Association of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with lung adenocarcinoma: A radiologist’s perspective

Gorospe, Luis; Muñoz-Molina, Gemma María; Farfán-Leal, Frank Eric; Cabañero-Sánchez, Alberto; García-Gómez-Muriel, Isabel; Benito-Berlinches, Amparo

doi:10.1016/j.lungcan.2017.06.010

Cited by 5 publications

(5 citation statements)

References 5 publications

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“…Although in our series, there is a family association of two sisters affected by DIPNECH, there is no clear evidence of family aggregation/germline genetic alterations in this entity. The possible association between DIPNECH and pulmonary adenocarcinoma is described [8], but not with other types of tumors. However, two of our patients (50%) have a metachronous tumor (perianal squamous cell carcinoma and gastric adenocarcinoma).…”

Section: Discussionmentioning

confidence: 99%

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience

Cabezón-Gutiérrez

Khosravi-Shahi

Palka-Kotlowska

et al. 2019

Cureus

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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder that is commonly underdiagnosed. In 2015, it was recognized by the World Health Organization (WHO) classification of lung tumors as a premalignant lesion. DIPNECH syndrome is characterized by cough, exertional dyspnea, wheezing, and, less frequently, hemoptysis. We report the clinical and histological features and imaging findings in four cases of DIPNECH from our institution (Torrejon University Hospital, Madrid, Spain) between the years 2012 and 2019. DIPNECH represents a rare and poorly understood pulmonary disorder. Our limited single-center experience shows the slow and stable evolution of the disease. However, some exceptional cases may progress poorly if distant metastases occur.

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Section: Discussionmentioning

confidence: 99%

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience

Cabezón-Gutiérrez

Khosravi-Shahi

Palka-Kotlowska

et al. 2019

Cureus

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“…In some cases, a familial link has been observed with two sisters affected by DIPNECH [ 11 ]. However, there is a lack of clear evidence supporting family aggregation or germline genetic alterations in this particular condition [ 11 , 12 ]. In our case, the patient's sister has experienced breast cancer, and their brother has been diagnosed with throat cancer, without any history of DIPNECH.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report

Pokhriyal,

Sapkota,

Al-Ghuraibawi

et al. 2024

Cureus

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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease characterized by the diffuse proliferation of neuroendocrine cells in the bronchial epithelium. It is considered a preinvasive precursor to carcinoid tumors and usually presents with obstructive symptoms. We present the case of a 71-year-old female, non-smoker, with a past medical history of asthma, osteoarthritis, allergic rhinitis, and hyperlipidemia who was referred to the pulmonology clinic in view of incidental chest CT findings of multiple pulmonary nodules. Physical examination and labs were unremarkable. CT of the chest showed scattered multiple noncalcified pulmonary nodules with a 10 mm dominant nodule in the inferior right middle lobe and several subcentimeter hypodensities in the left and right lobes of the lung. A PET scan confirmed the CT findings along with no abnormal hypermetabolic activity to suggest malignancy. The patient was followed up in the pulmonology clinic at six months, 12 months, and then 18 months. At 18 months owing to a slight increase in the size of the largest lung nodule, a CT-guided biopsy done was conclusive of a carcinoid. The tumor cells were positive for synaptophysin, chromogranin, insulinoma-associated protein 1 (INSM-1), and thyroid transcription factor 1 (TTF-1). The Ki-67 (Keil) index was <1%. A video-assisted thoracic surgery with right middle lobectomy along with mediastinal lymph node dissection was then done, and the patient was found to have stage pT1aN0 typical carcinoid tumor (1.0 cm), with multiple carcinoid tumors and neuroendocrine hyperplasia, consistent with DIPNECH. She has been under clinical follow-up for over three years at present and continues to be asymptomatic with complete remission following surgery. DIPNECH primarily affects middle-aged, non-smoking females who present with cough and dyspnea, and diagnosis is often delayed due to clinical features overlapping with those of obstructive lung disease. Imaging shows lung nodules, ground-glass opacities, and/or mosaic attenuation. Due to the rarity of the conditions, there are no established clinical trials, and therefore, there is a need to establish guidelines.

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“…1,3 To the best of our knowledge, there are few published reports concerning differentiating DIPNECH manifesting as multiple pulmonary nodules from multiple pulmonary metastases (Table ). 2,4 Whenever a patient has lung cancer with multiple pulmonary nodules, DIPNECH should be included in the differential diagnosis. 4 Reported features of DIPNECH are as follows: (1) most affected patients are women in their 50s to 70s; (2) 70% of affected patients have never smoked; (3) chronic cough is a characteristic symptom; (4) the condition is characteristically accompanied by obstructive pulmonary disease; (5) high serum concentrations of progastrinreleasing peptide; and (6) multiple pulmonary nodules of diameter 5 mm or less located around the lower lung field or bronchi.…”

Section: Discussionmentioning

confidence: 99%

“…2,4 Whenever a patient has lung cancer with multiple pulmonary nodules, DIPNECH should be included in the differential diagnosis. 4 Reported features of DIPNECH are as follows: (1) most affected patients are women in their 50s to 70s; (2) 70% of affected patients have never smoked; (3) chronic cough is a characteristic symptom; (4) the condition is characteristically accompanied by obstructive pulmonary disease; (5) high serum concentrations of progastrinreleasing peptide; and (6) multiple pulmonary nodules of diameter 5 mm or less located around the lower lung field or bronchi. 3,5 The current patient demonstrated all of these features except for obstructive respiratory disorder, and the past reported patients in Table seem to have some of those features.…”

Section: Discussionmentioning

confidence: 99%

Lung adenocarcinoma coexisting with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia manifesting as multiple pulmonary nodules: A case report

et al. 2022

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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), a rare condition, is characterized by pathological proliferation of neuroendocrine cells. Some of them are localized to the airway mucosa, and others locally infiltrate to form tumorlets and nodules. Here, we present a patient with lung adenocarcinoma accompanied by DIPNECH, making the latter difficult to distinguish from multiple pulmonary metastases. The patient, a 72-year-old Japanese woman, was diagnosed as having stage IVA lung adenocarcinoma because she had multiple nodules in both lungs. Mutation of epidermal growth factor receptor gene having been found in the primary tumor, treatment with osimertinib was started. This resulted in shrinkage of the primary tumor, but not the multiple pulmonary nodules. To determine whether these lung nodules were indeed lung metastases, we performed right upper lobectomy with lymphadenectomy and wedge resection of the right lower lobe. On pathological examination, the primary tumor was diagnosed as invasive adenocarcinoma, whereas the multiple pulmonary nodules were diagnosed as DIPNECH manifesting as tumorlets. Therefore, the final diagnosis was stage IA1 lung adenocarcinoma accompanied by DINPECH. The patient had no recurrences 1 year after the operation without any additional treatment. This is a rare case of lung adenocarcinoma accompanied by DIPNECH presenting as multiple pulmonary nodules. DIPNECH should be included in the differential diagnosis of multiple pulmonary nodules. K E Y W O R D Sdiffuse idiopathic pulmonary neuroendocrine cell hyperplasia, epidermal growth factor receptor, lung adenocarcinoma, tyrosine kinase inhibitor

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Association of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with lung adenocarcinoma: A radiologist’s perspective

Cited by 5 publications

References 5 publications

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report

Lung adenocarcinoma coexisting with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia manifesting as multiple pulmonary nodules: A case report

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