Conjunctival extranodal marginal zone B‐cell lymphoma with crystal‐storing histiocytosis

Localized ocular crystal-storing histiocytosis and associated lymphoma - Report of two cases and review of literature

“…There are only a few reports describing CSH within certain ocular structures such as the cornea, conjunctiva, lacrimal gland, and orbital soft tissues, with most patients having an underlying lymphoproliferative or a plasma cell disorder including MM, lymphoplasmacytic lymphoma, MALT lymphoma and MGUS. 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 A small proportion of CSH is noted to be related to benign disease that is autoimmune or inflammatory in nature. 1 , 3 In the two case reports that we present here, the lesions in the lacrimal gland and orbital soft tissues showed localized ocular CSH with associated lymphoma without any systemic involvement.…”

“… Local radiation with resolution of initial mass, recurrence was treated with incisional biopsy Kusakabe et al 4 2006 81/F MALT lymphoma Unilateral lower eyelid 5-month history of a slowly enlarging tumor on her left lower eyelid, Extension to inferior oblique and inferior rectus muscles Surgical excision and regional radiotherapy, no recurrence 1 yr post. de Alba Campomanes et al 10 2009 66/M MGUS Unilateral orbit, EOM, cornea 3-year history of progressive ptosis, proptosis, external ophthalmoplegia and corneal crystals Not reported Radhakrishnan et al 11 2014 53 M MALT lymphoma Unilateral orbit 8-year history of painless, gradually progressive right inferior orbital swelling and proptosis Local external beam radiation x 3weeks with complete resolution of mass and proptosis Yu et al 12 2014 32/M EMZL Unilateral conjunctiva Salmon coloured conjunctival patch lesion Excisional biopsy Mittal et al 13 2015 58/M EMZL Unilateral Conjunctiva Other: mediastinal and abdominal lymph nodes 2-year history of a subconjunctival pink ‘salmon-coloured’ tumor with prominent blood vessels Complete resolution with chemotherapy (rituximab 375 mg/m2 iv; cyclophosphamide 750 mg/m2 iv; vincristine 1.4 mg/m2 iv; prednisone 40 mg/m2 iv) Boudhabhay et al 2 2018 60/M MM Bilateral cornea, retina and optic nerve Other: kidney, bone marrow …”

Orbital Crystal-Storing Histiocytosis: A Clinicopathologic Study of 4 Cases

“…7 Crystal-storing histiocytosis with underlying ENMZL presenting as a salmon-patch conjunctival mass is distinctly rare, with 2 such reports. 1,8 Crystal-storing histiocytosis-related orbital masses can cause proptosis and reduced eye movements 6,9-12 : of particular interest is a patient with lacrimal gland CSH and ENMZL arising 5 years after radiotherapy for conjunctival lymphoma, 9 and another patient with known systemic CSH and lymphoplasmacytic lymphoma, who presented with bilateral proptosis. 12 The presence of localized B-cell lymphoma in three quarters of the authors' patients accords with the findings in 11 published cases of periocular CSH, of whom 5 of 11 had ENMZL, 1,8-11 2 had multiple myeloma, 3,4 2 had paraproteinemias, 5,6 1 had plasmacytic conjunctivitis, 7 and 1 had lymphoplasmacytic lymphoma.…”

“…I diopathic sclerosing orbital inflammation (ISOI) is a rare and specific condition, encompassing about 5% to 8% of idiopathic orbital inflammatory diseases. 1 Histologically, it is characterized by severe fibrosis with sparse, mixed chronic inflammatory cells. Treatment modalities such as immunosuppression, surgical excision, and radiotherapy are sometimes, but not consistently, effective.…”

“…It affects a broad age range, has equal gender incidence, and can involve any organ although most commonly the head and neck. 1 Crystal-storing histiocytosis is frequently associated with an underlying B-cell lymphoma, plasma cell neoplasm, or rarely inflammatory disorders, 2 and treatment and prognosis depends on the underlying disease.…”