Intramedullary spinal teratoma: An interesting case report and review of the literature :Manoj Kumar Sharma, Ratika B Agarwal, Karan Sankhla, Suresh K Sankhla, Journal of Spinal Surgery

CASE REPORT
Year : 2022 | Volume : 9 | Issue : 3 | Page : 170--172

Intramedullary spinal teratoma: An interesting case report and review of the literature

Manoj Kumar Sharma, Ratika B Agarwal, Karan Sankhla, Suresh K Sankhla
Department of Neurosurgery, Global Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Manoj Kumar Sharma
C/O Dr. Suresh Sankhla, Room No. 112, 1^st Floor OPD, Global Hospital, 35, Dr. E. Borges Road, Parel, Mumbai -a 400 012, Maharashtra
India

Abstract

Mature spinal intramedullary teratomas are benign germ cell tumors that are associated with an excellent long-term outcome following early treatment with radical surgical resection. However, the preoperative diagnosis is often difficult and delayed due to their rare occurrence in the spinal cord, especially when the typical clinical and imaging features are not present. We report a case of intramedullary teratoma in a young adult female who presented with unusual clinical and preoperative radiographic findings. The final diagnosis was made on histopathological examination postoperatively. Variations in clinical presentations, imaging, optimal treatment strategies and histopathological characteristics of this rare tumor are discussed here with a review of the literature.

How to cite this article:
Sharma MK, Agarwal RB, Sankhla K, Sankhla SK. Intramedullary spinal teratoma: An interesting case report and review of the literature.J Spinal Surg 2022;9:170-172

How to cite this URL:
Sharma MK, Agarwal RB, Sankhla K, Sankhla SK. Intramedullary spinal teratoma: An interesting case report and review of the literature. J Spinal Surg [serial online] 2022 [cited 2023 Apr 2 ];9:170-172
Available from: http://www.jossworld.org/text.asp?2022/9/3/170/356029

Full Text

Introduction

Intramedullary teratomas are rare spinal tumors that account for 0.2%–0.5% of all spinal neoplasms in children.[1] Males are affected more commonly than females (3:1) and comparatively adults are more affected than children.[2] We report an interesting case of intramedullary teratoma in an adult patient who presented with progressive scoliosis.

Case Report

A 21-year-old female presented with progressive dorsal scoliosis for the past few years with subtle ill-defined areas of hypoesthesia over the dorsum of her right foot without any impairment in motor power, reflexes and gait. Her scoliosis was more marked in the dorsal region with convexity toward the right side. She had no cutaneous markers of neurofibromatosis or signs of occult spinal dysraphism. Magnetic resonance imaging (MRI) showed a focal expansion of the cord at the level of D6-D9 due to a large heterointense intramedullary tumor with a thin peripheral enhancement following intravenous contrast administration [Figure 1]a, [Figure 1]b, [Figure 1]c. The lesion showed a small amount of fat component, few tiny cysts, and multiple hypointense foci of calcification within. A laminotomy at D5-D9 and midline myelotomy was performed with the removal of the grayish well-circumscribed mass, partly soft, cystic, and largely calcified under the guidance of intraoperative ultrasonography [Figure 2]a and [Figure 2]b. Histopathological findings were suggestive of a mature teratoma [Figure 3]. The tumor was composed of a haphazard admixture of tissue elements derived from all three germ layers. The cystic spaces and glands were lined by ciliated pseudostratified columnar epithelium. The goblet cell containing columnar epithelium was seen along with transitional and simple columnar epithelia. There were focal clusters of seromucinous glands and a few small glands with cuboidal lining. These epithelial structures were embedded within glial tissue along with focal nerve bundles, fibrocollagenous tissue and occasional islands of adipose tissue. There was abundant mature lamellar bone with interspersed marrow tissue and foci of calcification.{Figure 1}{Figure 2}{Figure 3}

The patient had an uneventful recovery and was discharged from the hospital after 5 days. Follow-ups at 1st and 3rd month postoperatively, she was found to be asymptomatic with stable scoliosis.

Discussion

Teratomas are slow-growing germ cell neoplasms that occur most commonly in the sacrococcygeal (57%), gonadal (29%), mediastinal (7%), and retroperitoneal (3%) regions and are rarely found in the spinal canal.[3] Spinal teratomas have a predilection of the thoracic spine and conus medullaris and involve the spinal canal as an extra- or intramedullary lobulated mass with a solid or cystic component.[4] Some patients have congenital skin abnormalities such as dermal sinus, tuft of hair, nevi and vertebral anomalies at the same level and are reported to have associated split cord malformations and other developmental spinal anomalies such as myelomeningoceles and tethered cord.[5] Although the exact etiology of origin is not known, it is believed that they arise from multipotential germinal cells misplaced in the dorsal midline between the yolk sac and gonadal ridges.[6] Walter and Kleinert[7] classified central nervous system teratomas into three groups: mature, immature and malignant types depending on the degree of differentiation. Mature teratoma is composed of fully differentiated adult-type tissue elements representative of the three germ layers, although the presence of only two layers does not exclude the diagnosis.[1] Intramedullary teratomas are usually mature and considered benign.

Most patients develop symptoms and signs of progressive cord or root compression.[2] However, our patient was an adult female who presented with a spinal deformity and had no clinical signs of spinal cord involvement or associated cutaneous or osseous anomalies. Preoperative diagnosis of spinal teratoma is often very difficult. Radiological signs such as widening of interpedicular space, erosion of vertebral bodies and associated vertebral anomalies such as spina bifida, vertebral body fusion and diastematomyelia on plain X-rays are nonspecific findings. The MRI is specific for fat, calcification and solid/cystic areas within the tumor. Enlargement of the spinal cord, high signal on T2-weighted images and the heterogeneous enhancement on postcontrast T1-weighted images are common characteristics of the intramedullary teratomas, as observed in the present case. Diagnosis of teratoma, however, depends on the histopathological identification of the tissues representing the three germinal layers (ectoderm, mesoderm and endoderm).[8]

Surgical removal of teratoma along with its capsule generally provides a good long-term outcome and therefore, radical resection should be the aim of the surgery. The tumor is approached through a laminectomy or laminotomy and is removed using standard microsurgical techniques. Laminotomy with laminoplasty is preferred in younger children and in those patients who have preoperative spinal deformity or instability, like in our patient.[9] A safe subtotal excision is an effective option in certain cases where tumor is having adhesions with the cord and roots and the risk of postoperative neurological morbidity is high. Tumor recurrence is generally uncommon and may need redo surgery to control clinical worsening. Adjuvant therapy should be considered when malignancy is suspected based on histopathological findings.[10] Long-term follow-up is mandatory.

Conclusion

Although rare, intramedullary spinal teratomas may occur in adolescents and adults with or without congenital spinal anomalies and should therefore be considered in the differential diagnosis of spinal tumors. A careful evaluation with MRI is essential for the diagnosis of teratoma in the early stage. Surgical resection is the best treatment modality to arrest further neurological deterioration and radical resection should be the primary goal of surgery.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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