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No known drug allergies&#46; No toxic habits or occupational exposure&#46; Student&#59; no significant family history&#46; He was referred by the medical oncology department to the respiratory medicine outpatient clinic due to the presence of mediastinal lymph nodes showing suspected tumor relapse on PET-CT&#46; Clinical examination showed some degree of asthenia&#44; but no other symptoms&#46; Physical examination was normal&#46; Clinical laboratory tests revealed completely normal serum biochemistry&#44; complete blood count&#44; and coagulation parameters&#46; A computed tomography of the chest&#44; abdomen and pelvis requested by the medical oncologist revealed a conglomerate lymph node mass in the subcarinal space&#44; with bilateral hilar lymphadenopathies&#44; and no other findings&#46; Given these results&#44; a PET-CT was requested&#44; revealing the presence of hypermetabolic foci on the right lung and right subcarinal and pulmonary hilar lymphadenopathies&#44; with higher metabolic intensity than the previous routine PET-CT &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Endobronchial ultrasound under anesthetist-controlled deep sedation was performed&#44; revealing a rounded subcarinal lymph node mass measuring 12<span class="elsevierStyleHsp" style=""></span>mm with well-limited borders&#44; homogeneous echostructure&#44; and absence of central hilar structure or signs of necrosis&#46; The lesion was aspirated 3 times for <span class="elsevierStyleItalic">in situ</span> cytopathology in the examination room&#44; and enough material was obtained for subsequent studies&#46; The pathological diagnosis was metastatic Ewing&#39;s sarcoma&#46; After diagnosis&#44; the patient received four chemotherapy cycles with the cyclophosphamide-topotecan regimen&#46; Tolerance was good and metabolic response was complete&#44; and autologous peripheral blood transplantation was performed&#46; Complete metabolic response persisted in subsequent check-ups&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Ewing&#39;s sarcoma is the second most common malignant bone disease in children&#46; Approximately 90&#37; of cases occur in the second decade of life&#44; peaking at the age of 13 years&#46; It is extremely rare in children younger than 5 years&#44; and occurs predominantly in boys&#44; at a ratio of 1&#46;5&#8211;2&#58;1&#46; The most common clinical presentation is pain and swelling of the affected region&#44; and a palpable mass may be present&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Occasionally &#40;more often in the case of metastatic disease&#41; systemic manifestations occur&#44; such as fever&#44; asthenia&#44; weight loss&#44; leukocytosis&#44; anemia&#44; and raised erythrocyte sedimentation rate&#44; which can lead to an erroneous diagnosis of acute osteomyelitis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The most common disease sites are the diaphysis of the long bones and the pelvis &#40;70&#37;&#8211;75&#37; of cases&#41;&#44; and it has a predilection for the scapula&#59; it occurs more rarely in the ribs and the vertebrae&#44; but it can affect any bone&#46; About 15&#37;&#8211;20&#37; of patients also have pulmonary metastasis on diagnosis&#44; and Ewing&#39;s sarcoma can metastasize during the disease course to bone and lymph nodes&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Currently&#44; standard chemotherapy for Ewing&#39;s sarcoma involves 4&#8211;6 cycles of vincristine&#44; doxorubicin &#40;Adriamycin&#41; and cyclophosphamide&#44; alternating with ifosfamide and etoposide &#40;VDC&#47;IE&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Prognosis is determined by various parameters that make up the prognostic index&#58; a poor prognosis is predicted by age &#62;18 years&#44; extraosseous site&#44; size &#8805;8<span class="elsevierStyleHsp" style=""></span>cm&#44; distant metastasis &#40;20&#37;&#41;&#44; post-therapy histology I&#44; IIA or IIB&#44; EWS-FLI1 type 2 translocation&#44; and raised LDH levels&#46; Our patient presented some features for poor prognosis&#44; such as mediastinal metastasis confirmed on cytology by EBUS and pleuro-pulmonary metastasis shown on PET-CT&#46; Nevertheless&#44; he was able to benefit from the correct treatment offered by an optimal multidisciplinary therapeutic strategy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> In the vast majority of cases&#44; the diagnosis of Ewing&#39;s sarcoma with pulmonary involvement described in the literature is reached by transthoracic biopsy of accessible pulmonary lesions&#44; so a successful approach to the mediastinal lesions by endobronchial ultrasound is essential&#46; Herein lies the interest in this case&#58; we were able to use a minimally invasive technique with high diagnostic yield to establish a differential diagnosis&#44; ruling out other entities&#46;</p></span>"
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Scientific Letter
Involvement of Mediastinal Lymph Nodes by Ewing's Sarcoma
Afectación adenopática mediastínica por sarcoma de Ewing
Alberto Caballero Vázquez
Corresponding author
, Paula García Flores, Ángela Herrera Chilla
Unidad de Gestión Clínica de Neumología, Complejo Hospitalario Universitario de Granada, Granada, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of 20-year-old man with a history of Ewing&#39;s sarcoma in the left humerus diagnosed at the age of 15&#44; who has been in medical oncological follow-up since then&#46; After the diagnosis&#44; he received 5 cycles of neoadjuvant VAC&#47;ifosfamide-VP-16&#44; followed by radical surgery&#44; resulting in pathological complete response according to pathology reports&#46; The patient completed 12 adjuvant cycles of the same regimen and proceeded to regular monitoring&#46; After 2 years free of disease&#44; he received a diagnosis of probable pleuro-pulmonary relapse on a PET-CT&#44; which showed hypermetabolic foci in the oblique fissure of the right lung and in the right subcarinal and hilar lymph nodes&#44; although the latter were described as probably of a non-specific inflammatory nature&#44; and close monitoring with imaging techniques was recommended&#46; No known drug allergies&#46; No toxic habits or occupational exposure&#46; Student&#59; no significant family history&#46; He was referred by the medical oncology department to the respiratory medicine outpatient clinic due to the presence of mediastinal lymph nodes showing suspected tumor relapse on PET-CT&#46; Clinical examination showed some degree of asthenia&#44; but no other symptoms&#46; Physical examination was normal&#46; Clinical laboratory tests revealed completely normal serum biochemistry&#44; complete blood count&#44; and coagulation parameters&#46; A computed tomography of the chest&#44; abdomen and pelvis requested by the medical oncologist revealed a conglomerate lymph node mass in the subcarinal space&#44; with bilateral hilar lymphadenopathies&#44; and no other findings&#46; Given these results&#44; a PET-CT was requested&#44; revealing the presence of hypermetabolic foci on the right lung and right subcarinal and pulmonary hilar lymphadenopathies&#44; with higher metabolic intensity than the previous routine PET-CT &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Endobronchial ultrasound under anesthetist-controlled deep sedation was performed&#44; revealing a rounded subcarinal lymph node mass measuring 12<span class="elsevierStyleHsp" style=""></span>mm with well-limited borders&#44; homogeneous echostructure&#44; and absence of central hilar structure or signs of necrosis&#46; The lesion was aspirated 3 times for <span class="elsevierStyleItalic">in situ</span> cytopathology in the examination room&#44; and enough material was obtained for subsequent studies&#46; The pathological diagnosis was metastatic Ewing&#39;s sarcoma&#46; After diagnosis&#44; the patient received four chemotherapy cycles with the cyclophosphamide-topotecan regimen&#46; Tolerance was good and metabolic response was complete&#44; and autologous peripheral blood transplantation was performed&#46; Complete metabolic response persisted in subsequent check-ups&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Ewing&#39;s sarcoma is the second most common malignant bone disease in children&#46; Approximately 90&#37; of cases occur in the second decade of life&#44; peaking at the age of 13 years&#46; It is extremely rare in children younger than 5 years&#44; and occurs predominantly in boys&#44; at a ratio of 1&#46;5&#8211;2&#58;1&#46; The most common clinical presentation is pain and swelling of the affected region&#44; and a palpable mass may be present&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Occasionally &#40;more often in the case of metastatic disease&#41; systemic manifestations occur&#44; such as fever&#44; asthenia&#44; weight loss&#44; leukocytosis&#44; anemia&#44; and raised erythrocyte sedimentation rate&#44; which can lead to an erroneous diagnosis of acute osteomyelitis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The most common disease sites are the diaphysis of the long bones and the pelvis &#40;70&#37;&#8211;75&#37; of cases&#41;&#44; and it has a predilection for the scapula&#59; it occurs more rarely in the ribs and the vertebrae&#44; but it can affect any bone&#46; About 15&#37;&#8211;20&#37; of patients also have pulmonary metastasis on diagnosis&#44; and Ewing&#39;s sarcoma can metastasize during the disease course to bone and lymph nodes&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Currently&#44; standard chemotherapy for Ewing&#39;s sarcoma involves 4&#8211;6 cycles of vincristine&#44; doxorubicin &#40;Adriamycin&#41; and cyclophosphamide&#44; alternating with ifosfamide and etoposide &#40;VDC&#47;IE&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Prognosis is determined by various parameters that make up the prognostic index&#58; a poor prognosis is predicted by age &#62;18 years&#44; extraosseous site&#44; size &#8805;8<span class="elsevierStyleHsp" style=""></span>cm&#44; distant metastasis &#40;20&#37;&#41;&#44; post-therapy histology I&#44; IIA or IIB&#44; EWS-FLI1 type 2 translocation&#44; and raised LDH levels&#46; Our patient presented some features for poor prognosis&#44; such as mediastinal metastasis confirmed on cytology by EBUS and pleuro-pulmonary metastasis shown on PET-CT&#46; Nevertheless&#44; he was able to benefit from the correct treatment offered by an optimal multidisciplinary therapeutic strategy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> In the vast majority of cases&#44; the diagnosis of Ewing&#39;s sarcoma with pulmonary involvement described in the literature is reached by transthoracic biopsy of accessible pulmonary lesions&#44; so a successful approach to the mediastinal lesions by endobronchial ultrasound is essential&#46; Herein lies the interest in this case&#58; we were able to use a minimally invasive technique with high diagnostic yield to establish a differential diagnosis&#44; ruling out other entities&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Caballero V&#225;zquez A&#44; Garc&#237;a Flores P&#44; Herrera Chilla &#193;&#46; Afectaci&#243;n adenop&#225;tica mediast&#237;nica por sarcoma de Ewing&#46; Arch Bronconeumol&#46; 2017&#59;53&#58;215&#8211;216&#46;</p>"
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ISSN: 15792129
Original language: English
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