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           Myxoid Tumours of Soft Tissue



 

              

Conjunctiva is the epithelium which covers the exposed part of the sclera and inner surface of the eyelids.

It is composed of stratified squamous epthelium with goblet cells in the surface layer.

 The conjunctival mucous secretions act as the protective layer on the exposed surface of the eye.

Diseases of  the Conjunctiva:

Conjunctival Dermoid Tumour

Pinguecula

Pterygium

Sarcoidosis of Conjunctiva

Trachoma

Ligneous Conjunctivitis

Chlamydial Conjunctivitis (Inclusion Conjunctivitis)

Conjunctival Actinic Keratosis

Conjunctival Dysplasia and Carcinoma in situ

Conjunctival Squamous papilloma

Conjunctival Squamous Cell Carcinoma

Conjunctival Mucoepidermoid Carcinoma

Conjunctival Melanocytic Tumours

Primary Acquired Melanosis

Conjunctival Nevus

Conjunctival Melanoma

             

Conjunctival lesions in adults. A clinical and histopathologic review.Cornea. 1987;6(2):78-116.

A total of 2,455 conjunctival lesions in adults (over 15 years old) obtained during a 61-year period were reviewed and histopathologically classified as congenital, acquired epithelial, acquired subepithelial, pigmented, inflammatory/degenerative, and miscellaneous. The most common lesions in decreasing order of frequency were: pterygium, nevus, dysplasia, nonspecific nongranulomatous inflammation, and epithelial inclusion cyst. The most common conjunctival malignancy in adults was squamous cell carcinoma, followed by melanoma, and pagetoid change associated with sebaceous gland carcinoma. Squamous cell carcinomas arise from progressive stages of mild, moderate, and severe dysplasia. These tumors have a low malignant potential but may behave more aggressively in the spindle cell and mucoepidermoid variants. Melanomas arise de novo, from nevi or, most commonly, from acquired melanosis and have an associated 14-32% mortality rate by metastatic spread. Sebaceous carcinomas are often initially clinically misdiagnosed, involve the conjunctiva by pagetoid spread, and can have a low rate of metastasis and death if diagnosed early and excised with frozen-section monitoring of the surgical margins.

Conjunctival tumors in children. A histopathologic study of 42 cases.J Fr Ophtalmol. 2005 Oct;28(8):817-23.

PURPOSE: The aim of this study was to describe the clinicopathological characteristics of 42 conjunctival tumors surgically removed in children. PATIENTS AND METHODS: Records of all conjunctival tumors surgically removed in children during the 11-year period 1990-2001 were collected from the records of the Department of Ophthalmology, Edouard Herriot Hospital, Lyon. In all cases, the tumor was resected with no additional treatment. Sections of all cases were reviewed by the same pathologist. RESULTS: A total of 42 cases (40 patients) were included in the study. The mean age of the subjects at the time of surgical excision was 10 years, with a range of 1-17 years; 45% were male. The most frequent indication for tumor removal was suspected growth. The clinical diagnosis was accurate in 91% of cases. The tumor was localized at the limbus in 57% of cases. Most tumors were pigmented nevi, accounting for 83% of the lesions. The histopathological diagnoses in decreasing order of frequency were: nevi (35), angioma (2), dermolipoma (2), dermoid (1), papilloma (1), and squamous cell carcinoma (1). In one case, the histological study revealed squamous cell carcinoma in a child suffering from xeroderma pigmentosum. CONCLUSIONS: This retrospective study suggests that malignancy of pigmented conjunctival lesions is extremely rare in children. Although squamous cell carcinoma is rare in children, surgical excision and histological study are necessary when a conjunctival lesion is associated with xeroderma pigmentosum or immunodeficiency.

Incidence of benign and malignant lesions of eyelid and conjunctival tumors.Nippon Ganka Gakkai Zasshi. 2005 Sep;109(9):573-9.

PURPOSE: To examine the incidence of benign and malignant eyelid lesions and conjunctival tumors. SUBJECTS AND METHODS: One-hundred-and twenty-eight cases (131 eyes) which were treated during the period from January 1990 to February 2004 were histopathologically diagnosed for eyelid or conjunctival tumors (87 cases of eyelid tumors and 41 cases of conjunctival tumors) in retrospective evaluations. The incidence of benign or malignant lesions, the pathological classification, age, sex, and clinical diagnostic accuracy were all investigated. RESULTS: Sixty-four (73%) of the tumors were found to be benign eyelid tumors. The common benign eyelid tumors were 14 nevocellular nevi, 9 seborrheic keratosis, 7 epidermoid cysts, and 6 papillomas. Twenty-four (27%) eyelid tumors were malignant. These included 9 basal cell carcinomas, 9 sebaceous gland carcinomas, 4 malignant lymphomas, and 2 metastatic tumors. Thirty-four (79%) conjunctival tumors were benign, and the common benign conjunctival tumors were 9 nevocellular nevi and 7 papillomas. Nine (21%) conjunctival tumors were malignant, comprising 7 malignant lymphomas and 2 squamous cell carcinomas. The mean ages of malignant eyelid and conjunctival tumor patients were significantly older than those of benign tumor patients. Clinical accuracy in predicting basal cell carcinoma and sebaceous gland carcinoma was 11.1% and 44.4%, respectively. CONCLUSIONS : Approximately 70 approximately 80% of all eyelid and conjunctival tumors are benign. Clinicians should suspect that the lesions are malignant when seeing elderly patients with eyelid or conjunctival tumors. Excised eyelid lesions should be submitted for histopathologic confirmation because there are some cases where clinical diagnosis does not match pathological diagnosis.

Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors.Ophthalmology. 2004 Sep;111(9):1747-54.

OBJECTIVE: To report the spectrum and frequency of melanocytic and nonmelanocytic conjunctival tumors in an ocular oncology practice. DESIGN: Retrospective noninterventional case series. PARTICIPANTS: One thousand six hundred forty-three consecutive patients with a conjunctival mass evaluated at an ocular oncology department. METHODS: A chart review was conducted to obtain the clinical features of the patient and tumor and to tabulate and categorize the diagnoses. MAIN OUTCOME MEASURES: Tumor diagnosis overall and relative to patient age, race, and gender and relative to tumor location and laterality. RESULTS: In 1643 consecutive patients, the tumor was classified as melanocytic in 872 cases (53%) and nonmelanocytic in 771 cases (47%). The nonmelanocytic categories included congenital choristomatous (n = 40 [2%]), epithelial (n = 219 [13%]), vascular (n = 63 [4%]), fibrous (n = 7 [<1%]), neural (n = 1 [<1%]), xanthomatous (n = 1 [<1%]), myxomatous (n = 1 [<1%]), lipomatous (n = 23 [1%]), lacrimal gland origin (n = 12 [<1%]), lymphoid (n = 128 [8%]), leukemic (n = 3 [<1%]), metastatic (n = 13 [<1%]), secondary (n = 54 [3%]) tumors, and non-neoplastic lesions simulating a tumor (n = 206 [13%]). Of the 872 melanocytic lesions, the specific tumor diagnosis was nevus in 454 cases (52%), melanoma in 215 (25%), and primary acquired melanosis in 180 (21%). Patients with choristomatous, vascular, fibrous, xanthomatous, and myxomatous tumors presented at a mean age of <40 years, and those with malignant epithelial, lipomatous, leukemic, and secondary tumors presented at a mean age of >60. Of the 219 patients with epithelial tumors, 80% occurred in males, whereas the incidence of melanocytic lesions was equal in males and females. African-American patients represented only 7% of epithelial tumors, <1% of melanomas, and 8% of lymphoid tumors. CONCLUSION: Conjunctival tumors were of melanocytic origin in 53% of cases and nonmelanocytic origin in 47%. Overall, melanocytic tumors, epithelial tumors, and lymphoid tumors accounted for 74% of all cases. These tumors were far more common in Caucasian patients, and epithelial tumors were found more frequently in men.

Tumors of the conjunctiva and cornea.Surv Ophthalmol. 2004 Jan-Feb;49(1):3-24.

Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management.

Evaluation of apoptosis markers in conjunctival and eyelid benign and malignant tumors.Ann N Y Acad Sci. 2003 Dec;1010:748-51.

The balance between cell proliferation and programmed cell death plays a crucial role in malignant development. Bcl-2 family proteins, including proapoptosis protein Bak and antiapoptosis protein Bcl-2, regulate the apoptotic process. Mutation of the p53 gene, which results in P53 protein accumulation, was observed in many types of human cancer. The aim of our study was to evaluate immunohistochemical Bcl-2, Bak, and P53 protein expression and the relation between these proteins in conjunctival and eyelid benign and malignant tumors. We examined a series of 42 papillomas (CEP), 12 squamous cell cancers (SCC), and 19 cases of basal cell cancer (BCC). The age in the CEP group ranged from 18-94 years, and in the SCC and BCC groups from 42-87 years. Staining patterns were correlated with sex, age, and tumor localization. P53 protein-positive immunostaining was observed in 71% of cases, Bcl-2 in 83.9%, and Bak in 74.2 cases in the SCC and BCC groups. In the CEP group, P53 overexpression was observed in 90.5% of cases, Bcl-2 in 71.4%, and Bak in 76.2%. No statistically significant correlation was found between examined protein expression and sex, age, and tumor localization. An inverse correlation was observed between P53 and Bak protein expression in the CEP group. No statistically significance correlation was noted between Bcl-2 and P53 and Bcl-2 and Bak protein expression in both examined groups. The obtained data suggests that P53 and Bcl-2 protein expression coupled with decreasing Bak expression are associated with apoptosis and proliferation as well as malignant progression in conjunctival and eyelid tumors.

Amniotic membrane transplantation for conjunctival tumor.Yan Ke Xue Bao. 2003 Sep;19(3):165-7, 145.

PURPOSE: To evaluate the possibility of amniotic membrane transplantation (AMT) for the treatment of conjunctival tumor. METHODS: Preserved AMT was performed in 26 patients (26 eyes) with conjunctival tumor, including 9 eyes (34.62%) with malignant tumor (conjunctival malignant melanoma, corneal and conjunctival squamous cell carcinoma, conjunctival lymphoma), 17 eyes (65.38%) with benign tumor (conjunctival papilloma, conjunctival dermoid tumor, conjunctival nevus, hemangioma etc.). RESULT: All the patients are followed up for 1-53 months. No acute rejection was observed after preserved AMT. Ideal healing was found in conjunctiva wound. CONCLUSION: Preserved AMT is a very effective method to repair wound after giant conjunctival tumor operation. Complete removal of tumor and perfect fixation are the key of ocular surface reconstruction.

HPV type 16 in conjunctival and junctional papilloma, dysplasia, and squamous cell carcinoma.J Clin Pathol. 1995 Dec;48(12):1106-10.

AIMS--To clarify the role of human papillomavirus (HPV) infection in the development of papilloma, dysplasia, squamous cell carcinoma, and basal cell epithelioma arising from the eyelids, including the tunica conjunctiva palpebrum (conjunctiva), its junction to epidemis of eyelid skin (junction), and eyelid skin. METHODS--Sixteen cases of papilloma, four of dysplasia, four of squamous cell carcinoma, and 12 of basal cell epithelioma were examined using formalin fixed and paraffin embedded samples. Detection of HPV-DNA was performed by PCR-RFLP and in situ hybridisation (ISH) methods. RESULTS--HPV-16 was detected in 12/16 papillomas (75%), 2/4 dysplasias (50%), and 1/4 squamous cell carcinomas (25%) but in none of the basal cell epitheliomas. No other HPV subtypes were found. ISH assay showed positive signals in only two cases of dysplasia and squamous cell carcinoma. The mean age of HPV-16 positive dysplasia and squamous cell carcinoma cases (81.7 years) was significantly higher than that of HPV-16 positive papilloma cases (p < 0.01). CONCLUSIONS--Based on the presence of HPV-16 in both benign and malignant lesions and the age distribution, it seems likely that HPV-16 alone may be incapable of causing development of conjunctival and junctional dysplasia and squamous cell carcinoma, and that any correlation between the papilloma-squamous cell carcinoma sequence and HPV infection may be due to rare events.

Suture reaction masquerading as a conjunctival malignancy.Can J Ophthalmol. 2006 Apr;41(2):207-9.

CASE REPORT: We report a case of conjunctival inflammation secondary to a retained suture masquerading as a neoplastic lesion. Excisional biopsy was performed in the right eye on a superior bulbar conjunctival lesion that appeared to be a conjunctival malignancy. A past history of ptosis surgery was obtained. Careful repeated examination with anesthesia, applying traction to the forniceal conjunctiva, revealed an occult polypropylene suture. Despite clinical features typical of malignancy, histologic examination revealed only chronic inflammatory cells. COMMENTS: Patients with suspicious conjunctival lesions and a history of ptosis surgery should be carefully explored for retained suture fragments. Examination with anesthesia may be needed to find an occult suture.

Human papillomavirus in corneal and conjunctival carcinoma.Aust N Z J Ophthalmol. 1997 Aug;25(3):211-5.

PURPOSE: To investigate the role of human papillomavirus (HPV) types, 6, 11, 16 and 18 in corneal and conjunctival carcinoma, we examined 88 dysplastic corneal and conjunctival specimens and 66 controls that had been formalin-fixed and paraffin-embedded. METHODS: Sections were graded for histological abnormality by light microscopy and the presence of HPV DNA was determined by polymerase chain reaction using LI consensus primers. RESULTS: Human papillomavirus DNA was detected in 34 (39%) dysplasias and in five (7.5%) controls. Of dysplasias that were HPV-positive, 20 (59%) contained either types 16 or 18, 13 (38%) contained only types 6/11, while combinations of HPV types were present in 11 (32%). A histological correlation was found with HPV positivity (all genotypes) and unusually large ('epithelioid') dysplastic cells. CONCLUSION: The present study demonstrates a lower incidence of HPV in corneal and conjunctival carcinoma than previously reported, but shows an unexpectedly high incidence of HPV 6/11 in conjunctival carcinomas.

Retrospective study of ocular surface squamous neoplasia.Aust N Z J Ophthalmol. 1997 Nov;25(4):269-76.

BACKGROUND: Ocular surface squamous neoplasia (OSSN) encompasses the conditions of simple dysplasia to carcinoma in situ to invasive squamous cell carcinoma. It has a high rate of recurrence after treatment and the potential to metastasize. The present retrospective study was aimed at further defining the characteristics and clinical course of OSSN. METHODS: With ethical approval, the records of all major pathology laboratories in Queensland were surveyed. Two hundred and eighty-eight cases were identified: 155 dysplasia, 71 carcinomas in situ and 62 invasive squamous cell carcinoma. The records were analysed and an attempt was made to contact and re-examine the patients. RESULTS: Ocular surface squamous neoplasia occurs mainly in males (78.5%) with a mean age of 60.1 years (range 20-88 years). They present as irritation (40.1%) and are located usually at the limbus (87.8%). The majority of OSSN are treated by simple excision (87.5%), after which there is a high rate of recurrence (23.3%). The main predictors for recurrence include histological grade of the lesion, corneal location and larger size (> 2 mm). CONCLUSIONS: Management of OSSN requires adequate excision and careful follow up to monitor any recurrence. As with other ultraviolet light-related conditions, preventative measures must remain the key to disease control.

Tumors of the cornea and conjunctiva.Curr Opin Ophthalmol. 1997 Aug;8(4):55-8.

This article reviews the literature published in 1996 regarding conjunctival and corneal tumors. There is an increased incidence of squamous cell carcinoma of the conjunctiva in patients with HIV infection, especially in young individuals and Africans. Presumed causative factors for conjunctival squamous cell carcinoma include ultraviolet light, HIV infection, human papillomavirus and other unknown factors. Metastatic tumors to the conjunctiva appear at an advanced stage of the disease, and metastases to the conjunctiva are a poor prognostic sign for survival. Reviews of premalignant lesions of the conjunctiva, including diagnosis, treatment, and recurrence, are discussed. Spindle cell and mucoepidermoid variants of squamous cell carcinoma of the conjunctiva have a worse prognosis for intraocular extension compared with usual conjunctival squamous cell carcinoma.

Differential diagnosis and prognosis of 112 excised epibulbar epithelial tumors. Klin Monatsbl Augenheilkd. 1995 Oct;207(4):239-46.

BACKGROUND: Epithelial tumors of the epibulbar conjunctiva are rare in northern Europe. There are no reliable clinical criteria to predict the histological nature of the lesion. Even histologically proven malignant tumors typically do not show ocular invasion or metastasis. PATIENTS AND METHODS: From 1980 to 1993, 112 epithelial epibulbar tumors were classified in our ophthalmopathological laboratory. Surgical management of 50 papillomas (mean age 46 +/- 18 years, 58% males), 49 conjunctival/corneal intraepithelial neoplasias (CIN) (28 mild, 21 severe) and 12 squamous cell carcinomas (mean age 63 +/- 13 years, 69% males) included excisional biopsy (61%), with additional lamellar keratectomy (14%), epithelial abrasion (10%), cauterisation (3%), cryotherapy (2%), excimer laser ablation (2%). Other management regimes were lamellar keratoplasty (2%), penetrating keratoplasty (2%), epithelial abrasion (2%). One eye with intraocular invasion of a mucoepidermoid carcinoma was enucleated. RESULTS: Fifty five per cent of the papillomas were located at the caruncle or semilunar fold. Seventy eight percent of the papillomas had a pedunculated appearance. Two lesions involved the epibulbar conjunctiva as well as the opposite tarsal conjunctiva ("kissing papilloma"). Eighty eight percent of CIN/carcinoma were located at the limbus. The clinical differential diagnoses of CIN/carcinoma were squamous cell carcinoma (26%), papilloma (18%), leukoplakia (10%), pinguecula/pterygium (10%), dysplasia (8%), malignant melanoma (5%), trachoma (3%), chronic keratoconjunctivitis with corneal pannus (2%), "granuloma" (2%), non-pigmented naevus (2%). After a mean follow-up of 6.3 +/- 3.5 years, 13% of the papillomas showed a local recurrence. After a mean follow-up of 4.8 +/- 3.7 years, there were 30% local recurrences of CIN/carcinoma (50% each in severe CIN and carcinoma, 9% in mild CIN). But there was no recurrence of CIN/carcinoma in patients who had received a complete tumor resection initially. If the surgical margins were involved, there was a 64% recurrence rate (80% each in severe CIN and carcinoma). Thirteen per cent of the patients with CIN/carcinoma showed an additional malignant tumor elsewhere in the body. CONCLUSIONS: Local recurrences after excision of CIN/carcinoma did occur only if the surgical margins where involved, and most often within the first year following surgery. Dedifferentiation of epithelial tumors in recurrences was not observed. The diagnosis of CIN/carcinoma of the conjunctiva requires the exclusion of an additional extraocular neoplasia. Histopathological classification and assessment of resection margins of epibulbar tumors is indispensable, especially in view of the more aggressive treatment necessary in mucoepidermoid carcinomas.

August 2007

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Pigmented epithelial tumours of the conjunctiva.Br J Ophthalmol. 1992 May;76(5):294-6.

Two out of 60 conjunctival epithelial tumours reviewed between 1973 and 1989 were found to be pigmented. One tumour was a pigmented papilloma and the other a pigmented squamous cell carcinoma. The melanin pigment was found in epithelial tumour cells as well as in macrophages, dendritic melanocytes, and Langerhans cells. The distinction between the latter two types of cells was possible in one of the tumours only. Both tumours were found in dark-skinned white patients without any evidence of conjunctival acquired melanosis.

Demonstration of papillomavirus capsid antigen in human conjunctival neoplasia.Arch Ophthalmol. 1986 Dec;104(12):1801-5.

To investigate the association of human papillomavirus with conjunctival neoplasia, we identified 50 resected papillomas from 47 patients. Papillomas were composed of papillary or, less commonly, flat proliferations of predominantly nonkeratinizing squamous epithelium with admixed goblet cells. Koilocytosis was focally present in 30 tumors (60%). Atypia that ranged from mild to severe was present in ten lesions (20%). In addition, we examined specimens of conjunctival dysplasia or carcinoma from 61 patients. The lesions were predominantly flat proliferations of atypical epithelial cells. Twenty biopsies performed for suspected sarcoidosis were used as controls. Papillomavirus capsid antigen was demonstrated using an immunoperoxidase technique in nuclei of mature superficial epithelial cells of 23 papillomas (46%) and five dysplasias or carcinomas (8.2%) but not in the control biopsy specimens. These results suggest that papillomavirus may play a role in the etiology of conjunctival papilloma, dysplasia, and carcinoma.

Laser therapy of eyelid and conjunctival tumors, especially in AIDS patients. Ophthalmologe. 1994 Oct;91(5):691-3.

We treated patients with Kaposi's sarcoma of the lid or conjunctiva, molluscum contagiosum and papilloma of the lid with dye laser photocoagulation. This therapeutic device is extremely efficient as the chosen wavelength of 577 nm is absorbed selectively by haemoglobin. Since Kaposi's sarcoma is a haemoglobin-containing tumour composed of endothelial cells and papilloma is a benign tumour composed of connective tissue and branching vessels, dye laser photocoagulation causes selective damage to abnormal vessels and surrounding connective tissue. After local anaesthesia a small incision was made at the margin of the lesion five patients each with papilloma and with molluscum contagiosum. In six patients with Kaposi's sarcoma no incision had to be done. Starting from the incision or the margin of the Kaposi's sarcoma 50-100 shots of 0.3-0.5 mm diameter and 1 s duration were applied until the lesion was completely gone. We used a dye laser with 577 nm wavelength and 1 W energy. In patients with papilloma, the tumour basis was coagulated and the tumour itself underwent histological examination. There was a marked decrease of the size of the lesion or even complete disappearance. Since Kaposi's sarcoma in AIDS patients can be relatively fast growing, resulting in cosmetically irritating manifestations and, rarely, haemorrhages, treatment may become necessary. AIDS patients with molluscum contagiosum may also benefit from treatment, as spreading of DNA virus particles may result in viral follicular conjunctivitis. Dye laser photocoagulation, however, cannot protect against relapse.