ATYPICAL EXTRALOBAR SEQUESTRATION:
CT-PATHOLOGICAL CORRELATION
JULIE K. SHAFFREY, MD, DAVID A. BRINKER, MD,
KAREN M. HORTON, MD, RICHARD F. HEITMILLER,
AND ELLIOT K. FISHMAN, MD
Pulmonary sequestration is a malformation com-
prised of dysplastic lung tissue without normal com-
munication with the tracheobronchial tree and with
an anomalous systemic arterial supply. Pulmonary
sequestration is classified into two types, intralobar
and extralobar based on the location of the malfor-
mation and the venous drainage. Extralobar seques-
tration is less common than intralobar sequestration
and usually has systemic venous drainage into the
azygos system. Most patients with extralobar seques-
tration are diagnosed before the age of 10. We pres-
ent an interesting and unusual case of extralobar se-
questration which presented as a retroperitoneal
mass in an asymptomatic adult.  Elsevier Science
Inc., 1999
KEY WORDS:
Lung; Extralobar pulmonary sequestration;
Intraabdominal pulmonary sequestrationcomputed
tomography; 3D imaging
INTRODUCTION
Pulmonary sequestration is a congenital or acquired
malformation comprised of nonfunctioning dysplas-
tic lung tissue with a characteristic anomalous sys-
temic arterial supply and no normal connection with
the tracheobronchial tree. Anatomically, this malfor-
From the Russell H. Morgan Department of Radiology and Ra-
diological Science (J.K.S., K.M.H., E.K.F.), Department of Pathol-
ogy (D.A.B.), Department of Thoracic Surgery (R.F.H.), Johns Hop-
kins University School of Medicine, Baltimore, Maryland.
Address correspondence to: Elliot K. Fishman, MD, Depart-
ment of Radiology, Johns Hopkins Hospital, 600 N. Wolfe Street,
Baltimore, MD 21287.
Received April 29, 1999; accepted June 24, 1999.
CLINICAL IMAGING 1999;23:223–226
 Elsevier Science Inc., 1999. All rights reserved.
655 Avenue of the Americas, New York, NY 10010
MD,
mation is classified into two types, intralobar and ex-
tralobar. Intralobar sequestration (ILS) is more com-
mon, and is located within the pulmonary visceral
pleura with venous drainage most often via the pul-
monary veins. Extralobar sequestration (ELS) is in-
vested by its own visceral pleura, and commonly has
systemic venous drainage, often into the azygos sys-
tem. ELS almost exclusively presents before the age
of 10 and is most commonly located between the
lower lobe and the diaphragm (1, 2).
We present an unusual case of an extralobar se-
questration presenting as an incidental finding in the
retroperitoneum of an adult with the an atypical CT
appearance of a heterogeneous high-attenuating non-
enhancing mass.
CASE REPORT
A healthy, asymptomatic 38-year-old male presented
to our hospital for evaluation of a left-sided mass de-
tected on a preoperative chest radiograph performed
prior to minor orthopedic surgery at an outside hos-
pital. An outside contrast-enhanced chest computed
tomography (CT) scan demonstrated a 6-cm high-
attenuation mass that appeared to be located in the
left hemithorax. At that time, the mass was thought
to be an arteriovenous malformation (AVM).
At our institution, a spiral CT of the chest was
performed before and after the administration of in-
travenous contrast, using the CT angiographic tech-
nique. The unenhanced scan demonstrated a 6-cm
mass of high attenuation with foci of calcification
(Figure 1A). CT scans obtained in the arterial phase
demonstrated no measurable enhancement and no
feeding vessels (Figure 1B). The exact relationship of
this mass to the thoracic cavity was difficult to deter-
0899-7071/99/$–see front matter
PII S0899-7071(99)00137-0
224 SHAFFREY ET AL.
mine, and it appeared to be inseparable from the left
hemidiaphragm on the 3D reconstruction views (Fig-
ure 1C). Bases on the CT appearance, a foregut dupli-
cation cyst was considered, as it often contains high-
density material. The differential diagnosis included
pulmonary sequestration, although this was felt to be
less likely due to the lack of a definable vascular sup-
ply. AVM was not considered to be a diagnostic pos-
sibility due to the lack of enhancement.
At surgery, the intrathoracic cavity was found to
be unremarkable except for a focal bulge in the left
CLINICAL IMAGING VOL. 23, NO. 4
hemidiaphragm. The diaphragm was incised, and a
5 3 6 3 7-cm mass was found in the superior left ret-
roperitoneum. The mass was excised in its entirety.
At pathological evaluation, the specimen consisted
of a 132-gram spongy structure filled with thick yel-
low-white fluid (Figure 2A). Microscopic examina-
tion revealed dilated bronchioles lined by ciliated
pseudostratified columnar epithelium (Figure 2B).
Alveolar spaces and cartilaginous bronchi were
noted. Amorphous material filled the air spaces; ba-
sophilic polygonal and rod-shaped calcifications
FIGURE 1. 38-year-old man presenting with a high-
attenuation mass originally diagnosed as an AVM at
an outside institution. (A) Unenhanced CT scan
demonstrates a 6-cm left-sided mass with high CT at-
tenuation (arrow). (B) Contrast-enhanced CT scan
demonstrates no significant enhancement of the
mass (arrow). (C) CT angiographic technique with 3D
reconstruction views better demonstrates the loca-
tion of the mass (arrow), which was difficult to sepa-
rate from the diaphragm. No feeding vessels were
identified.
JULY/AUGUST 1999
FIGURE 2. Pathologic examination of an extralobar pul-
monary sequestration. (A) Gross photograph of opened
specimen shows smooth cyst walls. (B) Photomicrograph
shows epithelial-lined spaces filled with amorphous mate-
rial (340). Inset shows ciliated columnar epithelium and
luminal calcific material (3400).
were numerous. Chronic inflammatory cells (lym-
phocytes, plasma cells, and histiocytes) were identi-
fied in the interstitium and alveoli.
DISCUSSION
This is an unusual case of extralobar sequestration.
First, this patient is an adult. ELS occurs almost ex-
clusively presents before the age of 10, and most of-
ten presents within the first 6 months of life with
dyspnea and cyanosis (1, 2). However, it is estimated
that 10% of ELSs are asymptomatic, and may, there-
fore, go undetected (3). On the rare occasions that an
ELS presents as an adult, it is usually as an inciden-
ATYPICAL EXTRALOBAR SEQUESTRATION 225
tal finding, as in this case. Second, the retroperito-
neal location is unusual for ELS (4). ELS is most com-
monly located between the lower lobe and the
diaphragm. However, ELS has been described in a
variety of locations, including the mediastinum,
abdomen, retroperitoneum, pericardium, and even
with the substance of the diaphragm itself (3). The
difficulty in defining the exact location of the ELS in
this case is explained by its intimate association with
the diaphragm demonstrated at surgery. The poste-
rior sulcus of the left hemithorax, the superior ret-
roperitoneum, and the diaphragmatic parenchyma
were all considered possible sites on preoperative
imaging studies. Third, the CT appearance of this
ELS is atypical. Although the CT appearance of se-
questrations is varied, it usually appears as a homo-
geneous mass or may demonstrate solid and cystic
components along with areas of an atelectatic lung
(1). Radiographically detectable calcification in a se-
questration is rare and, to our knowledge, has been
described in only six cases—only one of which was
an extralobar sequestration (4). Explanations for the
presence of calcification are varied. In the single case
of ELS involving calcification, islands of bone were
identified, suggesting a hamartomatous component
(4). In one case of ILS, the calcification involved a
thrombosed systemic arterial supply rather than the
lung tissue (5). Another of the ILS cases involved a
patient with renal failure and suspected metastatic
calcification (6). In the three other cases the authors
explained the presence of calcification as the result
of chronic and recurrent infection of the sequestered
tissue (7, 8). In our case, thick secretions with
embedded calcifications filled the air spaces, ac-
counting for the high CT attenuation of the mass on
noncontrast images. In the setting of chronic inflam-
matory cells, dystrophic calcification as the result of
chronic infection is the most likely mechanism.
This case is also interesting because it demon-
strates the potential usefulness of 3D CT in defining
the location and vascular supply of these complex
malformations. Arterial supply in sequestrations
has classically been delineated by angiography. How-
ever, with the advent of more advanced scanners, CT
has been shown to be increasingly accurate as a non-
invasive method of vascular evaluation and often ob-
viates the need for conventional angiography (1).
Ikezoe et al. (1) used CT to demonstrate a vascular
supply in 16 of 24 cases of sequestration. In one of
the eight cases where CT was unsuccessful, surgery
demonstrated the sequestration to be fed by many
small branches of an intercostal artery. In our pa-
tient, the lack of a definable vascular supply at im-
226 SHAFFREY ET AL.
aging is not surprising considering the surgical find-
ings of numerous small vascular pedicles and no
dominant vascular stalk.
In summary, an outside CT scan performed with
i.v. contrast also demonstrated high attenuation
within the mass; however, at that time, it was felt the
high attenuation was due to enhancement. This mis-
conception led to the original diagnosis of AVM in
this patient. Further workup with a pre- and postcon-
trast scan at our institution demonstrated the high at-
tenuation to be a primary feature of the mass and not
the result of enhancement. Although the presence of
high CT attenuation due to radiographically detect-
able calcification has been described only once be-
fore in an ELS (4), we assert that it remains important
to fully evaluate such lesions with both pre- and
postcontrast scans as the treatment plans for various
diagnostic possibilities may differ significantly.
CLINICAL IMAGING VOL. 23, NO. 4
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