Hindawi Publishing Corporation
Case Reports in Medicine
Volume 2011, Article ID 262674, 6 pages
doi:10.1155/2011/262674
Case Report
Primary Pulmonary Epithelioid Hemangioendothelioma:
A Rare Cause of PET-Negative Pulmonary Nodules
Riccardo Cazzuffi,
1
Nunzio Calia,
1
Franco Ravenna,
1
Claudio Pasquini,
1
Sara Saturni,
1
Giorgio Narciso Cavallesco,
2
Francesco Quarantotto,
2
Rosa Rinaldi,
3
Annaluisa Cogo,
1
Gaetano Caramori,
1, 4
and Alberto Papi
1
1
Sezione di Malattie dell’Apparato Respiratorio, Dipartimento di Medicina Clinica e Sperimentale, Universit` a di Ferrara, Italy
2
Dipartimento di Scienze Chirurgiche, Anestesiologiche e Radiologiche, Modulo di Chirurgia Toracica, Universit` a di Ferrara, Italy
3
Sezione di Anatomia, Istologia e Citologia Patologica, Dipartimento di Medicina Sperimentale e Diagnostica,
Universit` a di Ferrara, Italy
4
Centro per lo Studio delle Malattie Infiammatorie Croniche delle Vie Aeree e Patologie Fumo Correlate dell’Apparato Respiratorio
(CEMICEF; formerly Centro di Ricerca su Asma e BPCO), Universit` a di Ferrara, Via Savonarola 9, 44121 Ferrara, Italy
Correspondence should be addressed to Gaetano Caramori, gaetano.caramori@unife.it
Received 11 February 2011; Accepted 16 May 2011
Academic Editor: A. Curt
Copyright © 2011 Riccardo Cazzuffi et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man,
presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography.
At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was
made on a pulmonary wedge resection (performed during video-thoracoscopic surgery).
1. Introduction
Pulmonary epithelioid hemangioendothelioma (PEH) is a
rare, low- to intermediate-grade tumor of endothelial origin
[1] with around 120 cases reported in the literature [2]. At
the onset, the patients are usually asymptomatic or present
with nonspecific symptoms (such as weight loss, fatigue,
dyspnoea, cough, and chest pain). Chest imaging usually
shows the presence of multiple, bilateral small pulmonary
lesions. The diagnosis usually requires a lung biopsy. The
treatment is still not standardized. Surgery is suggested in
presence of a solitary nodule or few unilateral nodules. In-
stead if the lesions are unresectable or multiple and bilateral
several chemotherapy protocols have been used. The prog-
nosis is variable with a median survival of 4-5 years.
2. Case Report
A 67-year-old Caucasian man, lifelong nonsmoker, with no
prior history of lung diseases, was referred to our university
hospital for the presence, in the last 3 months, of exertion
dyspnoea (grade 1 according to the Medical Research Coun-
cil scale [3]) and dry cough. He had worked as an employee
without occupational exposures of clinical relevance. His
past medical history was characterized by an herniated lum-
bar disc, systemic arterial hypertension (treated with calcium
blockers), diabetes mellitus (treated with oral hypoglycaemic
agents), and polycythemia vera (diagnosed in 2007 and treat-
ed from 2008 with hydroxyurea). Physical examination was
unremarkable. Arterial blood gases analysis performed with
the patient breathing room air demonstrated normal val-
ues (pH 7.40, PaCO
2
43 mmHg, PaO
2
91 mmHg, and bicar-
bonate level 26 mmoL/L). Routine laboratory tests were
within the normal range except for those listed in Table 1.
Serum levels of neoplastic markers were within normal
range, including carcinoembryonic antigen (2.7 ng/mL),
prostate-specific antigen (3.75 ng/mL), and CA19.9 (15 U/
mL). Also the serum levels of angiotensin-converting en-
zyme (11 IU/mL) and β
2
microglobulin (2.50 mg/mL) were
within normal limits.