A case presentation

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A 17yrs old male came with H/O low backache since 1month; it is gradual in onset and progressive     in nature, radiating along the rt lower limb, aggravated during walking and relieved by taking rest. Occasional numbness along rt LL.
No other significant history is available.
Neurologically, there was no neurological deficit. His SLR was restricted on the right.

MRI of LS SPINE:



Surgery:
At surgery, the lamina exposed by dissecting the fascia and paraspinal muscle.
After performing laminectomy  it was seen that there is slight bulging of DURA. On incising the dura, observed that the conus medullaris had a sausage-like appearance.
Its dorsal surface was lined with intact pia matter. There was no evidence of cyst rupture.
The cyst contained soft deep-ivory material with a creamy consistency, mixed with hairy tissue.
The contents were removed without significant spillage into the cerebrospinal fluid (CSF) and sent for HPE.

Histopathological examination:
Lumbar cyst revealed a stratified squamous epithelium with keratin material, pseudo stratified  ciliated columnar epithelium, hair follicle, nerve bundle , thin walled blood vessel   sebaceous glands, and mature fat.
Foci of respiratory epithelium were found.
These findings are consistent with a diagnosis of Mature Teratoma .



Discussion
The word 'teratoma' means  “Monstrous Tumor”
Willis defined teratoma as neoplasm with the power of autonomous growth, i.e., malformation composed of multiple tissues, foreign to their localization and lacking organ specificity.
 Teratoma is an encapsulated tumor composed of fully differentiated, ectodermal, mesodermal, and endodermal elements.
The more common ectodermal components include skin, brain, and choroid plexus.
 Mesodermal representatives include cartilage, bone, fat, and muscle.
Cysts lined by a respiratory or enteric type epithelium are the usual endodermal components.
Teratomas accounts for only 0.2-0.5% of all spinal cord tumors

Teratomas commonly  are classified using the Gonzalez-Crussi grading system:
Grade 0 or mature (benign);
1 or immature, probably benign;
2 or immature, possibly malignant (cancerous); and
3 or frankly malignant. (cancer);
Teratomas are also classified by their content:
a solid teratoma contains only tissues
a cystic teratoma contain only pockets of fluid or semi-fluid such as cerebrospinal fluid, sebum, or fat;
a mixed teratoma contains both solid and cystic parts.
 
Review Of Literature
The first spinal teratoma was described by Gowers and Horsley  in 1888.
The origin of teratomas of the spinal cord is controversial. Kubie and Fulton speculated that these tumors were ependymomal diverticula of the central canal of the spinal cord.
Rewcastle and Francoeur  thought they were misplacements of multipotential germinal cells.
The best accepted theory proposed by Willis, which states that tumors arise from the detachment and proliferation of cells that escape primary inducer control during early embryonic development and grow elsewhere chaotically.
In a survey of the literature, 83 cases of teratomas were found till 1996. Of these 83 teratomas, 31 were intramedullary.
The male/female ratio among adult patients with intramedullary  teratomas was 0.83, but the ratio was the inverse (0.15) among children (≤15 yr).
The locations of the teratomas involved the entire spinal cord, with a thoracolumbar predominance (particularly the conus medullaris region)
A Journal published in 2010 on Spinal Teratomas with study on 157 cases  with age distribution of birth to 84yrs, the incidence of intramedullary  is 46%, intradural extramedullary is 32%.
33% of tumors originated in Conus Medullaris, where as 20% each from thoracic and lumbar.
Most commonly  associated anomaly with spinal teratoma was Spina Bifida.
96% of tumors were Mature Teratomas.
Clinical features
The most frequently encountered symptoms were pain, weakness of the legs, sensory abnormalities, sphincter disturbanes and reflex abnormalities .
These symptoms, which are caused by either spinal cord or nerve root compression, occur in a progressive or intermittent-progressive mode.
The periods of remission and exacerbation can best be explained as the result of intermittent increases in the secretory activity of the glandular elements of the tumor.
Investigations
As reported by Hamabuchi et al., CT is a sensitive method to differentiate teratomas from other tumors, on the basis of the heterogenecity of the teratomatous contents.
Monajati et al. reported the superiority of MRI, compared with computed tomography.
The relatively high signal from fat in MRI, especially on T1-weighted images, makes the identification of heterogenous lesion with calcification and mixed csf, lipid & soft tissue mass in central canal of the spinal cord.
The exact diagnosis could only be made postoperatively, with respect to both the type of tumor and the presence of malignancy by HPE.

Complications:
If the cyst ruptures, acute symptoms related to chemical or aseptic meningitis, headache, or seizures may develop due to dissemination of the lipid droplets in the CSF.
Lumbar arachnoiditis may develop as a result of the leakage of fat and proteinaceous material into the subarachnoid space.
The highly irritative lipid content of a mature teratoma may cause a severe inflammatory response, although the spread of fat into the CSF may also be silent clinically.
Management:
Treatment of intramedullary teratomas is Surgical, especially because an exact diagnosis of benign or malignant cannot be made preoperatively.
Radical evisceration should be the aim of surgical intervention whenever possible.
Teratomas normally are well encapsulated and non-invasive of surrounding tissues, hence they are relatively easy to resect from surrounding tissues
For malignant teratomas, usually, surgery is followed by chemotherapy
Recurrences were most commonly encountered with immature or malignant teratomas.