Review: Orbital Amyloidosis

Primary localized amyloidosis presenting as diffuse amorphous calcified mass in both orbits: case report

“…Localized amyloidosis is an extremely rare condition generally occurring in the head and neck. Only 4% of such cases occur in the orbital region, mostly in the eyelid and conjunctiva (1,2) . The clinical picture can be quite variable with possible involvement of the lacrimal gland, the extraocular muscles, the anterior orbit (localized mass) and the retrobulbar space (diffuse process).…”

“…Primary localized amyloidosis is an uncommon condition of the orbit with only 40 cases previously reported, 33 of which unilateral (1)(2)(3)(4)(5)(6) . Most cases presented as isolated extraocular muscle enlargement or as a mass in the area of the lacrimal gland or elsewhere in the orbit, usually in the upper part of the anterior orbit (1,2,6) .…”

“…Most cases presented as isolated extraocular muscle enlargement or as a mass in the area of the lacrimal gland or elsewhere in the orbit, usually in the upper part of the anterior orbit (1,2,6) . The purpose of the present report was to document a patient with a unique presentation of primary localized amyloidosis characterized by a diffuse and densely calcified mass in both orbits.…”

Multiple myeloma with lacrimal gland amyloidosis and sarcoidosis

“…Previous nomenclature has divided amyloidosis into either primary or secondary forms. In primary amyloidosis, immunoglobulin light chain fragments (either kappa or lambda) are precursors to the amyloid light forms of amyloidosis that are either idiopathic or related to light chain-producing monoclonal gammopathy, as in multiple myeloma [6][7][8]. Secondary amyloidosis, also referred to as amyloid associated, or reactive amyloidosis results from longstanding inflammation or infection derived from an acute phase protein known as amyloid A with deposition leading to hepatosplenomegaly and renal dysfunction.…”

“…In patients with primary amyloid deposition in the lacrimal gland, involvement is usually unilateral, whereas in systemic cases the involvement tends to bilateral [6] as seen in our patient. Lacrimal gland amyloidosis is a rare phenomenon with few case reports in the available literature [5][6][7][8]. The majority of reported cases of lacrimal gland amyloidosis are primary and unilateral; bilateral lacrimal gland involvement is unusual.…”