Case Report
Recurrent meningeal inflammatory myofibroblastic tumor: A case report and literature review Maria Pascual‑Gallego, Miguel Yus‑Fuertes1, Manuela Jorquera1, Alfredo Gonzalez‑Maté2, Luis Ortega3, Armando Martínez‑Martínez3, Horacio Zimman Departments of Neurosurgery, 1Image Diagnosis, 2Nuclear Medicine and 3Anatomic Pathology, Clínico San Carlos University Hospital, Madrid, Spain
Abstract Address for correspondence: Dr. María Pascual‑Gallego, Department of Neurosurgery, Hospital Universitario Clínico San Carlos, Prof. Martín Lagos s/n, 28040, Madrid, Spain. E‑mail: [email protected] Received: 27‑06‑2013 Review completed: 28‑10‑2013 Accepted: 03‑12‑2013
An inflammatory myofibroblastic tumor (IMT) is a rare lesion with an uncertain prognosis and a disorder difficult to classify. IMTs are a heterogeneous group of lesions, sometimes indistinguishable from meningiomas and other expanding or inflammatory lesions of the central nervous system. This report presents a patient with IMT, who presented with recurrent retroocular pain radiating to the occipital region and no neurologic deficits. He had early recurrence in spite of total resection of the lesion. The clinical profile of 18 patients with either progression or recurrence has been reviewed Key words: Inflammatory pseudotumor, meningeal inflammatory myofibroblastic tumor, plasma cell granuloma, somatostatin receptors
Introduction Inflammatory myofibroblastic tumor (IMT) is a rare lesion with uncertain prognosis and is difficult to classify. Recently, World Health Organization defined IMT as a soft tissue tumor.[1] This report presents a case of IMT with recurrence despite gross total removal.
Case Report The present case is about a 47‑year‑old woman who was admitted with a 1‑year history of recurrent headaches, characterized by retroocular pain radiating to the occipital region. These attacks were associated with occasional nausea and visual disturbances. Neurologic Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.125273
644
examination was normal. Blood biochemistry and complete blood picture were normal. Brain magnetic resonance imaging (MRI) revealed a single extraxial mass in the left temporal‑occipital lobe with features suggestive of meningioma [Figure 1a‑c]. Cerebral single‑photon emission computed tomography (SPECT) with 122 MBq of pentetreotide‑I111 showed a moderate uptake by the tumor, indicating somatostain receptors [Figure 1d]. A left temporal‑occipital craniotomy revealed an extraxial and well‑encapsulated mass adherent to the transverse sinus. Gross total resection of the lesion was done with coagulation of the implantation site (Simpson grade 2). Histological evaluation revealed a spindle cell monomorph neoplasm with sparse myxoid regions and collagenous stroma, intermingled with inflammatory infiltrate and numerous plasma cells [Figure 2a]. Inflammatory cells expressed CD45 [Figure 2b] with epithelial membrane antigen (EMA) negative reactivity [Figure 2c], whereas spindle cells showed positive immunoreactivity for α‑smooth muscle actin [Figure 2d]. Ki 67 labeling index was less than 1%. The pathological diagnosis was IMT. The post‑operative course was uneventful and the immediate post‑operative contrast computed tomography scan showed no residual tumor [Figure 2e]. MRI performed 4 months after surgery revealed a 23 mm recurrence adherent to the transverse Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
a
c
b a
b
c
d
e
f
d
Figure 1: Pre-operative neuroradiological and single-photon emission computed tomography (SPECT) findings. (a) Axial basal T1-weighted magnetic resonance imaging (MRI) showing an isointense extraxial dural based temporal-occipital mass attached and (b) post-contrast which reveals an intense enhancement of the lesion, (c) Axial T2-weighted Fatsaturated Fast Spin Echo MRI coronal image with iso and hypointense areas with respect to the grey matter. Associated vasogenic edema found in the adjacent brain parenchyma. (d) Axial view of the cerebral SPECT with pentetreotide-I111 showing an uptake in the left temporaloccipital region matching the MRI location
sinus [Figure 2f]. The patient returned to her homeland and no subsequent follow‑up exam was possible. The literature review was based on a PubMed search for the key words “inflammatory myofibroblastic tumor”, “inflammatory pseudotumor”, “plasma cell granuloma” and further references cited in previous PubMed articles, including articles in English, French and Spanish.
Discussion IMTs include a heterogeneous group of lesions characterized by spindle shape cells and a variable amount of inflammatory cell infiltration and fibrosis. IMTs are divided into three subtypes:[2] (1) Compact spindle cells with inflammatory cells, (2) inflammatory areas resembling nodular fasciitis, (3) dense plate‑like collagen resembling scar. Recurrence is infrequent and malignant transformation and metastases are even more uncommon.[3] IMTs were initially reported in the lung,[4] but can occur in any organ. IMTs have been described in the brain [5‑7] and spine. [8] Most importantly, the symptoms and imaging of IMTs are indistinguishable from meningiomas and other expanding or inflammatory lesions of the central nervous system (CNS). Only histopathology can help diagnose an IMT. As or our literature review, there had been approximately 100 cases of IMT of CNS reported and only 19, including our case, have shown either progression or recurrence.[5,9‑22] IMTs of the brain are rare, with a small number of cases confirmed by histopathological diagnosis in children Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Figure 2: Histopathological findings. (a) Monomorphic spindle cells with bland nuclei, lying in a collagenous background containing plasma cell infiltrates (H and E) (b) CD45 positive immunostaining inflammatory cells with (c) epithelial membrane antigen negative reactivity. (d) Spindle cells showed positive immunoreactivity for alfa-smooth muscle actin staining. (e) Immediate post-operative contrast computed tomography scan showed no residual tumor. (f) Contrast T1-weighted magnetic resonance imaging 4 months later showed a recurrence adherent to the transverse sinus
and adults. Pathology includes spindle cell proliferation with myofibroblastic differentiation, collagenous stroma and an inflammatory infiltrate of lymphocytes, eosinophils and plasma cells.[1] The CNS lesion location can be meningeal,[6] intraparenchymatous,[5] pineal,[23] hypothalmus[15] intraventricular.[7,24] Lesions with sella turcica,[19] skull base,[25] orbit and external cranium including mastoid[6] and paranasal sinuses[26] have been reported. Clinically, IMTs are undistinguishable from other lesions of the brain and meninges. Clinical features of IMTs include headaches, seizures and focal deficits.[27] This patient can be classified as meningeal tumor close to the transverse sinus. Meningeal IMT usually is a mass attached to the dura mater and sometimes in the vicinity of dural venous sinuses. On MRI imaging IMT presents an extraxial mass isointense to hypointense relative to brain parenchyma in both T1 and T2‑weighted images with homogeneous uptake on postcontrast series.[20,28] The radiologic features of IMT include: (1) Localized diffuse dural thickening, (2) dural based masses (3) single or multiple intraxial 645
646
16, F
19, F
48, M
57, M
57, F
42, F
62, M
36, F
41, M
15, M
17, F
19, F
Ferrer (1989)
Chang (1991)
Hsiang (1994)
Hirohata (1996)
Hudson (1996)
Cho (2001)
Brandsma (2003)
Brandsma (2003)
Häusler (2003)
Häusler (2003)
Jochum (2004)
Headache, amenorrhea
Headache
Headache, hearing loss Decrease of visual acuity of the left eye Seizure, left homonymous hemianopia Seizure
Ptosis, double vision Visual loss, headache
Visual loss, headache, panhypopituitarism Headache, weakness, paresthesias, hemianopia Unsteady gait
Visual loss, headache
Age, Symptoms gender
Cannella (1988)
Author (year)
PCG
IP
Pathology
Meningeal PCG
IP
PCG
PCG
Sellar
Left frontal
Left occipital
IMT
IP
IP
Intra‑axial right PCG occipital lobe
Cavernous sinus, CPA
Right orbital and cavernous sinus, right temporal Petrous apex
Right CPA
Thoracolumbar PCG spine
Choroid plexus PCG or right lateral ventricle
Hypothalamus
Right frontal
Location of tumor
5 months
6 years
5 years
10 months
1 year
1 month
Biopsy
GTR
GTR+ chemotherapy+ RT (36 Gy) STR+RT (20 Gy)
6 months
2 years
2, 5 months
11 years
STR+RT
GTR
Steroids
Chemotherapy
RT (50 Gy)
RT
NA
NA
STR
NA
No treatment
NA
Time to Second recurrence/ treatment progression
Biopsy+steroids+ NA RT (50 Gy) STR 1, 5 years
Steroids
STR+RT (20 Gy)
STR
GTR+steroids
Biopsy+ RT (44 Gy)
GTR
First treatment
s‑100+
s‑100+
NA
NA
NA
NA
NA
NA
NA
NA
Vimentin+, SMA+, ALK+, negative: Desmin, myogenin, CD34, S100 prot, HMB45, cytokeratin, EMA, GFAP, Ki‑67 5‑10%
(Contd...)
B and T lymphocites, plasma cells and macrophages B and T lymphocites, plasma cells and macrophages Lymphocytes, plasma cells, histiocytes and multinucleated giant cells
Polyclonal plasma cells, histiocytes with lymphophagocytosis Polytipic B linphocites
NA
NA
Policlonal plasma cells, lymphocytes and histocytes NA
Plasma cells, lymphocytes and histiocytes Lymphoplasmocytes and mature plasma cells NA
Inmunohistochemistry Inflamatory cells
Progressive SMA+, vimentin+ worsening of symptoms Recurrence CD68+, VIM+, SMA+, ALK+, 3 years after S‑100+, EMA−, Vimentin+
NA
Progression
Progression
NA
NA
Metastases after STR
NA
Lost
NA
Outcome
Table 1: Literature review of 19 cases of recurrent/progressive intracranial inflammatory pseudotumors or plasma cell despite treatment
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
65, F
14, F
49, M
47, M
47, F
47, F
Jeon (2005)
Shah (2005)
Lin (2009)
Brito (2010)
Puntambekar (2012)
Present report (2013)
Orbit, falx, superior sagittal sinus, tentorium and mastoid with brain invasion Occipital
Migranious attacks
Reduced left visual acuity, hearing loss, dificulty in walking, urinary retention Seizure
Dizziness, hoarseness
Left temporo‑ occipital lobe
CPA, supra and parasellar, cavernous sinus Left parietal
Left CPA
Headache, partial Right right seventh nerve cavernous palsy sinus and Meckel’s cave
NA
Dizziness
Location of tumor STR+RT
First treatment 12 years
IMT
PCG
PCG
IP
GTR
Biopsy+steroids
Biopsy+ steroids+RT
GTR
4 months
6 weeks
Inmediate progression
6 months
NA
Recurrence 15 years after
Outcome
No treatment
GTR+steroids
Continued the first treatment
RT (12 Gy)
CD20+, CD3+, S‑100+
SMA+, ALK−, p53−, EMA−
SMA+, ALK−, p53−, Cyclin D1−, p16+, CD21−, c‑Kit−, EMA−
Lost
ASMA+, CD45+, EMA−, GFAP−and ALK−, Ki‑67 1%
No CD68+, S‑100−, CD1−, recurrence at GFAP+, Ki‑67 10‑20% 22 months
B and T lymphocites, plasma cells, macrophages and proliferating fibroblasts Inflammatory infiltrate with numerous plasma cells
Polyclonal plasma cells and lymphocytes
Plasma cells, aggregates of lymphocytes, and polymorphonuclear leukocytes Lymphocytes
A few plasma cells and lymphocytes
Plasma cell dominant
Inmunohistochemistry Inflamatory cells
No CD68+, T‑200+, S‑100−, recurrence at GFAP− 2 years Progressive NA worsening of symptoms
Methotrexate and Recurrence 6‑mercaptopurine+ 3 years after steroids
NA
NA
Time to Second recurrence/ treatment progression
IMT GTR 7 years fibrohistiocytic variant PCG Biopsy+steroids+ 7 months RT (20 Gy)
IMT PCG‑like
Pathology
M ‑ Male, F ‑ Female, NA ‑ Not available, CPA ‑ Cerebellopontine angle, IP ‑ Inflammatory pseudotumor, PCG ‑ Plasma cell granuloma, IMT ‑ Inflammatory myofibroblastic tumor, GTR ‑ Gross total resection, STR ‑ Subtotal resection, RT ‑ Radiotherapy, SMA ‑ Smooth muscle actin, ALK ‑ Anaplasic lymphoma kinase, EMA ‑ Epithelial membrane antigen, ASMA ‑ Alpha‑smooth muscle actin, GFAP ‑ Gliofibrilar acid protein, VIM ‑ Vimentin
43, M
Age, Symptoms gender
Jeon (2005)
Author (year)
Table 1: Contd....
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
647
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
masses (4) intraventricular/choroid plexus masses.[12] Other findings include dural venous sinus thrombosis, leptomeningeal involvement, extracranial involvement (mastoid, orbit). Cranial bone involvement has also been described with focal neurological deficit.[29] These neuroimaging features are rather non‑specific. SPECT with pentetreotide‑In111 shows a moderate uptake of radionucleide, which is less than the uptake observed in meningiomas, breast cancer metastasis and neuroendocrine tumors. There was only one report of pentetreotide‑In111 SPECT imaging in IMTs located in the pterygopalatine fossa.[15] These findings suggest for the potential role of somatostatin analogs in the treatment of IMTs.[30] Surgical resection remains the treatment of choice for mass lesions and a total resection can be a cure. However, in this patient, total removal of the lesions did not prevent early tumor recurrence, suggesting the aggressive biological behavior of the lesion. Such early recurrence has been described in patients with IMT in other organs. Carswell and Chataway[31] in their study have reported the efficacy of long‑term steroid therapy in controlling symptoms related to intracranial IMT. Chemotherapy regimens used for IMT include carboplatin and paclitaxel[32] methotrexate and 6‑mercaptopurine[28] Radiotherapy has also been tried in some case with variable degrees of success.[6,20,33] However, the determining factor for progression or recurrence of these tumors remains unclear and these lesions have been sometimes classified as inflammatory pseudotumors or plasma cell granulomas. Table 1 describes the clinical profile of patients with recurrence or progression of the lesion. Classification of different subtypes of IMT remains a challenge because of fewer numbers of cases and histological complexity of the tumor. In our patient the predominant pathological features spindle‑shape cells and inflammatory infiltrates. Reactivity for α‑smooth muscle actin was positive and EMA and anaplastic lymphoma kinase (ALK) was negative. These findings rule out other conditions, such as plasma cell meningioma, Rosai‑Dorfman disease, pachimeningitis, tuberculosis and fungal disease. ALK is positive in nearly half of patients with extra‑pulmonary IMT, particularly in children and is associated with local recurrence and distant metastasis.[5,34] However, ALK was negative in our case and in all cases of the CNS‑IMT reported by Kim et al.,[20] whereas Clarke et al.[23] found a pineal IMT with positive ALK reactivity.
2.
3. 4. 5.
6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24.
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Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859‑72. Jeba J, John S, Backiyanathan S, Christopher DJ, Kurian S. Inflammatory pseudotumour of the lung with sarcomatous brain metastasis. Eur J Cancer Care (Engl) 2008;17:412‑4. Bahadori M, Liebow AA. Plasma cell granulomas of the lung. Cancer 1973;31:191‑208. Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B. Inflammatory pseudotumors of the central nervous system: Report of 3 cases and a literature review. Hum Pathol 2003;34:253‑62. Jeon YK, Chang KH, Suh YL, Jung HW, Park SH. Inflammatory myofibroblastic tumor of the central nervous system: Clinicopathologic analysis of 10 cases. J Neuropathol Exp Neurol 2005;64:254‑9. Maeda Y, Tani E, Nakano M, Matsumoto T. Plasma‑cell granuloma of the fourth ventricle. Case report. J Neurosurg 1984;60:1291‑6. Zemmoura I, Hamlat A, Morandi X. Intradural extramedullary spinal inflammatory myofibroblastic tumor: Case report and literature review. Eur Spine J 2011;20 Suppl 2:S330‑5. Brandsma D, Jansen GH, Spliet W, Van Nielen K, Taphoorn MJ. The diagnostic difficulties of meningeal and intracerebral plasma cell granulomas–Presentation of three cases. J Neurol 2003;250:1302‑6. Brito CC, Lopes FC, Chimelli L, Gasparetto EL. Intracranial cell plasma granuloma. Arq Neuropsiquiatr 2010;68:127‑9. Cannella DM, Prezyna AP, Kapp JP. Primary intracranial plasma‑cell granuloma. Case report. J Neurosurg 1988;69:785‑8. Chang Y, Horoupian DS, Lane B, Fross RD, Smyth LT Jr, Seiling RJ. Inflammatory pseudotumor of the choroid plexus in Sjögren’s disease. Neurosurgery 1991;29:287‑90. Cho YS, Kim SM, Chung WH, Hong SH. Inflammatory pseudotumour involving the skull base and cervical spine. J Laryngol Otol 2001;115:580‑4. de Ruiter ED, Kwekkeboom DJ, Mooi WJ, Knegt P, Tanghe HL, van Hagen PM. Inflammatory pseudotumor of the fossa pterygopalatina: Diagnosis and treatment. Neth J Med 2000;56:17‑20. Ferrer I, Garcia Bach M, Aparicio MA, Acebes JJ, Twose J, Isamat F. Plasma cell granuloma of the hypothalamic region. Acta Neurochir (Wien) 1989;99:152‑6. Hirohata M, Sasaguri Y, Sugita Y, Tokutomi T, Kobayashi S, Morimatsu M, et al. Intracranial plasma‑cell granuloma: A case report. Noshuyo Byori 1996;13:133‑8. Hsiang J, Moorhouse D, Barba D. Multiple plasma cell granulomas of the central nervous system: Case report. Neurosurgery 1994;35:744‑7. Hudson HL, Hinton DR, Friedman DI, Apuzzo M, Chandrasoma P, Garbut M, et al. Pseudotumor associated with CNS lymphoma. J Neuroophthalmol 1996;16:208‑11. Jochum W, Hänggi D, Bruder E, Jeck T, Novotny H, Probst A, et al. Inflammatory myofibroblastic tumour of the sella turcica. Neuropathol Appl Neurobiol 2004;30:692‑5. Kim JH, Chang KH, Na DG, Park SH, Kim E, Han DH, et al. Imaging features of meningeal inflammatory myofibroblastic tumor. AJNR Am J Neuroradiol 2009;30:1261‑7. Lui PC, Fan YS, Wong SS, Chan AN, Wong G, Chau TK, et al. Inflammatory pseudotumors of the central nervous system. Hum Pathol 2009;40:1611‑7. Puntambekar P, Santhakumar S, Kupsky WJ, Tselis A, Mittal S. Primary intracranial plasma cell granulomas presenting as malignant neoplasms. J Neurooncol 2012;106:327‑37. Clarke AJ, Jacques TS, Galloway MJ, Thom M, Kitchen ND, Plant GT. ALK positive inflammatory myofibroblastic tumour of the pineal region. J Clin Pathol 2005;58:981‑3. Gandhi RH, Li L, Qian J, Kuo YH. Intraventricular inflammatory pseudotumor: Report of two cases and review of the literature. Neuropathology 2011;31:446‑54. McCall T, Fassett DR, Lyons G, Couldwell WT. Inflammatory pseudotumor of the cavernous sinus and skull base. Neurosurg Rev 2006;29:194‑200. Buccoliero AM, Caldarella A, Santucci M, Ammannati F, Mennonna P, Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
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Taddei A, et al. Plasma cell granuloma–An enigmatic lesion: Description of an extensive intracranial case and review of the literature. Arch Pathol Lab Med 2003;127:e220‑3. Saifudheen K, Jose J, Gafoor VA. Inflammatory pseudotumor of the head presenting with hemiparesis and aphasia. Case Rep Neurol Med 2011;2011:176546. Lin YJ, Yang TM, Lin JW, Song MZ, Lee TC. Posterior fossa intracranial inflammatory pseudotumor: A case report and literature review. Surg Neurol 2009;72:712‑6. Shah MD, McClain KL. Intracranial plasma cell granuloma: Case report and treatment of recurrence with methotrexate and 6‑mercaptopurine. J Pediatr Hematol Oncol 2005;27:599‑603. Lamberts SW, Krenning EP, Klijn JG, Reubi JC. The clinical use of somatostatin analogues in the treatment of cancer. Baillieres Clin Endocrinol Metab 1990;4:29‑49. Carswell C, Chataway J. The successful long‑term management of an intracranial inflammatory myofibroblastic tumor with corticosteroids. Clin Neurol Neurosurg 2012;114:77‑9.
32. Kubo N, Harada T, Anai S, Otsubo K, Yoneshima Y, Ijichi K, et al. Carboplatin plus paclitaxel in the successful treatment of advanced inflammatory myofibroblastic tumor. Intern Med 2012;51:2399‑401. 33. Crespo C, Navarro M, González I, Lorente MF, González R, Mayol MJ. Intracranial and mediastinal inflammatory myofibroblastic tumour. Pediatr Radiol 2001;31:600‑2. 34. Swain RS, Tihan T, Horvai AE, Di Vizio D, Loda M, Burger PC, et al. Inflammatory myofibroblastic tumor of the central nervous system and its relationship to inflammatory pseudotumor. Hum Pathol 2008;39:410‑9. How to cite this article: Pascual-Gallego M, Yus-Fuertes M, Jorquera M, Gonzalez-Maté A, Ortega L, Martínez-Martínez A, et al. Recurrent meningeal inflammatory myofibroblastic tumor: A case report and literature review. Neurol India 2013;61:644-9. Source of Support: Nil, Conflict of Interest: None declared.
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Recurrent meningeal inflammatory myofibroblastic tumor: A case report and literature review Maria Pascual‑Gallego, Miguel Yus‑Fuertes1, Manuela Jorquera1, Alfredo Gonzalez‑Maté2, Luis Ortega3, Armando Martínez‑Martínez3, Horacio Zimman Departments of Neurosurgery, 1Image Diagnosis, 2Nuclear Medicine and 3Anatomic Pathology, Clínico San Carlos University Hospital, Madrid, Spain
Abstract Address for correspondence: Dr. María Pascual‑Gallego, Department of Neurosurgery, Hospital Universitario Clínico San Carlos, Prof. Martín Lagos s/n, 28040, Madrid, Spain. E‑mail: [email protected] Received: 27‑06‑2013 Review completed: 28‑10‑2013 Accepted: 03‑12‑2013
An inflammatory myofibroblastic tumor (IMT) is a rare lesion with an uncertain prognosis and a disorder difficult to classify. IMTs are a heterogeneous group of lesions, sometimes indistinguishable from meningiomas and other expanding or inflammatory lesions of the central nervous system. This report presents a patient with IMT, who presented with recurrent retroocular pain radiating to the occipital region and no neurologic deficits. He had early recurrence in spite of total resection of the lesion. The clinical profile of 18 patients with either progression or recurrence has been reviewed Key words: Inflammatory pseudotumor, meningeal inflammatory myofibroblastic tumor, plasma cell granuloma, somatostatin receptors
Introduction Inflammatory myofibroblastic tumor (IMT) is a rare lesion with uncertain prognosis and is difficult to classify. Recently, World Health Organization defined IMT as a soft tissue tumor.[1] This report presents a case of IMT with recurrence despite gross total removal.
Case Report The present case is about a 47‑year‑old woman who was admitted with a 1‑year history of recurrent headaches, characterized by retroocular pain radiating to the occipital region. These attacks were associated with occasional nausea and visual disturbances. Neurologic Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.125273
644
examination was normal. Blood biochemistry and complete blood picture were normal. Brain magnetic resonance imaging (MRI) revealed a single extraxial mass in the left temporal‑occipital lobe with features suggestive of meningioma [Figure 1a‑c]. Cerebral single‑photon emission computed tomography (SPECT) with 122 MBq of pentetreotide‑I111 showed a moderate uptake by the tumor, indicating somatostain receptors [Figure 1d]. A left temporal‑occipital craniotomy revealed an extraxial and well‑encapsulated mass adherent to the transverse sinus. Gross total resection of the lesion was done with coagulation of the implantation site (Simpson grade 2). Histological evaluation revealed a spindle cell monomorph neoplasm with sparse myxoid regions and collagenous stroma, intermingled with inflammatory infiltrate and numerous plasma cells [Figure 2a]. Inflammatory cells expressed CD45 [Figure 2b] with epithelial membrane antigen (EMA) negative reactivity [Figure 2c], whereas spindle cells showed positive immunoreactivity for α‑smooth muscle actin [Figure 2d]. Ki 67 labeling index was less than 1%. The pathological diagnosis was IMT. The post‑operative course was uneventful and the immediate post‑operative contrast computed tomography scan showed no residual tumor [Figure 2e]. MRI performed 4 months after surgery revealed a 23 mm recurrence adherent to the transverse Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
a
c
b a
b
c
d
e
f
d
Figure 1: Pre-operative neuroradiological and single-photon emission computed tomography (SPECT) findings. (a) Axial basal T1-weighted magnetic resonance imaging (MRI) showing an isointense extraxial dural based temporal-occipital mass attached and (b) post-contrast which reveals an intense enhancement of the lesion, (c) Axial T2-weighted Fatsaturated Fast Spin Echo MRI coronal image with iso and hypointense areas with respect to the grey matter. Associated vasogenic edema found in the adjacent brain parenchyma. (d) Axial view of the cerebral SPECT with pentetreotide-I111 showing an uptake in the left temporaloccipital region matching the MRI location
sinus [Figure 2f]. The patient returned to her homeland and no subsequent follow‑up exam was possible. The literature review was based on a PubMed search for the key words “inflammatory myofibroblastic tumor”, “inflammatory pseudotumor”, “plasma cell granuloma” and further references cited in previous PubMed articles, including articles in English, French and Spanish.
Discussion IMTs include a heterogeneous group of lesions characterized by spindle shape cells and a variable amount of inflammatory cell infiltration and fibrosis. IMTs are divided into three subtypes:[2] (1) Compact spindle cells with inflammatory cells, (2) inflammatory areas resembling nodular fasciitis, (3) dense plate‑like collagen resembling scar. Recurrence is infrequent and malignant transformation and metastases are even more uncommon.[3] IMTs were initially reported in the lung,[4] but can occur in any organ. IMTs have been described in the brain [5‑7] and spine. [8] Most importantly, the symptoms and imaging of IMTs are indistinguishable from meningiomas and other expanding or inflammatory lesions of the central nervous system (CNS). Only histopathology can help diagnose an IMT. As or our literature review, there had been approximately 100 cases of IMT of CNS reported and only 19, including our case, have shown either progression or recurrence.[5,9‑22] IMTs of the brain are rare, with a small number of cases confirmed by histopathological diagnosis in children Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Figure 2: Histopathological findings. (a) Monomorphic spindle cells with bland nuclei, lying in a collagenous background containing plasma cell infiltrates (H and E) (b) CD45 positive immunostaining inflammatory cells with (c) epithelial membrane antigen negative reactivity. (d) Spindle cells showed positive immunoreactivity for alfa-smooth muscle actin staining. (e) Immediate post-operative contrast computed tomography scan showed no residual tumor. (f) Contrast T1-weighted magnetic resonance imaging 4 months later showed a recurrence adherent to the transverse sinus
and adults. Pathology includes spindle cell proliferation with myofibroblastic differentiation, collagenous stroma and an inflammatory infiltrate of lymphocytes, eosinophils and plasma cells.[1] The CNS lesion location can be meningeal,[6] intraparenchymatous,[5] pineal,[23] hypothalmus[15] intraventricular.[7,24] Lesions with sella turcica,[19] skull base,[25] orbit and external cranium including mastoid[6] and paranasal sinuses[26] have been reported. Clinically, IMTs are undistinguishable from other lesions of the brain and meninges. Clinical features of IMTs include headaches, seizures and focal deficits.[27] This patient can be classified as meningeal tumor close to the transverse sinus. Meningeal IMT usually is a mass attached to the dura mater and sometimes in the vicinity of dural venous sinuses. On MRI imaging IMT presents an extraxial mass isointense to hypointense relative to brain parenchyma in both T1 and T2‑weighted images with homogeneous uptake on postcontrast series.[20,28] The radiologic features of IMT include: (1) Localized diffuse dural thickening, (2) dural based masses (3) single or multiple intraxial 645
646
16, F
19, F
48, M
57, M
57, F
42, F
62, M
36, F
41, M
15, M
17, F
19, F
Ferrer (1989)
Chang (1991)
Hsiang (1994)
Hirohata (1996)
Hudson (1996)
Cho (2001)
Brandsma (2003)
Brandsma (2003)
Häusler (2003)
Häusler (2003)
Jochum (2004)
Headache, amenorrhea
Headache
Headache, hearing loss Decrease of visual acuity of the left eye Seizure, left homonymous hemianopia Seizure
Ptosis, double vision Visual loss, headache
Visual loss, headache, panhypopituitarism Headache, weakness, paresthesias, hemianopia Unsteady gait
Visual loss, headache
Age, Symptoms gender
Cannella (1988)
Author (year)
PCG
IP
Pathology
Meningeal PCG
IP
PCG
PCG
Sellar
Left frontal
Left occipital
IMT
IP
IP
Intra‑axial right PCG occipital lobe
Cavernous sinus, CPA
Right orbital and cavernous sinus, right temporal Petrous apex
Right CPA
Thoracolumbar PCG spine
Choroid plexus PCG or right lateral ventricle
Hypothalamus
Right frontal
Location of tumor
5 months
6 years
5 years
10 months
1 year
1 month
Biopsy
GTR
GTR+ chemotherapy+ RT (36 Gy) STR+RT (20 Gy)
6 months
2 years
2, 5 months
11 years
STR+RT
GTR
Steroids
Chemotherapy
RT (50 Gy)
RT
NA
NA
STR
NA
No treatment
NA
Time to Second recurrence/ treatment progression
Biopsy+steroids+ NA RT (50 Gy) STR 1, 5 years
Steroids
STR+RT (20 Gy)
STR
GTR+steroids
Biopsy+ RT (44 Gy)
GTR
First treatment
s‑100+
s‑100+
NA
NA
NA
NA
NA
NA
NA
NA
Vimentin+, SMA+, ALK+, negative: Desmin, myogenin, CD34, S100 prot, HMB45, cytokeratin, EMA, GFAP, Ki‑67 5‑10%
(Contd...)
B and T lymphocites, plasma cells and macrophages B and T lymphocites, plasma cells and macrophages Lymphocytes, plasma cells, histiocytes and multinucleated giant cells
Polyclonal plasma cells, histiocytes with lymphophagocytosis Polytipic B linphocites
NA
NA
Policlonal plasma cells, lymphocytes and histocytes NA
Plasma cells, lymphocytes and histiocytes Lymphoplasmocytes and mature plasma cells NA
Inmunohistochemistry Inflamatory cells
Progressive SMA+, vimentin+ worsening of symptoms Recurrence CD68+, VIM+, SMA+, ALK+, 3 years after S‑100+, EMA−, Vimentin+
NA
Progression
Progression
NA
NA
Metastases after STR
NA
Lost
NA
Outcome
Table 1: Literature review of 19 cases of recurrent/progressive intracranial inflammatory pseudotumors or plasma cell despite treatment
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
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65, F
14, F
49, M
47, M
47, F
47, F
Jeon (2005)
Shah (2005)
Lin (2009)
Brito (2010)
Puntambekar (2012)
Present report (2013)
Orbit, falx, superior sagittal sinus, tentorium and mastoid with brain invasion Occipital
Migranious attacks
Reduced left visual acuity, hearing loss, dificulty in walking, urinary retention Seizure
Dizziness, hoarseness
Left temporo‑ occipital lobe
CPA, supra and parasellar, cavernous sinus Left parietal
Left CPA
Headache, partial Right right seventh nerve cavernous palsy sinus and Meckel’s cave
NA
Dizziness
Location of tumor STR+RT
First treatment 12 years
IMT
PCG
PCG
IP
GTR
Biopsy+steroids
Biopsy+ steroids+RT
GTR
4 months
6 weeks
Inmediate progression
6 months
NA
Recurrence 15 years after
Outcome
No treatment
GTR+steroids
Continued the first treatment
RT (12 Gy)
CD20+, CD3+, S‑100+
SMA+, ALK−, p53−, EMA−
SMA+, ALK−, p53−, Cyclin D1−, p16+, CD21−, c‑Kit−, EMA−
Lost
ASMA+, CD45+, EMA−, GFAP−and ALK−, Ki‑67 1%
No CD68+, S‑100−, CD1−, recurrence at GFAP+, Ki‑67 10‑20% 22 months
B and T lymphocites, plasma cells, macrophages and proliferating fibroblasts Inflammatory infiltrate with numerous plasma cells
Polyclonal plasma cells and lymphocytes
Plasma cells, aggregates of lymphocytes, and polymorphonuclear leukocytes Lymphocytes
A few plasma cells and lymphocytes
Plasma cell dominant
Inmunohistochemistry Inflamatory cells
No CD68+, T‑200+, S‑100−, recurrence at GFAP− 2 years Progressive NA worsening of symptoms
Methotrexate and Recurrence 6‑mercaptopurine+ 3 years after steroids
NA
NA
Time to Second recurrence/ treatment progression
IMT GTR 7 years fibrohistiocytic variant PCG Biopsy+steroids+ 7 months RT (20 Gy)
IMT PCG‑like
Pathology
M ‑ Male, F ‑ Female, NA ‑ Not available, CPA ‑ Cerebellopontine angle, IP ‑ Inflammatory pseudotumor, PCG ‑ Plasma cell granuloma, IMT ‑ Inflammatory myofibroblastic tumor, GTR ‑ Gross total resection, STR ‑ Subtotal resection, RT ‑ Radiotherapy, SMA ‑ Smooth muscle actin, ALK ‑ Anaplasic lymphoma kinase, EMA ‑ Epithelial membrane antigen, ASMA ‑ Alpha‑smooth muscle actin, GFAP ‑ Gliofibrilar acid protein, VIM ‑ Vimentin
43, M
Age, Symptoms gender
Jeon (2005)
Author (year)
Table 1: Contd....
Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
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Pascual‑Gallego, et al.: Inflammatory myofibroblastic tumor‑recurrence review
masses (4) intraventricular/choroid plexus masses.[12] Other findings include dural venous sinus thrombosis, leptomeningeal involvement, extracranial involvement (mastoid, orbit). Cranial bone involvement has also been described with focal neurological deficit.[29] These neuroimaging features are rather non‑specific. SPECT with pentetreotide‑In111 shows a moderate uptake of radionucleide, which is less than the uptake observed in meningiomas, breast cancer metastasis and neuroendocrine tumors. There was only one report of pentetreotide‑In111 SPECT imaging in IMTs located in the pterygopalatine fossa.[15] These findings suggest for the potential role of somatostatin analogs in the treatment of IMTs.[30] Surgical resection remains the treatment of choice for mass lesions and a total resection can be a cure. However, in this patient, total removal of the lesions did not prevent early tumor recurrence, suggesting the aggressive biological behavior of the lesion. Such early recurrence has been described in patients with IMT in other organs. Carswell and Chataway[31] in their study have reported the efficacy of long‑term steroid therapy in controlling symptoms related to intracranial IMT. Chemotherapy regimens used for IMT include carboplatin and paclitaxel[32] methotrexate and 6‑mercaptopurine[28] Radiotherapy has also been tried in some case with variable degrees of success.[6,20,33] However, the determining factor for progression or recurrence of these tumors remains unclear and these lesions have been sometimes classified as inflammatory pseudotumors or plasma cell granulomas. Table 1 describes the clinical profile of patients with recurrence or progression of the lesion. Classification of different subtypes of IMT remains a challenge because of fewer numbers of cases and histological complexity of the tumor. In our patient the predominant pathological features spindle‑shape cells and inflammatory infiltrates. Reactivity for α‑smooth muscle actin was positive and EMA and anaplastic lymphoma kinase (ALK) was negative. These findings rule out other conditions, such as plasma cell meningioma, Rosai‑Dorfman disease, pachimeningitis, tuberculosis and fungal disease. ALK is positive in nearly half of patients with extra‑pulmonary IMT, particularly in children and is associated with local recurrence and distant metastasis.[5,34] However, ALK was negative in our case and in all cases of the CNS‑IMT reported by Kim et al.,[20] whereas Clarke et al.[23] found a pineal IMT with positive ALK reactivity.
2.
3. 4. 5.
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32. Kubo N, Harada T, Anai S, Otsubo K, Yoneshima Y, Ijichi K, et al. Carboplatin plus paclitaxel in the successful treatment of advanced inflammatory myofibroblastic tumor. Intern Med 2012;51:2399‑401. 33. Crespo C, Navarro M, González I, Lorente MF, González R, Mayol MJ. Intracranial and mediastinal inflammatory myofibroblastic tumour. Pediatr Radiol 2001;31:600‑2. 34. Swain RS, Tihan T, Horvai AE, Di Vizio D, Loda M, Burger PC, et al. Inflammatory myofibroblastic tumor of the central nervous system and its relationship to inflammatory pseudotumor. Hum Pathol 2008;39:410‑9. How to cite this article: Pascual-Gallego M, Yus-Fuertes M, Jorquera M, Gonzalez-Maté A, Ortega L, Martínez-Martínez A, et al. Recurrent meningeal inflammatory myofibroblastic tumor: A case report and literature review. Neurol India 2013;61:644-9. Source of Support: Nil, Conflict of Interest: None declared.
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