Hindawi Publishing Corporation Case Reports in Surgery Volume 2016, Article ID 7930521, 4 pages http://dx.doi.org/10.1155/2016/7930521
Case Report Ancient Schwannoma of the Cauda Equina: Our Experience and Review of the Literature Venceslao Wierzbicki,1 Alessandro Pesce,2,3 Luigi Marrocco,1 Emanuele Piccione,1 Alessandro Frati,4 and Riccardo Caruso1,5 1
Neurosurgery Department, Army Hospital of Rome “Celio”, Roma, Italy NESMOS Department, “Sapienza” University, Roma, Italy 3 Neurosurgery Department, Azienda Ospedaliera Sant’Andrea, Roma, Italy 4 Neurosurgery Division, IRCCS “Neuromed”, Pozzilli, Italy 5 Neurology and Psychiatry Department, “Sapienza” University, Roma, Italy 2
Correspondence should be addressed to Alessandro Pesce; ale [email protected] Received 17 July 2016; Revised 1 October 2016; Accepted 10 October 2016 Academic Editor: Jochen T¨uttenberg Copyright © 2016 Venceslao Wierzbicki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis.
1. Introduction To the best of our knowledge, there are only 2 cases previously reported of AS involving cauda equina [1, 2], an exceedingly rare schwannoma subtype for an extremely uncommon location. Although reported to affect head and neck, cervical region, limbs, pelvis, and retroperitoneum [1–5], it is uncommon is the spinal cord [6–10] but cauda equina involvement is exceptional. The authors report a case of AS involving cauda equina, an exceptionally rare lesion for an extremely rare location, and review all the relevant literature in order to outline clinical, radiological, and pathological remarks of this rare condition.
2. Case Presentation A 36-year-old man was admitted to our department complaining of one-year history of progressively worsening back pain with bilateral radiation to the anterior surface of the lower limbs, combined with paresthesias to both lower limbs
and urinary incontinence for about a month. The neurological examination reported a bilateral fall of both lower limbs in Mingazzini maneuver and Patellar and Achilles hyporeflexia and bilateral sensory deficit of sacral dermatomes. The patient underwent a lumbar spine MRI scan with TSE, FS, T1w, and T2w sequences and contrast enhanced T1 (Gadolinium) which demonstrated a voluminous intraduralextramedullary tumor (1.9 cm × 2.8 cm × 2.0 cm), with solid and cystic areas of heterogeneous intensity signal (Figures 1(a) and 1(b)) with a strong Gadolinium enhancement. The patient was operated on for D12-L1 laminotomy and en bloc resection of the lesion. Gross pathology was consistent with a spinal schwannoma (Figure 1(c)). The postoperative course was uneventful and the patient experienced a complete and immediate neurological recovery and was discharged in third postoperative day. Histology showed a mesenchymal neoplasm, with an alternation of hypercellular (Antoni A) and hypocellular areas (Antoni B) presenting degenerative traits like multiple microcystic areas filled with a tenuously basophilic colloid
2
Case Reports in Surgery
(a)
(b)
(c)
Figure 1: (a) Sagittal and (b) axial preoperative contrast enhanced MRI scan. (c) Intraoperative image of the lesion at gross examination resembles an ordinary schwannoma. Note the “target” pattern in (a) and the finely heterogenous contrast enhancement in (b), consistent with the typical aspect of an ancient schwannoma.
§
§ ∗
∘
∘
∗
(a)
(b)
(c)
Figure 2: (a) H&E 40x, (b) Ki67, and (c) S-100 immunostaining of the lesion. Note the presence of microcystic areas (∗) in a loose fibrillary stroma (§) finely admixed with hypercellular areas composed of bundles of spindle-cells (∘).
material in H&E stain, surrounded by a loose hypocellular fibrillary stroma with rare necrotic areas. The neoplasm was composed of spindle-cells with pyknotic nuclei and rare mitotic figures and a modest degree of nuclear pleomorphism. The neoplasm showed a remarkable S-100 immunoreactivity and a scarce reactivity for GFAP staining. The Ki67 immunoreactivity demonstrated an extremely low proliferating rate (less than 1%) (Figures 2(a), 2(b), and 2(c)). Histology was consistent with a typical AS. Before writing this paper patient was consulted and gave informed written explicit consent to this report.
3. Discussion Ancient schwannoma is a rare variant of schwannoma (
Case Report Ancient Schwannoma of the Cauda Equina: Our Experience and Review of the Literature Venceslao Wierzbicki,1 Alessandro Pesce,2,3 Luigi Marrocco,1 Emanuele Piccione,1 Alessandro Frati,4 and Riccardo Caruso1,5 1
Neurosurgery Department, Army Hospital of Rome “Celio”, Roma, Italy NESMOS Department, “Sapienza” University, Roma, Italy 3 Neurosurgery Department, Azienda Ospedaliera Sant’Andrea, Roma, Italy 4 Neurosurgery Division, IRCCS “Neuromed”, Pozzilli, Italy 5 Neurology and Psychiatry Department, “Sapienza” University, Roma, Italy 2
Correspondence should be addressed to Alessandro Pesce; ale [email protected] Received 17 July 2016; Revised 1 October 2016; Accepted 10 October 2016 Academic Editor: Jochen T¨uttenberg Copyright © 2016 Venceslao Wierzbicki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis.
1. Introduction To the best of our knowledge, there are only 2 cases previously reported of AS involving cauda equina [1, 2], an exceedingly rare schwannoma subtype for an extremely uncommon location. Although reported to affect head and neck, cervical region, limbs, pelvis, and retroperitoneum [1–5], it is uncommon is the spinal cord [6–10] but cauda equina involvement is exceptional. The authors report a case of AS involving cauda equina, an exceptionally rare lesion for an extremely rare location, and review all the relevant literature in order to outline clinical, radiological, and pathological remarks of this rare condition.
2. Case Presentation A 36-year-old man was admitted to our department complaining of one-year history of progressively worsening back pain with bilateral radiation to the anterior surface of the lower limbs, combined with paresthesias to both lower limbs
and urinary incontinence for about a month. The neurological examination reported a bilateral fall of both lower limbs in Mingazzini maneuver and Patellar and Achilles hyporeflexia and bilateral sensory deficit of sacral dermatomes. The patient underwent a lumbar spine MRI scan with TSE, FS, T1w, and T2w sequences and contrast enhanced T1 (Gadolinium) which demonstrated a voluminous intraduralextramedullary tumor (1.9 cm × 2.8 cm × 2.0 cm), with solid and cystic areas of heterogeneous intensity signal (Figures 1(a) and 1(b)) with a strong Gadolinium enhancement. The patient was operated on for D12-L1 laminotomy and en bloc resection of the lesion. Gross pathology was consistent with a spinal schwannoma (Figure 1(c)). The postoperative course was uneventful and the patient experienced a complete and immediate neurological recovery and was discharged in third postoperative day. Histology showed a mesenchymal neoplasm, with an alternation of hypercellular (Antoni A) and hypocellular areas (Antoni B) presenting degenerative traits like multiple microcystic areas filled with a tenuously basophilic colloid
2
Case Reports in Surgery
(a)
(b)
(c)
Figure 1: (a) Sagittal and (b) axial preoperative contrast enhanced MRI scan. (c) Intraoperative image of the lesion at gross examination resembles an ordinary schwannoma. Note the “target” pattern in (a) and the finely heterogenous contrast enhancement in (b), consistent with the typical aspect of an ancient schwannoma.
§
§ ∗
∘
∘
∗
(a)
(b)
(c)
Figure 2: (a) H&E 40x, (b) Ki67, and (c) S-100 immunostaining of the lesion. Note the presence of microcystic areas (∗) in a loose fibrillary stroma (§) finely admixed with hypercellular areas composed of bundles of spindle-cells (∘).
material in H&E stain, surrounded by a loose hypocellular fibrillary stroma with rare necrotic areas. The neoplasm was composed of spindle-cells with pyknotic nuclei and rare mitotic figures and a modest degree of nuclear pleomorphism. The neoplasm showed a remarkable S-100 immunoreactivity and a scarce reactivity for GFAP staining. The Ki67 immunoreactivity demonstrated an extremely low proliferating rate (less than 1%) (Figures 2(a), 2(b), and 2(c)). Histology was consistent with a typical AS. Before writing this paper patient was consulted and gave informed written explicit consent to this report.
3. Discussion Ancient schwannoma is a rare variant of schwannoma (
