The Turkish Journal of Pediatrics 2007; 49: 307-311Case
Successful treatment of retroperitoneal giant cell-type
malignant fibrous histiocytoma in a 5-year-old boy
Kudret Çağlar1, Sabiha Güngör1, Sezin Akansoy1, Ümit Sakallı2
Diclehan Orhan3, Melda Çağlar3
Departments of 1Pediatrics and 2Pediatric Surgery, Dr. Burhan Nalbantoğlu State Hospital, Nicosia, North Cyprus,
and 3Pathology Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
SUMMARY: Çağlar K, Güngör S, Akansoy S, Sakallı Ü, Orhan D, Çağlar
M. Successful treatment of retroperitoneal giant cell-type malignant fibrous
histiocytoma in a 5-year-old boy. Turk J Pediatr 2007; 49: 307-311.
Malignant fibrous histiocytoma, usually seen in patients older than 10 years, is
an aggressive soft-tissue sarcoma occurring mostly in the extremities and the
trunk, but it is extremely rare in children. We report the clinical, radiological
and pathologic features of a five-year-old boy who was diagnosed as a
retroperitoneally originated malignant fibrous histiocytoma. The patient with
unresectable mass was successfully treated with multidisciplinary approach,
with chemotherapy, surgery and radiotherapy, by using combined chemotherapy
consisting of vincristine, cisplatinum, adriamycin, cyclophosphamide,
actinomycin D and dacarbazine.
Key words: giant cell-type malignant fibrous histiocytoma, retroperitoneal, children.
Malignant fibrous histiocytoma (MFH) was
first introduced by Ozzello et al.1 in 1963,
and then more details of its histopathological
appearance were described by O’Brien, Stout
and Lattes1,2. Soft tissue sarcomas, malignant
tumors of extra-skeletal connective tissues,
which consist of rhabdomyosarcomas and non-
rhabdomyosarcomas, are relatively rare in the
childhood period1-5. Rhabdomyosarcoma is the
most common soft tissue sarcoma in the childhood
period4. Non-rhabdomyosarcoma soft tissue
sarcomas (NRSTS), such as synovial cell sarcomas,
MFH, fibrosarcomas, and malignant peripheral
nerve sheath tumors, are rare and heterogeneous
tumors sharing some biologic characteristics but
differing in histology, and account for only 3%
of all childhood malignancies3-5.
Malignant fibrous histiocytoma of soft tissue
origin, with the possibility of occurrence in the
retroperitoneal region, is extremely rare in the
childhood period1-5. Because this tumor is rare
in the pediatric population, many treatment
modalities of MFH are extrapolated from
the adult experience1-6. The approaches to
treatment of the disease and prognostic factors
that affect survival in pediatric patients have
not been well described in the literature1-6.
In this report, we describe clinical, pathological
findings and successful treatment of a five-year-
old boy presented with retroperitoneal huge
mass consisting of necrotic and calcified areas
who was diagnosed as a giant cell-type MFH.
A five-year-old boy with a two-day history
of painless abdominal mass was referred to
our department with no other symptoms. On
physical examination, an abdominal mass with
a diameter of 15x15 cm was palpated on the
left side of the umbilicus extending to the
pelvic aperture. No other remarkable findings
on the physical examination were detected.
Laboratory studies revealed a white blood
cell count of 13,400/mm³, a hemoglobin
level of 9.6 g/dl and a platelet count of
486,000/mm³. Renal and liver function tests
were within the normal limits, with a creatinine
clearance of 113 ml/min/m². Diagnostic
studies including radiographs of the lung and
skeletal survey showed no evidence of disease.
Abdominal ultrasonography demonstrated a
solid mass 113x99 mm in size having cystic
areas located on the whole left side of the
abdomen not involving surrounding visceral
organs. Abdominal computed tomography (CT)
demonstrated an 82x81x92 mm heterogeneous
mass, which caused a lytic lesion on the
corpus of L3 vertebra and consisted of calcified
and necrotic areas, originating from the left
retroperitoneal region and extending through
the right side of the abdomen without
infiltration of the left kidney or adrenal gland
(Fig. 1). No abnormality was detected on
the thorax tomography. Since the mass was
unresectable, a tru cut biopsy was done for
diagnosis. Bone marrow aspiration and biopsy
revealed no infiltration and on the pathological
evaluation no abnormality was found. Due to
the limitation of the tru cut biopsy specimen,
diagnosis could not be determined clearly
so incisional biopsy was performed. The
pathologic examination (Fig. 2a) revealed a
tumor composed of a mixture of histiocytes,
fibroblasts, foamy histiocytes and giant cells.
Fibroblasts and histiocytes showed mild
pleomorphism and mitotic activity. Two types
of giant cells were observed: osteoclast-like
giant cells and the malignant tumor giant
cells with enlarged and hyperchromatic nuclei.
There were no osteoid foci. No lipoblast was
observed. The tumor was infiltrating the
mature bone tissue at the periphery of the
lesion. Immunohistochemically, tumor cells
were negative for cytokeratin, HMB45, desmin,
alpha-smooth muscle actin, myoglobin, mdm2
and cdk4. Positivity for S-100 and CD68 was
observed in histiocytes and giant cells. Tumor
cells were diffusely positive for vimentin7-10.
With the diagnosis of giant cell-type MFH,
he was put on a combined chemotherapy
started with an induction period consisting
of vincristine (1.5 mg/m², day 1), cisp-
latinum (90 mg/m², day 1), adriamycin
(30 mg/m², days 1,2) and cyclophosphamide
(10 mg/kg, days 1,2,3) every three weeks and
then maintenance period including vincristine
(1.5 mg/m², day 1), cyclophosphamide
(10 mg/kg, days 1,2,3), and actinomycin
D (15 γ/kg, days 1,2,3,4) alternating with
cisplatinum (90 mg/m², day 1), adriamycin
(45 mg/m², day 1) and dacarbazine (400 mg//m²,
days 1, 2) every four weeks2-4. At the end of the
induction period (four courses), he showed
a minimal response with only 20% decrease
in size and enlargement of necrotic areas of
the tumor with no difference in the vertebral
lytic lesion. Gross total resection of abdominal
mass and removal of a part of vertebral corpus
with lytic lesion were done. On the evaluation
of the CT and F-18FDG whole body positron
emission tomography (PET), a residual disease
on the L3 vertebra was detected with absence
of abdominal mass (Fig. 3a). On the further
Fig. 1. Computerized tomography findings on admission.
308 Çağlar K, et al The Turkish Journal of Pediatrics • July - September 2007
Volume 49 • Number 3 Malignant Fibrous Histiocytoma in a 5-Year-Old Boy 309
pathologic examination (Fig. 2b), tumor tissue
was mostly necrotic and calcified. The non-
necrotic areas of the tumor showed similar
features with the incisional biopsy material.
The patient received radiotherapy on the left
hemi-abdomen with a total dose of 4000 cGy
and on the local area of L2-L3-L4 with an
additional dose of 1040 cGy. After 12 courses of
combined chemotherapy (4 courses of induction
period and 8 course of maintenance period)
completed in one year, no viable residual
lesion was detected on CT or PET (Fig. 3b).
He was off treatment for 20 months without
any evidence of disease.
Malignant fibrous histiocytoma, with an
incidence of 2-7% of all soft tissue sarcomas
in the pediatric population, is mostly
reported in the fifth and sixth decades as an
adult malignancy in the literature1-14. Male
predominance with a 2 to 1 male to female
ratio has been reported1-2. MFH has a typical
origin of subcutis and deep dermal layer of the
body with a most frequent occurrence in the
extremities followed by the retroperitoneum
in adult age, in contrast to the head and neck
regions in pediatric patients. However, data on
the etiology of MFH is limited; exposure to
Fig. 3a-b. Positron emission tomography (PET) findings of the patient.
Fig. 2a. Tumor composed of giant cells, fibroblasts and
histiocytes (hematoxylin and eosin-HE, X200).
Fig. 2b. Tumor with necrotic and calcified areas with
numerous giant cells after chemotherapy (HE X100).
ionizing radiation, xeroderma pigmentosum,
burn scars, scarring dermatosis, chronic ulcers
and previously diagnosed hematopoietic diseases
including Hodgkin’s lymphoma, melanoma
multiplex and malignant histiocytosis have
been described as etiologic factors of MFH in
At the time of the diagnosis, MFH is mostly
manifested as an enlarging painless soft
tissue mass and is typically larger than 5
cm in diameter1,2. Most lesions occur in the
extremities, with the possibility of remaining
painless for several years, or in the retro-
peritoneum with constitutional symptoms
including fever, malaise and weight loss1,2,13.
Retroperitoneal disease may cause expansion,
distension, hernia, varicocele with a mass
effect and hormonal abnormalities such as
hyperglycemia and hyperinsulinemia as a
result of insulin-like substance produced by
The histogenetic origin of MFH is supposed to
be either primitive mesenchymal stem cells or
primitive fibroblastic or histiocytic cells1,2,5,14.
MFH can be confused with pleomorphic
rhabdomyosarcoma, extrarenal rhabdoid
tumor, liposarcoma, large cell lymphoma and
anaplastic carcinoma1,7,10. It is very difficult
to differentiate the subtypes of MFH and
distinguish it from other malignant tumors7,8.
Specially five histologic subtypes of MFH are
defined, including storiform-pleomorphic type
as the most common form, myxoid type,
giant cell type with usually multinodular
morphology, inflammatory type as frequently
retroperitoneally originated, and angiomatoid
type, which is mainly seen in children and
young adults located more superficially than
It is reported that a retroperitoneal poorly
differentiated sarcoma resembling MFH or
fibrosarcoma should be investigated for a
liposarcoma component histologically and for
immunohistochemical positivity for mdm2 and
cdk4 to exclude a diagnosis of dedifferentiated
liposarcoma9,10. We eliminated the diagnosis of
dedifferentiated liposarcoma as the tumor did
not contain lipoblasts and the tumor cells were
negative for mdm2 and cdk4. The possibility
of rhabdomyosarcoma was also excluded as
the skeletal muscle markers were negative
Metastatic disease is demonstrated in 0-43%
of childhood MFH and 5-41% of adult MFH4.
The highest metastasis ratio among the MFH
subtypes is 50% in giant cell subtypes6. The
most common site of metastasis is lung (90%),
followed by the lymph nodes (35%), bones
(8%), and liver (1%), although spreading
to peritoneum, brain and other organs can
be seen1-6,11-14. The survival rate of illness
depends on some prognostic factors such as
deepness, which is still controversial, size
and grade of the tumor, localization, age of
patient, adequacy of surgical margins and also
cell types of the tumor1-6,11-14. Local recurrence
is common in MFH due to the behavior of
the tumor growing along facial planes, at a
rate of 27% that increases to 43% if there
is skeletal muscle involvement1-6,11-14. The
five-year survival rates can vary depending on
tumor size, such as smaller than 5 cm with
82%, 5-10 cm with 63% and above 10 cm
with 51%1-6,11-14. According to the reported
survival rates, children with MFH have a better
prognosis than adults2,4.
In many cases, the pediatric counterparts have
a different clinical behavior and outcome.
Because of the limited number of MFH cases
in childhood, the approach to the treatment is
extrapolated from the adult experience.
Although a high rate of local recurrence has
been reported, the most acceptable treatment
for MFH is a wide surgical excision1,2,6,13,14. It
is demonstrated that lymph node involvement
of the disease is very rare, so regional nodal
dissection is not recommended except in the
case of enlarged lymph nodes on examination
or scanning2,5,6,13,14. In the literature, the
effectiveness of adjuvant chemotherapy and
radiation therapy in pediatric patients is not
described clearly. As a result, their use is
recommended in some conditions such as
metastatic disease, disease of high metastatic
potential or in large unresectable tumors2,5,6,13.
Chemotherapeutic agents including vincristine,
actinomycin D and cyclophosphamide with or
without doxorubicin are mostly used in the
treatment of the disease. Use of cisplatinum,
ifosfamide, dacarbazine and VP-16 agents has
also been reported in the treatment of the
disease2,3,5,14. Moderate doses of radiation
therapy have been revealed to improve the local
control of inoperable or unresectable tumors
and incompletely excised tumors.
310 Çağlar K, et al The Turkish Journal of Pediatrics • July - September 2007
Volume 49 • Number 3 Malignant Fibrous Histiocytoma in a 5-Year-Old Boy 311
In our case, unresectable disease presenting
with painless abdominal mass originating from
the retroperitoneum in a pediatric patient
without any metastasis was successfully
treated with multidisciplinary approach. As
described in the literature, after the chemo
reduction of the disease, wide local excision
and radiotherapy were done to decrease the
local recurrence probability of the disease2-4.
The patient is now 20 months into his follow-
up for recurrence or metastasis of the disease.
At the last follow-up he had no evidence of
local recurrence or metastasis of the disease
on the conventional methods and PET.
We suggest that abdominal tumors originating
from the retroperitoneal region in pediatric
patients should be evaluated more carefully.
1. Repassy D, Csata S, Sterlik G, Hazslinszky P.
Retroperitoneal malignant fibrous histiocytoma. Int
Urol Nephrol 1999; 31: 303-311.
2. Köseoğlu V, Kürekçi AE, Kul M, Öztürk H, Günhan O,
Özcan O. Malignant fibrous histiocytoma in a child. A
case report and review of the literature. Turk J Pediatr
2000; 42: 72-75.
3. Pappo AS, Rao BN, Jenkins JJ, et al. Metastatic
nonrhabdomyosarcomatous soft-tissue sarcomas in
children and adolescents: the St. Jude Children’s
Research Hospital experience. Med Pediatr Oncol 1999;
4. Daw NC, Billups CA, Pappo AS, et al. Malignant
fibrous histiocytoma and other fibrohistiocytic tumors
in pediatric patients: the St. Jude Children’s Research
Hospital experience. Cancer 2003; 97: 2839-2847.
5. Belal A, Kandil A, Allam A, et al. Malignant fibrous
histiocytoma: a retrospective study of 109 cases. Am
J Clin Oncol 2002; 25: 16-22.
6. Rothman AE, Lowitt MH, Pfau RG. Pediatric cutaneous
malignant fibrous histiocytoma. J Am Acad Dermatol
2000; 42: 371-373.
7. Brooks JS. Disorders of soft tissue. In: Mills SE (ed).
Sternberg’s Diagnostic Surgical Pathology (4th ed),
Vol. 1. Philadelphia: Lippincott, Williams & Wilkins;
8. Oliveira AM, Dei Tos AP, Fletcher CD, Naschimento
AG. Primary giant cell tumor of soft tissues - a study
of 22 cases. Am J Surg Pathol 2000; 24: 248-256.
9. Gebhard S, Coindre JN, Michels JJ. Pleomorphic
liposarcoma: clinicopathologic, immunohistochemical
and follow-up analysis of 63 cases: a study from the
French Federation of Cancer Centers Sarcoma Group.
Am J Surg Pathol 2002; 26: 601-616.
10. Coindre JM, Mariani OM, Chibon F, et al. Most
malignant fibrous histiocytomas developed in the
retroperitoneum are dedifferentiated liposarcomas: a
review of 25 cases initially diagnosed as malignant
fibrous histiocytoma. Mod Pathol 2003; 16: 256-262.
11. Barthel H, Wilson H, Collingridge DR, et al. In vivo
evaluation of [18F]fluoroetanidazole as a new marker
for imaging tumour hypoxia with positron emission
tomography. Br J Cancer 2004; 90: 2232-2242.
12. Jadvar H, Fischman AJ. Evaluation of rare tumors
with [F-18] fluorodeoxyglucose positron emission
tomography. Clin Positron Imaging 1999; 2: 153-158.
13. Gambini C, Haupt R, Rongioletti F. Angiomatoid
(malignant) fibrous histiocytoma as a second tumour
in a child with neuroblastoma. Br J Dermatol 2000;
14. Shah SJ, Craver RD, Yu LC. Primary malignant fibrous
histiocytoma of the lung in a child: a case report and
review of literature. Pediatr Hematol Oncol 1996;