IgG4 Staining in Thyroid Eye Disease.

IgG4-related ophthalmic disease is increasingly widely recognized. Moreover, IgG4 staining can occur in other inflammatory diseases. The authors report a case of IgG4 staining of an enlarged, inflamed levator palpebrae superioris in a patient with a past history of thyroid eye disease. A 78-year-old woman with quiescent hyperthyroidism had clinical and radiological evidence of levator palpebrae superioris inflammation without superior rectus involvement. A biopsy was consistent with IgG4-related ophthalmic disease. There was a marked but incomplete response to an orbital injection of triamcinolone. The authors discuss the association between thyroid eye disease and IgG4 staining and the diagnostic issues that arise when IgG4-related ophthalmic disease criteria are fulfilled in patients with other orbital inflammatory conditions.

Cutaneous manifestations of peripheral T-cell lymphoma, not otherwise specified: a case series highlighting the diagnostic challenges for this heterogeneous group.

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a rare, heterogeneous group of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the defined T-cell entities, according to the World Health Organization classification. Most cases present with late stage nodal disease; however extranodal involvement is common. Skin and subcutaneous involvement is reported in approximately 20% of cases. Little attention has been given to the highly variable skin manifestations in the literature. It is our experience that lesions can present in ways other than previously described nodular or tumourous lesions that often ulcerate. We present a case series from a large tertiary institution of seven cases of PTCL, NOS with skin involvement, highlighting the variable presentations and diagnostic challenges for this heterogeneous group.

Unifocal Langerhans cell histiocytosis of the superior oblique muscle.

Orbital Langerhans cell histiocytosis (LCH) without bone involvement is rare. Isolated involvement of an extraocular muscle without bone change, to the best of the authors' knowledge, has not been previously reported. They describe a unique case of unifocal LCH of the superior oblique muscle with no bone involvement. A 16-year-old girl presented with a 2-week history of diplopia and headache. CT and MRI showed an enlarged right superior oblique muscle with no associated bone involvement. Biopsy with partial debulking revealed LCH and staging confirmed unifocal disease. The lesion underwent complete radiologic resolution following surgery. There was no recurrence after 16 months follow up.

Successful Giant Thymic Cyst Removal: Case Report and Review of the Literature.

Giant thymic cysts are a rare clinical entity evolving from smaller benign thymic cysts over many years. Benign thymic cysts account for approximately 3% of all mediastinal masses. There is a paucity of literature regarding benign thymic cyst management, especially when dealing with giant cysts. This can lead to potential confusion amongst clinicians on how to best treat these patients. We report the successful diagnosis and treatment of a 76 year-old female with a giant, benign thymic cyst. This cyst was discovered incidentally and after consultation of the literature it was found management strategies regarding this condition are scarce. After careful consideration of surgical principles, patient preference and potential complications of a conservative approach, the successful surgical removal of a 1.8 kg cyst took place. The patient improved symptomatically with improved exercise tolerance and lung function tests. This case demonstrates the benefits of giant thymic cyst removal thus confirming diagnosis, reducing potential serious complications and improving patient quality of life.

Immunoglobulin G4-related disease of the hard palate.

A 71-year-old woman presented with erythematous, nontender, bilateral hard palate nodules of 6-month duration. Biopsy showed collagenous sclerosis and a follicular lymphoplasmacytic infiltrate among the minor salivary glands. Immunoglobulin G (IgG) and IgG4 staining showed 280 IgG4(+) cells per high-power field and a ratio of IgG4(+) to IgG(+) cells of 0.8. The patient subsequently developed bilateral lacrimal gland and parotid gland enlargement associated with an increased serum IgG4 level of 3,031 mg/dL (≤ 135 mg/dL). Left lacrimal gland biopsy confirmed IgG4-related dacryoadenitis. The patient declined corticosteroid treatment for IgG4-related disease (IgG4-RD) and remained stable at 15 months after the first presentation. Spontaneous, partial resolution of the palatal lesion was observed during follow-up. IgG4-RD should be considered in the differential diagnosis of lymphoplasmacytic lesions of the hard palate.

Variant human T-cell lymphotropic virus type 1c and adult T-cell leukemia, Australia.

Human T-cell lymphotropic virus type 1 is endemic to central Australia among Indigenous Australians. However, virologic and clinical aspects of infection remain poorly understood. No attempt has been made to control transmission to indigenous children. We report 3 fatal cases of adult T-cell leukemia/lymphoma caused by human T-cell lymphotropic virus type 1 Australo-Melanesian subtype c.

Outcomes of lymphoma in South Australia, 1977-2007.

OBJECTIVE: To determine survival rates of patients with lymphoma in South Australia. DESIGN AND SETTING: De-identified data from the SA Cancer Registry on all patients with lymphoma were analysed, as well as the subgroup treated at the Royal Adelaide Hospital (RAH). For non-Hodgkin lymphoma (NHL), we used the International Working Formulation (IWF) grading. SA and RAH data on survival rates were compared with those for the whole of Australia and the United States. PATIENTS: All patients diagnosed with lymphoma and treated in SA in 1977-2007. OUTCOME MEASURES: 5-year survival rates for patients with lymphoma, by type of lymphoma and age. RESULTS: Of the total of 8651 patients with lymphoma, 939 were classified as having Hodgkin lymphoma (HL) and 7712 as having NHL. Of those with NHL, 1805 had low-grade, 3576 intermediate-grade, and 510 high-grade NHL. In another 1821 patients, the data were insufficient to make an IWF grading. There was a substantial increase in 5-year survival rates for patients with lymphoma between 1977 and 2007 in SA. While the increase in 5-year survival rates for HL was 7.6 percentage points, survival rates peaked at 88%. For NHL, there was an 18.7 percentage points increase in 5-year survival rates. The first significant increase of 7 percentage points was associated with the introduction of bone marrow transplantation; this was maintained with the increase in 5-year survival rates reaching 14 percentage points by 1995-1999. Since 1999, there has been a further increase of 5 percentage points in 5-year survival rates with the introduction of rituximab. CONCLUSION: Outcomes in patients with NHL have improved significantly, most likely because of the use of bone marrow transplantation and rituximab. Hospital- and state-based cancer registry data reflect the reality of population outcomes and the impact of new technologies.

Idiopathic Dacryoadenitis: Clinical Features, Histopathology, and Treatment Outcomes.

PURPOSE: To review the clinical and histologic features of idiopathic dacryoadenitis, and to assess prognostic factors associated with disease recurrence, treatment recalcitrance, and incomplete treatment response. DESIGN: Retrospective interventional case series. METHODS: setting: Tertiary referral centers. PATIENTS: Seventy-nine cases of biopsy-confirmed idiopathic dacryoadenitis. OBSERVATION PROCEDURES: The following data were reviewed: age, sex, laterality, symptom onset, clinical presentation, histopathology, treatment response, and recurrence. MAIN OUTCOME MEASURES: Rates of treatment recalcitrance, incomplete treatment response, and recurrence. RESULTS: Idiopathic dacryoadenitis patients had a mean age of 50 years, 57% were female, and 16% of cases were bilateral. Fifty-two percent had inflammation extending to adjacent structures on imaging. Twenty percent were recalcitrant to treatment, 17% had an incomplete treatment response, and 15% of patients had a recurrence during a mean follow-up time of 64 months. Risk factors for an incomplete treatment response were male sex (P = .01) and inflammation extending to extraocular muscle (P = .01). A clinical presentation of "classic" dacryoadenitis was a risk factor for treatment recalcitrance (P = .02). Bilateral cases were younger than unilateral cases (P = .004) and had an increased risk of recurrence (P = .05). Sclerosing cases were associated with an insidious onset of symptoms (P = .009), but neither histopathology nor the speed of symptom onset was associated with a poor prognosis. CONCLUSION: Thirty-seven percent of idiopathic dacryoadenitis had a poor response to treatment and 15% of cases recurred. The prognostic factors identified in this study have not been reported previously and may inform management.

An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD.

AIM: To determine the proportion of idiopathic orbital inflammation (IOI) and orbital benign lymphoid hyperplasia (OBLH) accounted for by immunoglobulin (Ig)G4-related orbital disease (IgG4-ROD) using the comprehensive diagnostic criteria for IgG4-related disease published by Umehara et al and the consensus diagnostic criteria published by Deshpande et al. Secondary aims were to compare the histological and clinical features of IgG4-ROD and non-IgG4-ROD cases, and to compare IgG4-ROD cases diagnosed using the comprehensive diagnostic criteria with those diagnosed using the consensus diagnostic criteria. METHODS: A retrospective histopathological review and clinical case series. 55 cases of biopsy-confirmed non-granulomatous IOI and 10 cases of biopsy-confirmed OBLH were included. The intensity of sclerosis, lymphoplasmacytic infiltration and eosinophilic infiltration was graded from 0 to 3+ using a standardised and validated scoring system. RESULTS: IgG4-ROD accounted for 50% and 40% of cases originally diagnosed as OBLH and 23.6% and 5.4% of cases originally diagnosed as IOI, using the comprehensive diagnostic criteria and the consensus diagnostic criteria, respectively. IgG4-ROD cases had numerous significant histological differences, but relatively few significant clinical differences, from non-IgG4-ROD cases. Compared with the comprehensive diagnostic criteria, the consensus diagnostic criteria identified a group of IgG4-ROD cases with a slightly higher ratio of IgG4+ to IgG+ (p=0.01) and a slightly longer duration of symptoms (p=0.02). CONCLUSIONS: This is the largest review of IgG4 staining among biopsy-confirmed IOI and OBLH. IgG4-ROD accounted for a substantial proportion of OBLH. The prevalence among cases of IOI was considerably reduced when the consensus diagnostic criteria were used in place of the comprehensive diagnostic criteria.

Thoracic outlet syndrome following breast implant rupture.

We present a patient with bilateral breast implant rupture who developed severe locoregional silicone granulomatous lymphadenopathy. Poly Implant Prothese silicone implants had been used for bilateral breast augmentation 5 years prior. Extracapsular implant rupture and bilateral axillary lymphadenopathy indicated explantation, capsulectomy, and selective lymph node excision. Histology demonstrated silicone lymphadenopathy with no evidence of malignancy. Over the subsequent 12 months, she developed progressive locoregional lymphadenopathy involving bilateral cervical, axillary, and internal mammary groups, resulting in bilateral thoracic outlet syndrome. We report the unusual presentation, progression, and the ultimate surgical management of this patient.

IgG4-related orbital disease: a meta-analysis and review.

IgG4-related orbital disease (IgG4-ROD) is a recently described condition that may account for a significant proportion of idiopathic lymphoplasmacytic or sclerotic orbital lesions. This study is the first meta-analysis of published cases and reveals several differences between IgG4-related disease affecting the orbit and that affecting the pancreas. IgG4-ROD affects a slightly younger group of patients, affects men and women approximately equally, is commonly associated with salivary gland lesions, is associated with a relatively higher serum IgG4 and may confer an increased risk of non-Hodgkin Lymphoma. Its pathogenesis may involve an immune response to antigen exposure in the upper aerodigestive tract.