J Korean Soc Spine Surg.  2017 Jun;24(2):109-114. 10.4184/jkss.2017.24.2.109.

A Long, Solitary, Rosary-Shaped Spinal Neurofibroma

Affiliations
  • 1Department of Orthopaedic Surgery, Soonchunhyang University Seoul Hospital, Seoul, Korea. schsbj@schmc.ac.kr

Abstract

STUDY DESIGN: Case report.
OBJECTIVES
We report the case of a long, solitary, rosary-shaped neurofibroma that was misdiagnosed as another disease due to the patient's surgical history involving repetitive procedures and its abnormal appearance. SUMMARY OF LITERATURE REVIEW: Neurofibroma is an intradural-extramedullary spinal tumor. It is generally not difficult to diagnose due to its frequent occurrence and specific magnetic resonance imaging (MRI) findings. However, to date, neurofibromatosis stigmata and long, solitary, rosary-shaped neurofibromas have rarely been reported.
MATERIALS AND METHODS
A 60-year-old woman was admitted to our hospital due to persistent pain, despite previous surgery and repetitive procedures. On physical examination, vision loss, hearing loss, skin discoloration, or subcutaneous nodules were not observed. A neurologic examination revealed normal motor and sensory function and voiding sensation. No pathologic reflexes such as the Babinski sign were observed. Previous sequential MRIs revealed intradural lesions that progressed from the thoracic vertebra 11 to the lumbar vertebra 3. She had no signs of neurofibromatosis stigmata, and the neurologic examination was unremarkable. The initial diagnosis was based on serial MRIs, which revealed a parasite infestation, a spinal cord tumor (myxopapillary-type ependymoma with hemorrhage), arachnoiditis, and vascular malformations. Total mass excision was performed, and the final diagnosis was neurofibroma.
RESULTS
There were no signs of a tumor remnants or local recurrence in a 1-year follow-up MRI study.
CONCLUSIONS
Although intradural spinal tumors are very rare, their clinical features are nonspecific and resemble other degenerative spinal diseases, including spinal stenosis and disc herniation. These diseases may easily be overlooked by physicians.

Keyword

Neurofibroma; Spinal cord neoplasms; Parasites; Diagnostic errors

MeSH Terms

Arachnoid
Arachnoiditis
Christianity
Diagnosis
Diagnostic Errors
Ependymoma
Female
Follow-Up Studies
Hearing Loss
Humans
Magnetic Resonance Imaging
Middle Aged
Neurofibroma*
Neurofibromatoses
Neurologic Examination
Parasites
Physical Examination
Recurrence
Reflex
Reflex, Babinski
Sensation
Skin
Spinal Cord Neoplasms
Spinal Diseases
Spinal Stenosis
Spine
Vascular Malformations

Figure

  • Fig. 1. Serial T2-weighted sagittal magnetic resonance imaging revealed that the mass-like lesion grew or moved caudally. (A-B) 27 months, (B-C) 12 months, (C-D) 5 months.

  • Fig. 2. A post-myelographic computed tomography scan revealed multiple filling defects in the dural sac, from the T11 to L3-4 disc levels. (A, B) Coronal and sagittal images show a tortuous lesion in the spinal canal with an elongated shape.

  • Fig. 3. Preoperative sagittal magnetic resonance imaging demonstrated that the mass-like lesion extended from the T11 level to the L3-4 disc levels. It was shaped like a rosary or pea in the dural sac. (A) Isointensity on T1; (B) mixed or slightly low intensity on T2; (C) a heterogeneous well-enhanced image post-contrast.

  • Fig. 4. Photographic findings. The mass had a serpentine shape with firm consistency. It measured 1.0×1.5×14 cm and weighed 10 g. (A) Gross sur-face, (B) longitudinal cross-section.

  • Fig. 5. Histological findings. A high-power section revealed a proliferation of spindle cells with wavy nuclei, consistent with a neurofibroma (hematoxylin and eosin staining, ×400 magnification).

  • Fig. 6. Postoperative 1-year follow-up magnetic resonance imaging. There was no evidence of tumor remnants or local recurrence of the tumor.


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