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Synovial sarcoma metastasis to the thoracic spine: A rare case where timely intervention leads to neurological recovery
Article · September 2012
DOI: 10.1007/s12570-012-0155-1
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Pradyumna Raval Vikas Gupte
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Eur Orthop Traumatol (2013) 4:161–164 DOI 10.1007/s12570-012-0155-1
CASE REPORT
Synovial sarcoma metastasis to the thoracic spine: a rare case
where timely intervention leads to neurological recovery Pradyumna Raval & Vikas Gupte
Received: 30 September 2012 / Accepted: 12 December 2012 / Published online: 27 December 2012 # EFORT 2012
Introduction His radiographs of the cervical and thoracic spine were
normal (Figs. 1 and 2). Computed tomography and magnetic Synovial sarcomas are derived from synovial tissues found resonance imaging of the cervicothoracic region showed epi- around fascial planes, periarticular structures and rarely in joints dural soft tissue at T2–T3 levels with altered signal in T2, T3, [1]. They represent 5 % to 10 % of all soft tissue sarcomas and T8 and L4 vertebral bodies (Figs. 3 and 4). These were sus- affect the adolescent and young adults more commonly than pected to be metastases. The thoracic spinal cord was swollen older individuals, with an average age around 30 years. Most and edematous from T2–T3 levels. Incidental metastatic lesions commonly involved is the lower extremity followed by the were also noted in the lung and the right second rib. upper extremity [1–4]. The trunk, abdomen, head and neck are The pre-operative staging of the mass was not done rare sites of involvement [1, 2, 5, 6]. Primary spinal synovial because of the extensive involvement and sudden deteriora- sarcoma and metastatic disease are extremely rare with literature tion of the patient by the time he presented to us. He consisting of case reports. Metastasis is about 3 % with extra- underwent a posterior decompression and fusion in the form spinal soft tissue sarcomas, with involvement of the lumbosacral of T2–T3 laminectomy and instrumentation from C7 to T7 spine, thoracic spine and cervical spine in that order [7–13]. We (Figs. 5 and 6). We placed lateral mass screws in the C7 report a case of metastasis to the thoracic spine in a 59-year old. vertebra without using the image intensifier and pedicle screws in the T7 vertebra. Thus, it was a cervicothoracic fusion after a thoracic decompression. The tumour was Case report removed piecemeal and spinal chord was decompressed. Histopathological evaluation was done on the tissue, which A 59-year-old gentleman presented to us with the chief was initially thought to be undifferentiated malignant small complaint of a dull aching backache, which had worsened round cell tumour involving the T2 vertebra. An immunohisto- over a period of 20 days. There was also a recent onset of chemistry (IHC) investigation was sought to differentiate be- associated neck pain of 7-day duration. This was accompa- tween metastatic small cell carcinoma and haemato–lymphoid nied by a weakness and paraesthesia in both lower limbs malignancy. The IHC test reported it to be a synovial sarcoma. with an altered bowel and bladder habitat. Post-operatively, the patient had a neurological recovery to On examination, he had tenderness at the T1–T2 level grade 4/5. Oncology opinion was sought and the patient re- and altered sensation from T4 downwards. He had a motor ceived radiotherapy as per the advice of the oncologist. The power of grade 2/5 in his lower limbs. The rest of the patient had a further neurological recovery after the radiothera- neurological examination was normal. py. He received radiotherapy cycles of 30 gy in ten fractions, as an outpatient. The patient died 3 months after the diagnosis was P. Raval first made. Our Lady of Lourdes Hospital, Drogheda, Republic of Ireland
V. Gupte FORTIS Hospitals, Mulund, Maharashtra, India Discussion
P. Raval (*) Synovial sarcoma is the fourth most common soft tissue 76, Fitzwilliam Court Apartments, Dyer Street, Drogheda, Ireland sarcoma. It has an incidence of 2.75 cases per 100,000 people e-mail: [email protected] [1, 9] and represent only 5 % to 10 % of all adult soft tissue 162 Eur Orthop Traumatol (2013) 4:161–164
Fig. 1 Lateral radiograph of cervical spine (pre-operative)
Fig. 3 CT scan image of the cervicothoracic spine sarcomas and 12 % to 15 % of all adult extremity soft tissue sarcomas [1, 2, 9]. In the paediatric population, they represent by Ki-67 immunoreactivity, the Ki-67 index of cellular around 8 % of all malignancies [14]. They are most commonly proliferative activity) and the gene fusion transcript (SYT– seen in the adolescent and the young population [1, 3, 14]. SSX) fusion type [2, 14, 16–19]. Their primary manifestation may be a slowly enlarging The histology has two forms: biphasic (epitheloid) and juxtaarticular painless mass [1]. They typically arise in the monophasic (spindle cell type). The biphasic form is com- legs and knee (knee is the most common site). They are posed of both epithelial cell and spindle cell components, often close to a joint and in the upper extremity they can be where as the monophasic form can be either epithelial cell or on the palmar surface [1]. The word ‘synovial’ is a misno- spindle cell type. There is a poorly differentiated type as mer and they are almost never intra-articular. The cell of well [1, 14]. Our patient had a biphasic type (Fig. 7). origin has been determined to be epithelial [2]; however, The immunohistochemistry markers for synovial sarco- they have also been reported to be malignant tumours of ma in our study were cytokeratin (focal), mic-2, bcl-2 and non-epithelial tissue by paediatric oncologists [14]. calponin. Although cytokeratin is regarded as the most They have a cytogenetic translocation of (X;18) (p11; useful single marker for synovial sarcoma, it is not always q11) and stain positively for keratin [1, 15]. This transloca- reliable. Cytokeratin subsets, such as bcl-2, CD56, calponin, tion involves the SYT gene on chromosome 18 and one of beta-catenin, have been useful in synovial sarcoma and mic- several highly homologous genes (SSX 1, SSX 2 and SSX 2 has been associated with PNET/ES [15, 19]. 4) on X chromosome [1, 2, 14]. Disease-specific survival in Our patient underwent radiotherapy cycles consisting of synovial sarcoma depends on age, primary tumor size, mar- 30 gy in ten fractions. This was in accordance to the stan- gin of resection, mitotic activity, bone or neurovascular dard radiotherapy administered to such patients [20, 21]. invasion, histologic subtype, overexpression of the cellular The recurrence rate is high in synovial sarcomas, and the tumor antigen p53, the rate of cell proliferation (as assessed lesion characteristically metastasizes to the lymph nodes, bones
Fig. 2 Lateral radiograph of thoracic spine (pre-operative) Fig. 4 MRI image of the cervicothoracic spine Eur Orthop Traumatol (2013) 4:161–164 163
Fig. 7 Histopathology picture of synovial sarcoma
Fig. 5 Anteroposterior radiograph of the cervicothoracic spine (post-
operative) in one case. Fourteen patients died of disease at a median of 18 months from diagnosis, and two were alive with progres- sive disease at the time of the report [16]. Thus, metastatic and lungs [1, 2, 9]. Metastases of synovial sarcoma to the spine synovial sarcoma has a poor prognosis. are extremely rare, and data in the literature consist primarily of Complete surgical resection of primary synovial sarcoma, case reports. Spinal cord compression due to spinal metastasis, if possible remains the treatment of choice [2, 26]. Excellent an emergent oncologic event, occurs in approximately 3 % of control by adjuvant radiotherapy has been reported [26, 27]. patients with extraspinal soft tissue sarcomas, with the lumbar– How a patient would respond to chemotherapy is an objec- sacral spine being the most common level (42 %), followed by tive assessment. However, there is one study which showed the thoracic spine (46 %) and the cervical spine (12 %) [7]. After no detectable beneficial effects on survival in the subset of the diagnosis of spinal cord compression in soft tissue sarcomas, patients treated with chemotherapy vs. those who received the survival rate is 1 to 21 months, with a median rate of no chemotherapy [18]. Some other studies showed an im- 5 months [7]. There is only one reported case of synovial provement in metastasis-free survival in patients treated sarcoma originating from the lumbar spine [22] and few cases with chemotherapy [16, 17]. If there are any local recurren- of metastasis to the spine [7–9, 22]. In a series of 59 patients, ces, they can be treated with limb sparing surgery. Bilsky et al. [23] noted three spindle cell sarcoma metastases to In our opinion, synovial sarcoma metastasis to the spine the spine but did not specify the levels. In a series of 19 patients, should be a possibility which one should keep in mind when Merimsky et al. [7] reported three patients with spinal metasta- dealing with patients who present with back pain and sud- ses, to T7–T8, C2–C3 and S1–S3. Three separate case reports den neurological deterioration. As in our case, a timely documented metastasis to the lumbar vertebra [5, 24] and to the surgical interventional and adequate decompression may thoracic vertebra [8, 25]. result in neurological recovery. In a study of 271 patients with synovial sarcoma done by Ferrari et al., 16 developed metastases. Metastases affected Acknowledgments We acknowledge the valuable inputs given by the lung in 14 cases, bone and lung in one case and the liver Dr. John Ryan (consultant histopathologist at Our Lady of Lourdes Hospital, Drogheda, Ireland) and Dr. Rupal Chedda (consultant radio- therapist at FORTIS Hospitals, Mulund, Maharashtra, India).
Funding No funding was received in support of this study.
References
1. Clifford R, Wheeless III. Synovial Sarcoma. Wheeless’ Textbook
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