HISTOPATHOLOGY INDIA.COMMyxoid Tumours of Soft Tissue


 

                  

 Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes.

 Visit:  Sarcoidosis  ; Cutaneous Sarcoidosis ; Pathological Diagnosis of Granulomatous Lung Diseases ; Non-necrotising Granulomatous Inflammation of the lung.

Ocular manifestations of sarcoidosis vary enormously. They include the conjunctiva, lacrimal gland, orbit, intraocular structures and eye-lid, either isolated or combined.

Sarcoid immune reaction in the conjunctiva is a dynamic process in which proliferation of immunocompetent mononuclear cells precedes the stage of granuloma formation.

Conjunctiva is involvement in about 30% of patients with sarcoidosis.

Conjunctival biopsy is considered to be a simple, safe and specific diagnostic procedure for sarcoidosis. Step sections should be done through the entire specimen since granulomas may be focal.

Bilateral, focal, multilobular, multiple pale yellow deposits (the size of millet grains) have been noted in the bulbar conjunctiva.

Unusually large, bilateral conjunctival tumours have been reported as a primary manifestation of sarcoidosis.

Microscopic features:   Noncaseating granulomatous epithelioid cell inflammation without evidence of acid fast bacilli or any other microorganisms.  Together with noncaseating granulomas the biopsy specimen may show prominent asteroid bodies.

Differential diagnosis :  Foreign body granuloma ; Mycobacterium Tuberculosis ; Cat Scratch Disease  ; Tularemia  ; Syphilis .

             

Conjunctival biopsy in sarcoidosis.J Chin Med Assoc.2006 Oct;69(10):472-7.

BACKGROUND: Conjunctival biopsy is considered to be a simple, safe and specific diagnostic procedure for sarcoidosis. This study was designed to determine the value of this procedure in Taiwan. METHODS: This study was conducted from December 2003 to April 2005 at the uveitis clinic of Taipei Veterans General Hospital. Blind sampling was conducted, obtaining a biopsy sample measuring 1 cm long by 3 mm wide from both lower fornices. A positive result was defined as the presence of non-caseating granuloma when other granuloma-forming processes had been excluded. RESULTS: Twenty-nine patients (7 men, 22 women) were enrolled. Mean age at diagnosis was 47.8 +/- 12.4 years. The most common initial symptom was eye-related problems in 19 (65.5%) patients. Of 58 biopsies, 15 (25.9%) specimens in 11 (37.9%) patients proved to be positive. Four patients experienced bilateral involvement; 7 patients had unilateral involvement. No prominent conjunctival nodules or follicles were noted. Gender, age, presence of uveitis, initial symptoms, and chest condition comparisons revealed no association between positive and negative conjunctival biopsies. CONCLUSION: Blind and bilateral conjunctival biopsy, due to its ease, safety and specificity, could be the first biopsy inpatients with clinical or chest X-ray abnormalities suggesting sarcoidosis. None of our patients with positive biopsy had nodular lesions.

Non-invasive detection of multinucleated giant cells in the conjunctiva of patients with sarcoidosis by in-vivo confocal microscopy.Ocul Immunol Inflamm. 2006 Aug;14(4):203-6.

AIM: To explore the use of in-vivo confocal microscopy (IVCM) as a potential non-invasive adjunctive tool for diagnosing sarcoidosis. METHODS: Conjunctivae were imaged using confocal microscopy in 10 patients with sarcoidosis and 27 control subjects. We utilized the ASL-1000 Scanning Confocal Microscope (Advanced Scanning Ltd., New Orleans, LA) and the Confoscan 3 (Nidek Co. Ltd., Gamagori, Japan). Two masked observers reviewed the in-vivo confocal images of the conjunctivae in these subjects. One masked observer was experienced in reviewing confocal images. The most striking and obvious feature seen in granulomatous inflammation on confocal microscopy is the presence of multinucleated giant cells (MGCs). RESULTS: Unmasked observation of the scans revealed MGCs in six of the 10 sarcoid patients and no MGCs in the controls. One experienced masked observer found MGCs in five of the 10 patients with sarcoidosis and had no false-positive results (Fisher's exact test, p = 0.001; specificity = 1; sensitivity = 50% for the diagnosis of sarcoidosis and 83% compared to the unmasked observer). The second less-experienced masked observer detected MGCs in three of the 10 patients and three of the 27 controls (11.1% of the controls) (p = 0.186; specificity = 0.89; sensitivity = 30% of all patients with sarcoidosis and 50% compared to the unmasked observer). CONCLUSIONS: The utilization of IVCM to visualize the basic histology and pathology in sarcoidosis of the conjunctiva is novel. Initial results indicate that trained observers can detect MGCs in granulomatous inflammation. The ASL-1000 microscope tends to have better resolution and deeper penetration of the conjunctiva compared with the Confoscan 3.

Conjunctival biopsy: a useful procedure for the diagnosis of sarcoidosis.J Fr Ophtalmol. 2006 May;29(5):579-82.

PURPOSE: To highlight the advantages of conjunctival biopsy in order to confirm the diagnosis of sarcoidosis. CASE REPORT: A 78-year-old woman presented with unilateral vitreitis. The examination of the conjunctive of right and left eyes revealed multiple, translucent, pale yellow nodules the size of millet grains located in the lower fornix. The diagnosis of sarcoidosis was suspected by an increased level of angiotensin-converting enzyme and bilateral hilar lymphadenopathies. A biopsy from conjunctival nodules showed noncaseating epithelioid and gigantocellular granulomas, confirming the diagnosis of sarcoidosis. CONCLUSION: Although underused, conjunctival biopsy seems to be both a safe and effective tool in the diagnosis of sarcoidosis.

Bilateral large conjunctival tumours as primary manifestation of sarcoidosis--successful treatment with steroid-depot-injections.Klin Monatsbl Augenheilkd. 2006 Apr;223(4):326-9.

INTRODUCTION: Ocular manifestations of sarcoidosis vary enormously. They include the conjunctiva, lacrimal gland, orbita, intraocular structures and eye-lid, either isolated or combined. We describe a female patient who presented with unusually large, bilateral conjunctival tumours as a primary manifestation of sarcoidosis. PATIENT: A 79-year-old white woman was referred to us for further management of a persisting "conjunctivitis", which had been refractory to treatment with multiple medications. Initial examination disclosed swollen eye-lids and bilateral large hard tumours of the inferior fornix. The obtained brush smear, which was cytopathologically evaluated, revealed epitheloid cells and multinucleate giant cells. After 4 weeks she developed three reddish-brown maculopapular lesions on her face. The subsequent biopsy from the left inferior fornix and the skin showed histopathologically a granulomatous epitheloid cell inflammation without central necrosis and without acid-proof bacilli. Therefore a sarcoidosis was included into the differential diagnosis. The systemic evaluation revealed no other manifestation. At first we tried to reduce the chronically inflammatory tumours with different immunomodulating local treatment forms. Only the repeated intralesional injection of a steroid depot showed a complete disappearance of all conjunctival and skin tumours. CONCLUSION: An isolated bilateral primary manifestation of sarcoidosis with large massive conjunctival tumours is very rare and clinically not typical. The non-invasive, cytopathological examination by means of brush smears offers a new perspective in the fast diagnosis of conjunctival manifestation of sarcoidosis. The tumours respond excellently to the intralesional injection of steroid depots.

Conjunctival deposits as the first sign of systemic sarcoidosis in a pediatric patient. Eur J Ophthalmol. 2006 Jan-Feb;16(1):168-70.

PURPOSE: Sarcoidosis is an inflammatory disease of unknown cause, characterized by noncaseating granulomas. In this study, the authors present a sarcoidosis patient without systemic signs diagnosed by histopathologic analysis of conjunctival deposits. METHODS: A 10-year-old girl had bilateral, focal, multilobular, multiple pale yellow deposits in bulbar conjunctiva for a year. In the patient's biomicroscopic examination, numerous 0.50- to 2-mm diameter pale yellow deposits were determined in both bulbar conjunctivas. The patient had no other systemic or ocular complaints. A conjunctival biopsy was performed. RESULTS: The biopsy specimens showed noncaseating granulomas with prominent asteroid bodies. Serum angiotensin-converting enzyme levels were increased in the patient. Conjunctival deposits were the first manifestation of sarcoidosis in the patient and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. CONCLUSIONS: To the authors' knowledge, this is the second study that reports a sarcoidosis patient with non-systemic involvement diagnosed after histopathologic analysis of conjunctival deposits.

Value of conjunctival biopsy in the diagnosis of sarcoidosis.Wiad Lek. 2005;58(3-4):252-4.

The eye signs of sarcoidosis may be observed in patients with no evidence of any other symptoms. In this paper a patient with diagnosis of sarcoidosis was presented. The proper diagnosis and treatment were possible after results of conjunctival biopsy. Conjunctival biopsy could be a useful method in the diagnosis of many systemic diseases, including sarcoidosis.

Conjunctival biopsy in the diagnosis of ocular sarcoidosis. Ocul Immunol Inflamm. 2001 Mar;9(1):59-64.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes. A definitive diagnosis of sarcoidosis requires that a biopsy be performed. A specimen can be obtained from any affected ocular structure, including conjunctiva, lacrimal gland, eyelid skin, and orbit. Among them, conjunctival biopsy has been suggested as a sensible and safe procedure for confirming suspected sarcoidosis. We describe three patients in whom ocular symptoms were the sole initial manifestations of sarcoidosis and who were diagnosed based on the results of a conjunctival biopsy. We also discuss the efficacy of and indications for conjunctival biopsy.

Value of conjunctival biopsy in diagnosis of sarcoidosis.Ophthalmologe. 1999 Nov;96(11):728-30.

PURPOSE: Retrospective study concerning the value of conjunctival biopsy in the diagnosis of sarcoidosis. PATIENTS AND METHODS: Between 1990 and 1996 we performed conjunctival biopsy in 11 patients (mean age 42.7 +/- 16.4 years) with suspect of sarcoidosis. RESULTS: In 8 of the 11 patients the diagnosis of sarcoidosis was established during the clinical course. In four of these eight patients conjunctival biopsy was positive. Five of the eight were under systemic steroids at the time of biopsy. Of the four patients with clinically established sarcoidosis and negative biopsy, three were under systemic steroids at the time of biopsy. In two patients diagnosis of sarcoidosis was established primarily by conjunctival biopsy. CONCLUSION: Conjunctival biopsy is a simple tool in the diagnostic of sarcoidosis. If possible, biopsy should be undertaken before systemic steroid treatment. We consider conjunctival biopsy to be useful as the first diagnostic tool before other invasive methods.

Conjunctival deposits as an initial manifestation of sarcoidosis.Am J Ophthalmol. 1999 Sep;128(3):361-2.

PURPOSE: To report conjunctival deposits as an initial manifestation of sarcoidosis. METHODS: Case reports. RESULTS: Two patients with bilateral bulbar conjunctival white deposits underwent conjunctival biopsy. The biopsy specimens showed noncaseating granulomas with prominent Schaumann bodies. Serum angiotensin-converting enzyme levels were increased in both patients. The first patient had abnormal results of pulmonary function tests. Sarcoidosis was diagnosed in both patients. CONCLUSIONS: Conjunctival deposits may represent the initial clinical manifestation of sarcoidosis.

Phenotypes of conjunctival inflammatory cells in sarcoidosis.Br J Ophthalmol. 1992 Feb;76(2):101-6.

Phenotypes of the infiltrating mononuclear cells of the lower fornix conjunctiva of nine patients with sarcoidosis and six controls were studied using monoclonal antibodies and a modified immunoperoxidase method. Four patients had sarcoidosis of recent onset (duration of 2 years or less) and five patients had a chronic disease (duration of 3 or more years). The inflammatory cells in the sarcoid conjunctival specimens were predominantly T lymphocytes, the vast majority of which were of T helper/inducer subtype expressing Leu-3a + 3b positivity. The ratio of T helper/inducer cells to T suppressor/cytotoxic cells was 3.9 on average but only 0.9 in controls. Epithelioid cell granulomas were seen in three specimens in one case of recent onset and in two chronic cases comprising a marked amount (more than 15 cells/visual field) of cells bearing phenotypes of macrophages, T cells, T helper/inducer cells and HLA-DR antigen, and in smaller quantities of T suppressor/cytotoxic cells. The mean number of all immunocompetent cell subtypes of specimens from newly diagnosed patients exceeded that of specimens from chronic patients. We believe that the sarcoid immune reaction in the conjunctiva is a dynamic process in which proliferation of immunocompetent mononuclear cells precedes the stage of granuloma formation.

Conjunctival biopsy in the diagnosis of sarcoidosis.Br J Ophthalmol. 1990 Aug;74(8):469-71.

We prospectively studied 47 sarcoidosis suspects and compared conjunctival and transbronchial lung biopsies in these patients. Thirty-four patients had positive findings on biopsy by either method. The transbronchial biopsy was positive in 31 patients, and the conjunctival was positive in 19. The transbronchial lung biopsy was more likely to be positive in black patients (p = 0.009) and in patients with pulmonary infiltrates on chest x ray (p = 0.0044). In comparison, the conjunctival biopsy was more likely to be positive in patients with conjunctival follicles (p = 0.036), ocular abnormalities consistent with sarcoidosis (p = 0.02), and in patients with pulmonary infiltrates on chest x ray (p = 0.029). Iritis was present in 12 patients, enlarged lacrimal glands in three, and vitritis in five. We conclude that the conjunctival biopsy is an effective means of diagnosing sarcoidosis and that every sarcoidosis patient should have an ophthalmic examination.

Immunopathology of ocular sarcoidosis.Int Ophthalmol. 1990;14(1):1-11.

Sarcoidosis is a multisystem disease characterized by enhanced immune responses at sites of involvement. To elucidate the immunopathogenesis of ophthalmic lesions, cell infiltrates in biopsies from conjunctiva and other tissues involved (lungs, lymph nodes, skin) were studied in 26 patients with active sarcoidosis in order to define the surface phenotype and the distribution of cells in granulomatous lesions. Biopsy specimens were also stained for detection of immunoglobulins, complement and fibrinogen deposits. The data demonstrate a lymphocytes/macrophages interaction in the central core of granulomatous areas as the crucial event that initiates the maintains the state of inflammation: at all sites of disease activity is present a compartmentalization of T-cells expressing a helper-related phenotype which account for the great majority of infiltrating cells both in the early lesions (aggregate of macrophages surrounded by lymphocytic infiltrate) and in well-organized sarcoid granulomata. The presence of plasma cells and immunoglobulin deposits may represent an epiphenomenon in line with the helper infiltration, suggesting a local hyper-reactivity of the B-cells immune system. This study suggests some immunopathogenetic mechanisms leading to the formation and growth of conjunctival sarcoid granulomata.

Ophthalmologic peculiarities of sarcoidosis.Klin Monatsbl Augenheilkd. 1987 Oct;191(4):253-9

The present paper deals with the frequency of different ophthalmic sarcoid changes and their morphological and photographic characteristics. In addition, the diagnostic value of conjunctival biopsy is evaluated. The findings were based on repeated ophthalmological examinations of an unselected group of 281 patients with histologically confirmed sarcoidosis. Conjunctival (37/218) and lacrimal gland (33/254) changes were encountered more often than sarcoid uveitis (22/281), and a conjunctival granuloma was the most frequent single sarcoid ophthalmic finding. Sarcoid changes in the eyes and the adnexae were often characterized by a scarcity of symptoms and a typical outward appearance. In some cases of sarcoid uveitis fluorescein angiography revealed in the iris and the retina nodules which showed features suggesting the proliferative nature of the disease. The iris infrared transillumination technique illustrated the affinity of sarcoid nodules in the pupillary area. Conjunctival biopsy showed epithelioid cell granulomas compatible with sarcoidosis in nearly half of the patients in whom it was suspected in a slit-lamp examination of the conjunctivae. In the diagnosis of sarcoidosis, conjunctival biopsy is a procedure to be recommended before more demanding methods are tried.

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Normal Anatomy and histology of Eye

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