Fungating synovial sarcoma at the posterior aspect of neck: a case report

Abstract

Background

In this report, we describe an uncommon instance of fungating synovial sarcoma affecting the posterior aspect of the neck. Although the existing literature has documented a limited number of cases, this particular case contributes to the knowledge about it, which is scarce.

Case presentation

A total of 5 months before the examination, a Pakistani-Asian male, age 20 years, complained of a malodorous fungating swelling on the posterior aspect of his neck. An examination revealed a foul-smelling, 10 × 13 cm fungating enlargement surrounded by maggots and hemorrhaging at the site of the incision. A hemoglobin level of 6 and a total leukocyte count (TLC) of 23,000 indicated the patient’s disoriented and pallid appearance. He was expeditiously admitted, and preoperatively, the general well-being of the patient was optimized. After a comprehensive discussion with the medical team, a strategy for marginal excision and coverage with a latissimus dorsi (LD) flap and grafting was devised. The tumor was successfully excised, and an LD flap with graft was conducted on the patient during surgery; however, the infection caused the failure of half of the graft. Following that, the lesion was debrided, and re-grafting was performed. The patient was subsequently administered 5 cycles of chemotherapy and 32 cycles of radiotherapy. He was diagnosed with pulmonary metastasis 2 years later. Sadly, the patient died during a follow-up visit 3.5 years later.

Conclusions

The patient’s unfavorable prognosis after surgical intervention, radiotherapy, and chemotherapy, despite undergoing all-encompassing treatments, underscores the importance of early detection and intervention in fungating tumor cases.

Introduction

Synovial sarcoma (SS) is a high-grade malignant tumor with a global prevalence of 0.0001–0.0002%, constituting only 800–1000 cases reported annually [1]. Synovial sarcoma is a misnomer primarily arising from primitive mesenchymal cells around joints instead of intra-articular synovium [2]. SS is commonly soft tissue sarcoma, but bony cases are also reported [2]. The lower limb is frequently involved, especially around the knee joint.

Only 3–10% of SS arise from the head and neck, contributing to only 2.5–3.5% of all sarcomas in the region [3]. However, SS can be primarily distinguished from other neck tumors on the basis of the histological appearance and presence of the characteristic chromosomal translocation; t(X;18). The translocation resulting from the fusion of the SYT gene with the SSX1 or SSX2 gene drives several histopathological variants, including monophasic, biphasic, and poorly differentiated tumors [2]. Although only a few cases have been reported on SS arising from the head and neck region, SS occurrence at the posterior aspect of the cervical spine is an extremely rare presentation [4, 5]. Therefore, due to the limited literature availability on the subject, the optimal management strategy for cervical spine SS has not been established. However, total surgical excision with a wide margin followed by postoperative radiation is widely recommended in the previously published reported cases. In this case report, we present a usual case of a large fungating SS originating from the posterior aspect of the spine managed with marginal excision and highlighting the diagnostic and management challenges.

Case report

A 20-year-old Pakistani-Asian male presented with fungating swelling at the posterior aspect of his neck for 5 months, with foul-smelling discharge from the swelling for 2 months. The swelling was initially small and progressed rapidly once operated by a local center. As the tumor progressed, it became fungated out of the skin (Figs. 1 and 2). The swelling was associated with foul smells, fever, weight loss, and continuous fever for the last few days of presentation. The patient’s attendant also noted bleeding from the tumor on and off.

Fig. 1

Foul-smelling fungating swelling of 15 × 10 cm (black arrow)

Fig. 2

Swelling extends from the occipital region to the base of the neck

The patient was a laborer by profession, with no known co-morbidities. He was taking antipyretics and antibiotics for this problem. His systemic examination was unremarkable. His hemoglobin was 6.0 g/dl, his total leukocyte count (TLC) 20,000, his erythrocyte sedimentation rate (ESR) 52, and his C-reactive protein (CRP) 15. Other labs were within normal limits. The x-ray of his cervical spine was normal, and magnetic resonance imaging (MRI) cervical spine contrast showed a mixed density soft tissue tumor arising from the paraspinal muscle extending from the occiput to the C7 vertebrae (Figs. 3 and 4). Computed tomography (CT) scan of the chest at the time of presentation was negative for metastasis. His bone scan was also unremarkable. His biopsy suggested monophasic synovial sarcoma.

Fig. 3

MRI showing mixed density soft tissue tumor arising from the paraspinal muscle extending from the occiput to the C7 vertebrae

Fig. 4

Axial MRI image showing vertebrae were spared with paraspinal cervical muscle involvement. The tumor did not extend into the cord

Preoperatively, the general well-being of the patient was optimized, and antibiotics prophylactically started. The case was discussed in a tumor board meeting including the neurosurgeon, plastic surgeon, orthopedic and once-radiologist team, and a plan was made to do marginal/wide margin resection and cover the defect with a latissimus dorsi flap and skin graft. After consent and counseling, the patient was operated on under general anesthesia in the prone position. The tumor was slowly dissected, and the wound was washed and hemostasis secured. A split-thickness skin graft (SSG) was taken from the thigh and applied over the flap.

The patient’s immediate recovery from anesthesia was smooth, but at 2 weeks, he developed discharge from the wound. The skin graft failed, and part of the LD got infected and debrided (Figs. 5 and 6). The patient was kept on vancomycin for 2 weeks, and vacuum-assisted closure (VAC) dressing was applied. The wound was granulated, and re-skin grafting was done (Fig. 7). His musculoskeletal tumor score was 28.

Fig. 5

First infected SSG. Necrosis of graft is circled

Fig. 6

Wound after debridement

Fig. 7

Status of the wound after the second SSG

The patient recovered normally within 3 months of surgery, and then underwent 30 cycles of radiotherapy and 5 cycles of chemotherapy. He was kept under routine follow-up with a CT scan chest. Despite all the treatment, he developed lung metastasis after 20 months of surgery, for which he received treatment from an oncologist. He died of lung metastasis after 3.5 years of surgical follow-up.

Discussion

Synovial sarcoma (SS) mostly impacts young individuals, with the extremities, particularly around the knee, which is the most common location (70%). While uncommon in the head and neck, SS in this area is known for its aggressive nature and high likelihood of recurrence. The precise etiology of SS remains uncertain; however, variables such as radiation and genetic susceptibility have been linked to an elevated risk [6].

There is limited information available on SS in the posterior cervical area. Shah et al. reported a case of SS that originated from the cervical spine and resulted in neurological impairments characterized by widespread paralysis and loss of sensation at the T4 level. Nevertheless, the author’s ability to provide comprehensive follow-up information was hindered by a lack of connection with the center. Tragically, the patient succumbed to fever and diarrhea after 2 years [5]. Foreman et al. reported a case of biphasic synovial sarcoma that was managed with surgical intervention, radiation treatment, and chemotherapy. The patient was free of disease recurrence for 6 years. Their approach included performing subtotal resection to remove the small-sized tumors, followed by postoperative radiation treatment and chemotherapy. There were no instances of tumor recurrence reported throughout the 6-year follow-up period. Kim et al. documented the effective management of SS using MRI, which revealed a tumor measuring 6.6 × 4 cm in the cervical spine muscles. The treatment included surgical intervention and further radiation. Remarkably, there was no recurrence of the tumor after a 2-year follow-up [7]. Spinal synovial sarcoma is an uncommon ailment that might be challenging to promptly detect in the absence of contemporary imaging methods.

The detection of cancer at a smaller size is believed to influence the long-term prognosis, which was seen to be more advantageous in individuals whose tumors were 4 cm or less [6].

A literature analysis by Standbouly et al. emphasized that surgical excision is the primary treatment for 91 cases of head and neck SS. Additional radiation and chemotherapy may be used depending on the size of the tumor. The overall survival rates at 1, 3, and 5 years were 87.5%, 61.4%, and 40.9%, respectively [8].

Synovial sarcoma poses distinct issues due to its tendency to return much later in comparison to other types of sarcomas. Krieg et al. (2011) found that local recurrence usually occurs around 3.6 years on average (ranging from 0.5 to 15 years), whereas metastases tend to arise around 5.7 years on average (ranging from 0.5 to 16.3 years). Furthermore, the location of the tumor also influences the prognosis, since synovial sarcomas found outside of the extremities are often linked to lower overall survival rates. However, this might be attributed to a delay in the appearance of symptoms and the presentation of the disease at a more advanced stage [2].

Although the surgical excision was successful, the patient eventually died because of lung metastases. Although synovial sarcoma is not common in certain areas, it has the potential to be life-threatening. Hence, it is important to promptly evaluate it as a probable diagnosis, while also seeking immediate advice and adopting a thorough strategy to promptly diagnose lesions and enhance prognosis.

Conclusions

The patient’s poor prognosis, caused by delayed presentation, a large tumor size upon arrival, and the negative effects of erroneous first excision, highlights the crucial significance of the early discovery and timely, suitable management in instances with fungating synovial sarcoma in the cervical region.