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Clinical note
Unsuspected bronchial carcinoid tumor detected in a somatostatin receptor scintigraphy in a patient with multiple endocrine neoplasia syndrome type 1 and hypergastrinemia
Hallazgo incidental de un tumor carcinoide bronquial en gammagrafía de receptores de somatostatina de paciente con síndrome de neoplasia endocrina múltiple tipo 1 e hipergastrinemia
V. Marín-Oyagaa,
Corresponding author
vicmar40@hotmail.com

Corresponding author.
, J.L. Tirado-Hospitala, J.I. Cuenca-Cuencaa, R. Guerrero-Vázquezb, D. Luján-Rodríguezc, R. Vázquez-Albertinoa
a Servicio de Medicina Nuclear, UDIM, Hospital Universitario Virgen del Rocío, Sevilla, Spain
b Servicio de Endocrinología y Nutrición, Hospital Universitario Virgen del Rocío, Sevilla, Spain
c Servicio de Anatomía Patológica, Hospital Universitario Virgen del Rocío, Sevilla, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The multiple neoplasia syndrome type 1 &#40;MEN1&#41; is an infrequent disease with an estimated prevalence of 1 in 20&#44;000&#8211;40&#44;000 persons&#46; Transmission is autosomal dominant by mutations of a tumor suppressor gene &#40;MEN1&#41; located in chromosome 11q13&#46; It is characterized by the presence of tumors in the parathyroid glands&#44; anterior pituitary gland&#44; endocrine pancreas and duodenum&#46; In addition it may be present in association with other tumors such as adrenocortical adenomas&#44; carcinoid tumor and thyroid nodules &#40;follicular adenomas&#44; goiter or non medullary carcinomas&#41;&#44; among others&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The carcinoid tumors associated with the MEN1 syndrome are fundamentally found in the gastrointestinal tract&#44; being infrequent in other localizations&#46; The prevalence of a bronchopulmonary localization is of 5&#37;&#44; although more recent studies have reported that this may reach 31&#37; and are preferentially presented in women&#46; Several studies have also described that their incidence is greater in patients with elevated gastrin levels&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a patient with a history of MEN1 syndrome and hypergastrinemia &#40;clinical suspicion of Zollinger&#8211;Ellison syndrome&#41; and the incidental detection of a typical bronchial carcinoid tumor in a somatostatin receptor scintigraphy with histological confirmation&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical case</span><p id="par0020" class="elsevierStylePara elsevierViewall">In 2005 a 40-year-old ex-smoker male with a history of essential hypertension was diagnosed with hyperparathyroidism due to detection of an inferior left parathyroid adenoma detected in a parathyroid scintigraphy with <span class="elsevierStyleSup">99m</span>Tc-MIBI&#46; Hyperprolactinemia and elevated levels of IGF-1 secondary to a prolactin and growth hormone producing hypophysary microadenoma were also observed&#46; In the extension study only an unspecific pancreatic lesion was observed&#46; According to these findings MEN1 was diagnosed and confirmed with a genetic study&#44; and the patient was treated with parathyroidectomy and resection of the hypophysary adenoma&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Four years later &#40;2009&#41; the patient presented intense epigastralgia and diarrhea&#44; receiving medical therapy&#46; An endoscopic study showed gastroduodenal ulcer leading to clinical suspicion of gastrinoma &#40;Zollinger&#8211;Ellison syndrome&#41;&#44; albeit without pathological findings in the morphological and imaging studies undertaken&#46; Blood analysis only showed elevated serum chromogranin A levels &#40;1&#46;140<span class="elsevierStyleHsp" style=""></span>ng&#47;ml&#41;&#46; The patient continued with pyrosis which was difficult to control&#44; requiring follow up&#46; In new morphological studies &#40;CT&#44; MR&#41; only small hepatic lesions of vascular origin were observed in segments vii and v&#46; A cholangio-MR did not demonstrate any further significant findings&#46; The previously mentioned pancreatic lesion was not visualized in posterior tests&#46; Serum gastrin levels were 760<span class="elsevierStyleHsp" style=""></span>pg&#47;ml &#40;normal up to 100<span class="elsevierStyleHsp" style=""></span>pg&#47;ml&#41; and performed without suppression of proton pump inhibitors due to intolerance &#40;in normal conditions diagnostic levels of gastrinoma without the use of inhibitors are &#62;1000<span class="elsevierStyleHsp" style=""></span>pg&#47;ml&#41; with continuation of clinical suspicion of a non localized gastrinoma&#44; leading to the performance of a scintigraphic study with somatostatin receptors and&#44; if necessary&#44; exploratory surgery with intraoperative echography&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">The urinary levels of catecholamines and indolacetic 5-OH were normal&#46; Baseline hormone levels were also normal and another abdominal MR did not show changes on comparison with previous morphological studies &#40;performed&#44; up to then&#44; only in the abdominal area&#41;&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The patient was referred to our department for a somatostatin receptor scintigraphic study &#40;09&#47;2010&#41;&#46; He was administered 185<span class="elsevierStyleHsp" style=""></span>MBq of <span class="elsevierStyleSup">111</span>In-Pentetreotide &#40;Octreoscan<span class="elsevierStyleSup">&#174;</span>&#41;&#46; A scintigraphic scan was performed up to the proximal third of the thighs with sectorial images of the abdomen and thorax in anterior and posterior projections at 6 and 24<span class="elsevierStyleHsp" style=""></span>h post-injection&#46; An abdominal and thorax SPECT&#47;CT &#40;Symbia T6&#44; Siemens<span class="elsevierStyleSup">&#174;</span>&#41; was also carried out using medium-energy collimators&#46; The most interesting finding was the pathological and focal increase of tracer uptake in the bronchial&#8211;peribronchial territory of the right pulmonary hilum compatible with bronchial&#47;bronchopulmonary neuroendocrine tumor &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41; as well as a doubtful lesion in the hepatic segment vii&#46; These findings were observed in the images acquired at both 6 and 24<span class="elsevierStyleHsp" style=""></span>h post-injection&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Taking into account the findings of the scintigraphic study&#44; a spiral CT of the thorax was performed with contrast&#44; which also showed the lesion to be suggestive of a right bronchial carcinoid tumor&#46; The doubtful lesion in hepatic segment vii was not observed in a Doppler echography with a signal potentiator&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Fibrobronchoscopy with bronchial aspirate&#44; brushing and bronchial biopsies were performed for histological and immunohistochemistry studies&#44; confirming the presence of a neuroendocrine&#44; and probably&#44; carcinoid tumor&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Lastly&#44; the patient underwent middle right lobectomy by video-assisted thoracoscopy&#44; confirming the definitive diagnosis of typical carcinoid tumor of 2<span class="elsevierStyleHsp" style=""></span>cm &#40;well differentiated neuroendocrine tumor&#44; grade ii&#41; with peribronchial growth &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">A thoracic and upper abdominal CT was performed with contrast 6 months after surgery with no evidence of local recurrence of adenopathies and without significant findings at the abdominal level&#46; A control somatostatin receptor scintigraphy carried out at 15 months did not present pathological findings at either a thoracic or abdominal level&#44; and no hepatic lesions were observed &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; The chromogranin A levels remained elevated &#40;1080<span class="elsevierStyleHsp" style=""></span>ng&#47;ml&#41; but without changes compared to previous levels&#44; thereby continuing the clinical suspicion of non localized gastrinoma and the patient remaining stable with medical management&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0060" class="elsevierStylePara elsevierViewall">Bronchial carcinoid tumors &#40;BCT&#41; are low grade neuroendocrine neoplasms with slow growth and with the capacity of producing local invasion and distant metastasis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">From a histological point of view there are 2 well defined types of BCT&#58; typical carcinoid and atypical carcinoid&#46; Both types of BCT derive from the Kulchitsky neuroendocrine cells localized in the bronchial mucosa and are differentiated by the grade of cellular pleomorphism&#44; mitotic activity and the presence of necrosis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Compared with other malignant diseases of the bronchioalveolar tree&#44; these well differentiated neuroendocrine tumors are infrequent &#40;1&#37; of all lung cancers&#41;&#44; they are not related to smoking&#44; are more frequent in women&#44; have a good prognosis and are generally presented as an isolated tumor&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Several studies have reported the association between the MEN1 syndrome and the appearance of BCT &#40;5&#8211;8&#37; with histologic confirmation&#44; but possibly up to 31&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">It is known that hypergastrinemia may stimulate the proliferation of neuroendocrine cells and that it is associated with the appearance of disseminated gastroenteropancreatic tumors as well as the progression of malignant gastric carcinoid tumors&#46; Considering the demonstrated relationship between the elevated serum gastrin levels present in patients with the MEN1 syndrome and the appearance of BCT&#44; it has been suggested that this hormone may also stimulate the bronchial neuroendocrine cells&#44; inducing hyperplasia&#44; dysplasis and malignant transformation&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Somatostatin receptor scintigraphy is an excellent test to detect carcinoid tumors&#44; having a sensitivity of 72&#8211;93&#37;&#44; achieving better results in bronchial lesions and worse results in gastric and intestinal tumors&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In the present case&#44; the somatostatin receptor scintigraphy identified an unsuspected BCT as an incidental finding in the search for a gastrinoma of difficult localization&#44; thereby allowing adequate&#44; opportune treatment&#46; Despite its prevalence and natural history&#44; the role of screening in the detection of BCT in patients with MEN1 and hypergastrinemia has not been systematically studied and currently remains unknown&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">It is therefore important for the professionals in permanent contact with this type of pathologies to keep this possible relationship in mind &#40;MEN1 syndrome&#44; hypergastrinemia and BCT&#41;&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
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    "fechaRecibido" => "2012-05-16"
    "fechaAceptado" => "2012-07-10"
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          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec106910"
          "palabras" => array:4 [
            0 => "Bronchial carcinoid tumor"
            1 => "Multiple endocrine neoplasia type 1 syndrome"
            2 => "Hypergastrinemia"
            3 => "Somatostatin receptor scintigraphy"
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          "palabras" => array:4 [
            0 => "Carcinoide bronquial"
            1 => "Neoplasia endocrina m&#250;ltiple tipo 1"
            2 => "Hipergastrinemia"
            3 => "Gammagraf&#237;a de receptores de somatostatina"
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Multiple endocrine neoplasia type 1 syndrome &#40;MEN1&#41; is characterized by the presence of tumors in parathyroid glands&#44; anterior pituitary gland&#44; endocrine pancreas and duodenum&#46; However&#44; other tumors may also occur&#46; One of them is the carcinoid tumor&#44; which in this context&#44; is more common in the gastrointestinal tract&#46; Less common is the presence of carcinoid tumors of bronchial origin&#44; which with histologic confirmation&#44; may occur in 5&#8211;8&#37; of cases and that appears more frequently in patients with hypergastrinemia&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report a patient with MEN1 syndrome&#44; hypergastrinemia and an incidental finding in a somatostatin receptor scintigraphy of an unsuspected bronchial carcinoid tumor that was confirmed histologically&#46;</p>"
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      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de neoplasia endocrina m&#250;ltiple tipo 1 &#40;MEN 1&#41; se caracteriza por la presencia de neoplasias en gl&#225;ndulas paratiroides&#44; hip&#243;fisis anterior&#44; p&#225;ncreas endocrino y duodeno&#46; Sin embargo&#44; otro tipo de tumores tambi&#233;n se pueden presentar&#46; Uno de ellos es el tumor carcinoide&#44; que en este contexto&#44; se localiza m&#225;s frecuentemente en el tracto gastrointestinal&#46; Menos frecuente es la aparici&#243;n de tumores carcinoides de origen bronquial que&#44; con confirmaci&#243;n histol&#243;gica se pueden presentar en el 5-8&#37; de los casos y que se han encontrado con m&#225;s frecuencia en pacientes que cursan con hipergastrinemia&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Presentamos el caso de un paciente con antecedente de s&#237;ndrome MEN 1&#44; hipergastrinemia y el hallazgo incidental en un estudio gammagr&#225;fico de receptores de somatostatina de un tumor carcinoide bronquial confirmado histol&#243;gicamente&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Mar&#237;n-Oyaga V&#44; et al&#46; Hallazgo incidental de un tumor carcinoide bronquial en gammagraf&#237;a de receptores de somatostatina de paciente con s&#237;ndrome de neoplasia endocrina m&#250;ltiple tipo 1 e hipergastrinemia&#46; Rev Esp Med Nucl Imagen Mol&#46; 2013&#59;32&#58;107&#8211;10&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Somatostatin receptor scintigraphy at 24<span class="elsevierStyleHsp" style=""></span>h post-injection in anterior &#40;A&#41; and posterior &#40;B&#41; projections&#46; Focal pathological uptake of the tracer in the right hemithorax compatible with bronchopulmonary neuroendocrine tumor &#40;horizontal arrows&#41; and doubtful lesion in hepatic segment vii &#40;vertical arrow&#41;&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Axial &#40;a&#41; and coronal slices &#40;b&#41; of SPECT&#47;CT &#40;A&#41; and SPECT images &#40;B&#41; in the upper half of the abdomen and lower half of the thorax&#46; Focal increase in tracer uptake in the right pulmonary hilum &#40;arrow&#41; at the bronchial&#8211;peribronchial level with no other pathological findings&#46;</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Typical carcinoid tumor with infiltrating peribronchial growth constituted by monomorphous cells of normal size&#44; scarce cytoplasm&#44; with acinar and trabecular growth&#44; and a low mitotic index &#40;&#60;2 mitosis per 10 fields of great magnification&#41;&#46; Staining with hematoxylin&#8211;eosin at a magnification of 40&#215; &#40;a&#41; and 10&#215; &#40;b&#41;&#46; The immunohistochemistry study showed cytoplasmatic tumoral positivity for neuroendocrine markers&#46; Synaptophysin &#40;c&#41; and chromogranin &#40;d&#41;&#46;</p>"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Control somatostatin receptor scintigraphy images &#40;15 months after the first study&#41; performed at 24<span class="elsevierStyleHsp" style=""></span>h post-injection in anterior &#40;A&#41; and posterior &#40;B&#41; projections with no evidence of lesions&#46;</p>"
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      "titulo" => "References"
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        0 => array:2 [
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Multiple endocrine neoplasia syndromes"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
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                            0 => "G&#46;G&#46; Callender"
                            1 => "T&#46;A&#46; Rich"
                            2 => "N&#46;D&#46; Perrier"
                          ]
                        ]
                      ]
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                  ]
                  "host" => array:1 [
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                    0 => array:2 [
                      "titulo" => "Carcinoide bronquial y s&#237;ndrome de neoplasias endocrinas m&#250;ltiples Tipo 1&#46; Aportaci&#243;n de un caso"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "C&#46; Montero"
                            1 => "P&#46; Sanju&#225;n"
                            2 => "M&#46;M&#46; Fern&#225;ndez"
                            3 => "I&#46; Vidal"
                            4 => "H&#46; Verea"
                            5 => "F&#46; Cordido"
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                          "etal" => false
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                  ]
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            3 => array:3 [
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              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Atlas de gammagraf&#237;a de receptores de somatostatina en tumores neuroendocrinos de p&#225;ncreas y tumores carcinoides"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
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                            0 => "J&#46; Banzo"
                            1 => "E&#46; Prats"
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              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Tumor carcinoide bronquial y gammagraf&#237;a de receptores de somatostatina&#58; detecci&#243;n de met&#225;stasis &#243;seas"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
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                            0 => "J&#46; Banzo"
                            1 => "M&#46;D&#46; Ab&#243;s"
                            2 => "E&#46; Prats"
                            3 => "F&#46; Garc&#237;a"
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Article information
ISSN: 22538089
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos