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Melanocytic Tumours 

Acquired Melanocytic Naevus  Nevi are noted in  either the skin or the conjunctival surface of the eyelids, particularly along the lid margin. Junctional and compound type of melanocytic naevi are the most common types.

Congenital melanocytic naevi

Naevus of Ota Nevus of Ota included congenital and acquired cases. Skin lesions mainly occurred in adolescence for the acquired cases. The main colours of lesions were brown and blue. The most local lesions were zygomata, temporal regions, and lower eyelids.

Nevus of Ota. Presentation of a case associated with a cellular blue nevus with suspected malignant degeneration and review of the literature.Pathologica. 1997 Apr;89(2):168-74.

BACKGROUND: Nevus of Ota is a melanotic pigmentary disorder ("dermal melanocytosis"), mostly congenital or acquired, involving the skin innervated by the first and the second branch of the trigeminal nerve, hence its descriptive label of "nevus fuscus coerulaeus ophthalmic and maxillaris". In more than half of patients this condition is associated with "ocular melanocytosis" ("melanosis oculi") involving the conjunctiva, the sclera, the uveal tract and possibly the optic nerve. In some cases a condition of "orbital melanocytosis" with involvement of orbital fat and periosteum by dendritic melanocytes is on record as well as in some other patients an analogous condition of "leptomeningeal melanocytosis" is present. At histology tissues from the above sites are seen infiltrated by dendritic melanocytes which can vary in number from so scarce up to so numerous that a diagnosis of a blue nevus of the common type is warranted. Sometimes the finding of a variously pigmented typical cellular blue nevus in the skin and alternatively that of heavily pigmented melanocytoma in the eye ("nevus magnocellularis") or in the meninges (so-called "melanotic meningioma") are respectively observed. MATERIALS AND METHODS: A case of cellular blue nevus with histologically uncertain malignant potential in a nevus of Ota of 30 years duration in a white female patient aged 59 is described. The lesion which was surgically totally removed grossly appeared nodular shaped and 2 cm sized. Histologically it consisted of a fairly well-circumscribed proliferation of melanocytic spindle-shaped cells growing in a vaguely fascicular pattern. On the basis of random nuclear atypicalities and pleomorphism and additionally by virtue of the presence of a few scattered mitoses (one of which was atypical) but in absence of frank necrosis a diagnosis of unpredictable biologic behaviour seemed to be warranted. The patient was closely followed-up but no adjunctive therapy given. Four years after the excision and diagnosis no local recurrence or distant metastasis has been discovered. A computerized search of previously recorded cases of melanomas in nevus of Ota was made. CONCLUSIONS: Forty-eight cases of malignant melanomas complicating this clinico-pathological setting are on record, mostly in the uveal tract, followed by locations in central nervous system, skin, and retro-orbital fatty tissue. Melanomas arising in nevus of Ota tend to be low grade lesions that do infiltrate locally but rarely metastasize. The importance of a closely dermatological and ophthalmic surveillance of patients with nevus of Ota is emphasized.

Melanosis Oculi : (Ocular melanocytosis)- A rare congenital anomaly. It is characterized by hyperpigmentation of conjunctiva, episclera, sclera, uveal tract and rarely the optic nerve.

Lentigo maligna melanoma

Superficial spreading melanoma

Nodular melanoma

Acral lentiginous melanoma

Clinicopathologic features and behavior of cutaneous eyelid melanoma.
Ophthalmology. 2002 May;109(5):901-8.

OBJECTIVE: To study the clinical and histopathologic features of cutaneous eyelid melanomas and identify prognostic factors in the behavior of such tumors. DESIGN: Retrospective observational case series. PARTICIPANTS: Twenty-three patients with cutaneous eyelid melanomas without conjunctival involvement. METHODS: Patients' charts were reviewed for clinical information, treatment procedure, and disease course (updated at the time of study). Histopathologic sections from all surgical procedures were reviewed. MAIN OUTCOME MEASURES: Histologic type of melanoma, tumor growth phase, Clark's level of invasion, tumor thickness, and other microscopic features were evaluated in each case. The width of excision margins was considered and measured histologically when possible. RESULTS: There was no gender predilection. The lower eyelid was more frequently involved than the upper eyelid or canthi. Seventeen cases (74%) were invasive, and six (26%) were in situ melanomas. Lentigo maligna melanoma was the most common histologic type, accounting for 61% (14 cases) of all melanomas and 53% (9 cases) of invasive melanomas. Superficial spreading melanoma accounted for 22% (5 cases) and nodular melanoma for 17% (4 cases) of all melanomas. Surgical excision, as the treatment of choice, was incomplete in nine cases, two thirds of which were lentigo maligna melanoma (in situ or invasive). Tumor reappeared in 77.8% of these cases. Fourteen patients had initial narrow excisions, and three of them (21.4%) had local recurrences. Although recurrence occurred in one each of our "in situ," "thin," and "thick" melanomas, it proceeded to distant metastases and death only in the "thin" one. Adjuvant radiotherapy was used in six patients with successful disease control in two cases. CONCLUSIONS: Eyelid skin melanomas have a relatively good clinical prognosis. The histologic type and thickness of the primary melanoma were not clearly related to the clinical behavior once they were completely excised. The use of very narrow excisions of 5 mm or less was associated with greater frequency of local recurrence. Lentigo maligna melanomas were the largest tumors at presentation and, despite being thinner, were a greater surgical challenge. This type of melanoma is almost certainly underdiagnosed by ophthalmologists.

Prognostic factors for survival in malignant melanoma of the eyelid skin.Ophthal Plast Reconstr Surg. 2000 Jul;16(4):250-7

PURPOSE: This study aimed to determine the prognostic factors for survival and disease-free interval for malignant melanoma of the eyelid skin. METHODS: This was a retrospective, nonrandomized, clinical review. Twenty-four patients with eyelid skin melanoma were identified through a search of the tumor registry at M. D. Anderson Cancer Center. Patients were treated between 1953 and 1994. The follow-up ranged from 3 to 18 years (mean = 9.6 years). Primary treatment in all cases entailed wide local excision of the tumor. Patients in whom regional lymph node metastasis developed underwent parotidectomy or neck dissection, with or without adjuvant chemotherapy or external beam radiation. Descriptive statistics were used to characterize the patients. Survival analysis in terms of disease-free survival and recurrence-free survival was performed using age, sex, location of tumor (upper lid, lower lid, or both), histologic type of melanoma, Breslow thickness, and Clark's level as independent variables for survival. RESULTS: Age, sex, location, and the histologic type of tumor were not significant prognostic indicators for survival in this cohort. Clark's level > or = IV by itself was a statistically significant predictor of decreased survival. In addition, tumors with either Clark's level > or = IV or Breslow thickness > or = 1.5 mm were associated with increased mortality. CONCLUSION: Clark's level > or = IV or Breslow thickness > or = 1.5 mm are poor prognostic indicators for malignant melanomas of the eyelid skin. Clinicians should have a high level of suspicion for occult regional lymph node metastasis when treating patients with these tumor features.

           

Congenital divided compound nevi of the eyelids.Klin Monatsbl Augenheilkd. 1999 Oct;215(4):263-5

BACKGROUND: Congenital divided nevi of the eyelids are a rare melanocytic lesion. Only 30 patients are reported in literature. We report on a 6-year-old boy and a 41-year-old male with this lesion and give a short overview of the literature. CASE REPORTS: Patient 1: A 6-year-old boy presented with a pigmented divided nevus of the left eye involving the upper and lower lid naselly. The lesion had increased in size in the last one year. Otherwise the results of ophthalmological examination were unremarkable. The divided nevus was completely excised. Histopathological studies revealed a melanocytic compound nevus. Patient 2: A 41-year old male presented in our department with a divided pigmented nevus on his left eye involving the upper and lower lid laterally. The lesion had gradually increased since birth. Otherwise the results of ophthalmological examination were unremarkable. A subtotoal excision and debulking procedure was performed. The defect in the lower eyelid was covered using a transposition flap from the upper eyelid. Histopathological examination revealed a melanocytic compound nevus. CONCLUSION: Divided nevi of the eyelids may be disfiguring and include the possibility of deprivation amblyopia when reaching a certain size in childhood. Malignant changes of this lesion were not observed so far. Total excision or subtotal excision and a debulking procedure may be helpful.

Cutaneous melanomas of the eyelid.Semin Ophthalmol. 2006 Jul-Sep;21(3):195-206

Cutaneous eyelid melanomas are very rare lesions. The lentiginous subtypes are the most frequent melanocytic lesions of the eyelid and can be likened to conjunctival melanocytic lesions like PAM, PAM with atypia and conjunctival melanoma. Compared to melanomas elsewhere on the body, eyelid melanomas have special considerations. Eyelid skin is very thin, the mucocutaneous junction at the lid margin can affect prognosis, the lymphatic drainage pattern is very variable and there is an inherent difficulty to excise wide margins without sacrificing important structures. A customized excision approach, using tissue-sparing "Slow-Mohs" technique, is suggested. Sentinel lymph node dissection has an evolving therapeutic role but remains controversial.

Treatment and outcomes of malignant melanoma of the eyelid: a review of 29 cases in Australia.Ophthalmology. 2007 Jan;114(1):187-92.

PURPOSE: To review the treatment and outcomes of malignant melanoma (MM) of the eyelid skin. DESIGN: Retrospective case series review. PARTICIPANTS: All consecutive patients who had MM arising from eyelid skin treated by 2 regional tertiary referral oculoplastic surgeons were included. METHODS: Patient charts were reviewed to collect information on the main outcome measures. MAIN OUTCOME MEASURES: Demographics, clinical and histological features of the lesion, treatment, and outcomes. RESULTS: Twenty-nine patients between 22 and 88 years old (mean, 65) were included. The most common site of MM occurrence was the lower eyelid. Seventeen cases arose in an area of pigmentation, 4 arose de novo, and 8 were of unknown origin. The most common histopathological types were lentigo maligna melanoma (19 cases), followed by superficial spreading MM (8 cases). Fourteen patients had in situ disease and therefore had no Breslow thickness. Another 7 patients had Breslow thickness of <0.76 mm. Thirteen patients had Clark level II or higher. According to the American Joint Committee on Cancer staging system for cutaneous melanoma, 14 patients were clinically stage 0 and 6 patients were stage IA, with thickness < or = 1 mm and no ulceration. Treatment included wide excision in all cases, one of which underwent anterior exenteration. Pathological techniques used included mapped serial excision with standard or overnight paraffin sections or Mohs' micrographic surgery. Most patients had a good outcome, although 2 died of the disease. Five patients had local recurrence, and 4 had distant metastases. Median postoperative follow-up was 3 years (range, 1 month-9 years, 9 months). CONCLUSIONS: Lentigo maligna melanoma compared with other forms of MM was relatively more common in the periocular region than in other body locations. Our pathologists preferred paraffin sections to frozen section for accurate assessment of melanocytic atypia and margin status. Initial wide excision margins of 10 mm from the macroscopic edge of the tumor are suggested, as histological margins may be less than this. Margin control by mapped serial excision or a modified Mohs' micrographic surgery using paraffin sections is a useful technique to ensure complete excision and minimization of local recurrence.

Benign pigmented lesions of the eyelids. J Fr Ophtalmol. 2005Oct;28(8):889-95.

We describe benign pigmented tumors of the lids, including benign lesions of the epithelium such as papillomas, seborrheic keratosis, and inverted follicular keratosis; precancerous lesions of the epithelium such as xeroderma pigmentosum and actinic keratosis; melanocytic lesions such as Ota nevus, lentigo, and lentigo maligna; and nevi and other lesions simulating a melanoma such as pigmented hidrocystoma, angiomas, and epithelial cyst. For each of these lesions, we describe the clinical and histopathological aspect as well as the therapeutic guidelines.

Margins of excision for cutaneous melanoma of the eyelid skin: the Collaborative Eyelid Skin Melanoma Group Report. Ophthal Plast Reconstr Surg 2003 Mar;19(2):96-101

PURPOSE: To evaluate the practice patterns among surgeons who treat melanomas of the eyelid skin with respect to margins of excision and to look for possible correlation between margins of excision and the incidence of local and regional recurrence and distant metastasis. METHODS: A retrospective survey of the members of the American Society of Ophthalmic Plastic and Reconstructive Surgery and the European Society of Ophthalmic Plastic and Reconstructive Surgery yielded 44 cases. The patients' age, sex, date of diagnosis, histologic classification of melanoma, Breslow thickness, Clark level, location of melanoma, size of margins of excision, and findings of local or regional recurrence or distant metastasis were recorded in each case. Patients were stratified on the basis of margins of excision: </=5 mm; >5 mm but <10 mm; and >/=10 mm. Patients were also stratified by Breslow thickness. A Cox regression model was used to evaluate the predictive value of each factor for recurrence. Main outcome measures were the incidences of local and regional recurrence and distant metastasis as a function of margins of excision and Breslow thickness. RESULTS: The majority of patients for whom reliable information was available had excision margins of </=5 mm. The Breslow thickness of most of the tumors was </=1 mm. Eleven patients (25%) had local recurrence. Five patients (11%) had regional lymph node metastasis. All patients with regional nodal metastasis were men. Distant metastasis developed in 3 patients (7%)-2 men and 1 woman. The follow-up times ranged from 10 to 108 months (mean, 34 months; median, 21 months). The incidence of local recurrence was higher among patients with melanomas at least 2 mm thick and margins of excision </=5 mm than among patients with melanomas at least 2 mm thick but with margins >/=10 mm, but this difference was not statistically significant because very few patients had melanomas at least 2 mm thick. Breslow thickness was the only statistically significant predictor of local, regional, and distant metastasis. Margins of excision did not have a statistically significant effect on local, regional, or distant recurrence. CONCLUSIONS: Breslow thickness is an important prognostic indicator for eyelid skin melanomas. A 5-mm margin of excision may be adequate for thin melanomas of the periocular skin, but because of the small number of patients in this series who had >5-mm margins, a definitive comparison of outcome with larger margins of excision cannot be made. For melanomas >/=2 mm, wider margins of excision may be prudent, and careful surveillance for local and regional recurrence is indicated.

Periorbital melanocytic lesions: excision and reconstruction in 40 patients. Plast Reconstr Surg. 1998 Jul;102(1):19-27.

The treatment of melanoma arising in the periorbital region is a difficult reconstructive problem. The abundance of vital structures in close proximity to one another makes the resection and subsequent reconstructive procedures extremely challenging. Reported here is experience with periorbital melanocytic lesions in 40 patients with the emphasis on the types of reconstruction performed. Forty patients with periorbital melanocytic lesions were treated between 1984 and 1995. The periorbital region was subdivided into five zones. These zones are the following: zone I, upper eyelid; zone II, lower eyelid; zone III, medial canthus; zone IV, lateral canthus; and zone V, contiguous structures. Ocular melanomas were not included in this study. The distribution of the lesions in our 40 patients was zone I (n = 1), zone II (n = 14), zone III (n = 1), zone IV (n = 9), and zone V (n = 31). The ages of the patients ranged from 3 to 84 years at the time of reconstruction, with an average age of 57 years. Resection and reconstruction were performed simultaneously in all patients. Thirty-six of the patients were reconstructed with one procedure, three patients required two procedures, and one patient required five procedures. The tumor type was superficial spreading melanoma in 15 patients, melanoma in situ in 17 patients, malignant spindle cell neoplasm in 2 patients, desmoplastic melanoma in 2 patients, amelanocytic melanoma in 1 patient, epithelioid melanoma in 1 patient, and atypical melanocytic nevus in 2 patients in which an early, evolving melanoma could not be excluded. Elective lymph node dissection was performed in four patients for intermediate thickness lesions (1.5 to 4.0 mm). The types of reconstructions performed included full-thickness skin grafts, upper lid myocutaneous flaps, cheek advancement flaps, cervicofacial flaps, inferiorly based nasolabial flaps, tarsoconjunctival flaps, frontalis muscle flaps, medial transposition Z-plasty, and primary closure. The resection of periorbital melanomas can be difficult because of the number of important anatomic structures in the region. The challenge to the surgeon in handling head and neck melanomas in general lies in the need to provide the best functional and aesthetic result while still resecting the primary lesion with the intent of effecting a cure. We present our series to demonstrate that the adequacy of margins of resection need not be compromised to facilitate reconstruction and that excellent results are obtainable with reconstructive procedures performed after adequate resections. Several different types of flaps and grafts can be used, with the indications varying depending on the location of the lesion and the extent of resection. The major reconstructive options will be reviewed in detail.

                  

 
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Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Lipomatous tumours

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Melanocytic tumours
              
1. Acquired Melanocytic Naevus CLICK

Ancient Naevus CLICK
Halo naevus  CLICK
Balloon cell naevus CLICK
Combined Naevus CLICK
Recurrent melanocytic naevus 
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2. Spindle and epithelioid cell naevi

Spitz naevus   CLICK
Pigmented spindle cell naevus  CLICK

3. Blue naevi

Common blue naevus
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Cellular blue naevus
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4. Dermal melanocytoses

Naevus of Ota  
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Naevus of Ito     CLICK
Mongolian spot CLICK

5. Congenital melanocytic naevi  CLICK                                              
6. Dysplastic melanocytic naevi  CLICK

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

Selected pigmented fundus lesions of children.J AAPOS. 2005 Aug;9(4):306-14.

BACKGROUND: Ocular cells that accumulate melanin pigment are derived from 1 of 2 sources, the optic vesicle or the neural crest. Migration and distribution of pigment containing cells may go awry during fetal development or these cells may be altered before or after birth either by local or systemic stimuli. Specific recognition patterns of pigment distribution often exist and may relate directly to a single disease process. METHODS: Records of pediatric patients with disorders of pigment distribution in the ocular fundi who had been examined by the author were reviewed. RESULTS: Five disorders with recognizable patterns of retinal pigment epithelium (optic vesicle derivation) disturbance (congenital hypertrophy of the retinal pigment epithelium, Gardner syndrome, chronic granulomatous disease, preserved para-arteriole retinal pigment epithelium in (autosomal-recessive) retinitis pigmentosa, and combined hamartoma of the retina and retinal pigment epithelium), and 5 disorders of cells originating from the neural crest (choroidal nevi, choroidal melanoma, melanocytoma, ocular melanosis, and oculodermal melanosis), were selected for illustration and discussion. CONCLUSIONS: These arbitrarily selected groups of disorders affect or involve patterns of pigment deposition in the ocular fundus. These patterns are recognizable and distinguishable one from another but have different implications for treatment and follow-up. Progress in recognizing distinguishing characteristics, diagnostic implications, understanding, and treatment of these disorders during the past 40 years is compared and contrasted between the 2 groups.

Pigmented conjunctival and scleral lesions.Mayo Clin Proc. 1994 Feb;69(2):151-61.

OBJECTIVE: The multiple causes of pigmentations of the conjunctiva and sclera are reviewed, and the recommended therapeutic modalities are discussed. DESIGN: Information from personal experience and the recent literature is summarized to determine the optimal diagnostic and treatment approaches for suspicious pigmented conjunctival and scleral lesions. MATERIAL AND METHODS: Clinical descriptions and illustrations are presented to characterize these ocular lesions. RESULTS: Pigmented lesions of the conjunctiva and sclera arise from either melanocytes or nonmelanocytes and have a diverse differential diagnosis. These lesions can be classified into congenital melanosis, conjunctival nevi, acquired melanosis (secondary or primary), and conjunctival melanomas. In secondary acquired melanosis, the increased conjunctival pigmentation is caused by irradiation, hormonal changes, chemical irritation, or chronic inflammatory conjunctival disorders. The biologic behavior of primary acquired melanosis of the conjunctiva is a controversial topic with important implications because it may progress to melanoma. In patients with primary acquired melanosis, a biopsy is recommended in order to grade the disease, offer a prognosis, and direct further treatment. Conjunctival melanomas may arise from primary acquired melanosis, from nevi, or de novo, or they may be metastatic lesions. CONCLUSION: Of the wide spectrum of melanocytic conjunctival lesions, those with malignant potential are melanosis oculi, nevus of Ota, junctional nevus, compound nevus, primary acquired melanosis, and melanomas.


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