Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report
© Warth et al; licensee BioMed Central Ltd. 2008
Received: 14 August 2007
Accepted: 25 January 2008
Published: 25 January 2008
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis. It has become generally accepted that DIPNECH is a precursor lesion to pulmonary carcinoid tumors.
Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.
This case contributes to a better understanding of the disorder and its associated pathologies.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an exceedingly rare disorder and only 40 cases have been described in the literature to date . According to the current WHO classification, this disorder is characterized by one of the following: a generalized proliferation of scattered single cells, small nodules or linear proliferations of pulmonary neuroendocrine cells ; in addition, it is considered to be a precursor for pulmonary carcinoid tumors.
DIPNECH is an exceedingly rare disease involving generalized proliferation of pulmonary neuroendocrine cells, which leads to an occlusion of the bronchial lumina and consequent clinical symptoms such as shortness of breath. Due to its neuroendocrine origin, its similar morphology to pulmonary carcinoids and particularly due to its association with pulmonary carcinoids, the disease is considered to be a precursor lesion for theses entities. Only 40 cases of DIPNECH have been reported in the literature to date  and there are no predictive histological or genetic data available so far. However, it has become generally accepted that DIPNECH is a precursor to pulmonary carcinoid tumors . In a recent study including 1090 patients, who received resections for primary lung tumors, Ruffini and colleagues found that the overall prevalence of pre-invasive lesions for lung carcinomas was 6.7%. Only 3 of these 1090 cases were associated with DIPNECH and the primary tumors were carcinoids in all of these cases . We have recently reported a similar case in which DIPNECH was associated with a carcinoid . The current report represents the first case of a patient with DIPNECH accompanied by a pulmonary adenocarcinoma of mixed subtype with partial neuroendocrine differentiation. The adenocarcinoma was positive for typical markers such as CK7, CK18, TTF1, and SPA and additionally, it was positive for NSE and CEA, which were also measured to be elevated in the patient's serum. Interestingly, besides typical carcinoid markers such as CD56, synaptophysin, and chromogranin A, the DIPNECH lesions were also positive for NSE. However, it remains elusive if the elevated NSE levels in the patient's serum belong to the adenocarcinomas, the DIPNECH lesions or a combination of both. Nevertheless, our findings raise the hypothesis of a common pathogenic background of pulmonary tumors with neuroendocrine differentiation, which should further be investigated. Although it is unlikely that DIPNECH is a precursor lesion for other tumors of the lung with neuroendocrine differentiation besides carcinoid tumors , this possibility cannot yet be excluded considering the small number of the cases described to date. Since it has been suggested that DIPNECH represents an underrecognized spectrum of disease and since it is being increasingly diagnosed , we report this case to contribute to a better understanding of the disorder and its associated pathologies. However, the association of DIPNECH with a higher overall cancer incidence should be regarded carefully, since there is evidence that malignancies at other sites lead to an increased use of imaging and thereby to a more frequent detection of DIPNECH . Therefore, more data are needed to accurately draw a conclusion on the incidence of associated malignancies.
DIPNECH is a rare disorder and it is considered to be a precursor lesion for pulmonary carcinoid tumors. Information on the disease is still limited, especially with regard to management and prognosis. This case is the first report of a patient with DIPNECH in association with a pulmonary adenocarcinoma. Since an increasing incidence of DIPNECH cases has been noted in the past few years, we report this case to contribute to a better understanding of the disorder and its associated pathologies.
Written consent was obtained from the patient for the publication of the report.
diffuse idiopathic neuroendocrine cell hyperplasia
thyroid transcription factor 1
surfactant protein A
neuron specific enolase.
- Ge Y, Eltorky MA, Ernst RD, Castro CY: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Ann Diagn Pathol. 2007, 11: 122-126. 10.1016/j.anndiagpath.2005.12.008.View ArticlePubMed
- Gosney JR, Travis WD: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. In Tumours of the lung, pleura, thymus and heart. Edited by: Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC. 2004, IARC Press, 76-77.
- Kerr KM: Pulmonary preinvasive neoplasia. J Clin Pathol. 2001, 54: 257-271. 10.1136/jcp.54.4.257.PubMed CentralView ArticlePubMed
- Ruffini E, Bongiovanni M, Cavallo A, Filosso PL, Giobbe R, Mancuso M, Molinatti M, Oliaro A: The significance of associated pre-invasive lesions in patients resected for primary lung neoplasms. Eur J Cardiothorac Surg. 2004, 26: 165-172. 10.1016/j.ejcts.2004.03.044.View ArticlePubMed
- Johney EC, Pfannschmidt J, Rieker RJ, Schnabel PA, Mechtersheimer G, Dienemann H: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and a typical carcinoid tumor. J Thorac Cardiovasc Surg. 2006, 131: 1207-1208. 10.1016/j.jtcvs.2005.12.064.View ArticlePubMed
- Gosney JR: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia as a precursor to pulmonary neuroendocrine tumors. Chest. 2004, 125: 108-10.1378/chest.125.5_suppl.108S.View Article
- Davies SJ, Gosney JR, Hansell DM, Wells AU, du Bois RM, Burke MM, Sheppard MN, Nicholson AG: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 2007, 62: 248-252. 10.1136/thx.2006.063065.PubMed CentralView ArticlePubMed
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