title of the case

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TITLE OF THE CASE
“Giant cell tumour of the rib, a rare differential diagnosis of chest wall tumour”
Authors of the case
Dr Manoj Kumar Pattnaik
Dr Paresh Chandra Majhi
Dr Sameer Kumar Panigrahy
Dr Anil Kumar Nayak
Dr Ipsita Dhal
Abstract
A young adult male referred from a peripheral hospital with a painless
swelling over anterolateral aspect of left lower chest wall. Initial
evaluation revealed giant cell tumour (GCT) of rib & following surgical
excision histopathological study confirmed the same.
Giant Cell Tumor is a benign, slow growing tumor with some frankly
malignant variant, commonly involve ends of long bones like lower end
of femur ,upper end of tibia & lower end of radius after epiphyseal fusion
in third and fourth decade with female preponderance [1-3]. On rare
occasion it may involve the ribs to produce symptomatology, that of a
chest wall tumour. Here we present a rare case of huge giant cell tumor
of rib at its unusual location creating a well differential diagnosis of chest
wall tumour.
Background
GCT of rib, arising from anterior arch with such a huge palpable swelling in
an adult male is very unusual & sparsely reported [4-8].More commonly
it arises from its posterior arch usually from the head and tubercle [8-11].
Whenever such a presentation faced, it is worthwhile to exclude common
entities of chest wall tumor, and then managed accordingly.
Case presentation
A young adult male presented to our hospital with one year history of slow
growing, painless, palpable, hard swelling over left lower chest wall,
anterolaterally with no associated symptoms (figure-1, figure-2).
Overlying skin was healthy with no local rise of temperature.
Investigation
All routine blood parameters were within normal limits except serum acid
phosphatase (moderately elevated) which was done after Fine Needle
Aspiration Cytology(FNAC). Cytosmears showed stromal cells in large
fragments with good number of giant cells attached to periphery of
fragments in the background of osteoclastic cells. Plain radiograph was
not apparent (figure-3). Contrast enhanced Computed tomography
showed heterogeneously enhancing mass arising from left 11th rib causing
rib destruction with large soft tissue component showing central necrosis
& few bony spicules, infiltrating the parietal abdominal muscle &
abutting descending colon, left kidney, spleen and left psoas muscle
(figure-4, figure-5).No lung pathology was detected.
Differential Diagnosis
Benign Lesions
Fibrous dysplasia
Enchondroma
Eosinophilic granuloma
Brown tumor
Aneurysmal bone cyst
Simple bone cyst
Chondromyxoid fibroma
Giant cell tumour
Malignant Lesions
Metastasis
Solitary myeloma
Chondrosarcoma
Osteosarcoma
Treatment
Keeping in mind, the high recurrence of GCT, wide excision was done with
involved portions of the parietal abdominal muscles. Peritoneal cavity
was not entered, but left pleural cavity was opened due to diaphragm
involvement (figure-6). Diphragm rent was closed with 2-0
polypropelene suture. Around one litre of intra operative blood loss was
replenished by blood transfusion. Tumor mass was approximately
10x8x7cm in size with irregular greyish trabeculated friable tissue
(figure-7). Histopathology showed biphagic pattern of both stromal cells
& osteoclastic giant cells in isomorphic distribution with areas of
hemorrhage & necrosis (figure-8, figure-9).
Outcome & follow up
Patient was uneventfully discharged on 10th post-operative day with no signs
of recurrence after five months of follow up. He has not received any
adjuvant therapy as the histopathology revealed no features of cellular
atypia or mitotic activity. Serum acid phosphatase returned to base line on
repeat evaluation.
Discussion
GCT of bone represent about 5% of all primary bone tumor. Scapula,
sternum, patella, vertebra, skull, and talus have been reported as other
rare sites [12]. Most of the cases are usually asymptomatic with mild pain
or soft tissue swelling. Sometime it may present as pathological fracture
due to thinning of cortical bone. Due to its rare incidence, GCT arising
from the rib is difficult to diagnose, especially when the tumor is located
in the anterior arc. Preoperatively FNAC can be used to enhance
diagnostic certainty along with radiological survey. In current case,
diagnosis was made by aspiration cytology preoperatively. Treatment of
GCT may vary from simply curettage with or without use of alcohol,
liquid nitrogen, phenol to wide excision with chest wall reconstruction [8-
11, 13]. However no standard treatment has been established for GCT of
rib. The present case was subjected to liberal wide excision with primary
reconstruction of diaphragm and chest wall. Literature reports high
recurrence after surgical treatment, up to 50% [12].Though estrogen and
progesterone receptors have been identified, role of steroid yet to be
decided [14]. Role of radiotherapy was not much encouraged as
malignant transformation in GCT has been reported with previous
radiotherapy.
Learning points
As such cases have been reported and will be reported in future, we can
never deny to put GCT among the differential diagnosis of chest wall
tumour, at least when a bony hard and fixed mass stands out clearly in
this age group even though its occurrence in rib is very unusual.
Whenever a surgical excision is planned, wide excision to be done with
expectation of healthy amount of blood loss as the tumour stroma is
highly vascular.
References
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