Systemic sarcoidosis initially presenting as an orbital tumour

Abstract

Clinical examination and computed tomographic (CT) scan of the orbit revealed a postero-ocular mass, the biopsy of which showed a noncaseating, giant cellular, epithelioid granuloma compatible with sarcoidosis. The patient had no respiratory complaints, but the thoracic CT scan showed multiple bilateral hilar and mediastinal adenopathies. Gallium scan showed increased uptake in both hilar regions, in the left orbit and in the right parotid gland. Multiple bronchial biopsies revealed sarcoid granulomas, with a bronchoalveolar lavage (BAL) cytological pattern of active sarcoidosis. The evolution was favourable with prednisolone given at a dose of 0.5 mg·kg -1 daily.It is suggested that in an unusual presentation of sarcoidosis, a thorough search should be made for localization at other sites, lungs in particular, even in the absence of respiratory complaints. Eur Respir J., 1995, 8, 474-476 Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology most commonly affecting young adults, and presenting most frequently with bilateral hilar lymphadenopathy, with or without pulmonary infiltrates and skin or eye lesions [1]. Although virtually any part of the visual apparatus may be affected [2], orbital sarcoidosis taking the form of a tumour, without eyelid, eyeball or lacrymal gland involvement, is rare [3][4][5].We report a case of systemic sarcoidosis that initially presented as an orbital tumour. Case reportA 73 year old obese woman with recently detected non-insulin-dependent diabetes presented with redness and itching of the left eye of 3 months' duration. Treatment for conjunctivitis had proved useless. The patient was referred to the ophthalmological clinic. On examination, the left conjunctiva was red and chemotic. There was proptosis of the left eye, with a Hertel exophthalmometer reading of 24 mm, and the ocular movements were diminished in all directions, without diplopia. A firm to hard, nonreducible and nontender mass was palpable on the medial sides of the superior and inferior parts of the orbital cavity. Visual acuity of the left eye was 8/10, and that of the right eye was 10/10. There was no evidence of uveitis, and ocular tension was normal in both eyes. On fundoscopy, papillary congestion with hypervascularity was seen in the left eye, which was confirmed by angio-fluoro-retinography. Goldman's perimetry did not show any defects in visual fields.Computed tomographic (CT) scan of the orbits showed a posterio-ocular mass extending to medial and superior regions and probably invading the left optic nerve ( fig. 1). There was no osteolytic lesion. Magnetic resonance imaging (MRI) of the orbit showed that the posterior ocular mass surrounded the left optical nerve, without invasion. Based upon these clinicoradiological findings, left orbital lymphoma was highly suspected. Biopsy CASE REPORT

A Case of Orbital Sarcoidosis with Caseating Granulomatous Inflammation

“…2,6 While past studies have presented a wide range of orbital manifestations of sarcoidosis including lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath, and dural involvement to extraocular muscle involvement, the vast majority of patients described have typical, non-caseating granulomas on histologic examination. 2,3,5,10–13 In the largest case series of orbital sarcoidosis with 26 patients, no patients presented with caseating inflammation, only with reports of non-caseating inflammation. 3 Among 3 large case series on ophthalmic manifestations of sarcoidosis, orbital involvement was only seen in 2 of 202 patients with non-caseating inflammation in 1 series, 14 and in none of the patients in the other 2 large series (>150 patients) without any mention of non-caseating inflammation.…”

A Case of Orbital Sarcoidosis with Caseating Granulomatous Inflammation

“…2,6 While past studies have presented a wide range of orbital manifestations of sarcoidosis including lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath, and dural involvement to extraocular muscle involvement, the vast majority of patients described have typical, non-caseating granulomas on histologic examination. 2,3,5,10–13 In the largest case series of orbital sarcoidosis with 26 patients, no patients presented with caseating inflammation, only with reports of non-caseating inflammation. 3 Among 3 large case series on ophthalmic manifestations of sarcoidosis, orbital involvement was only seen in 2 of 202 patients with non-caseating inflammation in 1 series, 14 and in none of the patients in the other 2 large series (>150 patients) without any mention of non-caseating inflammation.…”

Mikulicz Syndrome Revealing Sarcoidosis: About Two Observations

“…Anterior uveitis is the most frequent ocular manifestation and is noted in 21 to 70% of cases of ocular sarcoidosis depending on the series [2]. Patent orbital manifestations are rarer and are poorly described in the literature [3][4][5]. Among them, the lacrimal gland is the most frequent and is associated with systemic damage in the majority of cases.…”

Bilateral Exophthalmos caused by Systemic Sarcoidosis: a case report

“…When it occurs in this context, it is usually unilateral and is the initial symptom. It can be bilateral-as in our case-only in rare cases [3,[5][6][7][8].…”

A Case of Orbital Sarcoidosis with Caseating Granulomatous Inflammation

“…2,6 While past studies have presented a wide range of orbital manifestations of sarcoidosis including lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath, and dural involvement to extraocular muscle involvement, the vast majority of patients described have typical, non-caseating granulomas on histologic examination. 2,3,5,10–13 In the largest case series of orbital sarcoidosis with 26 patients, no patients presented with caseating inflammation, only with reports of non-caseating inflammation. 3 Among 3 large case series on ophthalmic manifestations of sarcoidosis, orbital involvement was only seen in 2 of 202 patients with non-caseating inflammation in 1 series, 14 and in none of the patients in the other 2 large series (>150 patients) without any mention of non-caseating inflammation.…”

Mikulicz Syndrome Revealing Sarcoidosis: About Two Observations

“…Anterior uveitis is the most frequent ocular manifestation and is noted in 21 to 70% of cases of ocular sarcoidosis depending on the series [2]. Patent orbital manifestations are rarer and are poorly described in the literature [3][4][5]. Among them, the lacrimal gland is the most frequent and is associated with systemic damage in the majority of cases.…”

Bilateral Exophthalmos caused by Systemic Sarcoidosis: a case report

“…When it occurs in this context, it is usually unilateral and is the initial symptom. It can be bilateral-as in our case-only in rare cases [3,[5][6][7][8].…”

A Case of Orbital Sarcoidosis with Caseating Granulomatous Inflammation

“…2,6 While past studies have presented a wide range of orbital manifestations of sarcoidosis including lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath, and dural involvement to extraocular muscle involvement, the vast majority of patients described have typical, non-caseating granulomas on histologic examination. 2,3,5,10–13 In the largest case series of orbital sarcoidosis with 26 patients, no patients presented with caseating inflammation, only with reports of non-caseating inflammation. 3 Among 3 large case series on ophthalmic manifestations of sarcoidosis, orbital involvement was only seen in 2 of 202 patients with non-caseating inflammation in 1 series, 14 and in none of the patients in the other 2 large series (>150 patients) without any mention of non-caseating inflammation.…”

Mikulicz Syndrome Revealing Sarcoidosis: About Two Observations

“…Anterior uveitis is the most frequent ocular manifestation and is noted in 21 to 70% of cases of ocular sarcoidosis depending on the series [2]. Patent orbital manifestations are rarer and are poorly described in the literature [3][4][5]. Among them, the lacrimal gland is the most frequent and is associated with systemic damage in the majority of cases.…”

Bilateral Exophthalmos caused by Systemic Sarcoidosis: a case report

“…When it occurs in this context, it is usually unilateral and is the initial symptom. It can be bilateral-as in our case-only in rare cases [3,[5][6][7][8].…”