CC BY-NC-ND 4.0 · Indian Journal of Neurosurgery 2018; 07(03): 216-219
DOI: 10.1055/s-0037-1603810
Case Report
Neurological Surgeons' Society of India

Extradural Dorsal Spinal Paraganglioma: A Rare Case Report and Literature Review

Mukesh K. Bhaskar
1  Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India
,
R. Kumar
1  Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India
,
Sunil K. Singh
1  Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India
,
Mukta Meel
2  Department of Pathology, RNT Medical College, Udaipur, Rajasthan, India
› Author Affiliations
Further Information

Address for correspondence:

Mukesh Kumar Bhaskar, MS
Department of Neurosurgery, King George's Medical University
Lucknow, Uttar Pradesh 226003
India   

Publication History

Received: 06 June 2016

accepted: 20 June 2016

Publication Date:
22 September 2017 (online)

 

Abstract

Paragangliomas are neuroendocrine tumors originating from specialized cells of neural crest derivative. They are mostly found in the carotid and jugulotympanic region. Paragangliomas are extremely rare in the spine, and in literature, most cases are described as intradural extramedullary lesion in the cauda equine or filum terminale region. Extradural dorsal spine paragangliomas are even rarer, and till date, only 11 cases are reported in available literature. We describe a case of an adult woman who presented with mid dorsal region pain and sensory-motor spastic paraplegia. Imaging features were suggestive of D5 Pott's spine with epidural granulation tissue. Perioperatively, the tumor was moderately vascular and complete excision done. On HPE and IHC, diagnosis of paraganglioma was made. There was no evidence of clinical recurrence of the lesion at 6 months follow-up.


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Introduction

Paragangliomas are neuroendocrine tumors of the extra-adrenal paraganglionic system. They are derived from chemoreceptor cells and usually seen in the carotid body and glomus jugulare, and their location in the spinal canal is rare. Most of them are found intradural, predominantly at the cauda equina.[1] [2] Epidural compression of the spinal cord by paraganglioma involving the dorsal spine occurs very rarely, and only a few number of cases have been reported in literature.[3] These tumors are very rarely encountered in adolescents and adults, with the peak incidence in the fifth decade.[4] We report a case of extradural dorsal spine paraganglioma in a 32-year-old woman.


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Case History

A 32-year-old woman presented with a history of backache in the mid-dorsal region for 7 months and weakness of both the lower limbs for 1 month. General physical and systemic examinations were within normal limits. Neurologic examination showed spastic paraplegia (0/5), hyperreflexia, and complete sensory loss in D5 and below. All the routine laboratory investigations including viral markers and chest X-ray were normal. Magnetic resonance imaging (MRI) of the dorsal spine revealed D5 vertebral body collapsed with lost intervening disc space and end plate irregularities with heterogenous postcontrast enhancement along with D4–6 pre- and paravertebral soft tissue heterogenous enhancement ([Fig. 1A–E]). There was a preoperative impression of Pott's spine, and the patient prepared for surgery.

Zoom Image
Fig. 1 Magnetic resonance images: (A) T1 sagittal image, (B, C) T2 sagittal and coronal image, and (D, E) contrast sagittal and axial image.

D5 laminectomy with complete excision of lesion, and D3–7 pedicle screw and rod fixation was done. The tumor was extradural, moderately vascular, bluish red, and soft to firm in consistency with complete collapse of D5 vertebral body. It was contiguous with the spinal roots but not adherent to the dura mater. The patient had an uneventful postoperative period and some sensory improvement in early postoperative period. The histopathologic examination (HPE) of the specimen showed neoplastic chief cells arranged in organized pattern, peripherally lined by sustentacular cells separated by fibrous septa (Zellballen's pattern). Cells have nuclei with stippled chromatin, inconspicuous nucleoli, and eosinophilic granular to clear cytoplasm ([Fig. 2A]). Immunohistochemistry (IHC) was performed for confirmation; positive reaction for chromogranin in the chief cells and S100 nuclear positivity in sustentacular cells ([Fig. 2B, C]) were seen. Based on HPE and IHC, morphologic diagnosis of paraganglioma was established. There was no evidence of clinical recurrence of the lesion at 6 months follow-up, but later on the patient was lost to follow-up.

Zoom Image
Fig. 2 Histopathology and immunohistochemistry images: (A) HPE ×100 and ×200, (B) S100 positive ×100 and ×200, and (C) chromogranin positive.

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Discussion

Paraganglioma is a rare neuroendocrine neoplasm that may develop at various locations such as the head, neck, chest, and abdomen. The term “paraganglioma” was first given by Lerman and colleagues in 1972.[5] Extra adrenal tumors are mainly found in the jugulotympanic region, and unusual intracranial locations include sellar, pineal, and petrous ridge regions.[1] [6] The spinal location is rare. Most paragangliomas are found intradural, predominantly at the lumbosacral region.[2] Only 11 patients with extradural dorsal spine paraganglioma have been reported in the literature ([Table 1A–E]).[3] [7] [8] [9] [10] [11] [12] [13] [14]

Table 1

Extradural dorsal paraganglioma: review of literature

No.

Year

Author

Sex

Age

Site

Clinical features

Surgical treatment

Abbreviations: F, female; M, male; mo, month; wk, week.

1

1983

Böker et al[7]

F

35

T4–5

2 mo back pain, 4 wk paraparesis and sensory loss below T4

Total resection

2

1983

Böker et al[7]

F

36

T11

10 mo back pain, sudden paraparesis and sensory loss below T11

Incomplete resection

3

1991

Cybulski et al[3]

M

34

T8

4 mo midthoracic back pain, sudden paresthesia and stiffness at lower limbs

Stage 1: Incomplete resection

Stage 2: Embolization

Stage 3: Total resection

4

1996

Fitzgerald et al[8]

M

30

T4

Back pain, numbness below T5, sustained ankle clones in both legs

Total resection

5

1996

Noorda et al[9]

F

52

T7–9

2 y neuralgia in thoracic spine and lower extremities weakness, T7 local tenderness and decreased sensation

Stage 1: Embolization

Stage 2: Total resection

Stage 3: Anterior stabilization

6

2001

Shin et al[10]

M

43

T6

1 y recurrent back pain, sudden paresthesia of the lower extremities, urinary incontinence

Stage 1: Embolization

Stage 2: Resection

7

2001

Shin et al[10]

F

67

T11

Back pain for several years, Lower abdominal pain for 1 y

Incomplete resection

8

2002

Houten et al[11]

M

41

T9

2 wk gait disturbance and lower limbs, weakness, diminished sensation under T10, lower limbs hyperreflexia

Incomplete resection

9

2003

Jeffs et al[12]

F

53

T12

10-mo history of headaches, facial flushing and palpitations associated with hypertension, mild hypesthesia in the right T12 dermatome

Grossly complete excision

10

2006

Conti et al[13]

F

43

T1–4

Sudden spastic incomplete paraplegia and paresthesia at the lower limbs

Resected en bloc

11

2012

Mauricio et al[14]

M

26

T10

Diffuse pain in thoracic region, with progressively diminished muscle strength and paresthesia in the left lower limb

Tumor resection with decompression and stabilization

Common clinical presentation of patients with spinal paragangliomas are back pain radiating down the leg, motor-sensory deficits, and bowel and bladder irregularity.[7] [15] The clinical presentation depends on the level of affected spinal cord and the degree of cord compression. Our patient presented with back pain in the mid-dorsal region and weakness of both the lower limbs. MRI finding of paragangliomas is common with other spinal tumors such as schwannomas, ependymomas, meningiomas, and dermoid, and paragangliomas appear isointense on T1-weighted images whereas hyperintense on T2-weighted images with contrast enhancement.[16]

Microscopically paragangliomas show typical nuclear monomorphism and “Zellballen's” pattern (large polyhedral chief cells arranged in nests), with granular argyrophilic cells. Perivascular pseudorosettes, areas of hemorrhage, and necrosis may also be seen. Immunohistochemistry is positive for chromogranin, synaptophysin, neuron-specific (gamma) enolase, and S-100.[17] Most paragangliomas are benign and cured by surgical removal. Only 3% have malignant potential with tendency to distant metastasis.[18] Therefore, after gross total excision of tumor prognosis is good, and radiotherapy is required in case of locally invasive tumors or when complete excision of tumor is not achieved.

Although extremely uncommon, extradural dorsal spine paraganglioma should be kept as a differential diagnosis for epidural compressive lesions. Spinal paragangliomas are mostly benign neoplasms, and a complete surgical resection provides recurrence-free survival. Generous reporting of such type of cases helps us in better understanding of such a rare entity.

Note

The data required for the manuscript was provided by Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India.


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Conflict of Interest

None.

Acknowledgment

The authors sincerely thank Prof. B. K. Ojha, Prof. Anil Chandra, Dr. C. Srivastava, Dr. Manish Jaiswal, and Dr. Somil Jaiswal for their constant support and help in preparation of the manuscript including submission procedures.


Address for correspondence:

Mukesh Kumar Bhaskar, MS
Department of Neurosurgery, King George's Medical University
Lucknow, Uttar Pradesh 226003
India   


  
Zoom Image
Fig. 1 Magnetic resonance images: (A) T1 sagittal image, (B, C) T2 sagittal and coronal image, and (D, E) contrast sagittal and axial image.
Zoom Image
Fig. 2 Histopathology and immunohistochemistry images: (A) HPE ×100 and ×200, (B) S100 positive ×100 and ×200, and (C) chromogranin positive.