Calcifying Fibrous Pseudotumor of
3 cm in
the Pleura
Ki Seok Jang, MD, Young-Ha Oh, MD,
Hong Xiu Han, MD, Soon-Ho Chon, MD,
Won Sang Chung, MD, Chan Kum Park, MD, and
Seung Sam Paik, MD
Departments of Pathology and Thoracic and Cardiovascular
Surgery, College of Medicine, Hanyang University, Seoul,
Korea
Calcifying fibrous pseudotumor is an uncommon benign
lesion that has unique histologic features. We report a case
of calcifying fibrous pseudotumor of the pleura occurring
in a 31-year-old woman. A computed tomographic scan
revealed a pleural mass in the right anterior costophrenic
angle. The excised mass was well circumscribed, nonencap-
sulated, solid, and firm. The tumor showed dense hyalin-
ized collagenous tissue interspersed with spindle cells,
psammomatous calcifications, and a predominantly lym-
phoplasmocytic infiltrate. Most spindle cells were diffusely
positive for vimentin, focally positive for CD34, and nega-
tive for desmins, smooth muscle actin, S-100 protein, and
anaplastic lymphoma kinase-1.
(Ann Thorac Surg 2004;78:e87 8)
2004 by The Society of Thoracic Surgeons
C alcifying fibrous pseudotumor (CFP) is an uncom-
mon benign fibrous lesion that was originally de-
scribed by Rosenthal and Abdul-Karim [1], and the term
itself was introduced by Fetsch and associates [2]. The
lesion is relatively common in children and young adults,
but its pathogenesis is still unclear. Recently it has been
postulated that CFPs may represent a late sclerosing
stage of inflammatory myofibroblastic tumor (IMT) [3, 4].
Lesions most commonly arise in the extremities, followed
by the trunk, inguinal and scrotal regions, and the head
and neck. Rare cases have been reported in the medias-
tinum, pleura, and visceral peritoneum [4]. We report a
case of pleural CFP with immunohistochemical studies.
A chest wall mass was incidentally found in a simple
chest roentgenogram of a healthy 31-year-old woman
with clavicular fracture due to a traffic accident. The
patient was transferred to our hospital for further evalu-
ation of the chest wall mass. Computed tomographic scan
revealed a well demarcated calcifying mass, measuring 8
7 5 cm in the right anterior costophrenic angle. The
mass was shown to compress the dome of her liver (Fig
1). This mass was not associated with her ribs or her
adjacent lung. Percutaneous needle biopsy was per-
formed and a piece of grayish white tissue was obtained.
Histopathologic examination of the needle biopsy re-
vealed cicatricial tissue with diffusely distributed calcifi-
cations without evidence of malignancy. Right lateral
thoracotomy was performed and the pleural cavity was
entered through the sixth intercostal space. The mass,
which originated from the parietal pleura, was resected
en-bloc with surrounding tissue, including portions of
periosteum from the overlying ribs. There was no exten-
sion to the visceral pleura and adhesions were absent. The
Accepted for publication March 25, 2004.
Address reprint requests to Dr Paik, Department of Pathology, College of
Medicine, Hanyang University, 17, Haengdang-dong, Seongdong-ku,
Seoul 133-791, Korea; e-mail: sspaik@hanyang.ac.kr.
2004 by The Society of Thoracic Surgeons
Published by Elsevier Inc
completely excised mass measured 8.5 7.5 4.
dimension. The mass was attached to the parietal pleura.
The outer surface was smooth and lobulated. The cut
surface showed a well circumscribed, nonencapsulated,
solid, and firm appearance (Fig 2). Microscopically, the
tumor was composed largely of dense interwoven collagen
bundles, scattered wavy spindle-shaped cells, and evenly
distributed psammomatous calcifications with inflamma-
tory infiltrates including lymphocytes, plasma cells, and
macrophages (Fig 3A). Most infiltrating lymphocytes were
of T-cell origin. The spindle cells were diffusely positive for
vimentin (Fig 3B) and focally positive for CD34 (Fig 3C), but
negative for desmins, smooth muscle actin, anaplastic lym-
phoma kinase-1, and S-100 protein. The patients postoper-
ative recovery was uneventful, and the patient was well at
her 12-month follow-up examination.
Comment
Calcifying fibrous pseudotumor is a rare benign soft
tissue entity with unique histologic features originally
reported by Rosenthal and Abdul-Karim [1] as a child-
hood fibrous tumor with psammoma bodies in 1988. In
1993, Fetsch and associates [2] introduced the term calci-
fying fibrous pseudotumor with an analysis of 10 cases.
This tumor most commonly appears as a solitary lesion
and primarily affects children and young adults with a
slight female predilection [3]. This tumor most commonly
arises in the extremities [4]. However, CFP of the pleura
is extremely rare. We believe that only 5 cases have been
reported to have CFP of the pleura [57]. Most patients
present a slow growing, painless mass in the subcutane-
ous or deep soft tissues. Most have been treated by
simple local excision, and there was a report of only a
single recurrence that occurred 7 years after the initial
excision. There have been no reports of metastasis.
The pathologic features of a CFP are well established.
The features include a nonencapsulated, densely hyalin-
ized, fibrous proliferation with variable degrees of infil-
tration of chronic inflammatory cells. Two types of calci-
fication have been described, including psammomatous
and dystrophic [1 4]. The present case showed typical
histologic features of a CFP with many psammomatous
calcifications and areas of inflammatory infiltrates, in-
cluding lymphocytes, plasma cells, and macrophages in
densely hyalinized fibrous stroma.
Fig 1. Computed tomographic scan reveals a pleural mass in the
right anterior costophrenic angle (arrow).
0003-4975/04/$30.00
doi:10.1016/j.athoracsur.2004.03.100
e88 CASE REPORT JANG ET AL
CALCIFYING FIBROUS PSEUDOTUMOR
Fig 2. The cut surface of the tumor shows a well circumscribed,
nonencapsulated, solid, and firm appearance.
The main differential diagnoses include IMTs and
localized fibrous tumors of the pleura. Inflammatory
myofibroblastic tumors are usually more cellular and less
hyalinized. However, to distinguish them from IMTs may
be difficult, because spindle cells, inflammatory infiltrate,
and calcification sometimes characterize both CFPs and
IMTs. There is a histologic overlap of these lesions, thus
it has been proposed that CFPs represent a late scleros-
ing phase of IMTs [3, 4]. Recently, a subset of IMTs has
been shown to have clonal cytogenetic abnormalities
involving the anaplastic lymphoma kinase-1 gene on
chromosome 2p [4]. Unlike IMTs, CFPs rarely express the
anaplastic lymphoma kinase-1 gene. Our presenting case
was negative for anaplastic lymphoma kinase-1 immu-
nostaining. This result partly supports the hypothesis
that CFPs have a different clinicopathologic entity when
comparing them with IMTs. This hypothesis opposes the
idea that CFPs represents a late sclerosing stage of IMTs.
Localized fibrous tumors of the pleura also show benign
Fig 3. (A) Microscopically, the tumor is com-
posed largely of dense interwoven collagen
bundles, scattered spindle-shaped cells, and
psammomatous calcifications with inflamma-
tory infiltrate, including lymphocytes and
plasma cells. The spindle cells were positive
for (B) vimentin and (C) CD34.
Ann Thorac Surg
2004;78:e87 8
fibrous proliferation with hyalinized collagenous stroma,
but psammomatous or dystrophic calcifications and in-
flammatory infiltrates are not combined. Characteristi-
cally, the fibrous tumor cells of localized fibrous tumors
of the pleura are positive for CD34 immunostaining.
Most reported CFPs are negative for CD34, except for
rare cases [8]. Our case was positive for CD34 immuno-
staining. Our CD34 positive finding deviates from the
other greater part of CD34 negative immunostaining
found in most CFPs. CD34 positive cases in the literature
have been found to develop in the neck, peritoneum,
mediastinum, and paratesticular sites. Our case had
developed in the parietal pleura and was shown to be
positive for CD34 immunostaining.
Calcifying fibrous pseudotumor of the pleura is ex-
tremely rare, and only 5 cases of CFT of the pleura have
been described in the literature. We report an additional
case of CFP of the pleura with immunohistochemical studies.
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