A 63-year-old woman presenting with a synovial sarcoma of the hand: a case report

Abstract

IntroductionSynovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma.Case presentationWe describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented.ConclusionAwareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.

Synovial Sarcoma of the Hand

“…Soft tissue sarcomas of the upper extremities are rare with perhaps only one or two undiagnosed soft tissue sarcomas encountered by hand surgeons throughout their entire career [3]. The most common site of soft tissue sarcoma is at the lower extremity (60%) (with tendency for the knee, ankle, and hip), followed by the upper extremity (23%) (with tendency for the shoulder), and the head and neck (9%) [1,2,4]. Further, it tends to be located close to the large joints of the extremities, especially the knee and ankle.…”

“…The incidence of synovial sarcoma, in general, is 1.548 per 1,000,000 [1]. Synovial sarcoma localized to the palmar surface should, therefore, be considered extremely rare [2]. In the unusual event in which sarcoma has been identified in the palmar aspect of the hand, the incidence of digit involvement is less common than that of the carpus [2].…”

Soft Tissue Sarcoma of the Hand and Wrist: Epidemiology and Management Challenges

“…With this in mind, it is fortuitous that these tumors are rare; for example, a consultant hand surgeon not specializing in sarcoma may be expected to encounter one synovial sarcoma over the course of their career. 2 When an "unplanned" excision of a sarcoma is performed outside of an STS MDT, initial histopathologic assessment is considered unreliable and should not be used to plan future treatment. 6 It should always be discussed at an MDT and followed by a re-excision of the affected area if appropriate, though this may be dependent on anatomical constraints such as adjacent neurovascular structures, the additional morbidity it may cause, and potential efficacy of adjuvant therapy.…”

“…Many studies of STS of the hand are limited to reports of single cases that provide little evidence to guide the modern sarcoma service. [2][3][4] We reviewed all cases of STS of the hand and wrist at our regional sarcoma service over a 15-year period. We present the demographics of the cohort, tumor epidemiology, and mode of presentation, along with individual treatment strategies and outcomes, and reconstructive details.…”

Initially Localized Synovial Sarcoma in Adults: A Retrospective Single-Center Analysis of 26 Patients Registered at the Department of Oncology, University of Vienna between 2004 and 2013

“…Adjuvant chemotherapy was administered to 12 adult patients (13.8%) and had no impact on survival [16]. Only few reports (case reports of adult patients with initially localized as well as initially metastatic SS) exist considering OS [19,20,21,22,23]. …”