Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease:A case report

BACKGROUND Immunoglobulin(Ig)G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases.IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions,such as Castleman disease,lymphatic follicular reactive hyperplasia,and lymphoma.CASE SUMMARY A 65-year-old male patient,with Guillain-Barre syndrome for 5 years,presented to our hospital complaining of bilateral orbital mass for 2 years.After hospitalization,the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L,but serum interleukin-6 was normal.The pathological morphology of orbital mass and cervical lymph node were consistent,which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles,and the interstitial fibrous tissue was proliferative.Immunohistochemistry showed that CD20(B cells)(+),CD3(T cells)(+),CD38(+),IgG(+),IgG4 positive cells>100/high powered field,and IgG4/IgG>40%.Combined with clinical and immunohistochemical results,lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease.Prednisone acetate treatment was given at 40 mg/d.After 2 wk,the superficial lymph nodes and orbital masses shrank,and the IgG4 level decreased.As prednisone acetate was regularly used at a reduced dosage,no recurrence of the disease has been observed.CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients,and immunoglobulin,complement,interleukin-6,C-reactive protein,and other examinations should be performed to confirm the diagnosis.

Corneal perforation and rare primary adenocarcinoma of the lacrimal gland: A case report

Rationale:Primary adenocarcinoma of the lacrimal gland is rare,and its presentation as corneal perforation is even rarer.Corneal perforation is an ocular emergency that warrants urgent ophthalmic surgery,yet complete staging of lacrimal gland tumor pre-operatively is essential for optimal oncological management.Patient's concerns:A 57-year-old man presented with left eye pain was found to have left eye proptosis and fleshy tissue mass around the eyeball.Uveal tissue was prolapsing over the perforated keratitis cornea,and the eye was full of discharge.Diagnosis:Bedside ultrasound B-scan confirmed vitreous haze,and emergency contrast computed tomography(CT)revealed soft tissue density mass(>40 mm)molding around the left globe and optic nerve without any rim enhancing abscess.Left exogenous endophthalmitis from exposure keratopathy secondary to proptosis caused by the bulky lacrimal tumor located in the confined orbital cone was diagnosed.Interventions:Emergency enucleation surgery of the left eyeball was done for this painful blind eye to control the infection from spreading.Orbital walls were biopsied intra-operatively,and tumor staging was completed by positron emission tomography-CT scan and magnetic resonance imaging.Without evidence of metastasis,left orbital exenteration was followed by adjuvant orbital chemoradiotherapy for the sake of close proximity of resection margin.Outcomes:Left exenterated orbit was fully epithelialized at around 2 months,and there was no recurrence of the disease up to present at the 1 year follow-up.Lessons:Thorough workup on the staging of the disease to minimize the number of operations for oncological patients is always a top priority,yet it may not always be possible as in our case presenting with corneal perforation.

Unusual occurrence of orbital hemangiopericytoma in the zygomatic bone of an adolescent: a case report

Background:Hemangiopericytoma and solitary fibrous tumor are considered related variants on the same spectrum and both may essentially be the same tumor.They are infrequently encountered in the orbital region while the zygomatic bone is an extremely rare location for these neoplasms to occur.Case presentation:A 14-year-old boy presented with complaint of deformity of left infraorbital area and a firm,regular mass in the region.Orbital CT scan revealed a well-defined round isodense intraosseous lesion in the lowermost portion of the lateral orbital wall(zygomatic bone),expanding the bone and protruding anteriorly and medially.MRI showed the mass to be heterogenous and strongly enhancing with contrast medium.Inferior transconjunctival orbitotomy was performed and the mass was removed.The histopathologic examination and immunohistochemistry staining results(positive for CD34,CD31 and smooth muscle actin,but negative for CD99,S100,B-cell lymphoma 2(bcl-2)and desmin)confirmed the diagnosis of hemangiopericytoma.The postoperative course was uneventful,with no evidence of recurrence after 5 years follow up.Conclusions:This case represents the second hemangiopericytoma reported in the zygomatic bone.Although extremely rare,hemangiopericytoma/solitary fibrous tumor might be considered in the differential diagnosis of intraosseous lesions of the orbital and zygomatic region.