Immunoglobulin G4 related systemic sclerosing disease involving the temporal bone

2010 ◽  
Vol 124 (10) ◽  
pp. 1106-1110 ◽  
Author(s):  
L Masterson ◽  
M Martinez Del Pero ◽  
N Donnelly ◽  
D A Moffat ◽  
E Rytina
Keyword(s):  
Temporal Bone ◽  
Plasma Cells ◽  
Rare Condition ◽  
Immunohistochemical Analysis ◽  
Surgical Excision ◽  
Serum Levels ◽  
Inflammatory Myofibroblastic Tumour ◽  
Inflammatory Pseudotumour ◽  
Immunoglobulin G4 ◽  
Anaplastic Lymphoma

AbstractObjective: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.

scholarly journals IgG4-Related Lymphadenopathy

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Yasuharu Sato ◽  
Tadashi Yoshino
Keyword(s):  
Lymph Node ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Lymph Node Involvement ◽  
Lymph Node Biopsy ◽  
Immunoglobulin G4 ◽  
Reactive Protein ◽  
Serum Igg4 ◽  
Node Involvement

Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (>135?mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4+plasma cells (IgG4+/IgG+plasma cell ratio >40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castleman’s disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

Benign necrotizing osteitis of the external auditory meatus

1997 ◽  
Vol 111 (3) ◽  
pp. 269-270 ◽  
Author(s):  
B. N. Kumar ◽  
R. M. Walsh ◽  
A. Sinha ◽  
R. G. Courteney-Harris ◽  
W. V. Carlin
Keyword(s):  
Temporal Bone ◽  
Otitis Externa ◽  
Rare Condition ◽  
External Auditory Meatus ◽  
Surgical Excision ◽  
The Other ◽  
External Meatus ◽  
Malignant Otitis Externa ◽  
Benign Process

AbstractBenign necrotizing osteitis of the external auditory meatus is a rare condition which occurs in non-diabetic healthy people. The aetiology of the necrotic process with the formation of sequestrum in the bony external meatus is unknown. It is important to differentiate this benign process from malignant otitis externa and radionecrosis of the temporal bone as the management of these conditions differs. We present two cases, one managed surgically and the other medically, and review the literature. Immediate surgical excision of the sequestrum is advocated instead of long-term medical therapy and waiting for it to separate spontaneously.

Eosinophilic angiocentric fibrosis of the nose and sinuses

2014 ◽  
Vol 128 (12) ◽  
pp. 1071-1077 ◽  
Author(s):  
J Rimmer ◽  
P Andrews ◽  
V J Lund
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Case Series ◽  
Rare Condition ◽  
Surgical Excision ◽  
Upper Respiratory Tract ◽  
Immunoglobulin G4 ◽  
Disease Spectrum ◽  
Complete Surgical Excision ◽  
Male Patients ◽  
Eosinophilic Angiocentric Fibrosis

AbstractBackground:Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.Methods:We report the largest single-institution case series of sinonasal eosinophilic angiocentric fibrosis to date, comprising nine patients. The current literature is reviewed, showing emerging evidence that this condition may belong to the immunoglobulin G4-related disease spectrum.Results:The series comprised five female and four male patients, with a mean age at presentation of 53 years. All were treated surgically. Six patients had no signs of recurrent disease after an average of 8.5 years. One patient went on to develop granulomatosis with polyangiitis (Wegener's granulomatosis), which required immunosuppressive therapy.Conclusion:The first-line management of this rare condition is complete surgical excision. Chronic granulomatous conditions, including granulomatosis with polyangiitis, should be excluded before a diagnosis is made, and patients should be carefully followed.

scholarly journals Temporal bone involvement of IgG4-related disease: a rare condition misleading to petrous apicitis causing lateral rectus palsy

2019 ◽  
Vol 12 (2) ◽  
pp. e228550 ◽  
Author(s):  
Sanathorn Chowsilpa ◽  
Sayanan Chowsilpa ◽  
Tanyathorn Teeranoraseth ◽  
Kannika Roongrotwattanasiri
Keyword(s):  
Temporal Bone ◽  
Plasma Cells ◽  
Rare Condition ◽  
Lateral Rectus ◽  
Pathological Examination ◽  
Tissue Mass ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Gradenigo’S Syndrome ◽  
Petrous Apicitis

IgG4-related disease (IgG4-RD) of temporal bone is rare and clinical manifestation mimics infection. A 19-year-old female presented with progressive left earache and intermittent left nasal obstruction. Then, she rapidly developed left lateral rectus palsy. The physical examination revealed mild redness of left tympanic membrane and a small nasal polyp from the left middle meatus. CT scan showed left petrous apicitis and enhancing sinonasal mucosa. Therefore, Gradenigo’s syndrome was first considered. The empirical intravenous antibiotic was immediately prescribed and surgery was performed. The intraoperative pale soft tissue mass in middle ear and polyp in the left nasal cavity were sent for pathological examination and found positive immunohistochemical stains for IgG4 in plasma cells. Systemic corticosteroid, the first-line treatment, was started and her symptoms were finally recovery.

Metachronous inflammatory myofibroblastic tumour in the temporal bone: case report

2009 ◽  
Vol 123 (10) ◽  
pp. 1184-1187 ◽  
Author(s):  
B S Goh ◽  
S P Tan ◽  
S Husain ◽  
I M Rose ◽  
L Saim
Keyword(s):  
Case Report ◽  
Temporal Bone ◽  
World Literature ◽  
Surgical Excision ◽  
Inflammatory Myofibroblastic Tumour ◽  
English Medical Literature ◽  
Metachronous Lesion ◽  
Canal Wall Down ◽  
One Year ◽  
The Right

AbstractObjective:We report an extremely rare case of metachronous inflammatory myofibroblastic tumour in the temporal bone.Method:Case report and review of the world literature on metachronous inflammatory myofibroblastic tumour.Results:Inflammatory myofibroblastic tumour in the temporal bone is rare, and metachronous inflammatory myofibroblastic tumour in the temporal bone has never been reported in the English medical literature. We report a case of inflammatory myofibroblastic tumour in the right temporal bone in a 27-year-old woman presenting with right-sided otalgia and progressive hearing loss. A metachronous lesion was discovered in the left temporal bone one year later. The patient underwent surgical excision of the tumour via canal wall down mastoidectomy for both lesions. Long term steroids were prescribed after both surgical procedures. At follow up three years after the last procedure, the patient remained free of disease.Conclusion:To the best of our knowledge, this is the first reported case of metachronous inflammatory myofibroblastic tumour in the temporal bone.

scholarly journals Plasma Cell Granuloma of the Thyroid: Review of an Uncommon Entity

2018 ◽  
Vol 142 (8) ◽  
pp. 998-1005 ◽  
Author(s):  
Pallavi A. Patil ◽  
Ronald A. DeLellis
Keyword(s):  
Plasma Cell ◽  
Inflammatory Myofibroblastic Tumor ◽  
Plasma Cells ◽  
Specific Treatment ◽  
Surgical Excision ◽  
Hashimoto Thyroiditis ◽  
Plasma Cell Granuloma ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Inflammatory Myofibroblastic Tumors

Plasma cell granuloma (PCG) is characterized by proliferation of polyclonal plasma cells with associated fibrosis and is often considered part of the heterogeneous group of inflammatory myofibroblastic tumors (IMTs). The thyroid is rarely affected by PCG. A patient having PCG associated with Hashimoto thyroiditis (HT) prompted our literature search that revealed 18 cases of PCG, 55% (n = 10) of which occurred together with HT. The etiopathogenesis of PCG is unknown and there is no specific treatment except surgical excision for compressive symptoms. This entity has an excellent prognosis with no evidence of recurrence or metastasis. Lesions of the thyroid with infiltrating plasma cells include HT, fibrous variant of HT, plasmacytoma, plasma cell myeloma, Riedel thyroiditis, IgG4 (immunoglobulin G4)-related disease, IMT, and PCG. Inflammatory myofibroblastic tumor has ALK gene rearrangements and is considered a neoplasm as opposed to PCG, which is a reactive polyclonal plasma cell proliferation. We believe IMT and PCG are distinct entities and consensus definitions are required for avoiding confusion in the literature.

Immunohistochemical analysis of CD138-positive Plasma cells in the endometrium

2016 ◽  
Vol 76 (05) ◽  
Author(s):  
M Weber ◽  
B Toth ◽  
E Schleußner ◽  
UR Markert
Keyword(s):  
Plasma Cells ◽  
Immunohistochemical Analysis

Inflammatory Myofibroblastic Tumour in Antimesenteric Border of Descending Colon of Children: A Case Report

2015 ◽  
Vol 3 (1) ◽  
pp. 47-50
Author(s):  
Shahnoor Islam ◽  
AKM Amirul Morshed ◽  
Afiqul Islam
Keyword(s):  
Soft Tissue ◽  
Spindle Cell ◽  
Inflammatory Myofibroblastic Tumour ◽  
Pathological Findings ◽  
Soft Tissue Lesion ◽  
Soft Tissue Tumours ◽  
Inflammatory Pseudotumour ◽  
Erythrocyte Sedimentation ◽  
Antimesenteric Border

Inflammatory myofibroblastic tumour (IMT) occurring at intraabdominal sites in children has rarely been described. Inflammatory pseudotumour is a soft tissue lesion that may be confused with a sarcoma. It is abbreviated as IMT. Inflammatory myofibroblastic tumour, also known as soft tissue tumours, atypical fibromyxoid tumours, pseudosarcomatous fibromyxoid tumour, plasma cell granuloma, pseudosarcomatous myofibrotic proliferation, post-operative spindle cell nodules. In this paper, we describe a case of inflammatory myofibroblastic tumour (IMT) with an unusual constellation of clinical, pathological findings. A 10-year-old girl had an 7-cm intraabdominal mass accompanied by severe anemia, fever, constipation, weight loss, thrombocytosis, elevated erythrocyte sedimentation rate. Laparotomy was performed. The final pathologic diagnosis was IMT. At the most recent follow up (12months) after excision of the tumour, the patient was symptom-free and there was no evidence of tumour recurrence.J. Paediatr. Surg. Bangladesh 3(1): 47-50, 2012 (January)

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

2021 ◽  
pp. 1-4
Author(s):  
Baher M. Hanna ◽  
Wesam E. El-Mozy ◽  
Sonia A. El-Saiedi
Keyword(s):  
Right Ventricular ◽  
Interventricular Septum ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Rare Condition ◽  
Surgical Excision ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
The Right

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

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