Mentions: CT guided biopsy of the mass was performed, microscopic examination (Figure 3, Figure 4) showed small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The nuclei are large and hyperchromatic. Mitotic activity is high. The immunohistochemistry study was strongly positive for CD99 (Figure 5) and NSE, but was negative for cytokeratin (CK), leukocyte common antigen (LCA) (Figure 6) and myogenin. Based on immunohistochemistry findings, the diagnosis of Askin tumor was made. Tumor staging procedure, including bone scintigraphy, abdominopelvic and brain CT, did not detect distant metastasis. The patient was given carboplatin-etoposide chemotherapy and has complete 2 cycles of treatment. His physical status worsened. The patient died 2 months after diagnosis, mainly attributed to tumor progression.

Bottom Line: There was associated pleural effusion and infiltration of the soft parts of the chest wall.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pneumology, Mohamed V Military Teaching Hospital, Faculty of Medicine and Pharmacy, Mohamed V University, Rabat, Morocco.

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus