Desmoplastic fibroma of the scapula: A case report and review of literature

M Harisankar; TS Gopakumar; C Vijesh; MS Bhuvitha

doi:10.4103/joasis.joasis_4_20

CASE REPORT

Year : 2020 | Volume : 17 | Issue : 1 | Page : 24-27

Desmoplastic fibroma of the scapula: A case report and review of literature

M Harisankar¹, TS Gopakumar², C Vijesh², MS Bhuvitha³
¹ Department of Orthopaedics, Baby Memorial Hospital, Calicut, Kerala, India
² Department of Orthopaedics, MES Medical College Hospital, Malappuram, Kerala, India
³ Department of Pathology, Government Medical College, Trivandrum, Kerala, India

Date of Submission	11-Oct-2020
Date of Acceptance	27-Oct-2020
Date of Web Publication	17-Nov-2020

Correspondence Address:
M Harisankar
Department of Orthopaedics, Baby Memorial Hospital, Calicut, Kerala
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joasis.joasis_4_20

Abstract

Desmoplastic fibroma (DF) of the bone is a very rare benign tumor of the skeletal system with a reported incidence <1.1% of all primary bone tumors. DF of bone is considered to be the skeletal counterpart of extra-abdominal desmoid tumors. The common sites are mandible and tibia. Here, we present a case report of DF of the scapula with a 14-year follow-up. The DF of the scapula is an extremely rare tumor with only six such cases reported in international literature till now.

Keywords: Benign bone tumor, desmoplastic fibroma, desmoplastic fibroma of scapula

How to cite this article:
Harisankar M, Gopakumar T S, Vijesh C, Bhuvitha M S. Desmoplastic fibroma of the scapula: A case report and review of literature. J Orthop Assoc South Indian States 2020;17:24-7

How to cite this URL:
Harisankar M, Gopakumar T S, Vijesh C, Bhuvitha M S. Desmoplastic fibroma of the scapula: A case report and review of literature. J Orthop Assoc South Indian States [serial online] 2020 [cited 2023 Apr 1];17:24-7. Available from: https://www.joasis.org/text.asp?2020/17/1/24/300757

Introduction

Desmoplastic fibroma (DF) of the bone is described by the WHO as a rare, locally aggressive, benign lesion of bone characterized by the formation of abundant collagen fibers by the tumor cells. DF is a rare bone tumor with a reported incidence of 0.11% of all primary bone tumors.^[1],[2]

Jaffe in 1958 was the first to describe this tumor and to note its uncanny histological resemblance to aggressive fibromatosis, aka desmoid tumor.^[3] Due to this resemblance, DF is considered as the osseous variant of extra-abdominal desmoid tumor.^[4] DF of bone is a rare entity by itself and DF of the scapula is an extremely rare location for a DF of bone, with only six cases published in the literature till now.^{[5],[6],[7],[8],[9]}

In this article, we report a case of DF of the scapula with 11 years of follow-up and discuss the review of relevant literature.

Case Report

A 16-year-old boy presented to us with swelling over the right scapula, pain, and limitation of movement of the right shoulder for 6 months. The swelling was insidious in onset and was slowly growing.

On examination, the patient had a hard, fixed, tender swelling of 6 cm × 4 cm fixed to the right scapula involving the lateral border and spine of the scapula. The skin over the swelling was normal. There were no distal neurovascular deficits. Blood investigations revealed normal results.

X-ray evaluation [Figure 1] showed a radiolucent osteolytic lesion with cortical erosion and sclerosis involving the lateral border and spine of the right scapula.

Figure 1: X ray showing the lytic lesion along the lateral border of the scapula

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Computed totmgraphy (CT) scan [Figure 2]a and [Figure 2]b of the region showed an expansile lytic lesion involving lateral aspect and spine of the right scapula with few thin internal trabeculae inside.

Figure 2: (a and b) Computed tomography showing lytic lesion lateral border of the scapula

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The patient was treated by curettage and hydroxyapatite application in 2006. Biopsy [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e report was indicative of DF.

Figure 3: (a-c) Histology – H and E staining low-power (×10) and high-power (×40) views; (d and e) Special staining (Masson's trichrome) low-power (×10) and high-power (×40) views showing a characteristic picture of desmoplastic fibroma – abundant collagen with few spindle cells and osteoid fragments

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The patient was on irregular follow-up. He presented again to the outpatient department 6 years later (in 2012) with recurrence of pain and swelling of the scapula. X-ray [Figure 4] and CT [Figure 5]a and [Figure 5]b evaluation showed recurrence of the lesion.

Figure 4: Recurrence of the lytic lesion

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Figure 5: (a and b) Computed tomography cuts showing recurrence of the lytic lesion right scapula

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He was treated with repeat curettage and biopsy [Figure 6]. The biopsy was consistent with DF, and there were no signs of malignant transformation.

Figure 6: Postoperative X-ray after the revision curettage and hydroxy appetite application of the lesion

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Following surgery, his pain reduced and the range of motion of the right shoulder was found to be improved. The patient is on regular follow-up for the past 7 years [Figure 7] and [Figure 8], and there are no signs of further recurrence.

Figure 7: X-ray on his latest OP visit showing healed lesion right scapula

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Figure 8: The patient is having a good range of movements of the right shoulder

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Discussion

DF is one of the rarest bone tumors comprising almost 0.11% of all primary bone tumors.^[1],[2] There is no specific sex predilection, with most of the cases occurring in the second decade.^[10]

Jaffe^[3] and Triantafyllou^[11] proposed that an antecedent trauma was responsible for the occurrence of the tumor. They also suggested the possibility of hormonal influence in the growth of the tumor as they noted the sudden proliferation of local recurrence during pregnancy. However, none of the case series showed any conclusive evidence with regard to the etiology of DF.

Most commonly involved bones are mandible (22%), femur (15%), pelvic bones (13%), radius (12%), and tibia (9%).^[10] The scapula is an extremely rare location with only six such cases published in the literature.

The clinical presentation of DF is very vague and is similar to other tumors. Patients present mostly with a slow-growing swelling which is palpable. Most of them are incidental findings during radiographic evaluation following a trauma.

Radiographically, the lesion of DF has been described as a purely lytic expansile lesion with lobulated margins and trabeculations without any mineralized matrix. The tumors mostly appear oval with the long axis aligned along the long axis of the bone. The lesion is usually metaphyseal and might extend into the epiphysis and even up to the subchondral bone in skeletally mature patients.^[10],[12]

In angiography, DF appears hypovascular. Computerized tomography is relevant in determining whether there is any cortical breach. Magnetic resonance imaging remains the single most important diagnostic imaging modality in determining and assessing the soft-tissue extension of the tumor and thus helps in the preoperative planning.^[10]

DF is usually classified as a benign tumor. The lesion never perforates the periosteum.^[5],[13] However, Dahlin and Unni classified DF as a borderline lesion with regard to malignancy because the lesion demonstrates local infiltration.^[1] Malignant transformation of DF is extremely rare.^[10]

Histological appearance of DF is very similar to that of extra-abdominal desmoid tumors. The appearance is of uniformly appearing slender, spindle, or stellate cells, mainly fibroblasts with minimal cytological atypia dispersed in an abundant collagenous matrix.^[12],[14] The lesion is histologically very similar to low-grade fibrosarcoma and it presents a challenge to even the most experienced pathologist to distinguish between the two.

The literature available is not enough to make any conclusive comments regarding ideal treatment, but considering the high recurrence rates associated with intralesional procedures, wide resection should be the goal of surgical intervention. Intralesional procedures lead to high recurrence because there are no effective adjuvant therapies available. Postoperative irradiation of the tumor site should be done under extreme caution when considering the risk of developing postradiation sarcomas of soft tissues and bone.^[10],[15]

Conclusion

DF is a rare benign lesion of the bone. The lesion, though benign, shows local infiltrative properties. The diagnosis presents a challenge to the treating clinician as the clinical features are vague and nonspecific and to the pathologist as the histological picture is very difficult to distinguish from low-grade fibrosarcoma.

Considering the very high local recurrence rates associated with intralesional procedures and the limited efficacy of the adjuvant therapies, wide resection of the lesion is the safest surgical goal. Postoperative radiation therapy if done should be done with extreme caution keeping in mind the risk of developing postradiation soft tissue or bone sarcomas.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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