CASE 11242 Published on 22.11.2013

A rare cause of bilateral pulmonary nodules

Section

Chest imaging

Case Type

Clinical Cases

Authors

Mesquita R, Sousa M, Ananias P, Lopes P, Costa N, Ribeiro A, Figueiredo L

Hospital de Santa Marta,
Centro Hospitalar Lisboa Central,
Radiologia;
Rua de Santa Marta, Lisboa, Portugal;
Email:romeu.mesquita@gmail.com
Patient

35 years, male

Categories
Area of Interest Thorax, Abdomen, Lung ; Imaging Technique Digital radiography, CT-High Resolution, CT-Angiography, CT, Experimental
Clinical History
A 35-year-old man, non-smoker, without symptoms or prior history of lung diseases. His past medical history was not significant, without occupational exposures or family history of clinical relevance. The physical examination was unremarkable.
Imaging Findings
A routine chest X-ray showed diffuse and bilateral micronodular pattern - multiple small, round noncalcified nodules in both lungs (Fig. 1). Serum levels of neoplastic markers and angiotensin converting enzyme were normal. Pulmonary function tests were also normal. Bronchoscopy performed with bronchoalveolar lavage, brushing and transbronchial biopsies were nondiagnostic. Chest HRCT examination showed multiple, bilateral small pulmonary nodules (< 10mm). These nodules were present in all pulmonary lobes and adjacent to bronchioles and medium/small vessels (Fig. 2 a, b). There were no enlarged lymph nodes, pleural effusion or other relevant pulmonary changes (Fig. c). Abdominal and pelvic CT revealed no malignant lesions (Fig. 3). Radiological skeletal study showed no bone involvement. Surgical lung biopsy and histological examination of the nodules revealed epithelioid cells with eosinophilic cytoplasm and intracytoplasmic vacuolization (Fig. a, b, c). On immunohistochemical studies, the tumour cells were immunoreactive to CD34 and negative for cytokeratin (Fig. 4 d).
Discussion
Pulmonary epithelioid haemangioendothelioma (EH) is a rare neoplasm of vascular origin, previously known as intravascular bronchioloalveolar tumour.
It is a low- to-intermediate tumour, with a borderline malignancy and a clinical course usually between haemangioma and angiosarcoma.
Pulmonary EH is more common in women than men and many patients are asymptomatic at presentation, so it is often an incidental finding on imaging studies.
The prognosis is very variable, with survival ranging from less than one year up to 30 years. Pulmonary symptoms and, especially, pleural haemorrhagic effusions and haemoptysis are generally considered the worst prognostic factors [1].
EH can affect many organs simultaneously or sequentially, and lungs and liver are the two organs most frequently involved. In patients with additonal hepatic involvement knowledge of the CT manifestations in the liver may be helpful to narrow the differential diagnoses.
There are three different patterns of CT findings identified in thoracic manifestation of EH: 1) multiple pulmonary nodules; 2) multiple pulmonary reticulonodular opacities; and 3) diffuse infiltrative pleural thickening [2].
The presence of multiple discrete pulmonary perivascular nodules with well- or ill-defined margins in both lungs on chest radiographs or CT is the characteristic finding. The nodules can range in size up to 3 cm, but most are less than 1 cm in diameter, and are usually found in relation to small and medium-sized vessels and bronchi. This presentation may appear in many lung diseases and is easily mistaken for metastatic carcinoma, which is usually the initial radiologic interpretation. However, little growth is shown on serial chest CT examinations [3]. Although histologic calcifications are common, radiologic visible calcifications are rare. In long-standing cases or after treatment, extensive calcification of the nodules can be seen.
PET/CT findings can demonstrate increased FDG-uptakes, but usually are not a pivotal tool for the diagnosis, and a negative PET cannot exclude pulmonary EH [4].
Histologic characteristics of EH include epithelioid cells with abundant eosinophilic cytoplasm in a fibromyxoid stroma, some of them with intracytoplasmic vacuoles, having a signet ring-like appearance. The diagnosis usually requires a lung biopsy, and is made on the basis of these histopathological features and confirmed by positive immunohistochemistry staining for vascular-endothelial markers, like CD31, CD34 and factor VIII [2].
There is no established standard treatment. Spontaneous partial regressions are reported, particularly in asymptomatic patients [5]. Surgery when tumour resection is feasible is usually the treatment of choice. Chemotherapy/immunostimulants in patients with disseminated disease and lung transplantation can also be an option [6].
Differential Diagnosis List
Pulmonary epithelioid haemangioendothelioma
Pulmonary metastases
Miliary granulomatous infection
Sarcoidosis
Silicosis
Primary lung malignancy
Lymphangitic carcinomatosis
Final Diagnosis
Pulmonary epithelioid haemangioendothelioma
Case information
URL: https://www.eurorad.org/case/11242
DOI: 10.1594/EURORAD/CASE.11242
ISSN: 1563-4086