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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 4 | Page : 802-805
Clear cell sarcoma of the paraspinal ligament – A rare tumor at an unusual location: A review
Sharad Pandey1, Anirudha Puntambekar2, Vivek Sharma1, Kulwant Singh1, Deepa Santhosh3, Amrita Ghosh3, Abhijeet Kunwar4
1 Department of Neurosurgery, Sir SunderLal Hospital, IMS, BHU, Varanasi, Uttar Pradesh, India
2 Department of Pathology, Ruby Hall Clinic, Grant Medical Foundation, Pune, Maharashtra, India
3 Department of Pathology, Sir SunderLal Hospital, IMS, BHU, Varanasi, Uttar Pradesh, India
4 Department of Orthopedics, Trauma Centre, Sir SunderLal Hospital, IMS, BHU, Varanasi, Uttar Pradesh, India
|Date of Web Publication||5-Jul-2016|
Department of Neurosurgery, Sir SunderLal Hospital, IMS, BHU, Varanasi, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pandey S, Puntambekar A, Sharma V, Singh K, Santhosh D, Ghosh A, Kunwar A. Clear cell sarcoma of the paraspinal ligament – A rare tumor at an unusual location: A review. Neurol India 2016;64:802-5
|How to cite this URL:|
Pandey S, Puntambekar A, Sharma V, Singh K, Santhosh D, Ghosh A, Kunwar A. Clear cell sarcoma of the paraspinal ligament – A rare tumor at an unusual location: A review. Neurol India [serial online] 2016 [cited 2019 Feb 16];64:802-5. Available from: http://www.neurologyindia.com/text.asp?2016/64/4/802/185353
Clear cell sarcoma (CCS) was first described by Enzinger in 1965. It is a rare, aggressive soft-tissue tumor comprising approximately 1% of all soft tissue sarcomas. Due to its close clinical and histological similarity with a malignant melanoma, Chung and Enzinger proposed the name “malignant melanoma of soft parts.” It is mostly seen in young adults with the median age of presentation around 30 years. This tumor typically arises in the deep soft tissues of the distal extremities particularly the foot and ankle, although cases presenting in the kidney, trunk, penis, gastrointestinal tract, and head and neck have also been reported. We report a CCS located extradurally at the thoracic spine (D3/D4) level in a 19-year-old female patient who presented with progressive paraparesis. On reviewing the literature, we found our case to be the second case of CCS in the paraspinal ligament and the fourth case of CCS of the thoracic region.
A 19-year-old female patient presented with complaints of backache over the upper thoracic region, and tingling and numbness in both lower limbs for 4 months. She also complained of weakness in bilateral lower limbs for the past 4 months, which was progressive in nature. She had no complaints in both the upper limbs. No bowel and bladder complaints were present. On neurological examination, power was 3/5 in both lower limbs. Light touch and pinprick sensations were present but other sensations were grossly absent. Deep tendon reflexes were exaggerated in both the lower limbs with ankle clonus. No cutaneous melanotic lesions, subcutaneous nodules around large joints, or abdominal mass (especially mass lesions in the kidneys) were found.
Plain and contrast magnetic resonance imaging (MRI) revealed a well-defined extradural mass lesion measuring 3.2 cm × 1.7 cm × 1.3 cm posterior to the thoracic spinal cord at D3–D4 level with a small right neural foraminal extension at the same level. The mass showed an isointense signal intensity on T1-weighted images and iso- to hypo-intensity on T2-weighted images, which on post-gadolinium study, showed a moderate, nonhomogenous enhancement [Figure 1]. There was associated mass effect leading to compressive thoracic myelopathy. Ultrasonography of abdomen, and X-ray chest and dorsolumbar spine were normal. There was no history of melanoma or other skin lesions.
|Figure 1: (a) Magnetic resonance image showing a well-defined extradural mass lesion posterior to the thoracic spinal cord at D3–D4 level. (b) Lesion showing an isointense to hypointense signal intensity on T1- and T2-weighted images with right neural foraminal extension. (c) Postgadolinium study-lesion showing moderate nonhomogenous enhancement|
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Elective neurosurgical intervention was planned and D3–D4 laminectomy with complete excision of the mass lesion was done. Intraoperatively, the mass lesion was firm, well-circumscribed, and grayish-white in color. The mass was extradural and showed a small right neural foraminal extension at the D3–D4 level. The excised mass on histopathological examination (HPE) was reported to be a tumor with tightly packed nested and fascicular arrangement infiltrating adjacent fibrocollagenous tissue. Nested tumor cells were large, polygonal, having a vesicular nuclei with a single prominent nucleolus and clear cytoplasm, with transition to spindle-shaped cells with a brisk mitotic activity and no neural differentiation. Tumor cells revealed melanin pigment at places. On cytohistochemistry, it showed a strong positivity for vimentin, S-100, and human melanoma black-45 (HMB-45) and was negative for actin and desmin [Figure 2]. Based on these findings, the patient was diagnosed as a case of a primary CCS of spinal ligament at the D3–D4 level. The postoperative period was uneventful and the patient was referred to the Radiotherapy Department for adjunctive radiotherapy.
|Figure 2: (a) Microphotograph showing nested pattern of polygonal tumor cells having a vesicular nuclei, a single prominent nucleolus, and clear cytoplasm. Melanin pigment is also noted (Hematoxylin and Eosin ×400), (b and c) Immunohistochemistry showing strong cytoplasmic reactivity with human melanoma black-45 (HMB-45) and S-100 stains (DAB, ×400)|
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CCS is a rare tumor, accounting for approximately 1% of all soft tissue sarcomas. It mainly affects young adults in the 20–40-year age group with a median age of 30 years. It presents as a slow-growing, often painful lesion. The principal sites of the neoplasm are the extremities, especially the region of the foot and ankle, followed by the knee, thigh, and hand., Nevertheless, a few reports of CCS involving unusual sites such as the pleura, chest wall, nerve roots of the spinal cord at different levels, scapula, retroperitoneum, buttock, and mediastinum have also been documented.,,,,,,,,
We are reporting the second case of CCS originating from the spinal ligament. In 2008 Gollard et al., reported the first case of CCS in a 38-year-old female presenting with a paravertebral mass at the D5–D6 level measuring 2.0 cm × 5.0 cm. In this case, the histopathological examination revealed a tumor with a clear cell pattern with no melanin pigment and a strong positivity for S-100 and HMB-45. Three more cases of CCS of different origin involving the thoracic region have been reported by Parker et al., Chung and Enzinger, and Kim et al. [Table 1].,,
|Table 1: Reported cases of clear cell sarcoma in the paraspinal region till date|
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Histologically, CCS consists of compact nests or fascicles of predominantly fusiform or spindled cells with a clear cytoplasm bordered and defined by a delicate framework of fibrocollagenous tissue contiguous with the adjacent tendons or aponeuroses. The cells have highly distinctive features consisting of nuclei with a vesicular nuclear chromatin pattern and prominent basophilic nucleoli reminiscent of a malignant melanoma. The cytoplasm varies from being clear to weakly eosinophilic and contains large amounts of intracellular glycogen. More than 50% of cases contain multinucleate (wreath-type) giant cells. Approximately two-thirds of the tumors contain a variable amount of melanin pigment demonstrable on hematoxylin and eosin or, more often, by the use of special stains.
The distinction of CCS from other melanin-producing lesions can be more problematic and may require correlation of the histological, clinical, and molecular data. Main differentials in the present case included melanotic tumors such as metastatic malignant melanoma, malignant blue nevus, malignant peripheral nerve sheath tumor, and paraganglioma-like dermal melanocytic tumor. Nonmelanotic differentials include a perivascular epithelioid cell tumor and an epithelioid leiomyosarcoma [Table 2].,,
CCS is characterized by a recurrent chromosomal translocation t (12; 22), which results in fusion of the EWS gene on 22q with the ATF1 gene on 12q. This genomic abnormality may represent a good marker for identifying these tumors.
CCS has traditionally been considered as an “ungradable” sarcoma and for that reason, several studies have attempted to identify other prognostic factors. Size of the tumor and the presence of necrosis have proved to be the most robust prognostic factors. Tumors >5 cm in size have a significantly worse outcome than those that are smaller. Other factors such as age, location, depth, or proliferation index have been found to be independent prognostic factors. Radical surgery is the mainstay of therapy, and chemotherapy has proven to have little efficacy.,,
CCS, a rare malignancy, should be considered in the differential diagnosis of patients presenting with a soft tissue mass. Although it is an uncommon neoplasm, it can involve any ligament, tendon, or aponeurosis. Thus, radiological and histopathological evaluation are the key to the diagnosis. Complete surgical excision with radiotherapy is the mainstay of treatment.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]