Eur J Pediatr (2006) 165: 736–738

DOI 10.1007/s00431-006-0149-5

YOU R DIAGN OSIS

Safak Gucer . Umran Caliskan . Canan Ucar .

Yavuz Koksal . Alaettin Dilsiz . Gulsev Kale

A suprarenal mass in a child

Received: 12 February 2006 / Accepted: 22 March 2006 / Published online: 16 August 2006
# Springer-Verlag 2006

Clinical information occupying mainly the left surrenal region (Fig. 1). The
adrenal gland could not be discerned in the mass. The
A 5-year-old boy was admitted to the hospital with lesion consisted of cystic and solid components. There
complaints of abdominal pain, weight loss (2 kg lost in were also a few lymph nodes varying from 0.5 cm to 1 cm
2 months) and sweating lasting for 2 months. Past medical in size in the para-aortic region. At laparotomy, a
history was unremarkable. On physical examination his 6 cm×5 cm×4 cm retroperitoneal mass was found in the
body temperature was 36.8°C (axillary), and the rest of the left suprarenal region.
physical examination was unremarkable. The peripheral
blood count showed a hemoglobin level of 14.2 g/dl, a
hematocrit value of 41.7%, a platelet count of 318,000/
mm3 and a white blood cell count of 5,900/mm3 with
normal differential. No atypical cells were seen in his
peripheral blood smear. The erythrocyte sedimentation rate
was 31 mm/h, and the serum biochemistries were normal
except for a temporary increase in transaminases. C-
reactive protein was 64.2 mg/l (N: 0–10). Levels of urinary
catecholamine metabolites, α-fetoprotein, and β-human
chorionic gonadotropin were normal. Bone marrow aspi-
ration revealed hypercellularity, but no malignant infiltra-
tion was seen.
Abdominal computed tomography and magnetic reso-
nance imaging studies showed a retroperitoneal pararenal
soft tissue mass extending to the pre-aortic area and
Fig. 1 Computed tomography shows the soft tissue mass
S. Gucer . G. Kale surrounding left kidney and suprarenal region
Pathology Unit, Department of Pediatrics, Faculty of Medicine,
Hacettepe University,
Ankara, Turkey

U. Caliskan . C. Ucar
Department of Pediatric Hematology, Meram Medical Faculty,
Selcuk University,
Konya, Turkey

Y. Koksal (*)
Department of Pediatric Oncology, Meram Medical Faculty,
Selcuk University,
42080 Konya, Turkey
e-mail: yavuzkoksal@yahoo.com
Tel.: +90-332-2236310

A. Dilsiz
Department of Pediatric Surgery, Meram Medical Faculty,
Selcuk University,
Konya, Turkey

What is your diagnosis?
It was supplied by an artery from the abdominal aorta, and
the venous drainage was to the left renal vein. The mass
was adherent to the tail of the pancreas, the left kidney
capsule, and the left surrenal gland, and extended to the left
hemidiaphragm. It was removed with the left adrenal gland
and some para-aortic lymph nodes.
Results
The histological pattern revealed two distorted bronchi
showing intraluminal acute inflammatory exudates, peri-
bronchial mononuclear inflammatory cells and fibrous
tissue. Two foci of non-caseating granulomas were also
noted (Fig. 2). Neither acid-fast bacteria nor caseation was
detected.
Diagnosis Upon these findings, we diagnosed extralobar
pulmonary sequestration (ELPS). The postoperative fol-
low-up period of 1 year was uneventful. No medication
was given.
Discussion
Pulmonary sequestration is a rare congenital anomaly
occurring with an incidence of 0.15–1.7% [2, 6, 9]. It is
characterized by non-functioning pulmonary tissue without
normal connection to the tracheobronchial tree and
supplied by one or more abnormal systemic arteries
(especially the aorta). It has classically been described as
intralobar, in which the sequestered lung tissue is contig-
uous with the adjacent normal lung covered by same
pleura, or extralobar, with a separate pleural covering.
Fig. 2 Two distorted bronchi showing intraluminal acute inflam-
matory exudates, peribronchial mononuclear inflammatory cells and
fibrous tissue. Two foci of non-caseating granulomas are also noted
(upper right). H&E, ×200
737
The lower respiratory organs, including the larynx,
trachea, bronchi and lung, begin to form during the fourth
week of gestation when lung buds appears at the caudal end
of the pharyngotracheal tube. These buds further divide
into right and left mainstem bronchi and form bronchi,
bronchioli and alveolar ducts with subdivisions [2, 6, 9].
Extralobar pulmonary sequestration originates, possibly,
from a separate outpouching of the foregut or by abnormal
separation of a segment of the lung during the development
[9].
Extralobar pulmonary sequestration has been reported to
be associated with various congenital anomalies, of which
congenital diaphragmatic hernia is the most frequent [2, 6].
The high occurrence of diaphragmatic hernia may suggest
that a defect leading to ELPS occurs before the sixth week
of gestation, when the diaphragm is fused. Other associated
anomalies include congenital heart and pericardial defects,
lung anomalies, skeletal malformations, foregut anomalies,
gastrointestinal fistulous complications and duplications
[2, 6, 9]. Extralobar pulmonary sequestration occurs mostly
in the left side, and its location is between the lower lobe of
the lung and the diaphragm in 63–77% of the cases. In the
child’s first year of life the development anomaly with
aberrant non-functioning pulmonary parenchyma tissue
can cause respiratory and feeding problems [1, 6].
Although rare, ELPS can also be subdiaphragmatic,
predominantly in a suprarenal location, and can easily be
mistaken for para-spinal neoplastic disorders [2, 3, 5, 7,
9]. Extralobar pulmonary sequestration is intra-abdominal
in 10–15% of cases and may remain asymptomatic for
years unless an infection supervenes [2, 6, 9]. It may
occur as an incidental finding during an X-ray or
ultrasonographic investigation or during the course of
corrective surgery of congenital diaphragmatic hernia. An
increasing number of ELPS cases have been antenatally
diagnosed with the widespread use of prenatal ultraso-
nography [1, 4, 7].
In our patient the ELPS had remained silent until he
reached 5 years of age, when it became symptomatic,
including abdominal pain, weight loss and sweating. A
number of diseases, particularly neoplastic, including
neuroblastoma, rhabdomyosarcoma, teratoma, and adrenal
and renal tumors were investigated, since the imaging
studies yielded a mass in the left suprarenal region. Less
commonly, duplication cysts, myolipoma, ELPS and
hemangioma were included in the differential diagnosis.
The main presumptive diagnosis was neuroblastoma or
teratoma in our patient.
Light microscopy of ELPS reveals well-formed bron-
chial structures or irregular lumens lined with a ciliated
pseudo-stratified columnar epithelium. In some cases there
may be histological features consistent with type-2
congenital pulmonary airway malformation [2, 6, 8, 9].
In the long term, once the infection had supervened, the
acute and chronic inflammatory changes would occur. A
few patients have been reported to have prominent
lymphatic dilatation [2]. It is interesting that our patient
showed mainly a granulomatous inflammation that
prompted us to search for a mycobacterial tuberculosis
738
and other mycobacterial infections. However, no acid-fast
bacilli were demonstrated, and polymerase chain reaction
results were negative, as were the other laboratory
investigations for tuberculosis, both on the patient and on
the family. To our knowledge, granulomatous inflamma-
tion has not previously been reported in patients with
ELPS. In our opinion the granulomatous inflammation is
the result of interstitial leakage of bronchial secretions.
In conclusion, although rare, ELPS, which is located
subdiaphragmatically, predominantly in a suprarenal loca-
tion, can easily be mistaken for para-spinal or surrenal
neoplastic disorders. The recommended treatment of
symptomatic ELPS is surgical resection. Laparoscopic
resection is the first choice of experienced surgeons.
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