An inflammatory pseudotumor in the thoracic epidural space presenting with progressive paraplegia: a histopathological diagnosis with clinical and radiological uncertainty. Case report with literature review

Abstract

We present here a 49-year-old female with 2 months history of progressive weakness in lower limbs, with no history suggestive of any traumatic, infective, inflammatory, or neoplastic pathology. Both clinical and radiological investigations were inconclusive. There was a mass lesion in the epidural space (predominantly in the posterior and right lateral space) at T1-T3 vertebral levels compressing the thoracic spinal cord. Considering the progressive nature of her neurological deficit, an emergency decompressive laminectomies of T1-T3 vertebrae were done with excision of the compressive mass lesion. Histopathological examination showed a rich lympho-plasmacytic cell infiltrates with storiform spindle cells and dense fibrosis, which was diagnostic of IPT. Post-operatively there was a rapid recovery in neurology and she became ambulatory at the end of 2 weeks. The purpose of this case report is to discuss the clinical, histopathological and radiological features, differential diagnosis, management, and prognosis of spinal IPT on the background of relevant literature review.

An Inflammatory Pseudotumor in the Costo-Vertebral Gutter: Case Report with Literature Review

“…Inflammatory pseudotumor was first described by Brunn in 1939 as a lesion in lung and was named as IPT by Umirker in 1954 [6,7]. It is a chronic inflammatory process of unknown origin and its histopathological presentation is very variable.…”

“…The IPT is hypointense in both T1 and T2-weighted MRI images showing strong enhancement with gadolinium contrast, > Iranian Journal of Neurosurgery as seen in our patient [1,4,6]. Histopathologically, the IPT includes inflammatory cell component made of plasma cells, phenotype B and T lymphocytes, histiocytes, eosinophils and macrophages sometimes taking a clear xanthomatous look [3,5,6].…”

“…Extra-pulmonary locations, apart from orbit are: nasal sinuses, liver, spleen, pancreas, intestine, kidney, urinary tract, bladder, testes, heart, and lymphatic system [1,5]. Only seven cases with epidural location in the spine have been reported to date [6]. In the nervous system, this condition is difficult to diagnose because its clinical and radiological presentation are not specific [2].…”

“…Histopathologically, the IPT includes inflammatory cell component made of plasma cells, phenotype B and T lymphocytes, histiocytes, eosinophils and macrophages sometimes taking a clear xanthomatous look [3,5,6]. It is associated to a fibroblast or myofibroblast component and a collagen background and more or less hyalinized [3,6].…”

“…

Inflammatory pseudotumor (IPT) is a benign tumor-like lesion of unknown cause and is originally described in the lung and orbit, but it has recently been reported throughout the body and only a small number of people [1,3,6]. We presented a rare case of costo-vertebral inflammatory pseudotumor with epidural invasion presenting with progressive cervico-brachial pain.

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