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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 4  |  Page : 802-805

Clear cell sarcoma of the paraspinal ligament – A rare tumor at an unusual location: A review

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 Publication5-Jul-2016

Correspondence Address:
Sharad Pandey
Department of Neurosurgery, Sir SunderLal Hospital, IMS, BHU, Varanasi, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.185353

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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 2020 Apr 9];64:802-5. Available from:


Clear cell sarcoma (CCS) was first described by Enzinger in 1965.[1] 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.”[2] 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.[3] 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.[4] 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.[5],[6] Nevertheless, a few reports of CCS involving unusual sites such as the pleura,[7] chest wall,[8] nerve roots of the spinal cord at different levels, scapula, retroperitoneum, buttock, and mediastinum have also been documented.[7],[8],[9],[10],[11],[12],[13],[14],[15]

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.[16] 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].[2],[9],[17]
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.[4] 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.[18]

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].[2],[4],[9]
Table 2: Differential diagnosis

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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.[19]

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.[20],[21],[22]

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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Conflicts of interest

There are no conflicts of interest.

  References Top

Katenkamp D, Perevoshchikov AG, Raikhlin NT. Soft tissue clear cell sarcoma. Morphology, differential and tumor classification. Zentralbl Allg Pathol 1984;129:521-31.  Back to cited text no. 1
Chung EB, Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell sarcoma. Am J Surg Pathol 1983;7:405-13.  Back to cited text no. 2
Dim DC, Cooley LD, Miranda RN. Clear cell sarcoma of tendons and aponeuroses: A review. Arch Pathol Lab Med 2007;131:152-6.  Back to cited text no. 3
Weiss MD, Goldblum JR. Clear cell sarcoma of tendon and aponeurosis. In: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. Philadelphia, PA: Mosby Elsevier; 2008. p. 926-33.  Back to cited text no. 4
De Beuckeleer LH, De Schepper AM, Vandevenne JE, Bloem JL, Davies AM, Oudkerk M, et al. MR imaging of clear cell sarcoma (malignant melanoma of the soft parts): A multicenter correlative MRI-pathology study of 21 cases and literature review. Skeletal Radiol 2000;29:187-95.  Back to cited text no. 5
Gandolfo N, Martinoli C, Cafiero F, Peressini A, Nicolò M, Dellachà A, et al. Malignant melanoma of soft tissues (clear cell sarcoma) of the foot. Is MRI able to perform a specific diagnosis? Report of one case and review of the radiological literature. Anticancer Res 2000;20:3993-8.  Back to cited text no. 6
Bury T, Hermans G, Alexis-Agnant R, Chevalier P, Limet R, Bartsch P. Clear cell sarcoma: An extremely rare cause of pleural disease. Eur Respir J 1997;10:2653-6.  Back to cited text no. 7
Fujiwara K, Nanjou S, Ishihara S. Clear cell sarcoma of the chest wall; report of a case. Kyobu Geka 2006;59:1119-22.  Back to cited text no. 8
Parker JB, Marcus PB, Martin JH. Spinal melanotic clear-cell sarcoma: A light and electron microscopic study. Cancer 1980;46:718-24.  Back to cited text no. 9
Sengöz A, Tasdemiroglu E, Togay H. Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report. Neurosurg Focus 2006;21:E11.  Back to cited text no. 10
Kazakos CJ, Galanis VG, Giatromanolaki A, Verettas DA, Sivridis E. Clear cell sarcoma of the scapula. A case report and review of the literature. World J Surg Oncol 2006;4:48.  Back to cited text no. 11
Katabuchi H, Honda R, Tajima T, Ohtake H, Kageshita T, Ono T, et al. Clear cell sarcoma arising in the retroperitoneum. Int J Gynecol Cancer 2002;12:124-7.  Back to cited text no. 12
Takamura S, Teraki Y. Case of clear cell sarcoma in the left buttock in which serum neuron-specific enolase was a useful marker for monitoring disease progression. J Dermatol 2015;42:621-4.  Back to cited text no. 13
Tirabosco R, Lang-Lazdunski L, Diss TC, Amary MF, Rodriguez-Justo M, Landau D, et al. Clear cell sarcoma of the mediastinum. Ann Diagn Pathol 2009;13:197-200.  Back to cited text no. 14
Tanaka Y, Yoshimasu T, Oura S, Hirai Y, Kawago M, Okamura Y. Primary clear-cell sarcoma in the mediastinum. Case Rep Oncol 2014;7:306-9.  Back to cited text no. 15
Gollard R, Hussong J, Bledsoe J, Rosen L, Anson J. Clear cell sarcoma originating in a paraspinous tendon: Case report and literature review. Acta Oncol 2008;47:1593-5.  Back to cited text no. 16
Kim DH, Choi KH, Cho YD. Clear cell sarcoma of the upper thoracic back muscle. J Korean Neurosurg Soc 2009;45:112-4.  Back to cited text no. 17
Fletcher CD. Peripheral neuroectodermal tumors. Diagnostic Histopathology of Tumors. 4th ed., Ch. 27. Philadelphia, PA: Elsevier/Saunders 2013. p. 2056-8.  Back to cited text no. 18
Mavrogenis A, Bianchi G, Stavropoulos N, Papagelopoulos P, Ruggieri P. Clinicopathological features, diagnosis and treatment of clear cell sarcoma/melanoma of soft parts. Hippokratia 2013;17:298-302.  Back to cited text no. 19
Lucas DR, Nascimento AG, Sim FH. Clear cell sarcoma of soft tissues. Mayo Clinic experience with 35 cases. Am J Surg Pathol 1992;16:1197-204.  Back to cited text no. 20
Montgomery EA, Meis JM, Ramos AG, Frisman DM, Martz KL. Clear cell sarcoma of tendons and aponeurosis: A clinicopathologic study of 58 cases with analysis of prognostic factors. Int J Surg Pathol 1993;1:89-100.  Back to cited text no. 21
Sara AS, Evans HL, Benjamin RS. Malignant melanoma of soft parts (clear cell sarcoma). A study of 17 cases, with emphasis on prognostic factors. Cancer 1990;65:367-74.  Back to cited text no. 22


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