Vol.2, No.7, 381-385 (2013) Case Reports in Clinical Medicine
Copyright © 2013 SciRes. OPEN ACCESS
Skeletal cystic angiomatosis: A rare cause of
unilateral lytic bone lesions
Lia Marques1*, Elisa Vedes2, Miguel Toscano Rico3
1Serviç o de Medici na III, Hospital Pulid o Valente, Centro Hospitalar Lisboa Norte, Lisboa, Portugal;
*Corresponding Author: marques.lia@gmail.com
2Unidade de Cuidados Intensivos, Hos p i t al da Luz, L i s b o a, Portugal
3Serviç o de Medicina, Hospit al de Santa Marta, Cen tro Hospitalar Lisboa Central, Lisboa, Portugal
Received 30 July 2013; revised 29 August 2013; accepted 10 September 2013
Copyright © 2013 Lia Marques et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Cystic angiomatosis is a rare, benign, multifocal
disorder of bone and viscera. Angiomatous de-
posits result in bone lysis and organ dysfunc-
tion. Bony cystic lesions occur in the axial and
proximal appendicular skeleton. Lesions may
cause bone pain or pathological fracture. Diag-
nosis is difficult, of exclusion and demands a
biopsy. The prognosis varies upon whether the
lesions are solely skeletal or there is visceral
involvement. A 71-year-old man reports increas-
ing symptoms of painful swelling in the right
thoracic wall for over a month. The swelling was
bony hard in consistency. Except for his bony
swelling, the patient’s physical examination was
within normal limits, as were all his laboratory
studies. X-ray imagery showed multicystic ex-
pansive lytic areas involving the right ribs. Com-
puterized tomography, magnetic resonance im-
agery and gallium bone scan revealed lytic le-
sions of multiple right ribs, and cervical, dorsal,
lumbar and sacrum iliac spine. A right rib biop sy
has shown a cystic formation with endothelial
walls. Five years later, the patient remained sta-
ble, with no clinical, laboratory or imagilogic
progression of disease and without visceral in-
volvement. This case is presented in his rarity
and differential diagnosis challenge.
Keywords: Cystic Angiomatosis; Bone Lytic
Lesions; Hi stiocitosis X
Cystic angiomatosis (CA) of bone is a rare pathologi-
cal entity characterized by multifocal hemangiomatous
and/or lymphangiomatous lesions of the skeleton with
possible visceral organ involvement, especially the spleen
[1-6]. It was first described in 1953 by Jullian E. Jacobs e
Paul Kimmelstiel [6,7].
The aetiology and exact pathogenic mechanism of the
disease are still unknown, but most authors believe th at it
is congenital malformation: a vascular hamartoma [1-3,
8,9]. It is characterized by substitution of bone tissue
with hamartomatous malformation of primitive vessels
disseminated and multifocal [1,10].
CA affects mainly young adults of the male gender
with half the cases being diagnosed around puberty [3].
Clinical signs are usually mild compared to radiological
changes, which justify the fact that most of the cases of
CA are revealed incidentally during radiological exami-
nation [1,3,11]. It is important to emphasise that a wide
range of affections exists, varying from a relatively mild
form in which the changes are limited to the skeleton,
with the possibility of a spontan eous reg r ession , to a very
severe form with extensive involvement of extraskeletal
tissues, leading at times to early death [9].
There has been a long standing confusion over the
terminology of this entity: a subgroup of patients pre-
senting with this condition present solely with skeletal
lesions. This subgroup on the follow up revealed spon-
taneous regression of skeletal lesions [1,3,6,12]. Hence,
the term “skeletal-extraskeletal angiomatosis” was coin-
ed for cases presenting with visceral manifestations [12].
Skeletal-extraskeletal angiomatosis is defined as a be-
nign vascular proliferation involving the medullary cav-
ity of bone and at least one other kind of tissue. It is also
known as CA [12].
CA radiological changes appear most frequently in the
axial skeleton in frequency order: ribs, pelvis, proximal
femora, proximal humerus, spine, skull, scapulas and
clavicles [3,13]. Hands and feet are rarely affected [3,13].
Radiologically the lesions appear as multiple osseous
L. Marques et al. / Case Reports in Clinical Medicine 2 (2013) 381-385
Copyright © 2013 SciRes. OPEN ACCESS
areas in the bone with well-defined edges surrounded by
a sclerotic ring with a honey-comb appearance [3]. The
intramedullary cysts are round or oval, and the cortex
may appear expanded without breaking unless a patho-
logical fracture occurs. Usually th ere is no periostitis [3].
Although the clinical and radiological features are
generally characteristic, the process is often mistaken
with other cystic lesions of the skeleton, especially his-
tiocytosis X (Table 1). Only a wide open biopsy, pref-
erably the partial excision of an affected rib establishes
the diagnosis [6,9].
Histologically angiomatosis consists of multiple di-
lated vascular canals, of cystic appearance and with walls
lined with a layer of flat endothelial cells. The cysts, in
various locations, may contain blood or proteinaceous
fluid, which often makes it impossible to determine if the
dilated canals are related to a haemangioma or a lym-
phangioma. Patients frequently have a lymphangioma-
tous lesion at one site and a typical haemangioma at an-
other [6].
In the soft tissues, the angiomas are usually located in
the neck or axillary regions [6], with some rare reports of
intrathoracic lung masses and gastro-intestinal angiodys-
plasia [8,9]. Visceral involvement reaches 60% - 70% of
all cases, more frequently in the lung, spleen, liver or
mediastinum [6].
Differentia l diagno sis of CA is presented in Table 1. A
definitive diagnosis of CA is difficult to establish and is
frequently one of exclusion [6]. It should be considered
in patients presenting with diffuse cystic lesions of the
skeleton on x-ray examination and with minimal or no
associated clinical or laboratory findings [6]. A bone bi-
opsy is needed, but is often unrevealing [3,6]. Typical
hystoimmunopathologic findings show the walls of the
cysts react to immunological markers of the histologic
endothelium (antigens related to factor VIII and CD 31).
Imagiologic methods are of limited value. Bone scan
underestimates the lesions extension. Even magnetic
resonance imagery (MRI) is not specific for CA. Indeed
Table 1. Differential diagnosis of cystic angiomatosis pre-
senting as multiple lytic bone lesions.
Differential diagnosis of cystic angiomatosis presenting
as multiple lytic bone lesions
Common causes Less frequent causes
Multiple myeloma Hystiocytosis X
Bone Metastasis Fibrous dysplasia
Lymphoma Gaucher’s disease
brown tumours Enchondromatosis
Amyloidosis Gorham disease
angiomas are variable in appearance on MRI: on T1
weighted images, the signal intensity varies from low to
high, depending on the amount of adipose tissue present
and the predominant tissue components—haemangioma
or lymphangioma. T2 weighted images usually show
areas of very high intensity corresponding to the vascular
or fluid components [3].
There is no specific treatment for CA. The treatment is
symptomatic. When there is osseous pain, analgesics
should be prescribed and if there is increased osseous
remodelling, bisphosphonates are recommended. Surgery
may be an option for bone lesions. Splenectomy is to be
considered if the spleen alone among the viscera is af-
fected [1,3 , 6 , 1 2 ].
CA has a variable prognosis. Some cases, with vis-
ceral involvement, produce death in childhood [3,14,15].
When the condition is confined to the skeleton, the
prognosis is good, as the lesions tend to remain static or
regress [1,3,6]. Usually the extend of soft tissue and vis-
ceral involvement dictates the morbidity and mortality
Up to 2008, no more than 200 cases of CA have been
described in the literature [1]. The authors present this
case report because of its extreme rarity and scientific
interest concerning differential diagnosis.
A 71-year-old man was admitted to an Internal Medi-
cine ward increasing symptoms of painfu l swelling in th e
ight posterior thoracic wall for over a month. The swell-
ing was bony hard in consistency and painful on deep
palpation. The pain was constant and worsed by any
movements, but didn’t wake up the patient during the
night. There was no paresthesia or muscular strength
alterations described. There was no anorexia or weight
lost, no fever, no urinary, respiratory or gastrointestinal
symptoms associated. There was no diaphoresis, no dys-
phonia, no dyspnea, no hemoptysis or other haemorrhage.
The patient had noticed the swelling for the first time
about 40 years before, but was never investigated and no
specific diagnosis was made.
Previews medical story revealed multiple traumas to
right ribs, smoke related chronic obstructive pulmonary
disease, arterial hypertension and diabetes mellitus. His
usual medications were an inalated beta 2 agonist, am-
lodipine and metformine. The patient had stopped smok-
ing nine years before the admission and had a daily al-
cohol consumption of 20 gr ethanol.
Physical examination revealed a good performance
status, apparent age was inferior to real age, no skin
lesions, no lymphadenophaty, digital hipocratism or
thyroid gland swelling were noticed. The right thoracic
posterior wall was swollen, weak, with motion limitation
and tender. There were no other inflammatory signs or
L. Marques et al. / Case Reports in Clinical Medicine 2 (2013) 381-385
Copyright © 2013 SciRes. OPEN ACCESS
mammary alterations. With no other abnormalities on
physical examination, namely the prostatic ex am was in-
The investigation s yielded the following results: white
blood cell count in the normal range, normocytic nor-
mochromic anaemia (Haemoglobin 11.5 g/dL), erythro-
cyte sedimentation rate (ESR) of 44 mm/first hour, lac-
tate dehydrogenase (LDH), alkaline phosphatase and
ionised calcium in the normal range. There were no renal,
hepatic or urinary abnormalities. X-ray imaging showed
multicystic expansive lytic areas involving the right ribs
(Figure 1).
Aetiological investigation yielded: seric proteinogram
with no abnormalities, no Bence Jones protein, parathy-
roid and thyroid hormones in the normal range. Skeletal
and skull films have shown no abnormalities. Thyroid,
testicular and prostatic ultrasounds were normal. Com-
puterized tomography (CT) has shown important lytic
lesions of multiple right ribs, mainly the second rib, ex-
pansive in nature, with cortical rupture and without le-
sions in the surrounding soft tissues. Lytic lesion of the
6th right rib with intra thoracic growth but without pleu-
ral and pulmonary involvement. The expanding mass
was multiloculated with loss of cortical definition in al-
most the entire length of bone with the lesion extending
into the adjacent soft tissues. There was no evidence of
calcification or host reaction (Figure 2). Spinal CT has
shown multiple lytic bone lesions lateralized to the right
(Figure 2). MRI of the spine, ribs and thorax: multiple
vertebral lesions with heterogeneous sign (central low
sign and peripheral high intensity sign on T1 and T2
weighted images). These lesions are of lytic nature and
are more prominent in D1 to D3. Multiple structural and
sign alterations over several right ribs with high intensity
in T2 weighted images, compatible with CA.
A gallium bone scan has shown osteolytic and os-
teoblastic lesions over multiple right ribs and dorsal
lumbar and sacrum iliac spine (Figure 3). Accentuated
bone fixation over right ribs (1st-6th), some of these
were pure lytic lesions. Other areas of anomalous fixa-
tion over 5th and 6th right condrocostal articulations,
12th dorsal vertebra, 1st and 2nd lumbar vertebrae and
right sacroiliac articulation (Fig u re 3).
CT guided right rib biopsy: bloody liquid with low
cellularity sediment with some histiocytes, lymphocytes
and neutrophils, cystic formation with endothelial walls
and without neoplastic cells. There was one giant multi-
nucleated cell (Figure 4). A diagnosis of CA was estab-
The patient was reassured, discharged, treated with
nonsteroidal anti-inflammatory drugs (NSAIDs) for pain
control and kept under surveillance. Five years later the
patient remained stable. Laboratory evaluation has
shown modest alkaline phosphatase elevation with no
Figure 1. First thoracic radiogram.
Figure 2. CT images document osteolytic lesions in right rib
and second cervical vertebra.
Figure 3. Bone scan with multiple right osteolytic lesions.
Figure 4. Right rib histopatologic characteristics—cystic for-
mation with endothelial walls and one giant multi-nucleated cell.
other abnormalities. CT and bone scan findings has
shown no progression or periosteal reaction (Figure 5).
L. Marques et al. / Case Reports in Clinical Medicine 2 (2013) 381-385
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Figure 5. CT and bone scan five years after the diagnosis was
The patient stared therapeutic bisphosphonates with pain
control and alkaline phosphatase normalization.
The patient remains stable, asymptomatic except for
occasional anterior chest pain without systemic disease
or visceral involvement by angiomatous lesions.
AC of the bone is a rare entity and its isolated sk eletal
involvement is even rarer [6,16]. The osseous lesions
produce symptoms when a pathological fracture occurs,
but as in the patient reported here, bone pain may exist
without fracture [1,3]. The interesting feature of this case
is the presence of multiple, well defined osseous lytic
lesions involving the axial skeleton, that were unilateral.
This bizarre appearance made the diagnosis a real chal-
The differential diagnosis initially considered for this
patient was that of multiple lytic lesion of the skeleton.
The authors reinforce that one of the most important first
steps in deriving a differential diagnosis when evaluating
osteolytic lesions is to keep in mind the patient’s age [6 ].
Considering the presented 71-year-old patient, the most
frequent causes to be considered were metastasis and
multiple myeloma (MM). These two diseases produce
involvement of the flat bones such as the pelvis with a
well-defined profile, but without reactional sclerosis or
radiodense matrix inside. In MM, the lesions are usually
the same size, that was not the case of the presented pa-
tient, and there were no laborato ry abnormalities suppor-
tive of this diagnosis.
Actually, considering medical story and X-ray findings
alone, in this case the diagnosis hypothesis was divided
into two main groups: neoplastic and non neoplastic le-
sions. The first group had in favour, apart from the pa-
tient’s age, raised ESR and anaemia. Favouring a benign
aetiology for these osseous lesions, there were the long-
time course of the swelling and the patient excellent
physical status. The differential diagnoses considered in
the benign group were primary and secondary hyper-
parathyroidism, polvostotic fibrous dysplasia, Gorham
disease, histiocytosis X and mastocytosis. However, the
clinical course, the physical examination, the laboratory
findings and the pathological specimen, all helped to ex-
clude these possible diagnoses. Of these, the most chal-
lenging one is histiocytosis X (multiple or disseminated
histiocytic granuloma) which produces cysts surrounded
by sclerosis in the pelvis, skull, vertebrae, and long
bones, having a similar osseous distribution as CA, but
with the skull bone s being more often affected. The main
pathological difference between CA and histocitosis X is
that in the last one there is usually a periosteal reaction,
typically absent in CA. The diagnosis must always be
confirmed by histological examination of the affected
bone, a rib or fibula being the most convenient for this
purpose [3]. In Gorham disease (disappearing bone dis-
ease), the histology of bone lesions is very similar to
those of CA, but it usually affects only one bone and
produces massive bone destruction, which was not the
case of this patient.
This case is similar to some of the cases of CA re-
ported in the literature except for the late age of onset
[6,7,9,16]. The authors highligh t that the rib swelling was
noticed 40 years before, at the age of 31, what is more
concordant with the classic age of CA. But previous
evaluation for this osseous swelling was not performed
and the patient used to relate it to previous trauma.
The roentgen features in this case were also concor-
dant with the literature, being usually lytic lesions, in
sclerotic areas, expanding cysts, and generalized skeletal
lucency [9,16]. The pathologic findings are also in agree-
ment with the literature for CA and usua lly are described
as a conglomeration of vascular channels in some areas,
and other areas showed empty cysts with fragments of
endothelial lining [9,16].
There is no specific treatment, though osseous lesions
can regress spontaneously. It is important to be familiar-
ized with the clinical, radiological and pathological fea-
tures of CA in order to avoid any unnecessary treatment
Concerning prognosis for the presented patient, there
is no compromised prognosis in the face of the patient’s
clinical stability, without lesions progression or visceral
involvement after a five-year-surveillance-period. In this
case, spontaneous regression of the cystic bone lesion
may occur, as happened in some reported cases [1,3,12].
In conclusion, the authors remember this rare clinical
entity and, that despite the rarity of the disease, CA
should be considered as a differential diagnosis in a pa-
tient presenting with skeletal diffuse cystic lesions with
minimal or no associated with clinical or laboratory find-
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