Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and multiple pulmonary epithelioid hemangioendothelioma (PEH): a case report
Summary: We report a case of a 76-year-old female with multiple lung nodules with history of cough. The lower left lobectomy showed four nodules that were described as pulmonary epithelioid hemangioendothelioma (PEH). In the remaining parenchyma of the lobe, multiple nests of neuroendocrine cells were observed and the diagnosis was diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare condition, with under 100 cases described in literature [1, 2].
Pulmonary epithelioid hemangioendothelioma (PEH) is a vascular tumor very rare in the lung parenchyma, is also of multicentric origin, and extrapulmonary lesions arise from liver, bone, soft tissue, and skin and most of the previously reported cases have been asymptomatic. Herein we describe a case with concomitant DIPNECH and PEH.
Case report: 76-year-old female with chronical cough and angina pectoris (one a month) was to our ambulatory on the 2013. Never fever or night sweating or dyspnoea. The chest x ray showed little bilateral nodules described since the 2005. The lung function test and gastroscopy were normal.
We treated the patient with PPI and inhaled bronchodilators ex adjuvantibus.
On the February 2014 because of the persistence of the cough a Tc scan was performed: “eleven nodular lesion the bigger in the LLL 18×14×15mm, some with calcification”. The bronchoscopy was negative. The patient performed a TTNA of the major nodule which revealed a non-small cell lung carcinoma, not otherwise specifed (NSCLC-NOS).
After the PET with sames results of the CT scan, the patient underwent to the VATS for LLL Lobectomy and linfadenectomy.
Histology: epithelioid hemangioendothelioma. The patient underwent lower left lobectomy.
The immunohistochemical stains showed that the neoplastic cells expressed CD34, CD31 and variable expression for factor VIII. The histological and immunohistochemical features were diagnostic for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Discussion: The recent WHO classification considers DIPNECH a precursor of carcinoid tumorlets and carcinoid tumors. DIPNECH was first described in the early 1950s  mostly occurs in middle aged or older adults with an average age of diagnosis of 58 years but the reported age range varies from 22 to 79 years. There is a female predilection (F:M 4:1) and most patients are nonsmokers. Clinical presentation is often insidious. HRCT often shows small airways obstruction evidenced by inspiratory mosaic attenuation and expiratory air trapping. The coexistence of multiple pulmonary nodule is the best clue to the diagnosis of DIPNECH. The diagnosis of DIPNECH requires histopathological confirmation of pulmonary neuroendocrine cell proliferation confined to the epithelium of large and small airways. The gold standard remains surgical lung biopsy.
Pulmonary epithelioid hemangioendothelioma (PEH)
In 1975 Dail and Liebow reported the first cases of an unusual pulmonary neoplasm that they called “intravascular bronchioloalveolar tumor” (IVBAT). This name reflected their original hypotesis that the lesion in question was an epithelial tumor: specifically, a bronchioloalveolar carcinoma variant showing prominent vascular invasion .
Conclusion: Herein we reported a case with the association between DIPNECH and PEH: both are rare conditions. The concomitant presence of both lesions in the same patient and in a same lobe is also a potential diagnostic pitfall in evaluating a multinodular lung disease, especially if only small biopsies are under examination. Accurate sampling of lung surgical specimen together with appropriate clinical-radiological correlations may help in correctly reveal both conditions.