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Extraskeletal Ewing's Sarcoma of the Little Finger, a rare case

Md. Jawed Akther

JiangYuan Hospital Affiliated to Jiangsu Institute of Nuclear Medicine, China

E-mail : dr_mdjawedakther@yahoo.com

L. Udaya Kumar

JiangYuan Hospital Affiliated to Jiangsu Institute of Nuclear Medicine, China

Shameem Unnisa Shaikh

Department of neurosurgery, No 101 Hospital of Chinese PLA, China

DOI: 10.15761/GOS.1000139

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Abstract

When Ewing’s sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing’s sarcoma (ESS). It is generally accepted to be between 15% and 20% of that of Ewing sarcoma of bone. Extraskeletal Ewing sarcoma usually manifests in young patients, with 85% of cases detected between 20 months and 30 years of age. The most commonly reported locations of extraskeletal Ewing sarcoma include the paravertebral region (32%), lower extremities (26%), chest wall (18%), retroperitoneum (11%), pelvis and hip (11%), and upper extremities (3%). Radiological features of Ewing’s sarcoma are nonspecific. It is confirmed by features on histological analysis. In young people who present with soft tissue tumours, ESS should be considered. In the management of patients with tumours, imaging techniques are useful for biopsy guidance, evaluating the possibility of resection, and tumour response to treatment.

Key words

extra skeletal ewing's sarcoma; little finger; MRI; histopathology

Introduction

Ewing's sarcoma (ES) was first described in 1921 [1]. It is the second most common primary bone tumor of childhood and adolescence [2]. Tumors morphologically indistinguishable from Ewing sarcoma of the skeletal system can present as soft tissue masses. In some cases, they simply represent soft tissue extensions of tumor originating in the underlying bone. In others, bone involvement is absent, and these are regarded as primary Ewing sarcomas of soft tissues [3-5]. The prevalence of extraskeletal Ewing sarcoma is generally accepted to be between 15% and 20% of that of Ewing sarcoma of bone [6,7]. Extraskeletal Ewing sarcoma usually manifests in young patients, with 85% of cases detected between 20 months and 30 years of age [7]. The most commonly reported locations of extraskeletal Ewing sarcoma include the paravertebral region (32%), lower extremities (26%), chest wall (18%), retroperitoneum (11%), pelvis and hip (11%), and upper extremities (3%). 8,5,9,10,11

Case Report

A 18 year old right hand dominant boy farmer by occupation presented with a 1 year history of swelling over left little finger with insidious onset and gradually progressive in size. It was associated initially with dull aching pain that later worsened with rapid increase in size since last 1 month. He had history of incision and drainage by a quack 2 months back presuming it to be abscess. There was no history of trauma, fever, cough, dyspnea, and body aches. There was history of reduced appetite and weight loss since 1 month. He had no other co-morbid medical illness.

On physical examination, the mass was 6×3.5×3 cm, immobile and fixed involving 5th finger extending upto distal palmar crease. Both 4th & 5th fingers were widely apart (Figure 1 & 2). There was restriction of little finger movements. There was no regional lymphadenopathy. All laboratory findings were within normal range.

Figure 1. Swelling of Lt. Little finger.

Figure 2. Swelling of Little finger.

Conventional radiographs revealed enlarged soft tissue opacity in left 4th web space, extending to proximal and middle phalanx of left 5th finger with evidence of well-defined osteolytic lesion associated with cortical break noted in lateral aspect of left 5th proximal metacarpal. Rest of metacarpals, phalanges and carpal bones appeared normal. Incision biopsy report was in favor of Ewings sarcoma.

MRI Lt. wrist & hand (Figure 3) showed evidence of well-defined soft tissue lesion along medial aspect of little finger of the left hand in between two tendons of extensor muscles of 4th and 5th finger. There was widening of the space between 4th and 5th finger. The lesion was seen abutting the medial medial aspect of proximal and middle phalanx of little finger with fine marginal erosion of the medial cortex of middle phalanx and altered signal intensity of the proximal phalanx could suggest possibility of secondary involvement. The lesion was showing area of central necrosis. Size of the lesion was 6 x 3.5 cm. with area of necrosis around 2 cm. On post contrast images it showed good contrast enhancement with circumferential pattern. Rest of the visualised bones, muscles appeared normal. Soft tissue mixed intensity lesion with areas of circular or whorled contrast enhancement along medial aspect of little finger suggested possibility of neoplastic aetiology than infective cause. Possible differential diagnoses were – Soft tissue Ewings sarcoma, other soft tissue sarcoma, Lymphangi