Infantile fibrosarcoma misdiagnosed as vascular tumors

Abstract

Clinical as well as radiologic diagnosis of infantile fibrosarcoma (IFS) is often a challenging problem due to similarities with tumors of vascular origin. Consequently, in the majority of cases, histological and immunohistochemical studies are considered gold standards for the final diagnosis. The two case reports and the review of literature discussed should increase the important features in the history and the presentation that increase the index of suspicion for IFS, as well as it highlights the important characteristics of imaging and laboratory studies that confirm its diagnosis.

Keywords: Infantile fibrosarcoma; Upper extremity tumors; Vascular tumors.

Fig. 1

Patient 1. a, b Upon presentation and before excision. c, d Immediate postoperative images after resection of the tumor

Fig. 2

3D CT angiography. a Soft tissue, bone and vascular component in color images; b, c, d the main blood supply of the tumor, useful for preoperative planning. There are hypertrophied branches of the brachial artery with tortuous enlarged draining veins and probable enlargement of the arm and subclavian veins

Fig. 3

a Left arm tumor upon presentation before the incisional biopsy. b, c During neoadjuvant chemotherapy, showing the chemotherapy induced necrosis and sloughing of the tumor. d Post neoadjuvant treatment and pre-surgical excision; tumor has shrunk by about 60 % of its initial mass

Fig. 4

Surgical excision. a Meticulous dissection revealing preservation of the median and ulnar nerves as well as the brachial artery (arrows). b Specimen removed