Hostname: page-component-76fb5796d-vfjqv Total loading time: 0 Render date: 2024-04-25T13:44:21.842Z Has data issue: false hasContentIssue false
  • English
  • Français

Immunoglobulin G4 related systemic sclerosing disease involving the temporal bone

Published online by Cambridge University Press:  02 June 2010

L Masterson ,
M Martinez Del Pero ,
N Donnelly ,
D A Moffat  and
E Rytina
L Masterson
Affiliation:
Department of Otolaryngology, Norfolk and Norwich University Hospital, UK
M Martinez Del Pero
Affiliation:
Department of Otolaryngology, Norfolk and Norwich University Hospital, UK
N Donnelly*
Affiliation:
Department of Skull Base Surgery, Cambridge University Hospital, UK
D A Moffat
Affiliation:
Department of Skull Base Surgery, Cambridge University Hospital, UK
E Rytina
Affiliation:
Department of Histopathology, Cambridge University Hospital, UK
*
Address for correspondence: Mr Neil Donnelly, Department of Skull Base Surgery, Cambridge University Hospitals, Hills Road, Cambridge CB2 0QQ, UK. E-mail: neil.donnelly@addenbrookes.nhs.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

To report a rare condition affecting the temporal bone. Immunoglobulin G4 related systemic sclerosing disease is a recently described autoimmune condition with manifestations typically involving the pancreas, biliary system, salivary glands, lungs, kidneys and prostate. Histologically, it is characterised by T-cell infiltration, fibrosis and numerous immunoglobulin G4-positive plasma cells. This condition previously fell under the umbrella diagnosis of inflammatory pseudotumour and inflammatory myofibroblastic tumour.

Case report:

We present the case of a 58-year-old woman with multiple inflammatory masses involving the pharynx, gall bladder, lungs, pelvis, omentum, eyes and left temporal bone, over a seven-year period. We describe this patient's unusual clinical course and pathological features, which resulted in a change of diagnosis from metastatic inflammatory myofibroblastic tumour to immunoglobulin G4 related systemic sclerosing disease. We also review the literature regarding the management of inflammatory pseudotumours of the temporal bone, and how this differs from the management of immunoglobulin G4 related systemic sclerosing disease.

Conclusion:

We would recommend a full review of all histological specimens in patients with a diagnosis of temporal bone inflammatory pseudotumour or inflammatory myofibroblastic tumour. Consideration should be given to immunohistochemical analysis for anaplastic lymphoma kinase and immunoglobulin G4, with measurement of serum levels of the latter. Management of the condition is medical, with corticosteroids and immunosuppression, rather than surgical excision.

Keywords

Temporal BoneIgG4Inflammatory Pseudotumor
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 124 , Issue 10 , October 2010 , pp. 1106 - 1110
Copyright
Copyright © JLO (1984) Limited 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Umiker, WO, Iverson, L. Postinflammatory tumors of the lung; report of four cases simulating xanthoma, fibroma, or plasma cell tumor. J Thorac Surg 1954;28:55–63CrossRefGoogle ScholarPubMed
2Neild, GH, Rodriguez-Justo, M, Wall, C, Connolly, JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Medicine 2006;4:23CrossRefGoogle ScholarPubMed
3Hamano, H, Kawa, S, Horiuchi, A, Unno, H, Furuya, N, Akamatsu, T et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–8CrossRefGoogle ScholarPubMed
4Kamisawa, T, Okamoto, A. IgG4-related sclerosing disease. World J Gastroenterol 2008;14:3948–55CrossRefGoogle ScholarPubMed
5Hamano, H, Kawa, S, Ochi, Y, Unno, H, Shiba, N, Wajiki, M et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 2002;359:1403–4CrossRefGoogle ScholarPubMed
6Schaeffer, CJ, Minai, OA, Sharma, N, Kanne, JP, Mohammed, TL. Inflammatory myofibroblastic tumor of the lung: recurrence after steroid treatment. J Thorac Imaging 2008;23:191–3CrossRefGoogle ScholarPubMed
7Coffin, CM, Watterson, J, Priest, JR, Dehner, LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumour). A clinicopathological and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859–72CrossRefGoogle ScholarPubMed
8Gleason, BC, Hornick, JL. Inflammatory myofibroblastic tumours: where are we now? J Clin Pathol 2008;61:428–37CrossRefGoogle ScholarPubMed
9Meis, JM, Enzinger, FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol 1991;15:1146–56CrossRefGoogle ScholarPubMed
10Cook, JR, Dehner, LP, Collins, MH, Ma, Z, Morris, SW, Coffin, CM et al. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study. Am J Surg Pathol 2001;25:1364–71CrossRefGoogle ScholarPubMed
11Tsuboi, H, Inokuma, S, Setoguchi, K, Shuji, S, Hagino, N, Tanaka, Y et al. Inflammatory pseudotumors in multiple organs associated with elevated serum IgG4 level: recovery by only a small replacement dose of steroid. Intern Med 2008;47:1139–42CrossRefGoogle Scholar
12Taguchi, M, Aridome, G, Abe, S, Kume, K, Tashiro, M, Yamamoto, M et al. Autoimmune pancreatitis with IgG4-positive plasma cell infiltration in salivary glands and biliary tract. World J Gastroenterol 2005;11:5577–81CrossRefGoogle ScholarPubMed
13Pickartz, T, Pickartz, H, Lochs, H, Ockenga, J. Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary Sjogren's syndrome. Eur J Gastroenterol Hepatol 2004;16:1295–9CrossRefGoogle ScholarPubMed
14Fukui, T, Okazaki, K, Yoshizawa, H, Ohashi, S, Tamaki, H, Kawasaki, K et al. A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjogren's syndrome. Pancreatology 2005;5:86–91CrossRefGoogle ScholarPubMed
15Brazier, DJ, Sanders, MD. Multifocal fibrosclerosis associated with suprasellar and macular lesions. Br J Ophthalmol 1983;67:292–6CrossRefGoogle ScholarPubMed
16Sugimoto, T, Tanaka, Y, Morita, Y, Kume, S, Uzu, T, Kashiwagi, A et al. Is tubulointerstitial nephritis and uveitis syndrome associated with IgG4-related systemic disease? Nephrology 2008;13:89CrossRefGoogle ScholarPubMed
17Sato, Y, Ohshima, K, Ichimura, K, Sato, M, Yamadori, I, Tanaka, T et al. Ocular adnexal IgG4-related disease has uniform clinicopathology. Pathol Int 2008;58:465–70CrossRefGoogle ScholarPubMed
18Armigliato, M, Paolini, R, Bianchini, E, Monesi, G, Zamboni, S, D'Andrea, EA et al. Hashimoto's thyroiditis and Graves’ disease associated with retroperitoneal fibrosis. Thyroid 2002;12:829–31CrossRefGoogle ScholarPubMed
19Schonermark, M, Issing, P, Stover, T, Ruhand, S, Lenarz, T. Fibroinflammatory pseudotumor of the temporal bone. Skull Base 1998;8:45–50CrossRefGoogle ScholarPubMed
20Strasnick, B, Vaughan, A. Inflammatory pseudotumor of the temporal bone: a case series. Skull Base 2008;18:49–52CrossRefGoogle ScholarPubMed
21Williamson, RA, Paueksakon, P, Coker, NJ. Inflammatory pseudotumor of temporal bone. Otol Neurotol 2003;24:818–22CrossRefGoogle ScholarPubMed
22Goh, BS, Tan, SP, Husain, S, Rose, IM, Saim, L. Metachronous inflammatory myofibroblastic tumour in the temporal bone: case report. J Laryngol Otol 2009;123:1184–7CrossRefGoogle ScholarPubMed
23Lee, JH, Jung, MK, Song, CE, Yeo, SW, Lee, HK, Pang, PS et al. Concomitant inflammatory pseudotumor of the temporal bone and lung: a case report. Ear Nose Throat J 2007;86:614–16CrossRefGoogle ScholarPubMed
24Gasparotti, R, Zanetti, D, Bolzoni, A, Gamba, P, Morassi, ML, Ungari, M et al. Inflammatory myofibroblastic tumor of the temporal bone. Am J Neuroradiol 2003;24:2092–6Google ScholarPubMed
25Kasliwal, MK, Suri, A, Gupta, DK, Suri, V, Rishi, A, Sharma, BS. Sphenoid wing inflammatory pseudotumor mimicking a clinoidal meningioma: case report and review of the literature. Surg Neurol 2008;70:509–13CrossRefGoogle ScholarPubMed
26Coulson, C, George, A, Biswas, A, Phelan, C, De, R. Pseudotumour of the temporal bone: an unusual cause of otorrhoea and facial palsy. Eur Arch Otorhinolaryngol 2008;265:713–15CrossRefGoogle ScholarPubMed