Amyloidosis of lacrimal gland

Primary localized amyloidosis of lacrimal gland is a rare occurrence. This report describes a female patient with isolated amyloidosis of the lacrimal gland. A 45-year-old Indian woman presented with a swelling over the left lacrimal gland region. Computed tomography showed uniform enlargement of the lacrimal gland. A lacrimal gland biopsy revealed amyloidosis. No systemic involvement was detected on further investigation. To our knowledge, this is the first report of lacrimal gland amyloidosis from India and our report also highlights the importance of lacrimal gland biopsy in diagnosing lacrimal gland masses.     

Amyloidosis of the orbit and adnexae.

Five patients representing a spectrum of orbital and adnexal amyloidosis are presented. One patient with conjunctival amyloidosis did not have antecedent infection nor systemic disease (primary localized amyloidosis), while another patient with conjunctival amyloidosis had a long history of repeated attacks of bacterial conjunctivitis (secondary localized amyloidosis). The third patient presented with serial bilateral lacrimal gland amyloid without systemic disease (primary localized orbital amyloidosis). Another patient had intraorbital amyloid associated with metastatic deposits or immunoglobulin-producing lymphoma cells in the orbit (secondary systemic amyloidosis) and a fifth patient, with borderline Congo red staining for amyloid, was shown by electron microscopy to have macrophages with intracellular amyloid-like fibrils and extracellular crystalline material resembling immunoglobulin deposits. Amyloidosis has been clearly shown to be associated with immunoglobulins and plasma cells. Electron microscopic findings in our two cases of primary localized orbital amyloidosis leave unresolved the suggestion that mesenchymal cells, in addition to plasma cells, may be responsible for the production of amyloid. It should be emphasized for the clinician that amyloidosis of the conjunctiva and orbit is almost always a benign disease without systemic implications. On the other hand, amyloid of the skin of the eyelids, is a hallmark of generalized amyloidosis.     

Primary localised amyloidosis of the orbit.

AIMS/BACKGROUND: Primary localised amyloidosis is rarely encountered in the orbit. The typical clinical and radiological appearances have not been clearly established, in particular the single site deposition of amyloid has never been proved. METHODS: Six cases were reviewed in detail and their clinical and radiological characteristics are described here. Four patients had scintigraphy with 123I serum amyloid P component and one patient had typing of the amyloid fibrils. RESULTS: All the patients had a firm mass in the upper orbit with a predilection for the region of the lacrimal gland. Computed tomography showed a homogeneous mass with thickening and irregularity of the adjacent bone and/or calcification within the mass. None was associated with systemic disease. Scintigraphy with 123I serum amyloid P component demonstrated that the amyloid was confined to the orbit. In one patient the amyloid fibrils were derived from an IgG4 heavy chain constant domain. The lesions were partially excised with subsequent clinical stability over 6 months to 18 years in all but one patient who had continued enlargement of the lesion. CONCLUSION: There is a characteristic clinical and radiological pattern for primary localised amyloidosis of the orbit. The disease process is truly local and not part of a systemic process. A majority respond to simple debulking with subsequent observation.     

Orbital and peri-orbital amyloidosis: a report of four cases

Our objective was to describe our experience with orbital amyloidosis, and illustrate the different forms of presentation. This was a retrospective case series of four patients with biopsy-proven orbital amyloi- dosis, over the period from 2014 to 2016. We describe its diagnostic and clinical characteristics, management and systemic study. The series comprised three women and one man of mean age 52 ± 9.4 years. Affected sites were the lacrimal gland, tarsal conjunctiva, lacrimal sac and orbit. In three of the four patients, calcifications were observed. Three patients had associated ptosis. The patient with orbital involvement suffered an unusual vascular complication during surgery and systemic disease was detected. Management included debulking and complete resection of the lesion. In conclusion, orbital amyloidosis presents as a wide variety of forms. Its diagnosis is biopsy-based. Calcifications in biopsy specimens or images should raise suspicion of amyloidosis. It is important to always check for systemic amyloidosis.     

Treatment of primary lymphoma of the lacrimal gland by surgical excision alone: A 5-year follow-up study

PURPOSE. To evaluate the long-term efficacy of surgical excision alone in the treatment of non-Hodgkin(1)s lymphoma localized to the lacrimal gland. MATERIALS AND METHODS. Thirteen patients with primary lacrimal gland lymphoma were included. The presumptive diagnosis was based on: (1) The painless and relatively slow onset of unilateral lacrimal gland swelling, mostly in elderly adults; (2) The CT pattern of a soft tissue mass in the lacrimal gland region with defined margins and molds to the globe; (3) The negative results of systemic work-up. The involved lacrimal gland was excised via an anterior trans-septal approach in 12 cases and by lateral canthotomy, upper cantholysis, and a trans-periosteal approach in one case. Following histopathologic confirmation of the diagnosis, no supplemental therapy was given. The patients were followed for a minimum of 5 years (5-8 years). RESULTS. In all patients, the excised lacrimal gland lymphoma was firm, nodular and appeared encapsulated. The histologic subtypes of the excised masses were: low-grade lymphomas in 11 cases (7 small lymphocytic, 3 plasmacytoid lymphocytic, 1 follicular small cleaved) and intermediate grade in 2 cases (1 follicular large cell and 1 diffuse small cleaved). No evidence of local recurrence or systemic dissemination was reported during the follow-up period in any of the patients. CONCLUSIONS. Surgical excision of the tumor is a new curative technique for lymphoma localized to the lacrimal gland without any other orbital or systemic involvement. By this technique, orbital radiotherapy and its potential ocular complications can be avoided. However, more study is recommended.     

Treatment of primary lymphoma of the lacrimal gland by surgical excision alone: A 5-year follow-up study

PURPOSE . To evaluate the long-term efficacy of surgical excision alone in the treatment of non-Hodgkin¹s lymphoma localized to the lacrimal gland. MATERIALS AND METHODS . Thirteen patients with primary lacrimal gland lymphoma were included. The presumptive diagnosis was based on: (1) The painless and relatively slow onset of unilateral lacrimal gland swelling, mostly in elderly adults; (2) The CT pattern of a soft tissue mass in the lacrimal gland region with defined margins and molds to the globe; (3) The negative results of systemic work-up. The involved lacrimal gland was excised via an anterior trans-septal approach in 12 cases and by lateral canthotomy, upper cantholysis, and a trans-periosteal approach in one case. Following histopathologic confirmation of the diagnosis, no supplemental therapy was given. The patients were followed for a minimum of 5 years (5–8 years). RESULTS . In all patients, the excised lacrimal gland lymphoma was firm, nodular and appeared encapsulated. The histologic subtypes of the excised masses were: low-grade lymphomas in 11 cases (7 small lymphocytic, 3 plasmacytoid lymphocytic, 1 follicular small cleaved) and intermediate grade in 2 cases (1 follicular large cell and 1 diffuse small cleaved). No evidence of local recurrence or systemic dissemination was reported during the follow-up period in any of the patients. CONCLUSIONS . Surgical excision of the tumor is a new curative technique for lymphoma localized to the lacrimal gland without any other orbital or systemic involvement. By this technique, orbital radiotherapy and its potential ocular complications can be avoided. However, more study is recommended.     

Systemically disseminated extranodal marginal zone B-cell lymphoma of lacrimal gland in a patient with systemic lupus erythematosus

A 33-year-old woman with systemic lupus erythematosus had bilateral eyelid swelling. On examination, bilateral lacrimal gland mass were palpable. The morphologic and the immunohistochemical features were supportive of the diagnosis of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue-type (MALT lymphoma). Further systemic evaluation revealed she had clinical stage IV disease with bone marrow involvement. She underwent local radiotherapy and several cycles of chemotherapy. However, the patient died of sepsis with the disease after 48 months. Systemically disseminated MALT lymphoma presenting initially in the lacrimal gland of a patient with systemic lupus erythematosus is rare, and the prognosis is poor.     

Non-Hodgkin-Lymphom der Tränendrüse

Background

Non-Hodgkin lymphoma is a systemic disease and various organs can therefore be affected. Ocular manifestations of non-Hodgkin lymphomas are possible but involvement of the eyelids or lacrimal glands are uncommon.

Case Report

A 63-year-old female patient suffered from a painless upper eyelid swelling of the left eye for 3 weeks. The anterior and posterior parts of the eyes showed no pathologic signs and X-ray examination of both orbits revealed no pathologic findings. Magnetic resonance imaging and computer tomography of the head revealed a neoplasm of the left lacrimal gland and also of the left parotid gland. The histologic examination revealed a recurrent non-Hodgkin lymphoma.

Conclusions

The causes of eyelid swellings can be multiple, however, painless swellings may also be caused by neoplasms. In the case described here it was interesting that behind a painless eyelid swelling even a systemic disorder was hidden, i.e. a recurrence of a non-Hodgkin lymphoma, which was localized in the lacrimal gland.     

Diverse clinical presentations of orbital sarcoid

PURPOSE: To review the clinical presentation, location, systemic features, management, and natural history of orbital sarcoid. DESIGN: Retrospective, noncomparative, interventional case series. METHODS: Twenty patients with sarcoid and sarcoidal reactions of the orbit underwent biopsy, excision of localized mass, and systemic and local treatment at a tertiary referral center. Age, gender, onset, symptoms and signs, characterization of disease process, location, systemic disease, associated systemic features, management, and recurrence of the disease were identified. RESULTS: Of the 20 patients studied, five were male and 15 were female. The mean age was 50.55 +/- 16.43 years (range, 18 to 77 years). The most common symptom was the presence of a palpable mass, followed by eyelid swelling. Review of the computed tomographic scans revealed four main categories of presentation: lacrimal gland infiltration (n = 11; 55%), orbital mass (n = 4; 20%), optic nerve sheath and dural involvement (n = 4; 20%), and extraocular muscle involvement (n = 1; 5%). Concurrent systemic sarcoidosis discovered after the diagnosis of orbital sarcoid was present in 10 cases (50%). The remaining showed no evidence of systemic disease at follow-up. Angiotensin converting enzyme analysis was performed in 10 cases; in only two (20%) was elevated, and in the remaining eight, it was within normal levels. CONCLUSIONS: Orbital sarcoid has a diverse clinical presentation varying from lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath and dural involvement, to extraocular muscle involvement. The orbital site most commonly involved was the lacrimal gland.     

Ocular adnexal lymphoma: Five case reports and a literature review

This article reports the clinical course and treatment of ocular adnexal lymphoma based on a retrospective review of five cases with a histologically approved ocular adnexal lymphoma at Kaohsiung Veterans General Hospital over 10 years. Extranodal B-cell lymphoma in the orbit, lacrimal gland, eyelid, or conjunctiva was found in these patients. Four of them were female, and they were aged 45–64 years. All patients were also consulted with hematologists for possible systemic involvement and therapeutic plan. The patient with retrobulbar and orbital apex involvement received systemic chemotherapy. The patient with lacrimal gland involvement experienced tumor recurrence after local excision, and therefore received adjuvant radiotherapy. The remaining three patients had localized lymphoma on the eyelid or bulbar conjunctiva, and they all showed no recurrence after surgical excision. The incidence of ocular adnexal lymphoma has risen worldwide over the last few decades. Although most cases are confined to ocular adnexal, some may also be associated with disseminated lymphoma. Accurate diagnosis and staging is mandatory for appropriate treatment. Generally speaking, localized and low-grade ocular adnexal lymphoma involved eyelid or conjunctiva seem to have good outcome after surgical excision only. Systemic chemotherapy should be considered in patients with advanced disease or systemic manifestations, and radiotherapy also offers a good choice for lacrimal gland lymphoma.     

Orbital Wegener granulomatosis without systemic findings

PURPOSE: To describe a case of orbital Wegener granulomatosis without systemic disease. METHOD: Case report. RESULTS: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with Wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically. CONCLUSION: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.     

Isolated lacrimal gland involvement in Rosai-Dorfman-Destombes disease

Rosai-Dorfman-Destombes (sinus histiocytosis with massive lymphadenopathy) disease is an uncommon disease characterized by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal disease. Orbital involvement occurs in 9-11% of cases. However, isolated Rosai-Dorfman-Destombes disease of the lacrimal gland without any systemic involvement is very rare with only three case reports. We describe here one such young male patient with unilateral lacrimal gland swelling. Excision biopsy revealed almost complete replacement of the lacrimal gland by lymphocytes, plasma cells and large pale histiocytes. The latter exhibited emperipolesis and stained positive for S-100 and CD68 on immunohistochemistry. Patient is well and has no other manifestation or recurrence of the disease during a follow-up of 24 months.     

IgG4-related dacryoadenitis

A 44-year-old female presented with bilateral eyelid swelling and painless, palpable mass over the lacrimal gland area for more than 1 year. A bilateral enlarged lacrimal gland with mild contrast enhancement was noted on computed tomography. Biopsy of the lacrimal gland showed lymphoplasmacytic infiltrate with focal hyaline and sclerotic change, and immunohistochemical staining revealed numerous IgG4-positive plasma cells, which was characteristic of IgG4 dacryoadentitis. Serum IgG and IgG4 levels were within normal range. The patient was treated with surgical excision instead of systemic steroid owing to poor tolerance. No tumor recurrence or systemic involvement was noted during the follow-up period. Our case highlights the importance of IgG4 dacryoadenitis in differentiating bilateral lacrimal gland inflammatory conditions. A normal serum IgG4 level does not rule out the diagnosis and surgical excision may be an alternative for those who are considered ineligible for corticosteroid treatment.     

Recurrent acute lymphoblastic leukemia presenting in the lacrimal gland

A 14-year-old girl with a history of acute lymphoblastic leukemia presented with periorbital swelling of the right eyelid associated with enlargement of the lacrimal gland and cervical lymphadenopathy. Radiographic imaging showed diffuse enlargement and enhancement of the right lacrimal gland. The patient underwent an incisional biopsy of the lacrimal gland that showed atypical lymphocytes consistent with recurrent acute lymphoblastic leukemia. The lacrimal gland is a rare extramedullary site for recurrence of this disease.     

Idiopathic eosinophilic orbital pseudotumor of the lacrimal gland in a child

BACKGROUND: Idiopathic orbital pseudotumor is a rare disease. 10 % of the cases occur in children. In most of these juvenile cases, the swelling is bilateral, and histologically, eosinophilic granulocytes are found. In contrast to adults, children very rarely develop an associated systemic disorder. The disease is generally treated with steroids. Little is known about the natural course of idiopathic orbital pseudotumors in childhood. PATIENT: A 2S-year old girl presented with a unilateral swelling of the right upper lid. Four weeks later, a comparable swelling of the left side was revealed. We took biopsies of the lacrimal gland on both sides. Histology displayed an eosinophilic pseudotumor of the lacrimal gland. Steroid treatment was started, but after 4 weeks the parents refused to continue the treatment. During the following year, the bilateral swelling remained unchanged. Surprisingly, the bilateral swelling disappeared 3 years later and no systemic disorder had occured. CONCLUSIONS: Our case shows that spontaneous resolution of a juvenile eosinophilic orbital pseudotumor is possible. Treatment with steroids does not appear to be compulsory in all cases.     

Rosai-Dorfman syndrome affecting the lacrimal gland

This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node biopsy produced a histological diagnosis compatible with Rosai-Dorfman disease. Later he developed an acute red proptotic eye. He had severe proptosis due to an enlarged lacrimal gland. He refused surgical excision of the tumour, which is suggested if there is an ocular adnexal involvement. Conservative treatment with systemic steroid resulted in the resolution of lacrimal gland swelling, nasal sinusal mucosal thickening and cervical lymphadenopathy. Previous studies have shown that patients with Rosai-Dorfman syndrome are often black males1 and require surgery.     

Rosai-Dorfman syndrome affecting the lacrimal gland

This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node biopsy produced a histological diagnosis compatible with Rosai-Dorfman disease. Later he developed an acute red proptotic eye. He had severe proptosis due to an enlarged lacrimal gland. He refused surgical excision of the tumour, which is suggested if there is an ocular adnexal involvement. Conservative treatment with systemic steroid resulted in the resolution of lacrimal gland swelling, nasal sinusal mucosal thickening and cervical lymphadenopathy. Previous studies have shown that patients with Rosai-Dorfman syndrome are often black males1 and require surgery.     

Mikulicz's disease: a new perspective and literature review

PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature. METHODS: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA. RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders. CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of Sj?gren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.     

Lacrimal gland abscess: two case reports

BACKGROUND: Bacterial dacryoadenitis is rare and suppuration leading to abscess formation within the lacrimal gland has been very rarely reported in the antibiotic era. METHODS: The medical records and investigation results, including computed tomography (CT), of two patients with lacrimal gland abscess were reviewed. RESULTS: Two cases of lacrimal gland abscess, one a 28-year-old male and the other a 64-year-old female, are described. Both demonstrated a characteristic low-density area within an enlarged lacrimal gland on CT. The first case had been treated with antibiotics and the abscess, when drained, was sterile. The second case settled spontaneously. Neither patient suffered any sequelae of dry eye. CONCLUSIONS: Although rare, lacrimal gland abscess may still occur and may require surgical drainage if spontaneous resolution does not occur.     

Makroglobulinämie Waldenström der Tränendrüse bei einer Patientin mit Sarkoidose

We report a case of bilateral lacrimal gland involvement as the first sign of Waldenstrom’s macroglobulinaemia in a patient with sarcoidosis. Histological analysis of an incisional biopsy revealed a lymphoplasmocytic lymphoma consistent with Waldenstrom’s macroglobulinaemia. No noncaseating granulomas were encountered. Systemic treatment was initiated and resulted in complete resolution of the lesions. In a patient with a systemic disease, such as sarcoidosis, and lacrimal gland involvement, a biopsy of the lacrimal gland mass should be taken to make a correct diagnosis and start appropriate treatment.