2013, Cilt 1, Sayı 1, Sayfa(lar)
Calcifying Fibrous Tumor: A Case Report
Mehmet GAMSIZKAN1, Cengiz YILDIRIM2, Koray DAŞ3, Ömer GÜNHAN1
1Department of Pathology, Gülhane Military Medical Academy, Faculty of Medicine, ANKARA, TURKEY
2Department of Orthopaedics and Traumatology, Tatvan Military Hospital, BİTLİS, TURKEY
3Department of General Surgery, Ankara Mevki Military Hospital, ANKARA, TURKEY
Anahtar Kelimeler: Kalsifikasyon, Fibröz doku tümörleri, Patoloji
Kalsifiye fibröz tümör genellikle çocuk ve genç erişkinleri etkileyen
nadir görülen benign fibröz bir lezyondur. Yirmi bir yaşında erkek
hastada sağ inguinal bölgeden başlayarak uyluk anteromedial düzeye
kadar uzanan, palpasyonda ele gelen çok sayıda kitleleri mevcuttu.
Hastaya tanısal eksizyonel biyopsi uygulandı. Histopatolojik incelemede
yoğun kollajenize bir stromada fibroblastlar, psammoma
cisimleri, distrofik kalsifikasyonlar ve odaklar halinde mononükleer
hücrelerden oluşan inflamatuar hücre infiltrasyonu dikkati çekti.
Sunulan olguda, kalsifiye fibröz tümörün klinik ve morfolojik özelliklerine
değinilmiş ve literatür eşliğinde tartışılmıştır.
Calcifying fibrous tumor is a rare benign lesion. It was
described for the first time in 1988 by Rosenthal et al. as
a “Childhood fibrous tumor with psammoma bodies”
in peripheral axial soft tissue1
and, in 1993, Fetsch et
al. re-named it as calcifying fibrous pseudotumor2
first, cases were diagnosed in subcutaneous and deep soft
tissues (extremities, trunk, inguinal and scrotal regions,
head and neck area). However, these tumors were gradually
reported in other areas such as the visceral regions (pleura,
mesentery, peritoneum), the oral cavity, and the adrenal
. Many cases in the literature have been described
as “calcifying fibrous pseudotumor”, however, we preferred
to use the term “calcifying fibrous tumor” in accordance
with World Health Organization (WHO) classification3
We herein present a case of calcifying fibrous tumor in the
right inguinal region extending to the anteromedial thigh
and discuss its clinical and morphological features with
regard to the literature.
A 21-year-old man presented with multiple masses which
were localized from the right inguinal region to the anteromedial thigh. He had gone to a hospital for treatment
at the age of six due to a painless mass in the right inguinal
region. The patient underwent an excisional biopsy for the
mass; however, he was not informed about the diagnosis.
Then, the masses recurred in the same region. There was
no family history. Physical examination revealed palpable,
nontender and semi-mobile multiple masses, from the right
inguinal region to the anteromedial thigh. The largest one
was about 3x3 cm (Figure 1A, B
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|Figure 1A,B: Clinical photograph of the palpable masses on the right thigh.
The patient's complete blood count and other routine biochemical
values were within normal limits. Ultrasonographically,
there were hypoechoic spherical-ovoid solid
masses with regular margins including punctate echogenicity
(Figure 2A). Magnetic resonance imaging (MRI)
of the subcutaneous fatty tissue showed hypointense solid
masses that were localized from the inguinal canal extending
to the anteromedial thigh, on T1-T2-weighted images
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|Figure 2: A) Ultrasonography demonstrating spherical-ovoid solid masses with regular margins including punctate echogenicity.
B) MR images of the lesions. C) Lymphoid aggregates and psammomatous calcification (H& E x50). D) Paucicellular and hyalinized
fibrosclerotic tissue (H&E x200).
We performed an excisional biopsy of one of the masses for
histopathological diagnosis. Macroscopically, the lesion was
a 3x3x2 cm, gray-white, regularly contoured, unencapsulated
solid mass. The cut surface was gritty in texture. Histopathologically, the tumor consisted of well circumscribed,
unencapsulated, paucicellular, hyalinized fibrosclerotic tissue.
There was neither cellular atypia nor mitotic figure in
the fibroblasts. Lymphoid aggregates consisting of lymphocytes
and plasma cells were also present. Psammomatous
and dystrophic calcifications were scattered throughout the
lesion. (Figure 2C, D). Immunohistochemically, spindle
cells showed diffuse positivity for vimentin (Figure 3) and
negativity for CD34, anaplastic lymphoma kinase (ALK),
S-100, cytokeratin, actin, desmin and CD117. The lesion
was diagnosed as calcifying fibrous tumor.
The patient was informed about his pathology and advised
to undergo total surgical excision. Unfortunately, he left
and never returned to complete the procedure and was
therefore lost to clinical follow up.
Calcifying fibrous tumor (CFT) is a rarely seen entity
that usually occurs in children and young adults, with
a slight increase in risk in women. Clinically, it appears
as a slow growing, nontender mass. Visceral symptoms
may occur depending on the localization of the lesions.
Radiographically, it is non-calcified and well circumscribed.
Punctate, thick or band-like calcifications can be seen on
Computed Tomography (CT). On MRI, the tumor may be
similar to fibromatoses3
. Their etiopathogenesis has not
been fully clarified yet. However, in the literature, there are
cases that may be related to trauma and Castleman's disease4-8
. CFT is a benign lesion and metastasis has not been
reported. Nascimento et al have observed local recurrences
in 3 of 10 patients in his study9
. In our case there was no
history of trauma, and the patient was otherwise healthy.
Macroscopically, CFT is well circumscribed and unencapsulated.
Its diameter ranges from 1 to 15 cm. In some cases
the tumor may have indistinct boundaries; hence infiltration
may be indistinguishable from the surrounding tissues.
On sectioning, the tumor reveals a solid, grey-whitish and
Histopathologically, it is characterized by abundant hyalinized
collagen tissue with lymphoplasmacytic mononuclear
inflammatory cell infiltrate and dystrophic - psammomatous
calcifications. Lymphoid aggregates can be found in
some cases. Although immunohistochemical analysis is not
necessary for diagnosis4, fibroblasts do express vimentin
and factor XIIIa and CD34 immunoexpression can be seen
on rare occasions10,11. Ultrastructural studies show that
calcifications occur as a result of cytoplasmic degeneration
in the fibroblasts6,9.
The histopathological features of CFTs are generally easily
recognizable from other reactive or benign neoplastic
lesions. Inflammatory myofibroblastic tumor (IMT),
reactive nodular fibrous pseudotumor (RNFP), nodular
fasciitis, desmoid fibromatosis, fibroma of tendon sheath
and calcifying aponeurotic fibroma might be considered in
the differential diagnosis12. IMT is composed of spindled
myofibroblasts, fibroblasts, and inflammatory cells. It is
more cellular and shows less hyalinization than CFT. In
addition, a polymorphic inflammatory cell infiltration and
occasional stromal calcification can be seen in IMT. Actin
and ALK immunoexpression is positive in IMT9,14. In
fact, some authors consider CFT to be a late sclerosing
stage of IMT4. In reactive nodular fibrous pseudotumor
(RNFP), actin, desmin and CD117 immunoexpression
is positive and CD34 is negative in addition to the
characteristic histopathological features of CFT. Desmoid
fibromatosis is poorly circumscribed with infiltration of
the surrounding structures and more cellular than CFT.
Nodular fasciitis is composed of plump myofibroblastic
cells in a myxoid stroma without calcification. Fibromas of
the tendon sheath generally occur in distal extremities such
as the thumb and the fingers. It is composed of paucicellular
spindled fibroblasts in a collagenous stroma and slit-like
vascular channels. Calcifying aponeurotic fibroma is usually
seen on the palms and soles of children. Its boundaries are
more irregular than CFT. Band-like calcification, chondroid
metaplasia and multinuclear giant cells can also be seen3,12.
There are few studies regarding cytogenetic abnormalities
in calcifying fibrous tumors. By using fluorescent in situ
hybridization, Hoffmann et al attempted to detect trisomy
7 and trisomy 8 which has been reported in other benign fibrous tumors. Due to the low signal intensity, they were
not able to complete the study.13. In another study,
Fukunaga et al found that CFT had a diploid DNA content
by flow cytometry14. Whether CFT is a true neoplasm
or a reactive process still remains unknown13-15. Future
molecular studies can be helpful for detecting the biologic
behavior of CFT.
As a result, CFT is a rare benign lesion and conservative
excision is sufficient to cure the patient. However, local
recurrence can be seen in some cases. Thus clinical followup
The authors thank Armağan Günal for immunohistochemical
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