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    Myxoid Tumours of Soft Tissue




                  

Nevi of the conjunctiva generally develop during the first 2

decades of life, and are almost entirely restricted to the epibulbar

surface, the plica, the caruncle, and the lid margin. 

These are benign pigmented lesions of the ocular surface and are freely

mobile over the surface of the globe.

Unless a biopsy is required for ruling out malignant melanoma,

a nevus is usually excised for cosmetic reasons only.

The common acquired conjunctival nevus usually undergoes progressive

maturation and only rarely gives rise to conjunctival melanoma.

Pure junctional nevi are rare except in childhood.

Histologically, however, a junctional nevus may be indistinguishable

from primary acquired melanosis (PAM) with atypia, a condition

of middle-aged and elderly individuals that has a tendency to evolve

into melanoma.

Nevi in adolescents may attract a vigorous lymphocytic response and

may cause clinical and histologic confusion with other entities,

particularly a regressing nodule of melanoma that occurs

predominantly in adults.

Rarely, congenital conjunctival nevi are identified, sometimes in

patients with adjacent congenital nevi of the eyelid.

Melanocytic nevi of the conjunctiva include :

(i) Intraepithelial  (junctional),

(ii) Subepithelial (equivalent of dermal nevus),

(iii)Compound (intraepithelial plus subepithelial),

(iv) Spindle/epithelioid cell (juvenile melanoma or Spitz nevus),

(v) Blue and cellular blue nevus.

Most conjunctival nevi are compound or subepithelial, and

only in young patients pure intraepithelial nevi (junctional nevi)

are likely to be observed.

 A variety of unusual nevi, including balloon-cell nevi, dysplastic nevi,

recurrent nevi, episcleral melanosis and the nevus of Ota, melanocytoma,

and composite or mixed nevi all may be identified in the conjunctiva.

Congenital nevi of the conjunctiva :

- Both clinically and histopathologically these lesions usually

appear as compound or subepithelial nevi.

- Congenital conjunctival nevi may be diffuse.

- They tend to cover larger geographic areas than acquired nevi.

- These  may grow with the patient and are variably  pigmented

from light to dark brown.

- The conjunctival component of these lesions is usually thick.

Nevi are formed by an abnormal benign proliferation of

melanocytes with retraction of their dendritic processes, which

results in the cells assuming a more rounded appearance.

- In the second to the third decades of life, the cells are distributed

 in the substantia propria to form a compound nevus.

Common conjunctival nevi are composed of variably pigmented,

round, or polygonal cells, similar to the composition of common

acquired conjunctival nevi, except that they are far more

hypercellular.

Pigmentation is variable in conjunctival nevi.

Some nevi are totally amelanotic and, when small, the intraepithelial

component may predominate.

As these lesions enlarge or become inflamed, they may be confused with

epithelial proliferations.

A series of changes frequently occur in conjunctival nevi around

the age of puberty. 

 - There is an increase in size of the nevoid nests and intraepithelial

component.

 - As the nevus becomes more prominent it causes irritation with

secondary inflammation.

 - The inflammation sometimes causes increased pigmentation, and the

inflammatory cellular infiltrate further increases the size and vascularity

of the nevus. 

Visit: Conjunctival Melanocytic Tumours ; Primary Acquired Melanosis ; Conjunctival Melanoma ;  Uveal Melanoma

             

Inflammatory juvenile conjunctival nevus (IJCN) Klin Monatsbl Augenheilkd. 2007 May;224(5):422-6

BACKGROUND: Pathologists may occasionally have difficulties in reliably assessing the dignity of tumour cells in histological sections, especially in nevi with junctional activity. PATIENT HISTORY: This case history of a boy suffering from an inflammatory juvenile conjunctival nevus (IJCN) is reported with a follow-up period of 25 years. Seven years after first surgical treatment of a histologically proven nevus, a recurrent pigmented lesion within the former operation area aroused the suspicion of it being a malignant melanoma. Logically, a second excision was performed followed by cryotherapy with liquid nitrogen (spray freezing). The histological diagnosis performed by a pathologist resulted in a malignant melanoma. A second recurrent pigmented conjunctival tumour developed fourteen years later. This lesion again aroused another strong clinical suspicion of malignant transformation into a melanoma. However, the histological examination of the biopsy at this time only showed benign nevus cells and areas of conjunctival melanosis without atypia. DISCUSSION: Especially in young patients, IJCN must be regarded as an independent type of nevus, which might lead even experts in ophthalmic pathology to over-diagnose this lesion as a malignant melanoma. This could mean that the wrong therapeutic steps were taken with surgical procedures that cause unnecessary mutilation. A reappraisal of the former histological specimens of the first recurrent tumour by other pathologists came to the conclusion that the initial diagnosis of melanoma could not be maintained. Nevertheless, we also strongly recommend follow-up examinations at regular intervals in cases of IJCN since we are aware of the fact that melanocytic tumours of the conjunctiva behave unpredictably.

Treatment of large conjunctival nevus by resection and reconstruction using amniotic membrane.Graefes Arch Clin Exp Ophthalmol. 2006 Jun;244(6):761-4.

BACKGROUND: Nevus of the bulbar conjunctiva is a benign pigmented lesion of the ocular surface. Unless a biopsy is required for ruling out malignant melanoma, a nevus is usually excised for cosmetic reasons only. However, with large lesions involving two or more quadrants of the bulbar conjunctiva, a total resection can be difficult. In this report, we present a case of a huge nevus treated by surgical excision and amniotic membrane transplantation for reconstruction of the bulbar conjunctiva. METHODS: A 54-year-old Japanese man was referred to our clinic with suspected malignant tumor of the bulbar conjunctiva in his right eye. A large and diffuse pigmented tumor with numerous small cysts was present mainly on the upper bulbar conjunctiva. Resection of the conjunctival tumor and amniotic membrane transplantation for reconstruction of the bulbar conjunctiva were performed. RESULTS: The histopathological diagnosis was conjunctival nevus. Epithelialization of the bulbar conjunctiva over the amniotic membrane sheet was completed 4 weeks after resection. At 44-month follow-up, there was no recurrence or any postoperative complication. CONCLUSION: Surgical resection combined with reconstruction by amniotic membrane transplantation is effective for the treatment of large conjunctival nevus.

Conjunctival nevi: clinical features and natural course in 410 consecutive patients.Arch Ophthalmol. 2004 Feb;122(2):167-75.

OBJECTIVES: To describe the clinical features of a conjunctival nevus and to evaluate the lesion for changes in color and size over time. DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Four hundred ten consecutive patients with conjunctival nevi. MAIN OUTCOME MEASURES: The 2 main outcome measures were changes in tumor color and size. RESULTS: Of the 410 patients, 365 (about 89%) were white, 23 (about 6%) were African American, 8 (2%) were Asian, 8 (2%) were Indian, and 6 (1%) were Hispanic. The iris color was brown in 55% (229/418), blue in 20% (85/418), green in 20% (83/418), and not indicated in 5% (21/418). The nevus was brown in 65%, tan in 19%, and completely nonpigmented in 16%. The anatomical location of the nevus was the bulbar conjunctiva (302 eyes, 72%), caruncle (61 eyes, 15%), plica semilunaris (44 eyes, 11%), fornix (6 eyes, 1%), tarsus (3 eyes, 1%), and cornea (2 eyes, <1%). The bulbar conjunctival lesions most commonly abutted the corneoscleral limbus. The nevus quadrant was temporal (190 eyes, 46%), nasal (184 eyes, 44%), superior (23 eyes, 6%), and inferior (21 eyes, 5%). Additional features included intralesional cysts (65%), feeder vessels (33%), and visible intrinsic vessels (38%). Cysts were clinically detected in 70% of histopathologically confirmed compound nevi, 58% of the subepithelial nevi, 40% of the junctional nevi, and 0% of the blue nevi. Of the 149 patients who returned for periodic observation for a mean of 11 years, the lesion color gradually became darker in 5% (7 patients), lighter in 8% (12 patients), and was stable in 87% (130 patients). The lesion size was larger in 7% (10 patients), appeared smaller in 1% (1 patient), and was stable in 92% (137 patients). There were 3 patients who developed malignant melanoma from a preexisting compound nevus (2 cases) or blue nevus (1 case) over a mean interval of 7 years. CONCLUSIONS: Conjunctival nevus is a benign tumor most often located at the nasal or temporal limbus and rarely in the fornix, tarsus, or cornea. Over time, a change in tumor color was detected in 13% (20/149) and a change in tumor size was detected in 8% (12/149).

Conjunctival balloon cell nevi--light- and electronmicroscopic findings in 2 patients.Klin Monatsbl Augenheilkd. 2001 Apr;218(4):269-72.

CASE REPORTS: Two 19-year-old patients presented with small nasal conjunctival tumors. Histologic examination revealed subepithelial tumors consisting of large cells with deeply basophilic, small, centrally-placed nuclei and abundant, slightly eosinophilic cytoplasm with microvesicles. Electron microscopy showed premelanosomes in some of the balloon cells. CONCLUSION: The balloon cell nevus is a rare variant of the conjunctival nevi. The characteristic intracellular microvesicles are probably degenerative remnants of premelanosomes.

Pigmented spindle cell naevus of reed presenting in the conjunctiva.Acta Ophthalmol Scand. 2000 Feb;78(1):104-6.

PURPOSE: This study aimed to present the clinical and histopathological features of a pigmented spindle cell naevus (PSCN) appearing in the conjunctiva. METHODS: Histopathological examination of the lesion following excision and review of the pertinent literature. RESULTS: The features were consistent with those previously recognized in the skin as the PSCN of Reed. CONCLUSION: The PSCN can appear in the conjunctiva and should not be confused with conjunctival melanoma.

Combined nevi of the conjunctiva. Trans Am Ophthalmol Soc. 1999;97:170-83;
discussion 183-5.

PURPOSE: To report the clinical and histologic features of combined nevi of the conjunctiva, a type of nevus that is not uncommon in the skin but has rarely been reported in the conjunctiva. METHODS: Conjunctival nevi and melanomas from the files of the University of California, San Francisco, eye pathology laboratory were reviewed from 1984 to 1999 for the presence of features of both standard nevocytic nevi and blue nevi. Clinical histories and, when available, clinical photographs were obtained. RESULTS: Thirty-one combined nevi were discovered during the 15-year period between 1984 and 1999. One case before 1984 had been incorrectly diagnosed as a junctional nevus. The dendritic and spindle-shaped blue nevus cells had been overlooked because they were not recognized as distinct from the standard nevocytic nevus cells. The recognition of a blue as well as a brown color, a deep as well as a superficial component in the lesion, or a history of pigmentation since birth may help to establish the correct clinical diagnosis and prevent an unnecessarily deep surgical resection. Although growth of the lesion or "satellites" in some patients may favor a clinical diagnosis of melanoma, none of the lesions in this series were malignant. CONCLUSION: Despite a paucity of reports of combined nevi of the conjunctiva in the medical literature, this type of nevus--a combination of a nevocytic and a blue nevus--is common and has been overlooked in the past.

Morphology of conjunctival progressive nevus.Vestn Oftalmol. 1999 Nov-Dec;115(6):20-3.

Structural features of 59 progressive nevuses of the conjunctiva were studied. The proliferation of the epithelium and melanocytes is partially compensated by spontaneous regression of the nevus structures. The growth of a nevus is structurally similar to tumor growth, but the nevuses lack the melanocyte dysplasia, the main sign of malignant degeneration. The immune reactions are involved in the tissue restructuring of the growing nevus. Permanent foci of photoelastosis reflect the significance of ultraviolet exposure as a factor of risk of the nevuses progress.

Conjunctival melanocytic nevi of childhood. J Cutan Pathol. 1999 May;26 (5):248-52.

Two young patients with conjunctival compound nevi are presented to illustrate two types of abnormalities that lead to difficulty in distinction of these nevi from invasive melanomas. In Case 1, inflammation is associated with disruption of the nevus cell architecture and cytologic atypia. In Case 2, the occurrence of a combined nevus (compound and blue nevus types) in the conjunctiva leads to diagnostic problems. Circumscription of the lesions, lack of mitoses in the substantia propria, and lack of pagetoid spread of atypical cells in the adjacent conjunctival epithelium support benign diagnoses in both cases.

Blue nevi of the conjunctiva.Klin Monatsbl Augenheilkd. 1994 Oct;205(4): 242-3.

PATIENT AND METHODS: A 54-year-old man presented with multifocal pigmentations of the left bulbar and palpebral conjunctiva. He had first noticed these changes 5 years ago without any evidence of progression. The pigmented lesions were located in the plica, caruncle, the lower fornix and the mucocutaneous junction of the lower lid. The right cornea showed a focal superficial scar paracentrally due to a herpes simplex keratitis in 1970. Visual acuity was 20/20, otherwise the eyes were unremarkable. Biopsies were performed in all areas. One year after surgery, there was no recurrence of the lesions. MICROSCOPY: Histopathologically, the conjunctival epithelium is essentially unremarkable. The substantia propria display mostly uniformly pigmented spindle-shaped melanocytic cells. Many cells contain coarsely clumped melanin granules. Other areas reveal poorly pigmented to unpigmented melanocytic cells, some of them with Schwannian features. Intranuclear vacuoles are commonly present. The tumor cells are often located in stromal areas displaying increased amounts of collagen. A few polyhydral melanophages are present within the lesion. DIAGNOSIS: Biopsy A (plica)--blue nevus with areas of cellular blue nevus. Biopsy B (caruncle)--cellular blue nevus. Biopsy C (lower fornix)--cellular blue nevus. Biopsy D (mucocutaneous junction of left lower lid)--Blue nevus.

Cellular blue nevus of the conjunctiva.Ophthalmology. 1992 Nov;99(11): 1714-7.

BACKGROUND: Cellular blue nevi of the conjunctiva are extremely rare, and their natural history and malignant potential have not been fully ascertained. METHODS: A report of an unusually well-documented case of growth of a cellular blue nevus is presented, along with a review of current knowledge of this lesion. RESULTS: A 71-year-old woman presented with a darkly pigmented raised lesion of the conjunctiva, which had slowly enlarged over 47 years. There were nonconfluent areas of involvement of the upper and lower lids. Results of biopsy showed the lesion to be a cellular blue nevus, with no evidence of malignancy. CONCLUSION: This well-documented case of slow growth and spread without malignant transformation adds to the knowledge of this rare lesion.

A case of conjunctival Spitz nevus: review of literature and comparison with cutaneous locations.Ann Ophthalmol. 1989 May;21(5):176-9.

We report a case of Spitz nevus of the bulbar conjunctiva in a 15-year-old boy. Clinically, the lesion was juxtalimbic, nodular, red, and 6mm in diameter. Only histologic examination provided the diagnosis. Perusal of the literature revealed seven cases of Spitz nevus of the conjunctiva, but for some of them the histology was incompletely described. We compare the clinical and histologic features in cutaneous and conjunctival nevi and stress the similarity between the two. The histologic criteria which permit differentiation of melanomas and Spitz nevi in conjunctival locations are identified.

Benign conjunctival melanocytic lesions. Clinicopathologic features. Ophthalmology. 1989 Apr;96(4):436-61.

The common acquired conjunctival nevus usually undergoes progressive maturation and only exceptionally gives rise to conjunctival melanoma. Pure junctional nevi are rare except in childhood. Histologically, however, a junctional nevus may be indistinguishable from primary acquired melanosis (PAM) with atypia, a condition of middle-aged and elderly individuals that has a tendency to evolve into melanoma. Nevi in adolescents may attract a vigorous lymphocytic response and may cause clinical and histologic confusion with other entities, particularly a regressing nodule of melanoma that occurs predominantly in adults. Rarely, congenital conjunctival nevi are identified, sometimes in patients with adjacent congenital nevi of the eyelid. A variety of unusual nevi, including balloon-cell nevi, Spitz nevi, epithelioid cell nevi, dysplastic nevi, recurrent nevi, episcleral melanosis and the nevus of Ota, blue and cellular blue nevi, melanocytoma, and composite or mixed nevi all may be identified in the conjunctiva. Concepts of histogenesis as well as the clinical, light microscopic, and ultrastructural features of these and other benign pigmentary conditions of the conjunctiva are described.

Melanocytic nevi of the palpebral conjunctiva. An extremely rare location usually signifying melanoma.Ophthalmology. 1988 Aug;95(8):1053-7.

Nevi of the conjunctiva generally develop during the first 2 decades of life, and are almost entirely restricted to the epibulbar surface, the plica, the caruncle, and the lid margin. Therefore, any elevated pigmented lesion of the forniceal or tarsal conjunctiva acquired later in life must be regarded with suspicion as a melanoma or melanoma precursor, and a biopsy should be done routinely. This report is, to the best of the authors' knowledge, the first histopathologic documentation of nevi located in the palpebral conjunctiva. One nevus was acquired and the other was probably congenital.

August 2007

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Melanocytic tumours
              
Acquired Melanocytic Naevus

Ancient Naevus 
Halo naevus 
Balloon cell naevus
Combined Naevus 
Recurrent melanocytic naevus

Spitz naevus

Pigmented spindle cell naevus

Common blue naevus 
Cellular blue naevus
 


Naevus of Ota
 
Naevus of Ito
Mongolian spot
 

Congenital melanocytic naevi                                   Dysplastic melanocytic naevi 

Pigmented melanocytic lesions causing diagnostic problems

Prognostic parameters of melanoma

Lentigo maligna melanoma

Superficial spreading melanoma

Nodular melanoma

Acral lentiginous melanoma

Desmoplastic /Spindle cell /Neurotropic melanoma

Naevoid melanoma

Balloon cell melanoma

 

Normal Anatomy and histology of Eye

Pathology of Aqueous Humor

Glaucoma

Conjunctival Pathology

Conjunctival Dermoid Tumour

Pinguecula

Pterygium

Sarcoidosis of Conjunctiva

Trachoma

Chlamydial Conjunctivitis (Inclusion Conjunctivitis)

Conjunctival Actinic Keratosis

Conjunctival Dysplasia and Carcinoma in situ

Conjunctival Squamous papilloma

Conjunctival Squamous Cell Carcinoma

Conjunctival Mucoepidermoid Carcinoma

Pathology of the Eyelid

Reporting of biopsies taken from lesions of the Eyelid

Inflammatory diseases of the Eyelid

Chalazion

Pseudorheumatoid nodule  

Necrobiotic Xanthogranuloma with paraproteinemia

Silica granuloma of the eyelid

Sarcoidosis

Leishmaniasis

Paracoccidioidomycosis

Molluscum Contagiosum

Dermoid cyst of eyelid

Trachoma

Chlamydial Conjunctivitis (Inclusion Conjunctivitis)

Onchocerciasis (River Blindness)

Loiasis

Dirofilariasis

Normal histology and diseases of the retina

Retinal Occlusovascular Disease

Central Retinal Artery Occlusion

Central Retinal Vein Occlusion

Comparison between central retinal vein and central retinal artery occlusions

Hypertensive Retinopathy

Retinopathy of Prematurity

Retinitis Pigmentosa

Retinal Detachment

Diabetic Retinopathy

Macular Degeneration

Cytomegalovirus infection

Toxoplasmosis

Visceral Larva Migrans

Retinal Tumours

Phakomatosis

Von Hippel Lindau Disease

Tuberous sclerosis (retinal hamartomas)

Neurofibromatosis

Comparison between ocular melanoma and retinoblastoma

Adenocarcinoma of the Retinal Pigment Epithelium

Tumour and tumour-like lesions of the Eyelid

Adnexal Tumours of the eyelid

Benign sweat gland tumour

Apocrine/Eccrine Hydrocystoma

Syringocystadenoma Papilliferum

Chondroid Syringoma

Sweat gland carcinoma

Adenoid cystic carcinoma

Mucinous carcinoma

Sebaceous Tumours

Sebaceous Adenoma

Sebaceous Carcinoma

Tumours of the Hair Follicle

Trichilemmoma

Trichilemmal Carcinoma

Pilomatrixoma

Trichoepithelioma

Trichofolliculoma

Epidermal tumours of the Eyelid

Squamous Papilloma

Seborrheic keratosis

Keratoacanthoma

Actinic keratosis

Bowen's disease

Basal Cell Carcinoma

Squamous Cell Carcinoma

Merkel cell  carcinoma

Melanocytic tumours of the Eyelid

Mesenchymal Tumours of the Eyelid

Lobular capillary hemangioma  

Infantile Hemangioma

Port wine stain  

Masson's Tumour  

Kaposi's sarcoma

Kimura's disease