Scientific letter
Patient with diffuse idiopathic hyperplasia of neuroendocrine cells of the lung (DIPNECH) and bilateral nodules: Therapeutic dilemma
Paciente con hiperplasia difusa idiopática de células neuroendocrinas de pulmón (DIPNECH) y nódulos bilaterales: dilema terapéutico
Eric Gutiérrez Pérez
, Carlos Alberto Rombolá
Corresponding author
Hospital Universitari Arnau de Vilanova, Lleida, Spain
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Some aspects of the aforementioned definition have even been questioned more recently, and histopathological confirmation could be dispensed with, while adding respiratory clinical criteria and considering this condition as a primary entity or simply reactive to other pulmonary conditions.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In addition, its diagnosis is usually accidental (incidental radiological or pathological finding of resected surgical pieces), this being an underdiagnosed pathology with few published cases.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The condition mainly affects adult females.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Clinically, it is characterised by coughing, dyspnoea, wheezing, and obstructive spirometric changes, due to hyperplastic obstruction of the bronchial lumen and the secretion of vasoactive amines. This fact is the one that most clearly indicates its prognosis. On the other hand, the possibility of progression to low- and medium-grade malignancies (classically typical and atypical carcinoid tumours, since progression to other histological lineages has not been demonstrated), may cast a shadow over their evolution.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Radiologically, this condition is characterised by the presence of a mosaic attenuation pattern (with or without bilateral nodules), although these findings lack great specificity.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Given the scarce scientific evidence and the absence of unified criteria for the management of DIPNECH,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> we present a clinical case, with the intention of opening up a debate on the best therapeutic approach.</p><p id="par0025" class="elsevierStylePara elsevierViewall">This case was a 67-year-old female patient, allergic to Ibuprofen, with a history of pulmonary tuberculosis infection treated in childhood, recurrent pneumonia and occasional dry cough, The patient received treatment at our centre for severe thoracic trauma with multiple rib fractures, while detecting the presence of pulmonary nodules in emergency tomography (CT scan). After adequate evolution, the patient was discharged pending an outpatient-directed study to run the following additional tests for the correct characterisation of lung lesions:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">•</span><p id="par0030" class="elsevierStylePara elsevierViewall">High-resolution thoracic CT scan: confirmed the presence of two polylobed solid central nodules, 14 mm in LID and another, 6 mm in LSI, both associated with areas with a ground glass pattern. No significant lymphadenopathy was detected.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">•</span><p id="par0035" class="elsevierStylePara elsevierViewall">PET-CT: described identical CT findings. Nodules with a slight increase in FDG uptake (SUVmax 1.79), with infiltrative characteristics. Gallium PET-CT was suggested in order to achieve greater sensitivity to possible hidden neuroendocrine tumours.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">•</span><p id="par0040" class="elsevierStylePara elsevierViewall">Echobronchoscopy: the only finding was a 4.8 mm oval adenopathy in 4R location, which was punctured, finding no evidence of malignancy.</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">•</span><p id="par0045" class="elsevierStylePara elsevierViewall">Gallium PET-CT: showing overexpression of somatostatin receptors (SUVmax 2.99) in the major nodule.</p></li></ul></p><p id="par0050" class="elsevierStylePara elsevierViewall">As a result of all these findings, neuroendocrine origin of the pulmonary nodules was suspected.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Respiratory function tests were compatible with a non-reversible obstructive pattern (FVC 123%, FEV1 71%, DLCO 86%).</p><p id="par0060" class="elsevierStylePara elsevierViewall">Presented to the multidisciplinary lung tumour committee, surgical resection of the larger lesion was indicated, performing an anatomical segmentectomy of the right segment 6 and robotic lymphadenectomy. The patient had good progression and was discharged on the third postoperative day.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The pathology report describes a typical 15 mm carcinoid tumour with free resection margins with no atypia, necrosis, or vascular invasion. The presence of foci of idiopathic diffuse hyperplasia of neuroendocrine cells in non-tumoral lung parenchyma (DIPNECH) was identified.</p><p id="par0070" class="elsevierStylePara elsevierViewall">The committee ruled out adjuvant treatment for the resected nodule, and follow-up was indicated. The indication to resect or not the remaining nodule in the central location of the LSI was discussed. Located between the lingula and the culmen and measuring 6 mm, the nodule was of probable synchronous neuroendocrine origin but without histological confirmation.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Taking into account the size of the lesion, it should be remembered that the size limit for tumorlets is 5 mm, and therefore the treatment of this lesion must be based on surgical excision in the event of a carcinoid tumour.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However, the presence of DIPNECH demonstrated in the study of the specimen suggested the possibility of successive appearance of new lesions of neuroendocrine origin in revisions; subsidiary to other resections in the future. Possibly, the idea of having to perform a lobectomy in the end, to avoid a probable typical subcentimetre carcinoid (while the condition remained stable) was excessive in this case, in addition to the possibility of exacerbating the effects on respiratory functional changes typical of DIPNECH.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Other therapeutic options such as stereotactic radiotherapy or radiofrequency could be discussed, although its central location and probable neuroendocrine histology ruled out these techniques in practical terms. Systemic treatments were also ruled out due to the patient's scarce symptoms. The patient will continue with clinical and tomographic surveillance. Several series have indicated symptomatic improvement through treatment with somatostatin analogues, especially Octreotide,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> which, in the case of coexistence with a neuroendocrine tumor, could decrease it in size. In the case of indolent progression, some recommend control by CT without contrast every 2 years, although in lesions larger than 1 cm, the possibility of closer control has been considered, given the greater possibility of malignancy.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Gutiérrez Pérez E, Rombolá CA. Paciente con hiperplasia difusa idiopática de células neuroendocrinas de pulmón (DIPNECH) y nódulos bilaterales: dilema terapéutico. Cir Esp. 2024. <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.ciresp.2024.05.013">https://doi.org/10.1016/j.ciresp.2024.05.013</span></p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "A.M. Marchevsky" 1 => "A.E. Walts" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1053/j.semdp.2015.08.002" "Revista" => array:6 [ "tituloSerie" => "Semin Diagn Pathol." "fecha" => "2015" "volumen" => "32" "paginaInicial" => "438" "paginaFinal" => "444" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26472691" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "B.F. Samhouri" 1 => "T.R. Halfdanarson" 2 => "C.W. Koo" 3 => "C. McCarthy" 4 => "E.S. Yi" 5 => "C.F. Thomas" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:3 [ "tituloSerie" => "Endocr Relat Cancer." "fecha" => "2023" "volumen" => "30" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: imaging and clinical features of a frequently delayed diagnosis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "B.P. Little" 1 => "J.C. Junn" 2 => "K.S. Zheng" 3 => "F.W. Sanchez" 4 => "T.S. Henry" 5 => "S. Veeraraghavan" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.2214/AJR.19.22628" "Revista" => array:6 [ "tituloSerie" => "AJR Am J Roentgenol." "fecha" => "2020" "volumen" => "215" "paginaInicial" => "1312" "paginaFinal" => "1320" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/33021835" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Gorshtein" 1 => "D.J. Gross" 2 => "D. Barak" 3 => "Y. Strenov" 4 => "Y. Refaeli" 5 => "I. Shimon" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1002/cncr.26200" "Revista" => array:6 [ "tituloSerie" => "Cancer." "fecha" => "2012" "volumen" => "118" "paginaInicial" => "612" "paginaFinal" => "619" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21751183" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "B.F. Samhouri" 1 => "T.R. Halfdanarson" 2 => "C.W. Koo" 3 => "C. McCarthy" 4 => "E.S. Yi" 5 => "C.F. Thomas" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1530/ERC-23-0051" "Revista" => array:3 [ "tituloSerie" => "Endocr Relat Cancer." "fecha" => "2023" "volumen" => "30" ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0030" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Comparison of gallium-68 somatostatin receptor and <span class="elsevierStyleSup">18</span>F-fluorodeoxyglucose positron emission tomography in the diagnosis of neuroendocrine tumours: a systematic review and metaanalysis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "X. Liu" 1 => "N. Li" 2 => "T. Jiang" 3 => "H. Xu" 4 => "Q. Ran" 5 => "Z. Shu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1967/s002449912108" "Revista" => array:5 [ "tituloSerie" => "Nucl Med." "fecha" => "2020" "volumen" => "23" "paginaInicial" => "188" "paginaFinal" => "200" ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0035" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An update on the Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "T. Al-Toubah" 1 => "S. Grozinsky-Glasberg" 2 => "J. Strosberg" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s11864-021-00828-1" "Revista" => array:5 [ "tituloSerie" => "Curr Treat Options Oncol." "fecha" => "2021" "volumen" => "22" "paginaInicial" => "28" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/33641079" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/21735077/0000010200000010/v1_202410180733/S2173507724001510/v1_202410180733/en/main.assets" "Apartado" => array:4 [ "identificador" => "24081" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Scientific letter" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/21735077/0000010200000010/v1_202410180733/S2173507724001510/v1_202410180733/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173507724001510?idApp=UINPBA00004N" ]
