The Turkish Journal of Pediatrics
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Osseous presentation of Hodgkin's disease: a case report and review of the literature
R. Doğan Köseoğlu1, Atilla Şenaylı2, Ünal Bıçakçı2, Nurper Onuk-Filiz1, Taner Sezer3, Tiraje Celkan4
1Departments of Pathology, Gaziosmanpaşa University Faculty of Medicine, Tokat, Turkey
2Departments of Pediatric Surgery, Gaziosmanpaşa University Faculty of Medicine, Tokat, Turkey
3Departments of Pediatrics, Gaziosmanpaşa University Faculty of Medicine, Tokat, Turkey
4Department of Pediatrics, İstanbul University, İstanbul Faculty of Medicine, İstanbul, Turkey
|Köseoğlu RD, Şenaylı A, Bıçakçı Ü, Onuk-Filiz N, Sezer T, Celkan
T. Osseous presentation of Hodgkin's disease: a case report and review of
the literature. Turk J Pediatr 2007; 49: 218-222.|
The bone involvement in the later stages of Hodgkin's disease is an expected
phenomenon, but it is very rare in early stages of the disease. About 49 cases
of Hodgkin's disease presenting with bone involvement have been reported
in the literature. We reported a 14-year-old boy initially evaluated with
pain localized at the left ilium. Although all the radiological examinations
suggested an osseous anomaly, histopathologic evaluation of the pelvic
lymphadenopathies provided definite diagnosis of the disease. We discuss the
possible differential diseases and review the literature regarding the osseous
presentation of Hodgkin's disease.
Hodgkin's disease, osseous presentation
|Hodgkin's disease usually presents with lymph
node or solid organ involvement, but bone is
another possible origin of the disease. In the
literature, to our knowledge, only 49 cases of
Hodgkin's disease with bone involvement at
the very beginning of the disease have been
reported, though bone involvement becomes
increasingly prevalent at the later stages[1,2].
Here, we report a case of Hodgkin's disease
presenting with bone involvement in a 14-
|Case Presentation |
|A 14-year-old boy was referred to the Pediatric
Surgery Department of Gaziosmanpasa
University Hospital with paroxysmal pain and
growing mass in his left hip for two months.
There was no family history of lymphoma or
another tumor disease. He had no systemic
symptoms or weight loss. In the physical
examination, there was no lymphadenopathy or
any mass palpated on the body except in the
left iliac region. Blood chemistry studies were
unremarkable, except for sedimentation rate
of 81 mm per hour. X-ray and computerized
tomography (CT) scan of the pelvis showed a
destruction in the left ilium with a soft mass protruding through the pelvis. Also, in the paraaortic
region, multiple lymphadenopathies were
detected in CT scan. Technetium scintigraphy
revealed hyperemic lesions with lytic and
sclerotic areas in ilium.|
Frozen examination of bone biopsy performed
during the operation was not diagnostic
and showed nonspecific inflammatory cell
infiltration with extensive myxoid stromal
changes and mesenchymal cells in atypical
appearance supporting a soft tissue tumor
in the iliac bone. Because of the nonspecific
diagnosis in frozen examination, the operation
was continued to excise the lymph nodes
from the pelvic and para-aortic region.
Histopathological examination of lymph
nodes revealed polymorphic inflammatory
cell infiltration separated by fibrous bands.
Inflammatory infiltration was composed
of reactive lymphocytes, eosinophils, and
plasmocytes. In this reactive cellular background,
scattered multilobulated and mononuclear
Reed-Sternberg cells and lacunary cells were
detected (Fig. 1). Reactive cell infiltration and
Reed-Sternberg cells were also observed in bone
biopsy (Fig. 1). The monoclonal antibodies against CD30, CD15 (LeuM1), CD3, CD20, and CD45 in both lymph node and bone tissue
were used for immunohistochemical analysis.
Reed-Sternberg cells showed positive staining
for CD30 and CD15; cells were negative for
CD45, CD3 and CD20. Histopathological and
immunohistochemical features of lymph nodes
and iliac bone were concordant with Hodgkin's
disease, nodular sclerosis type.
| ||Fig. 1: A: Lymph node demonstrates effacement of the
nodal architecture by cellular infiltrate (hematoxylin &
eosin-HE, x10). B: The cellular infiltration consisting of
mixed inflammatory cells and Reed-Sternberg cells in
lymph node (HE, x40). C: Bone biopsy demonstrating a
destructive cellular infiltrate (HE, x20).|
The patient has been treated with adriamycin,
bleomycin, vinblastine, dacarbazine (ABVD)
chemotherapy for one year. In addition,
radiotherapy has been performed for the last
three months. The bone lesion regressed and
lymphadenopathy was not present in the last
follow-up. In the last physical examination,
general condition of the patient was good and
the patient was in remission.
|Bone involvement in Hodgkin's disease occurs in
9% to 35% of the cases. The bone involvement
is seen particularly in the later stages, but very
rarely at the time of presentation[1-4]. In the
literature, 49 cases of Hodgkin's disease with
bone involvement were reported at the time
of initial diagnosis (Table I). The majority of these cases also had lymph nodes and other
organ involvement with bone lesions at staging
workup. Age of these cases ranged from 5 to
85 years and the average age was 37.6 years;
most cases were adults. Seven cases were
determined in the age range of 5-15 years, not
including our case. Twenty-six (53%) of the
cases were male and 23 (47%) were female.
The bones most involved at the initial diagnosis
were vertebrae, pelvic bones, femur, humerus,
sternum, tibia, scapula, mandible and rib.|
| ||Table I: List of the 50 Patients Reported in the Literature|
In Hodgkin's disease, radiographic evidence of
bone involvement is seen in 10-15% of cases. The bone lesions of Hodgkin's disease may be
lytic, sclerotic or mixed. Lytic lesions are more
common than sclerotic lesions. As the bone
involvement may be via hematogenous or direct
invasion, the type of spreading can be related
with the type of bone lesion. Lytic lesions
usually support hematogenous dissemination,
whereas sclerotic lesions support direct
invasion from adjacent lymph nodes. In the
literature, radiological examinations revealing
bone involvement at the initial diagnosis were
present for 32 of 49 cases. Fifteen of 32 cases
(46.8%) had lytic, nine cases (28.1%) had
sclerotic, and three cases (9.4%) had mixed
lesions. One case (3.1%) had periosteal reaction
in his X-ray examination. Radiographs of
four cases (12.5%) were normal. Radiological
features of lesions were not reported for 17
of 49 cases. In the retrospective study of
Ostrowski et al., although the radiological
features of the cases were analyzed, the details
were not given individually. They reported in
their series that osteolytic, osteosclerotic and
mixed lesions were present in 24%, 24% and
46% of the cases, respectively.
The histopathological diagnosis could be
obtained in only 22 of 49 cases. Ten of them
were nodular sclerosis type Hodgkin's disease
and nine cases were diagnosed as mixed
cellular type Hodgkin's disease. Only three
cases were reported as lymphocyte depletion
type Hodgkin's disease. Histological subtypes of Hodgkin's disease in 27 cases were not certain.
Thus, nodular sclerosis and mixed cellular
subtypes were determined at about equal
proportions in the literature. We determined
nodular sclerosis type Hodgkin's disease
in our case.
The differential diagnosis of Hodgkin's disease
presenting with primary or secondary bone
involvement is very important. Anaplastic
lymphoma or peripheral T-cell lymphoma
may show similar histologic picture, but
non-Hodgkin's osseous lymphomas, especially
peripheral T-cell lymphomas, may cause
fibrosis or sclerosis in bone marrow and bone.
Furthermore, all these lymphomas also have
a significant large cell component. Another
entity causing a similar diagnostic dilemma is
malignant fibrous histiocytoma of the bone.
Osteomyelitis is an important disease in
the differential diagnosis. Both clinical and
radiological features of osteomyelitis are very
similar to osseous Hodgkin's disease. In
osteomyelitis, small biopsies are not helpful
in the differential diagnosis. In the literature,
we determined that many cases of Hodgkin's
disease with bone involvement were diagnosed as osteomyelitis at the initial evaluation[1,2,5].
The other entities that should be regarded
in the differential diagnosis are eosinophilic
granuloma, chondrosarcoma, Paget's disease
and primary sarcoma of bone[1-3,5].
Most of the cases previously reported in the
literature were diagnosed before the utilization
of immunohistochemical studies. Therefore,
diagnosis of Hodgkin's disease may be suspect
in these cases. The immunophenotypes of
Reed-Sternberg cells and variants are similar
for nodular sclerosis, mixed cellular and
lymphocyte-depletion types. Nodular sclerosis
is the most frequent histological type in
Hodgkin's disease with bone involvement,
followed by mixed cellular type. For these
reasons, immunohistochemical analysis should
be performed to eliminate other diagnostic
possibilities and to confirm the diagnosis of
bone-involving Hodgkin's disease.
Whenever malignant lymphoid cell infiltration
is determined in bone, it is important to
decide whether this infiltration is primary
or secondary. Primary lymphoma of bone
is extremely rare. It constitutes 3-8% of
primary malignant bone tumors. Most of the primary bone lymphomas are non-Hodgkin's
lymphomas; primary Hodgkin's disease of
bone is seldom encountered[1,5]. In the reviewed
literature, only seven cases had been reported
as primary osseous Hodgkin's disease[1,2,7]. Four
of these cases had been reported before 1970. Therefore, the diagnosis of these cases should
be cautiously regarded due to inadequate
clinical evaluation and staging procedures in
the past. No organ or lymph node involvement
within six months from initial diagnosis must
be determined for the diagnosis of “primary
osseous Hodgkin's disease”.
As the chemotherapy in Hodgkin's disease with
bone involvement has an important effect on the
prognosis, survival is better than expected[1,5].
Here, we report Hodgkin's disease presenting
with bone involvement in a 14-year-old boy
who had pain complaint in his left hip region
for two months. In the preoperative period, the
case was interpreted as primary bone tumor
based on clinical and radiological evaluation.
Intraoperative frozen study of iliac bone biopsy
was not diagnostic. The diagnosis could be
reached by histopathological investigation of
pelvic and para-aortic lymph nodes. In the
histopathological examination, nodular sclerosis
type Hodgkin's disease was determined in our
case. Radiological bone lesions of Hodgkin's
disease are mostly lytic in character but they
may also be sclerotic or mixed. Radiological
findings of bone lesions were mixed in our
case. Therefore, Hodgkin's disease should be
remembered in the differential diagnosis of
lytic lesions involving especially vertebrae and
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