Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 4  |  Page : 520-524  

Paravertebral and epidural sarcoma with spinal cord compression in a child: Case report and review of the literature


1 Department of Radio-diagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
2 Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India

Date of Web Publication14-Jul-2015

Correspondence Address:
Pratiksha Yadav
Department of Radio-diagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.160825

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  Abstract 

Spinal cord compression is a rare but serious complication of malignancy in the children. Sarcoma presented with epidural mass, and spinal cord compression is very rare. We present a case of paraspinal and epidural rhabdomyosarcoma (RMS) causing acute spinal cord compression. Parents of an 18-month-old male child brought him with complaints that the child had difficulty in standing and he was unable to walk. There was a soft swelling on the upper back. Ultrasonography was done which showed a soft tissue mass. Radiograph also showed a swelling on the upper back. Magnetic resonance imaging was advised for further evaluation, and it was detected an epidural and left paraspinal mass which was causing severe spinal cord compression. Diagnosis of sarcoma was suggested; differential diagnosis of RMS and Ewing's sarcoma was given. Biopsy was done, and it showed RMS. Partial excision of epidural mass was done, and then chemotherapy and radiotherapy was done.

Keywords: Ewing′s, rhabdomyosarcoma, sarcoma, spinal cord compression


How to cite this article:
Yadav P, Gujrati A, Buch A. Paravertebral and epidural sarcoma with spinal cord compression in a child: Case report and review of the literature. Med J DY Patil Univ 2015;8:520-4

How to cite this URL:
Yadav P, Gujrati A, Buch A. Paravertebral and epidural sarcoma with spinal cord compression in a child: Case report and review of the literature. Med J DY Patil Univ [serial online] 2015 [cited 2024 Mar 28];8:520-4. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2015/8/4/520/160825


  Introduction Top


Cord compression can occur in children in 4% of malignant lesion.

Primary intraspinal sarcomas are very rare in children. [1] Ewing's sarcoma is the most common sarcomatous tumor that cause metastatic spinal cord compression. [2] Rhabdomyosarcoma (RMS) is most common sarcoma in pediatric patients, which is highly aggressive and rapidly growing tumor. Primary sarcoma rarely involves the epidural space in children. [3] Acute spinal cord compression in the children can occur in the malignancy. It can be due to direct spread of the tumor, spread to the epidural space by infiltration of the vertebral foramina or metastatic vertebral bone disease with cord compression. Acute cord compression usually caused by sarcoma (RMS and Ewing's account up to 65% of cases). Neuroblastoma, lymphoma, and leukemia accounts for most of the rest. Usually, cord compression occurs in the terminal stage of malignancy but rarely patient can present with cord compression. Pain is the most common symptom of the epidural spinal cord compression. Pain radiates to legs, arms or chest depending upon the level of the lesion. Pain is usually associated with upper or lower motor neuron weakness. Sensory disturbances were seen in severe spinal cord compression. Patients may have bladder or bowel complaints.


  Case Report Top


Parents of an 18-month-old male child brought him in our hospital that the child had difficulty to stand and were unable to walk since 4 months. His mother told that child was perfectly all right 4 months back and was able to stand and walk without support. Child was born full-term by normal delivery, and his birth weight was 2.8 kg. All the milestones were normal. On examination, there was swelling seen on the upper back at left paraspinal region. Swelling was firm on palpation, nonpulsatile, nontender. Dorsal spine radiograph was advised which showed a soft tissue swelling on the upper back however vertebrae were unremarkable [Figure 1]. Ultrasonography (USG) was done, and it showed an ill-defined soft tissue mass seen in the left paraspinal region, no cystic component or calcification was seen. Magnetic resonance imaging (MRI) was advised for further evaluation, which showed an extradural, extramedullary mass lesion at the level of D5, and it was extending till superior part of D11 vertebral body. It measured approx. 6.2 cm (craniocaudal) × 1.2 cm (anteroposterior) × 1.1 cm (transverse) dimensions [Figure 2]a]. It was isointense to the spinal cord in T1-weighted image (T1WI) [Figure 2]b] and showed medium signal intensity on T2-weighted image (T2WI) [Figure 2]d]. This mass was extending into the left neural foramina and was causing widening of the neural foramina. This extradural mass was severely compressing the spinal cord and obstructing cerebrospinal fluid flow, which was seen on MRI myelogram [Figure 2]c]. It showed restriction on diffusion weighted image [Figure 3]a and b]. There was a heterogeneous soft tissue mass seen in the left paraspinal region, which is continuous with the extradural mass. On postgadolinium (Gd) injection, MRI, extradural and paraspinal mass showed heterogeneous enhancement [Figure 3]c and d]. Dorsal vertebrae were normal. No vertebral destruction seen. On the basis of MRI findings, diagnosis of paravertebral and epidural sarcomatous mass was suggested. Differential diagnosis of RMS and extraskeletal Ewing's sarcoma was given. Biopsy of the mass was done and sent for histopathology evaluation, which has confirmed the diagnosis of RMS.
Figure 1: Radiograph dorsal spine (a) lateral view showing a soft tissue swelling over upper back. No calcifi cation seen. Dorsal spine is normal. (b) Antero-posterior view showing normal dorsal spine

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Figure 2: A soft tissue mass involving the epidural space, left foramina and left paraspinal space (a) T2-weighted image (T2WI) sagittal image showing well defi ned epidural mass at the level of D5-D11, it is severely compressing spinal cord. (b) T1-weighted image axial image showing the mass is infi ltrating into the left neural foramina, and it is in continuation with a left paraspinal mass. (c) Magnetic resonance imaging myelogram showed severe compression of the spinal cord by epidural mass. (d) T2WI axial image showing mixed signal intensity mass in the left paraspinal region infi ltrating the left neural foramina and in the epidural space

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Figure 3: (a) Diffusion weighted image showing hyperintense signal intensity of the epidural mass which showed corresponding low signals on ADC. (b-d) Axial and sagittal postcontrast images showing heterogenous enhancement

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Histopathology

The section showed sheets of oval to spindle-shaped cells having granular eosinophilic cytoplasm and large elongated hyperchromatic nuclei. Few cells were large, oval with abundant eosinophilic cytoplasm and eccentrically placed nuclei. Few large elongated cells resembled strap cells. There were large areas of hemorrhage and necrosis. The intervening tissue showed dense mixed inflammation. Lack of primitive round cells, presence of eccentrically placed nuclei, eosinophilic granular cytoplasm and presence of spindling and strap-like cells favored the diagnosis of RMS over Ewing's sarcoma on histopathology [Figure 4]. Immunohistochemistry (IHC) done outside was positive for myogenin and negative for CD99, confirming the diagnosis of RMS.
Figure 4: Histopathology: (a) Sheets of oval to spindle shaped neoplastic cells (H and E, ×100). (b) Cells having large hyperchromatic nuclei and eosinophilic cytoplasm (H and E, ×400). (c) Large oval cells with eccentric nuclei (arrow) (H and E, ×400). (d) Areas of hemorrhage and necrosis and strap like cell (arrow) (H and E, ×400)

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Patient underwent surgery for the partial excision of the epidural tumor to reduce the spinal cord compression surgery was done by posterior midline approach and excision of the epidural tumor was done, however the complete removal of the tumor was not possible approx. Seventy percent of the tumor was excised, and there was a significant reduction of the spinal cord compression.

Child was referred to the pediatric oncologist. Combined chemotherapy and radiotherapy was given. Vincristine, actinomycin D, and cyclophosphamide were given for six cycles. Radiotherapy 36 Gy was given to the involved site. Child was clinically improved after that.


  Discussion Top


Rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft tissue sarcoma of the pediatric age group accounting for 5-8% of childhood cancer. [4] In children, the age-standardized annual incidence rate for RMS is between 4 and 7/million. [5] Forty percent of RMS arise from the head and neck region; around 15% are genitourinary (GU) nonbladder prostate (BP) tumors (i.e., paratesticular, vaginal and uterine tumors), 10% are BP tumors, 15% occur in the limbs, and 20% occur in other sites (i.e., thoracic or abdominal tumors).

Rhabdomyosarcoma is a fast-growing, primitive, high-grade, malignant mesenchymal tumor. It presents in a wide range of histologic types. There are various patterns of spread of tumor. Histologically, it contains a mixture of rhabdomyoblasts, which are recognized by their typical cross-striations, and undifferentiated cells. Most extremity tumors are alveolar or undifferentiated histologic types, unlike the embryonal or botryoid types found in the face and neck and GU system. Prognosis is bad for patients with RMS of the extremities than for those with tumors arising from the GU system or the head and neck region. In the extremities, the tumors are deep and tend to spread along fascial planes.

These tumors present with a wide range of symptom, depending upon the location of the primary tumor, but in general presenting symptoms often are indolent, with nonspecific symptoms. Limb and trunk tumors often present with a painless swelling and/or with enlarged regional lymph nodes. In patients presenting with metastases more general symptoms of fatigue, weight loss, and low blood counts are noted. [6]

Imaging of the primary site with radiography, ultrasound (US), MRI or computed tomography (CT) scan is required in all patients as an initial investigation. [7] Once the diagnosis has been confirmed histologically, the most frequently involved metastatic sites should be investigated; CT of the lungs, imaging of regional lymph nodes, and a technetium bone scan are recommended in every patient.

Imaging findings

Radiography does not play any significant role in its diagnosis. Localized bony erosion adjacent to the primary site can be seen. This area may show increased uptake on technetium-99 m methylene diphosphonate (99 mTc-MDP) bone scan in the absence of metastatic disease in the skeleton. [8] USG is usually the first imaging investigation that is done in children with soft-tissue masses due to its easy availability. Lesion characterization can be done; its extent and vascularity of a mass can be assessed. On US, RMS in general seen as a well-defined, slightly hypoechoic inhomogeneous mass that can show significantly increased flow. In all other RMS locations, additional imaging using CT or MRI is needed. US can be useful in performing image-guided biopsies. [4] MRI is the imaging modality of choice for diagnosis of RMS due to its excellent soft tissue contrast resolution. The imaging characteristics of RMS are generally nonspecific. They have intermediate signal intensity on T1WI. On T2WI, they tend to be of intermediate-to-high signal intensity. If the tumor contains a high number of septa, it may present as a lobulated lesion. These tumors shows strong enhancement on postcontrast MRI imaging due to their highly vascular nature. In very rare instances, the tumor may show a predominantly cystic appearance. [8] Dynamic series are useful in order to assess tumor vascularity and to differentiate between postchemotherapy/surgery residual disease and fibrosis. [9] Vascular involvement is considered to be absent if there is a normal tissue plane visible between the tumor and the vessel, or if the tumor has a <180° circumferential relationship. If the tumor surrounds the vessel for more than 180°, it is considered to be encased. [4] Positron emission tomography-CT (PET-CT) evaluates uptake in metabolically active malignant cells, with the help of fluorine-18 fluorodeoxyglucose. The combination of PET with CT has a higher accuracy for the depiction of pulmonary metastases. [10] Differentiating RMS from other malignant tumors is difficult. Few differentials that should be considered are Ewing sarcoma, peripheral neuroectodermal tumors (PNET), infantile fibrosarcoma, and desmoplastic small round-cell tumors soft-tissue tumors in childhood.

Treatment of spinal RMS is a combined approach of surgery, radiotherapy and chemotherapy. Goal of surgery is excision of the epidural tumor with preservation of neurological function. [6] Prognosis depends on the age of the patient, extent of the tumor, tumor histology, and presence or absence of metastasis.

Ewing sarcoma

Ewing sarcoma, along with Askin tumor and PNET, is now regarded as a single entity (Ewing sarcoma family of tumors). [11] These lesions are characterized by various degrees of neuroectodermal differentiation and by common histologic, IHC, and molecular properties. [12]

Ewing sarcoma is the prototype of round, small cell malignancies of bone. It is the sixth most common malignant tumor, comprising approximately 11-12% of all malignant tumors of bone and soft tissues. [11] Conventional Ewing sarcoma is composed of densely packed small cells with round nuclei but without distinct cytoplasmic outlines. [12] Occasionally, these tumors can arise as primary neoplasms of soft tissue. These are known as extraskeletal or soft tissue Ewing sarcomas. [13] Ewing sarcoma occurs primarily in children. Approximately 90% of cases present before the age of 20 years, median age of presentation is about 13 years. [13] The tumor has a decisively male predominance (3:2), clinically, Ewing sarcoma may present as a localized, painful mass or with systemic symptoms such as fever, malaise, weight loss, leukocytosis, and increased erythrocyte sedimentation rate. [13] According to Ilaslan et al., 9.8% of all cases of Ewing sarcoma have a primary vertebral origin.

Imaging findings

In most cases, the radiographic presentation of Ewing sarcoma is quite characteristic. It consists of an ill-defined lesion with a permeative or moth-eaten pattern of bone destruction associated with a lamellated periosteal new bone formation that has an onion skin (or "onion peel") appearance, or, less commonly, a "sunburst" ("trimmed whiskers" effect) configuration, and a large soft tissue mass. Ewing sarcoma occasionally presents as a large area of geographic bone destruction, with or without matrix mineralization, simulating any other type of bone sarcoma. At times, the lesion in the bone is almost imperceptible, and the only prominent finding is a soft tissue mass. [12] MRI is essential for definite demonstration of the extent of intraosseous and extraosseous involvement by this tumor. [12] T1WI show intermediate to low signal intensity, which becomes bright on T2W. Hypocellular regions and areas of necrosis are of lesser intensity. [13] Imaging after injection of Gd-DTPA reveals signal enhancement of the tumor on T1W sequences. [12] Enhancement occurs only in the cellular areas, allowing differentiation of the tumor from the peritumoral edema. On radionuclide bone scan, Ewing sarcoma shows a very intense increase of 99 mTc-MDP accumulation. Gallium-67-citrate more readily identifies soft tissue tumor extension. [14]

Treatment of Ewing's sarcoma is multimodality approach. Surgical decompression for sarcoma cases which showed rapid neurological deterioration. A combined approach with chemotherapy, radiotherapy and surgery is the treatment of choice in the primary as well as metastatic spinal sarcoma.


  Conclusion Top


Rhabdomyosarcoma and extraskeletal Ewing's sarcoma are extremely rare epidural tumors in children. MRI is very a very important investigation for evaluation of tumor extent and severity of spinal cord compression, which is must for the treatment plan.

 
  References Top

1.
Klimo P Jr, Codd PJ, Grier H, Goumnerova LC. Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr 2009;4:222-9.  Back to cited text no. 1
    
2.
Klein SL, Sanford RA, Muhlbauer MS. Pediatric spinal epidural metastases. J Neurosurg 1991;74:70-5.  Back to cited text no. 2
    
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Khalatbari MR, Jalaeikhoo H, Hamidi M, Moharamzad Y. Primary spinal epidural rhabdomyosarcoma: A case report and review of the literature. Childs Nerv Syst 2012;28:1977-80.  Back to cited text no. 3
    
4.
Kim EE, Valenzuela RF, Kumar AJ, Raney RB, Eftekari F. Imaging and clinical spectrum of rhabdomyosarcoma in children. Clin Imaging 2000;24:257-62.  Back to cited text no. 4
    
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Ruymann FB, Grovas AC. Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas. Cancer Invest 2000;18:223-41.  Back to cited text no. 5
    
6.
Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Merks JH. Imaging findings in noncraniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2008;38:617-34.  Back to cited text no. 6
    
7.
Brisse H, McHugh K,Scaramuzza D. RMS and non-RMS soft tissue sarcomas. Radiological guidelines. EpSSG: Minutes of the ESPR Oncology Group 1 st Meeting Dublin 2005.  Back to cited text no. 7
    
8.
Sebire NJ, Roebuck DJ. Pathological diagnosis of paediatric tumours from image-guided needle core biopsies: A systematic review. Pediatr Radiol 2006;36:426-31.  Back to cited text no. 8
    
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Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma 1980. Clin Orthop Relat Res 2003;153:1006-20.  Back to cited text no. 9
    
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Jadvar H, Connolly LP, Fahey FH, Shulkin BL. PET and PET/CT in pediatric oncology. Semin Nucl Med 2007;37:316-31.  Back to cited text no. 10
    
11.
Bertoni F, Bacchini P, Ferruzzi A. Small round-cell malignancies of bone: Ewing's sarcoma, malignant lymphoma, and myeloma. Semin Orthop 1991;6:186-95.  Back to cited text no. 11
    
12.
Boyko OB, Cory DA, Cohen MD, Provisor A, Mirkin D, DeRosa GP. MR imaging of osteogenic and Ewing's sarcoma. AJR Am J Roentgenol 1987;148:317-22.  Back to cited text no. 12
    
13.
Coombs RJ, Zeiss J, McCann K, Phillips E. Case report 360: multifocal Ewing tumor of the skeletal system. Skeletal Radiol 1986;15:254-7.  Back to cited text no. 13
    
14.
Dehner LP. Primitive neuroectodermal tumor and Ewing's sarcoma. Am J Surg Pathol 1993;17:1-13.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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