Leiomyosarcoma in a cervical myelomeningocele: a rare complication in a neglected case
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CASE REPORT
Leiomyosarcoma in a cervical myelomeningocele: a rare complication in a neglected case Sandeep Mohindra 1
&
Aman Batish 1 & Manjul Tripathi 1 & Kaniyappan Nambiyar 2 & Kirti Gupta 2
Received: 14 May 2020 / Accepted: 12 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background and importance Asymptomatic myelomeningoceles are usually more of cosmetic deformities and left without evaluation and untreated, amongst low socio-economic strata. Such midline swellings, if left neglected may become the seat for malignancies, usually of squamous cell origin. Clinical presentation The authors report a case of an 18-year-old male patient who developed leiomyosarcoma within chronically neglected cervical myelomeningocele. Conclusion A non-healing ulcer of any aetiology is a bed for carcinogenesis. Neglected myelomeningoceles, for any reason, may convert a benign entity to a malignant pathology. To our best knowledge, this is the first case of a leiomyosarcoma arising de novo from myelomeningocele. Keywords Case report . Cervical . Dysraphism . Leiomyosarcoma . Meningomyelocele . Neglect
Background and importance Neural tube defect is next only to cerebral palsy causing disability amongst congenital neurological anomalies [4]. Cervical myelomeningocele (CMMC) accounts for 3–5% of all cases of spinal dysraphism [4]. Children with CMMC may be asymptomatic and may present only with a mass protruding from the posterior midline of the neck or with any radiological tethering. CMMC if left untreated may cause gradual neurological deterioration with predominant motor involvement in upper extremities [4]. Very rarely, prolonged neglect may predispose this benign entity to develop malignancy secondary to chronic irritation [2, 3, 6, 7, 9, 13, 14, 16, 17].
Reporting guidelines The authors have followed the EQUATOR NETWORK guidelines. * Sandeep Mohindra [email protected] 1
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India
2
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
In the present communication, we describe a unique case of neglected cervical MMC, developing malignancy of mesodermal origin.
Clinical presentation An 18-year-old boy belonging to remote hilly terrains of north India presented with a soft, fleshy swelling on the nape of the neck, since birth (Fig. 1a). As the swelling was asymptomatic per se except for cosmetic deformity, the patient disregarded any management advice including radiological scans even when he developed gradual spastic paraparesis in his adolescence. In the last 3 months, there was a rapid increase in size, with leakage of CSF and ulceration emitting foul smell. Examination revealed an 8 × 10-cm swelling in the posterior midline of the cervical region, which was fleshy in consistency, non-tender and fixed to the underlying structures. The overlying skin was excoriated with purulent discharge and freshly bleeding marg
