|Ahead of print
A rare case of spinal extradural teratoma with mediastinal extension
Dilip K Macharla1, Raveena Miryala2, Kritin Mehrotra3, Naveen Mehrotra1
1 Department of Neurosurgery, Sunshine Hospital, Secunderabad, Telangana
2 Government EJHS Clinic, Nalgonda, Telangana
3 Armed Forces Medical College, Pune, Mumbai, India
|Date of Submission||11-Aug-2021|
|Date of Decision||02-Feb-2022|
|Date of Acceptance||12-Mar-2022|
|Date of Web Publication||30-Jan-2023|
Department of Neurosurgery, Sunshine Hospital, PG Road, Secunderabad, Telangana 500003
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Introduction: Spinal extradural teratomas extending to posterior mediastinum are very rare and seldom reported.Case Report: A 4-year-old boy presented with progressive spastic paraparesis with sphincteric dysfunction was misdiagnosed as cerebral palsy. His magnetic resonance imaging showed D2-D5 extradural tumor, extending to the posterior mediastinum, causing severe thecal sac compression. Intraspinal part of the tumor was excised, and the child made a good recovery after the surgery. On histopathological examination, it was confirmed as a mature cystic teratoma.Conclusion: Spinal tumors with myelopathy may mimic cerebral palsy in children. To the authors’ knowledge, there are very few reported cases of spinal teratomas extending to the mediastinum.
Keywords: Cerebral palsy, extradural teratoma, spina tumor, spinal extradural teratoma, spinal immature teratoma
| Introduction|| |
Teratomas are rare multipotent germ cell tumors with components from all three germ cell layers. Spinal teratomas are rare and constitute only 0.1–0.5% of all spinal cord tumors, incidence is higher in infants and children., They are present in intradural and extradural locations; however, extradural spinal teratomas are quite rare. We report a rare case of thoracic epidural spinal teratoma, extending to the posterior mediastinum in a 4-year-old child causing paraparesis, who was initially misdiagnosed to have cerebral palsy.
| Case Report|| |
A 4-year old boy was referred to us with progressive spastic paraparesis and sphincteric dysfunction. He is the second child of his parents, born of non-consanguineous wedlock, after a normal full-term gestation, via cesarean section due to umbilical cord around the neck. Antenatal and perinatal periods were unremarkable. His parents noticed paucity of bilateral lower limb movements since the age of 3 months, diagnosed as cerebral palsy and advised physical therapy. He had normal developmental milestones except for those related to his lower extremities. His lower limb paresis worsened to grade 2/5 with patellar and ankle clonus at the time of surgery. His magnetic resonance imaging (MRI) of spine showed a well-defined, lobulated, dumb bell-shaped, cystic lesion in the thoracic spine at D2–D5 levels, located in the extradural location on the right side, causing severe compression of the thecal sac, through the widened D2-D3 foramen, extending into the right posterior mediastinum. The lesion appeared hypointense on T1W and hyperintense on T2W, with central diffusion restriction [shown in [Figure 1]], and showed heterogeneous enhancement upon contrast administration. Rest of the spinal cord appeared normal, and no abnormality was detected in the screening of brain.
|Figure 1: MRI of the thoracic spine showing a lobulated, intraspinal lesion from D5 to D6 levels, extradurally, extending into the right paravertebral region and posterior mediastinum. A: Lesion appeared hyperintense on T2W imaging; B: hypointense on T1W imaging. C, D: lesion compressing the thecal sac and displacing it to the left and extending into the posterior mediastinum|
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He was evaluated by a multidisciplinary team consisting of pediatric neurosurgeons, cardiothoracic surgeons, pulmonologists, anesthetists, and pediatrician; thereafter a two-stage surgery was planned: first, excision of the intraspinal tumor, in view of severe compressive myelopathy. Excision of the mediastinal portion to be planned at a later stage, as the child did not have any symptoms pertaining to it.
We performed a laminoplastic laminotomy from D1 to D5 levels, per-operatively, a well-defined, capsulated, extradural tumor noted on the right side, displacing the thecal sac toward left [shown in [Figure 2A]], extraspinal extension was noted through the D2-D3 foramen. Intra-spinal, foraminal, and paraspinal part of the tumor was excised. Thecal sac became lax and pulsatile after tumor excision.
|Figure 2: A: Intraoperative image showing a well-defined, extradural tumor compressing the thecal sac. B: Gross examination—grayish tumor with pultaceous material and hair|
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On macroscopic examination, tumor was light grayish brown, had a soft, doughy consistency. Tumor consisted of solid-cystic areas, filled with grayish white pultaceous material and abundant hair [shown in [Figure 2B]]. The microscopic examination showed cyst wall lined by stratified squamous epithelium with keratin layers, multiple sebaceous units, with hair follicles, and mature adipose tissue. Normal glial tissue was noted [shown in [Figure 3]]. The underlying stroma was edematous and loose. Focally, the wall was disrupted with possible extravasation of the cyst contents, showing focal granulomatous reaction along with collection of macrophages, foreign-body type giant cells, plasma cells, and lymphocytes. No definite well-defined granulomas seen. No evidence of immature elements or malignancy noted. Histopathology features were consistent with those of mature cystic teratoma.
|Figure 3: Microscopic examination showing (A and B) epidermis with adnexal structures on H&E 100× magnification. C: Adipose tissue. D: Normal glial elements on high magnification H&E 400×|
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The child made an uneventful recovery after tumor excision and was able to walk with minimal support. He was continent for both bowel and bladder and had residual myelopathy signs, improving on physiotherapy. After 18-month follow-up, the child has almost attained a normal gait and the residual mediastinal component remained status quo.
| Discussion and Literature Review|| |
Rudolf Virchow (1863) was the first to introduce the term “teratoma,” derived from the Greek work “teras” which means monster. These are a type of germ cell tumors, consisting of well-differentiated tissues derived from three germ cell layers (ectoderm, mesoderm, and endoderm). Willis has defined teratomas as tumors comprising of tissues foreign to the part in which it grows. When only two germ cell derivates were present, it was called a “teratoid.”
In their large series of 254 teratomas, Tapper and Lack reported only 4 (1.5%) intraspinal teratomas; approximately 40% were sacrococcygeal teratomas. Of all spinal tumors, intraspinal teratomas constitute less than 0.5%, their incidence is higher (5–9%) among the pediatric spinal tumors.
Pathogenesis of spinal teratomas is poorly understood. According to the misplaced germ cell theory, teratomas arise from aberrantly migrated multipotent primordial germ cells, which possibly explains the upper thoracic teratoma in our case.,, In contrast, abnormal differentiation of pluripotent cells in the caudal cell mass results is the formation of teratomas as per the dysembryogenic theory.,,
Spinal teratomas are predominantly intradural in location, with only 7.6% being extradural as per Seonget al. Moreover, the extension of a spinal teratoma into the posterior mediastinum is rarer. Our case has a rare combination of intraspinal extradural mature teratoma with mediastinal extension. Kaneko et al. reported a similar case of extradural teratoma with bilateral mediastinal extension in 1999.
The association between spinal teratomas and spinal dysraphism was reported by several authors; however, in our case, dysraphic elements were not present.,,
The teratomas have an indolent course with slow progression, and clinical features may mimic cerebral palsy as it happened in our case. A child with progressive myelopathy symptomatology without perinatal insult needs an evaluation with MRI of spine, irrespective of spinal dysraphic state. The advantage of doing an MRI in such a clinical situation is picking up of a potentially treatable cause of myelopathy, as was our case and the child showed significant recovery after excision of teratoma.
There are no pathognomic radiological features of teratoma; a heterogeneous, thick-walled, lobulated mass with fat and calcification should raise a suspicion of it. The treatment of choice is total surgical excision of the tumor, but if it is firmly adherent to neural tissues like spinal cord, conus, or cauda, safe maximal resection is the rule.
There is no significant risk of regrowth following subtotal removal, and as per Allsopp et al., the rate of recurrence after total and subtotal resection was 9% and 11%, respectively. Sharma et al. reported a case of recurrence 9 years after excision of a benign teratoma which required a re-surgery. Hence, regular clinical and radiological follow-up is required to detect early recurrences.
| Conclusion|| |
Spinal extradural teratomas are very rare, more so is their extension into the posterior mediastinum. We suggest to get an MRI of spine in a child with progressive myelopathy features, without perinatal history of hypoxia irrespective of dysraphic features.
We acknowledge Dr Mujeeb Siddiqui, MD for histopathological examination and images.
Statement of ethics
Written informed consent from the parents of the patient (as the patient is a minor) was obtained for publication of this case report and accompanying images.
Financial support and sponsorship
No funding has been received for this study.
Conflicts of interest
The authors have no conflicts of interest to declare.
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[Figure 1], [Figure 2], [Figure 3]