DESMOPLASTIC FIBROMA OF

SCAPULA-A RARE BONE TUMOR

CASE REPORT AND LITERATURE REVIEW

Author : Dr. VIJESH .C

Guide : Dr. T.S GOPAKUMAR
INTRODUCTION

• A rare beningn tumor of the bone first described by jaffe in 1958

• Common site-long tubular bones[metaphysis]
• Slight male predominance
• Age-10 to 20 yrs
• Incidence-0.11% of all primary bone tumors
• Connective tissue origin
• Locally aggressive with high rate of recurrence
• Bony counter part of soft tissue desmoid tumor
CASE REPORT
• 16 year old male patient with pain swelling and limitation of moving right
shoulder since 6 month
• On Examination-A hard fixed tender swelling of 6 * 4 cm involving lateral border
and spine of scapula
• Patient was investigated using x ray ,CT, MRI and biopsy
• X ray-radiolucent osteolytic lesion with cortical erosion and sclerosis involving
lateral border and spine of right scapula
• CT- Expansile lytic lesion involving lateral aspect and spine of right scapula
• MRI-Low intensity signal on T1 and T2 weighted images
• Excision Biopsy-Suggestive of Desmoplastic fibroma of right scapula
• Patient underwent wide resection and extended curettage
CT RIGHT SHOULDER
HISTOPATHOLOGY
.Hypocellular fibrous tissue with abundant collagen,predominant cells are fibroblast
GROSS
• On follow up his symptoms and ROM right shoulder found to be
improved. No recurrence during the follow up period of 6 years.
DISCUSSION
Desmoplastic fibroma of scapula is a rare benign tumor, presented
with following clinical features :
• Pain and Swelling
• Restriction of movement
• Localised Tenderness
• Presents as a slowly growing mass and closely mimicking desmoid
tumor
• It is associated with high rate of recurrence
• Recent literature supports the role of PET/CT in assessing disease
response to treatment and detecting recurrence
• Immunohistochemistry for beta catenin is useful for differentiating
desmoplastic fibroma from desmoid tumor.
INVESTGATIONS
X-ray ,MRI, CT, PET SCAN

Definitive diagnosis - Histopathology

TREATMENT

• Wide resection and extended curettage

• Adjuvant treatment-anti inflammatory agents , radiation, tamoxifen
and cytotoxic agents
References
• Dorfman HD, Czerniak B. Bone Tumors. Mosby; 1998. pp. 559–606.
• Dahlin DC, Unni KK. In: Bone tumors. Thomas , editor. Springfield: Charles C. Thomas;
1984. pp. 375–8.
• Gebhardt MC, Campbell CJ, Schiller AL, Mankin HJ. Desmoplastic fibroma of bone: a
report of 8 cases and review of the literature. J Bone Joint Surg Am. 1985;67:732–47.
• Smith SE, Kransdorf MJ. Primary musculoskeletal tumors of fibrous origin. Semin
Musculoskelet Radiol. 2000;4:73–88.
• Perlick L, Zander D, Wallny T, Zhou H. [Desmoplastic fibroma of the fibula. A difficult
clinical, radiological and histological diagnosis] . Zentralbl Chir. 2000;125:895–9.
• Bertoni F, Calderoni P, Bacchini P, Campanacci M. Desmoplastic fibroma of bone. A
report of six cases. J Bone Joint Surg Br. 1984;66:265–8.