Usual Tumour at an Unusual
location – Solitary Sporadic Scapular Sessile Osteochondroma
Neeraja Myreddy1, K. P. Varalakshmi2, P. Sravani3, B. Chaitanya4
1Neeraja Myreddy, M.D,2K.P.Varalakshmi, M.D,3P.
Sravani, M.D,4B. Chaitanya, M.D. All are affiliated to Dr. NTR
University of Health sciences, Vijayawada, Andhra Pradesh, India.
Address for
correspondence: Dr B Chaitanya, Email:
bharadwaj.chaitanya@yahoo.com
Abstract
Osteochondromas is one of the common bone tumour account for 35-46% of
cases. These are most commonly seen on the metaphysis of a long bone,
but they can arise from any bone which is preformed from cartilage and
also involves flat bones like ilium and scapula. Scapular involvement
is noted in 3 – 4.6 % of cases and patients usually are
affected by winging of scapula. It is common in young patients, usually
below 30 years of age, with a ratio of males to females of 2:1. We
present a male patient diagnosed with solitary asymptomatic scapular
osteochondroma with review of literature.
Keywords:
Osteochondroma, Scapula, Cartilage.
Manuscript received:
10th July 2014, Reviewed:
12th July 2014
Author Corrected:
25th July 2014, Accepted
for Publication: 11st August 2014
Introduction
Osteochondroma is a cartilage capped bony projection arising on the
external surface of bone containing a marrow cavity that is continuous
with that of the underlying bone. Synonymously known as exostosis, it
is most commonly located in the distal femoral, proximal tibial or
proximal humeral metaphysis of adolescents and young male adults [1].
Osteochondromas generally arise in bones preformed by cartilage.
Involvement of flat bones is less common with the ilium and scapula
accounting for most of the cases. Majority are asymptomatic per se
while a few are known to cause secondary complications. They may be
sporadic, solitary or multiple and inherited due to germline mutations
of EXT 1 and EXT 2 tumour suppressor genes [2]. We present a classical
case of solitary sporadic sessile asymptomatic osteochondroma in a
young male.
Case
Report
A 22 year old male patient presented to the Orthopaedics department
with complaints of a painless swelling over the scapula of six months
duration. The swelling progressed in size gradually. There was no
history of trauma and associated symptoms or other swelling elsewhere.
No rapid increase in size was observed. Family history was
insignificant. On examination a hard, irregular bony swelling of size 4
x 4 cm was palpable on the dorsum of right scapula [Figure 1]. The
swelling was fixed to the underlying bone. Skin over the swelling was
normal and freely mobile. There was no localized tenderness. There were
no other musculo‑skeletal abnormalities. Laboratory data was within
normal limits. X‑ray showed a sessile, cauliflower‑like calcified
growth on postero medial aspect of scapula. Computed tomography (CT)
scan described a well defined lytic lesion with irregular border and
medullary continuity measuring 4.1 x 2.3 x 4 cm noted arising from
posterior aspect of medial border of scapula just inferior to scapular
spine. There is a thin rim of cartilaginous cap surrounding the lesion
[Figure 2]. A clinical diagnosis of osteochondroma was considered and
surgery planned. Wide local excision of the lesion was done under T1,
T2, T3 paravertebral block. The tumour was sessile and friable and so
could not be removed in toto as a single mass. Grossly, the lesion was
friable, cartilage capped. All the tissue bits obtained were processed
and H & E sections revealed a cartilaginous cap with
haphazardly arranged chondrocytes with benign morphology and underlying
bony trabeculae with interspersed fatty, hematopoietic marrow [Figure
3]. A histopathological diagnosis of osteochondroma was rendered.
Follow up of the patient so far showed no recurrences.
Figure I: Clinical
photograph showing right scapular swelling in a young patient.
Figure II: Axial
CT film showing irregular sessile lesion on posteromedial aspect of
right scapula
Figure III:
Microscopic photograph showing cartilage capped lesion with marrow
elements within bony spicules, H & E,
Discussion
Osteochondromas are benign tumors composed of spongy bone covered by
cartilaginous cap. Majority of them are sporadic and solitary. Less
commonly, it occurs as multiple lesions with autosomal dominant pattern
of inheritance. It was long debated whether osteochondroma was a
developmental disorder or a true neoplasm. The etiology is not known.
Based on the resemblance of the cartilage cap to the growth plate,
several hypotheses have been offered. These include the possibility of
breakage, rotation and aberrant growth of the epiphyseal plate or
herniation of the plate in the metaphysic [3]. Cytogenetic studies have
implicated the Knudson’s two hit hypothesis on EXT 1 AND EXT
2 tumour suppressor genes resulting in truncated / non functional
protein. The mutations were described to induce cytoskeletal
abnormalities in osteochondroma chondrocytes [4]. DNA flow cytometry of
the cartilaginous cap demonstrated aneuploidy (DNA index range
0.88-1.17) [5].
Osteochondromas can be found in any bone preformed by cartilage. Most
lesions are found during the period of rapid skeletal growth, and their
growth usually ceases by skeletal maturity. Involvement of the flat
bones, ilium, and scapulae occurs in about 5% of patients. Majority are
asymptomatic, slow-growing mass detected as an incidental finding by
radiography. Pain is usually caused by compression of adjacent
structures or due to the fracture of the stalk. Grossly an
osteochondroma presents as an exophytic, sessile or pedunculated (with
a stalk) projection of bone capped by cartilage. Importantly, the
osteochondroma demonstrates both medullary and cortical components that
are contiguous with the medulla and cortex, respectively of the
underlying native bone. On imaging studies, the lesions appear as
either a flattened (sessile) or a stalk-like (exostotic) protuberance
on the bone shaft in a juxtaepiphyseal location. CT scan or MRI images
typically show continuity of the marrow space into the lesion. These
modalities may also predict the thickness of the cartilage cap [6].
Microscopically osteochondroma mimics an epiphyseal growth plate
beginning with a thin (usually <1 cm) cartilaginous cap
superficially, a zone of endochondral ossification followed by regular
trabecular bone surrounded by fatty, hematopoietic marrow.
Occasionally, degenerative change and calcification are seen in the
cartilage cap.
Osteochondromas can potentially transform into malignancy. The features
that should raise suspicion of malignancy include :
1. Clinically – any recent
rapid increase in size with associated pain, multiplicity with
involvement of proximal skeleton and multiple recurrences
2. Radiographically - irregularity of the
margin, inhomogeneous mineralization, an associated soft tissue mass.
3. Grossly – a thick irregular
cartilage cap greater than 2 cms
4. Microscopically – Loss of
the architecture of cartilage, wide fibrous bands, myxoid change,
increased chondrocyte cellularity, mitotic activity, significant
chondrocyte atypia, necrosis and permeative growth into surrounding
soft tissues.
Differential diagnosis include parosteal osteosarcoma, Nora’s
lesion (bizarre parosteal osteochondromatous proliferation) and tori
(Exostoses in the cranio-facial and jaw bones in response to
irritants). Histopathology with radiological correlation provides
conclusive opinion on diagnosis.
Surgical excision is often curative for osteochondromas. Arthroscopic
resection can also be successfully performed. Removal in toto may be
difficult in sessile tumours. Clean surgical margins help to prevent
recurrences and to manage chondrosarcomas [7]. Secondary complications
include mechanical obstruction, nerve impingement, pseudoaneurysm of an
overlying vessel, infarction of the osteochondroma or fracture of the
stalk of the lesion[2]. The thickness of the cap decreases with
advancing age and may trigger the formation of a bursa.
Conclusion
The present case report describes a considerably rare entity 5
‘S’ osteochondroma – Sporadic, Solitary,
Sessile, Symptomfree and Scapular.
Funding:
Nil,
Conflict of interest:
Nil
Permission from IRB:
Yes
References
1. K.Krishnan Unni. Cartilaginous lesions of bone. Journal of
Orthopaedic Science.2001;6 (5):457-472. [PubMed]
2. Jennes I, Entius MM, Van Hulm E, Parra P, Sangiorgi L, Wuyts W.
Mutation screening of EXT1 and EXT2 by denaturing high-performance
liquid chromatography, direct sequencing analysis, fluorescence in situ
hybridization, and a new multiplex ligation-dependent probe
amplification probe set in patients with multiple osteochondromas. Mol
Diagn. 2008; 10(1): 85–92.
3. Porter DE, Simpson AH. The neoplastic pathogenesis of solitary and
multiple osteochondromas. J Pathol .1999;188 (2):119-125. [PubMed]
4. Bernard MA, Hall CE, Hogue DA, Cole WG, Scott A, Snuggs MB, Clines
GA, Lüdecke HJ, Lovett M, Van Winkle WB, Hecht JT. Diminished
levels of the putative tumor suppressor proteins EXT1 and EXT2 in
exostosis chondrocytes. Cell Motil Cytoskeleton.2001. 48(2):149-162. [PubMed]
5. Bovée JV, Cleton-Jansen AM, Wuyts W, Caethoven G,
Taminiau AH, Bakker E, Van Hul W, Cornelisse CJ, Hogendoorn PC.
EXT-mutation analysis and loss of heterozygosity in sporadic and
hereditary osteochondromas and secondary chondrosarcomas. Am J Hum
Genet.1999. 65(3): 689-698.
6. Woertler K, Lindner N, Gosheger G, Brinkschmidt C, Heindel W.
Osteochondroma: MR imaging of tumor related complications. Eur
Radiol.2000.10(5): 832- 840. [PubMed]
7. Pérez D, Ramón Cano J, Caballero J,
López L: Minimally-invasive resection of a scapular
osteochondroma. Interact Cardiovasc Thorac Surg 2011,
13(5):468–470. [PubMed]
How to cite this article?
Neeraja Myreddy, K P Varalakshmi, P Sravani, B. Chaitanya. Usual Tumour
at an Unusual location – Solitary Sporadic Scapular Sessile
Osteochondroma. Int J Med Res Rev 2014;2(4):403-406.doi:10.17511/ijmrr.2014.i04.025