Scientific Letter
Askin's Tumor. A Rare Diagnosis in an Elderly Patient
El tumor de Askin. Un diagnóstico raro en un paciente de edad avanzada
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A Rare Diagnosis in an Elderly Patient" "tieneTextoCompleto" => true "saludo" => "Dear Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "642" "paginaFinal" => "643" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Liliana Ribeiro, Dulce Apolinário, Joana Cunha, Fernando Guimarães" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Liliana" "apellidos" => "Ribeiro" "email" => array:1 [ 0 => "liliana.sc.ribeiro@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Dulce" "apellidos" => "Apolinário" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Joana" "apellidos" => "Cunha" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Fernando" "apellidos" => "Guimarães" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Pulmonology, Centro Hospitalar de Trás-os-Montes e Alto Douro, Vila Real, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Internal Medicine, Centro Hospitalar de Trás-os-Montes e Alto Douro, Vila Real, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "El tumor de Askin. Un diagnóstico raro en un paciente de edad avanzada" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1432 "Ancho" => 2131 "Tamanyo" => 516566 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Chest CT showing a pre-vascular solid mass with 9<span class="elsevierStyleHsp" style=""></span>cm in the superior mediastinum (white arrow). (B) Chest CT showing a lesion in the anterior portion of the 7th right rib with 7.9<span class="elsevierStyleHsp" style=""></span>cm and bone destruction (white arrow). (C) Photomicrograph illustrating small-sized round cells with scanty cytoplasm. Stain: hematoxylin and eosin (HE); magnification: 20í (D) Immunohistochemistry photomicrograph showing tumor cells positive for CD99; magnification: 20í.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma are small round-cell tumors of soft tissues and bones with immunohistochemical and molecular similarities, reason why they have been categorized in the group of Ewing family tumors. PNETs can arise from primitive nerve cells of the central nervous system or outside the central nervous system, called peripheral PNETs.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Peripheral PNETs within the thoracopulmonary region were originally reported by Askin <span class="elsevierStyleItalic">et al</span>. in 1979,<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> and since then they have been defined as Askin's tumors.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 76-year-old Caucasian man, smoker, admitted to the emergency department with a clinical history of progressively worsening dyspnea, cough and bilateral pleuritic chest pain, asthenia and anorexia, with 6-months evolution. On physical examination, observations were normal, except increased respiratory rate. On inspection and palpation, a hard and painful mass on the right anterolateral hemithorax was detected. Laboratory investigations were normal except for a high lactate dehydrogenase (LDH 944<span class="elsevierStyleHsp" style=""></span>U/L). Chest X-ray revealed bilateral pulmonary opacities and left enlargement of the superior mediastinum. Chest computed tomography (CT) showed three pulmonary masses, one with 6.1<span class="elsevierStyleHsp" style=""></span>cm in the right inferior lobe and two in the left superior lobe (with 5.8 and 2.9<span class="elsevierStyleHsp" style=""></span>cm). The patient also had a pre-vascular solid mass with 9<span class="elsevierStyleHsp" style=""></span>cm in the superior mediastinum (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) and a 7.9<span class="elsevierStyleHsp" style=""></span>cm diameter lytic mass involving the anterior portion of the 7th right rib (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Eco-guided transthoracic core needle biopsies of the rib lesion were performed and the histopathological examination revealed a small-round cell tumor (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C). The immunohistochemistry study was positive to CD99 (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D), synaptophysin and vimentine, favoring the diagnosis of Askin's tumor.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The patient had a hospital-acquired respiratory infection with associated respiratory failure and died, without starting any antineoplastic therapy.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Askin's tumors are rare tumors that develops in the soft tissue of the thoracic wall and mainly occurs in children, adolescents and young Caucasian adults. Reported cases in patients over 60 years are extremely rare.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Our patient presented some clinical signs and symptoms commonly seen in Askin's tumors, as dyspnea, cough, chest pain, chest wall mass and anorexia. Fever and superficial lymph nodes are other clinical findings reported.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2•4</span></a> Typical radiographic findings include a large chest mass that can be associated with pleural effusion. Rib destruction is frequent. Pulmonary parenchymal involvement, hilar and mediastinal adenopathies are other associated findings.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2,5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">If Askin's tumor is suspected, diagnostic confirmation is obtained by histological and immunohistochemical analysis, as was performed in our case. Histological features include small round malignant cells that contain scant cytoplasm. Like the other Ewing's family tumors, Askin's tumor may express neural markers as CD99, neuron-specific enolase, vimentin and Leu7 (CD57). Tumor cells may also be positive for synaptophysin. Diagnostic confirmation can be alternatively made by detection of the typical cytogenetic mutation, a reciprocal translocation between chromosomes 11 and 22 (t[11;22][q24;q12]).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The ideal treatment of Askin's tumor should be multimodal, and included a combination of neoadjuvant chemotherapy, radical surgical resection and adjuvant chemotherapy and radiotherapy,<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3,4</span></a> however, metastases are frequently seen at the time of diagnosis, making impossible the optimal treatment. Local recurrences after treatment are also frequently seen, reason why Askin's tumor is generally associated with poor prognosis and short survival.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Adverse prognostic factors reported include metastatic disease at diagnosis, larger tumor volume, presence of pleural effusion and poor response to neoadjuvant chemotherapy.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4,6</span></a> Some studies showed older age to be associated with poorer outcome but other studies showed no association between age and survival.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> Our patient presented several adverse prognostic factors, as metastatic disease and large tumor volume, and we believe that his advanced age had an important contribution to the rapidly fatal evolution after the diagnosis. Early clinical suspicion is crucial, and Askin's tumor must be kept in mind in the differential diagnosis of small round cell malignant tumors of the thorax, even in elderly.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1432 "Ancho" => 2131 "Tamanyo" => 516566 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Chest CT showing a pre-vascular solid mass with 9<span class="elsevierStyleHsp" style=""></span>cm in the superior mediastinum (white arrow). (B) Chest CT showing a lesion in the anterior portion of the 7th right rib with 7.9<span class="elsevierStyleHsp" style=""></span>cm and bone destruction (white arrow). (C) Photomicrograph illustrating small-sized round cells with scanty cytoplasm. Stain: hematoxylin and eosin (HE); magnification: 20í (D) Immunohistochemistry photomicrograph showing tumor cells positive for CD99; magnification: 20í.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Askin's Tumor in an adult: case report and findings on 18F-FDG PET/CT" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "G.K. Gedik" 1 => "O. Sari" 2 => "T. Altinok" 3 => "L. Tavli" 4 => "B. Kaya" 5 => "P.O. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 1 | 7 |
| 2024 October | 53 | 23 | 76 |
| 2024 September | 47 | 21 | 68 |
| 2024 August | 62 | 37 | 99 |
| 2024 July | 31 | 26 | 57 |
| 2024 June | 57 | 24 | 81 |
| 2024 May | 61 | 32 | 93 |
| 2024 April | 46 | 27 | 73 |
| 2024 March | 45 | 24 | 69 |
| 2024 February | 29 | 18 | 47 |
| 2023 March | 9 | 4 | 13 |
| 2023 February | 57 | 13 | 70 |
| 2023 January | 38 | 28 | 66 |
| 2022 December | 60 | 32 | 92 |
| 2022 November | 73 | 24 | 97 |
| 2022 October | 64 | 31 | 95 |
| 2022 September | 66 | 30 | 96 |
| 2022 August | 58 | 40 | 98 |
| 2022 July | 45 | 42 | 87 |
| 2022 June | 52 | 38 | 90 |
| 2022 May | 45 | 29 | 74 |
| 2022 April | 60 | 30 | 90 |
| 2022 March | 64 | 50 | 114 |
| 2022 February | 55 | 23 | 78 |
| 2022 January | 63 | 40 | 103 |
| 2021 December | 74 | 50 | 124 |
| 2021 November | 74 | 46 | 120 |
| 2021 October | 74 | 54 | 128 |
| 2021 September | 67 | 50 | 117 |
| 2021 August | 85 | 45 | 130 |
| 2021 July | 47 | 28 | 75 |
| 2021 June | 75 | 40 | 115 |
| 2021 May | 97 | 39 | 136 |
| 2021 April | 218 | 120 | 338 |
| 2021 March | 158 | 30 | 188 |
| 2021 February | 52 | 26 | 78 |
| 2021 January | 72 | 18 | 90 |
| 2020 December | 77 | 13 | 90 |
| 2020 November | 49 | 25 | 74 |
| 2020 October | 55 | 11 | 66 |
| 2020 September | 55 | 11 | 66 |
| 2020 August | 40 | 15 | 55 |
| 2020 July | 43 | 26 | 69 |
| 2020 June | 37 | 15 | 52 |
| 2020 May | 59 | 15 | 74 |
| 2020 April | 46 | 20 | 66 |
| 2020 March | 46 | 13 | 59 |
| 2020 February | 63 | 19 | 82 |
| 2020 January | 53 | 18 | 71 |
| 2019 December | 39 | 20 | 59 |
| 2019 November | 46 | 28 | 74 |
| 2019 October | 30 | 9 | 39 |
| 2019 September | 37 | 12 | 49 |
| 2019 August | 37 | 20 | 57 |
| 2019 July | 31 | 26 | 57 |
| 2019 June | 20 | 12 | 32 |
| 2019 May | 47 | 20 | 67 |
| 2019 April | 57 | 50 | 107 |
| 2019 March | 45 | 29 | 74 |
| 2019 February | 44 | 21 | 65 |
| 2019 January | 30 | 19 | 49 |
| 2018 December | 38 | 17 | 55 |
| 2018 November | 69 | 29 | 98 |
| 2018 October | 72 | 23 | 95 |
| 2018 September | 22 | 9 | 31 |
| 2018 May | 4 | 2 | 6 |
| 2018 April | 17 | 6 | 23 |
| 2018 March | 12 | 5 | 17 |
| 2018 February | 14 | 9 | 23 |
| 2018 January | 1 | 0 | 1 |
| 2017 November | 1 | 0 | 1 |
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