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                  Myxoid Tumours of Soft Tissue 



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Benign sweat gland tumour

Apocrine/Eccrine Hydrocystoma

Eccrine hidrocystoma of the eyelid.Eye. 2005 Jan;19(1):77-9.

AIM: To report on the clinical features of eccrine hidrocystoma involving the eyelid. METHODS: Data on a series of consecutive patients with histopathologically confirmed diagnosis were reviewed. RESULTS: Among 34 patients, 69 tumours were identified. The mean age at diagnosis was 59 years (range 39-91 years). The majority (71%) of patients had only a single tumour. The tumours appeared as a small (median size=1 mm) clear cystic lesion with 87% located near the eyelid margin. CONCLUSIONS: The eccrine hidrocystoma is a benign small cystic tumour that characteristically occurs close to but does not involve the eyelid margin.

Syringocystadenoma Papilliferum

Syringocystadenoma papilliferum of the eyelid. Am J Ophthalmol.1994 May 15;117(5):647-50

Syringocystadenoma papilliferum is a benign adnexal tumor of the scalp and face. We treated syringocystadenoma papilliferum in a 31-year-old man who had a circumscribed cup-shaped lesion of the left upper eyelid with a central crater. On histologic examination, the epidermal edge showed hyperkeratosis and invasive acanthosis with papillary projections that filled the central crater. The deeper portions of the projections resembled ductal structures lined by an inner columnar epithelium, which demonstrated luminal apical decapitation secretions, and an outer layer of smaller cuboidal cells. The fibrovascular dermal tissue exhibited prominent plasmacytic infiltration underlying the papillary epithelium. Although rare, syringocystadenoma papilliferum should be considered in the differential diagnosis of umbilicated squamous or basal cell tumors, especially if noted in a young individual.

 Chondroid Syringoma (benign mixed tumour)

Cutaneous benign mixed tumor (chondroid syringoma) of the eyelid: clinical presentation and management. Ophthal Plast Reconstr Surg 2004 Mar;20(2):110-6

PURPOSE: To describe the clinical presentation of cutaneous benign mixed tumor of the eyelid and its management options. METHODS: Periocular cases of cutaneous benign mixed tumor were gathered from members of an oculoplastics specialty Internet discussion group. A total of 9 patients are described in this retrospective, interventional case series. The clinical presentation, histopathology, and management of these lesions is reviewed. RESULTS: Patients were typically asymptomatic, presenting with a slowly enlarging, nontender nodule of 2 to 8 years' duration. The lesions ranged from 4 mm to 17 mm in greatest dimension. Four of the lesions were on the eyelid margin, three in the sub-brow area of the upper eyelid, and two in the central lids. All six cases not involving the brow were fixed to the tarsus; one brow lesion was believed to be adherent to the skin. None of the lesions was associated with significant changes of the overlying epidermis, although one lesion showed overlying pigmentation. All patients underwent excisional biopsy for diagnostic or cosmetic reasons. On histopathologic examination, the tumors were biphasic, with an epithelial component exhibiting apocrine or hair follicle differentiation and a myxoid, adipocytic, chondroid, and/or fibrous stroma. The pathologic diagnoses were all consistent with cutaneous benign mixed tumor (chondroid syringoma, pleomorphic adenoma). Follow-up ranged from 2 weeks to 12 months, although several patients failed to keep scheduled follow-up appointments. No clinical recurrences were identified. CONCLUSIONS: Cutaneous benign mixed tumor may occur in the eyelid, and, although uncommon, should be included in the differential diagnosis of firm, nodular eyelid tumors. The histopathologic features are similar to those seen in this tumor type arising in other areas of the body. Preoperative consideration of this diagnostic possibility may allow the surgeon to plan for complete excision, thereby reducing the possibility of recurrence or malignant transformation.

A rare case of atypical chondroid syringoma of the lower eyelid and review of the literature.Surv Ophthalmol 2006 May-Jun;51(3):280-5

A 46-year-old man presented with a painless firm mass in the eyelid margin of the left lower eyelid, which had been present for 9 years. Biopsy nine years previously had not established a diagnosis. We performed excisional biopsy of the lesion and lateral advancement skin flap for reconstruction of the skin defect. Histopathologic and immunohistochemical findings were consistent with atypical chondroid syringoma with eccrine differentiation. The systemic metastatic work-up was negative, and no recurrence or metastasis was present at 30-month follow-up. Chondroid syringoma is a rare skin tumor that occurs, very rarely, in the eyelids, especially the lower eyelid. Only 26 cases of chondroid syringoma in the periorbital area, including our case, have been reported, with various characteristics. Chondroid syringomas are classified as two types, the apocrine type characterized by tubular and cystic branching lumina lined by two layers of epithelial cells, and the eccrine type, which has small tubular lumina lined by a single layer of epithelial cells. The tumor can have benign, atypical, and malignant variants. Our patient had the atypical variant, which is characterized by benign cytological features, as well as the presence of infiltrative margins and/or satellite nodules. Even benign chondroid syringomas, if incompletely excised, can recur with malignant transformation; thus, complete excision and regular follow-up is recommended for all chondroid syringomas.

Sweat gland carcinoma

Endocrine mucin-producing sweat gland carcinoma: twelve new cases suggest that it is a precursor of some invasive mucinous carcinomas. Am J Surg Pathol. 2005 Oct;29(10):1330-9.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is an underrecognized low-grade carcinoma with predilection to the eyelid. Only 4 cases of this entity have been described in the literature. Here, we describe 12 cases of EMPSGC. The lesions were twice as frequent in females than males with an average age of 70 years (range, 48-84 years). Clinically, they presented as a slowly growing cyst or swelling. The most common site of occurrence was the lower eyelid (8 cases). Two lesions occurred on the upper eyelid and 2 on the cheek. Histologically, they were well-circumscribed, typically multinodular tumors with solid or partially cystic nodules, frequently showing areas of papillary architecture. Focal cribriform arrangements were also present. The nodules were formed by uniform small- to medium-sized oval to polygonal epithelial cells with lightly eosinophilic to bluish cytoplasm. The nuclei were bland with diffusely stippled chromatin and inconspicuous nucleoli. Intracytoplasmic and extracellular mucin was usually present. Mitotic activity was present but never brisk. All tumors examined immunohistochemically expressed at least one neuroendocrine marker, synaptophysin or chromogranin. CD57 and neuron specific enolase, secondary markers of neuroendocrine differentiation, were expressed in most cases. All tumors tested expressed estrogen and progesterone receptors, cytokeratin 7, low molecular cytokeratin Cam5.2, and epithelial membrane antigen and were negative for cytokeratin 20 and S-100 protein. Calponin, smooth muscle actin, and p63 immunohistochemical stains did not disclose myoepithelial cells around larger tumor nests in most cases, supporting the notion that EMPSGC is an invasive carcinoma. In 10 cases, cystic areas lined by benign epithelium indistinguishable from eccrine ducts were present. In some foci, the benign ductal epithelium was undermined or replaced by carcinoma in situ with similar cytologic features to the solid or papillary areas of EMPSGC. Myoepithelial cells were preserved in the areas of in situ carcinoma. In 6 cases, EMPSGC was associated with invasive mucinous carcinoma. In situ carcinoma and mucinous carcinoma also expressed neuroendocrine markers. Clinical follow-up showed no recurrences or metastases, consistent with low-grade carcinoma. The series provides histologic evidence for a multistage progression of noninvasive sweat gland neuroendocrine carcinoma to EMPSGC and then to mucinous carcinoma of the eyelid. Although the data from this series support the notion that the prognosis of EMPSGC and mucinous carcinoma is good, longer follow-up is needed for better understanding of their pathogenesis and clinical behavior.

Signet ring cell carcinoma of the eccrine sweat glands in the eyelid.  Opthalmology. 1996 Nov;103(11):1788-93

BACKGROUND: Signet ring cell carcinoma of the eyelid is a rare variant of eccrine sweat gland carcinoma and has been reported previously in only five patients. METHODS: The authors report the clinical findings of a 55-year-old man with a signet ring cell carcinoma in the left eyelid as well as a clinical follow-up of 4.5 years. Several biopsies and the exenteration specimen were analyzed by routine light microscopy, electron microscopy, and comprehensive immunohistochemical stains on paraffin sections. RESULTS: Histologically, the tumor was shown to be a rare type of eccrine sweat gland carcinoma with signet ring cells and Indian file growth pattern reminiscent of invasive lobular carcinoma of the breast. Estrogen and progesterone receptors were identified immunohistochemically. On electron microscopy, intracytoplasmic pseudolumina with microvilli were positive for anti-human milk fat globulin and the lectin peanut agglutinin. Clinically, the tumor followed a malignant course with orbital invasion and lymph node metastases. CONCLUSIONS: Histologic recognition of this variant of eccrine sweat gland carcinoma is important because of its aggressive and malignant behavior and the wide range of differential diagnoses. Primarily, metastatic mammary carcinoma must be excluded. The treatment is primary excision with histologic control of the excision margins. In more advanced stages, radiation therapy, neck dissection, and anti-estrogen therapy should be considered.

Histiocytoid eccrine sweat gland carcinoma of the eyelid: report of a case.
Clin Exp Dermatol. 2006 Nov;31(6):786-9.

This report describes the 12th documented case of histiocytoid, diffuse or signet-ring cell eccrine sweat gland carcinoma of the eyelid, a rare neoplasm that characteristically affects older men and mimics distant metastasis. Clinically, the patient was thought to have a colonic metastasis. Histologically, the neoplasm comprised sheets and cords of polygonal, eosinophilic cells, with dispersed larger, rounded single cells resembling histiocytes, and cells with cytoplasmic lumina or a signet-ring appearance. Occasional duct formation was also seen. The features were reminiscent of both the histiocytoid and lobular variants of breast carcinoma. Ultrastructurally, the presence of nonintestinal type microvilli with a length : width ratio in excess of 16 were consistent with an eccrine origin, but also raised the possibility of metastatic mesothelioma. However, a metastatic lesion was excluded by clinical and imaging investigations. There has been no recurrence 3 years after primary excision.

Porocarcinoma of the eyelid. Opthalmology.1997 Oct;104(10):1626-8

PURPOSE: The purpose of this report is to describe the clinical and histopathologic findings in a patient with porocarcinoma of the eyelid. METHODS: The case of a 68-year-old woman with a nodular lesion of the eyelid was studied, and the pertinent literature reviewed. RESULTS: No previous description of porocarcinoma of the eyelid was found in the literature. The present case presented with a nodular lesion of the right lower eyelid of 1-year duration. The tumor was completely excised. Microscopic study revealed ductal structures within lobules of tumor cells. CONCLUSIONS: Porocarcinoma is a rare cutaneous adnexal tumor arising from the eccrine secretory apparatus. Because the clinical behavior of this cancer includes deep invasion, regional lymphatic spread, and distant metastases, complete surgical excision is recommended, and should be verified by either conventional frozen sections or Mohs' micrographic sections.

Eyelid microcystic adnexal carcinoma. Arch Ophthalmol.1995 Apr;113 (4):494-6

Microcystic adnexal carcinoma is an uncommon cutaneous tumor with multiple synonyms. On cursory microscopic examination, the tumor mimics syringoma and other benign skin adnexal tumors. However, the asymmetric, infiltrative growth pattern clearly sets the lesion apart as carcinoma. The tumor is locally aggressive, with recurrences common, but regional metastases are rare. Histogenesis is controversial. Optimal treatment consists of complete surgical excision with clear surgical margins.

Orbital and periorbital microcystic adnexal carcinoma.  Ophthal Plast Reconstr Surg. 2005 Mar;21(2):97-102

PURPOSE: To review the clinical and histopathologic features of patients with microcystic adnexal carcinoma of the orbital and periorbital tissues. METHODS: This study was designed as a noncomparative interventional case series of three patients seen and treated at two oculoplastic surgical departments and a review of the literature. Clinical presentation, histopathologic findings, outcome of surgery, and clinical recurrence are presented. RESULTS: Three patients underwent surgery for lesions temporal to the lateral canthus. All had initial histopathology diagnosed as squamous cell carcinoma but were subsequently diagnosed as microcystic adnexal carcinoma after clinical recurrence. All patients had multiple recurrences and extensive perineural spread. Two patients required radiotherapy. CONCLUSIONS: Review of the literature shows that microcystic adnexal carcinoma affecting the orbital and periorbital tissues is difficult to differentiate clinically and microscopically from other conditions including squamous cell carcinoma. Eighty-one percent of cases that have histopathology checked in the initial assessment are still misdiagnosed. A high degree of suspicion is necessary if the lesion extends beyond an apparently adequate surgical margin, multiple recurrences occur, or superficial perineural spread is present. Mohs technique is the treatment of choice and may be improved if paraffin sections or immunohistocytochemistry are performed. Wide excision should be carried out once clear margins are obtained. Radiotherapy may be necessary in cases with multiple recurrences.

Adenoid cystic carcinoma   Primary cutaneous adenoid cystic carcinoma, an uncommon tumor of the skin, should be added to the differential diagnosis of eyelid tumors.

Adenoid cystic carcinoma arising from the lower eyelid. Jpn J Ophthalmol. 2006 Jul-Aug;50(4):374-6.

 BACKGROUND: Primary cutaneous adenoid cystic carcinoma is one of the rarest eyelid tumors. CASE: A 39-year-old man presented with a small subcutaneous induration in his left lower eyelid close to the lower lacrimal punctum. OBSERVATIONS: Under a clinical diagnosis of chalazion, the eyelid induration was extracted surgically via the palpebral conjunctiva. Histopathological examination of the extracted tumor revealed proliferation of basaloid cells with a prominent cribriform pattern containing Alcian blue-positive and periodic acid Schiff-positive material, leading to a diagnosis of adenoid cystic carcinoma. The patient subsequently underwent extended radical excision including the lacrimal sac. No recurrence was observed during 20 months after surgery. CONCLUSIONS: Adenoid cystic carcinoma should be considered as a differential diagnosis of eyelid tumors simulating chalazion. Adenoid cystic carcinoma in the eyelid may have a more favorable prognosis than that in the lacrimal gland.

Mucinous carcinoma   Primary cutaneous mucinous carcinoma of the eyelid is an adenocarcinoma of the eccrine glands. It is rare and locally aggressive but the prognosis following local excision, confirmed with tumour-free margins, is good. This tumour is usually described in the elderly.

Mucinous eccrine adenocarcinoma of the periocular region.
Ophthal Plast Reconstr Surg. 2006 Jan-Feb;22(1):30-5.

PURPOSE: To report 4 patients with mucinous eccrine adenocarcinoma of the periocular region. METHODS: Interventional case series of 4 patients with mucinous eccrine adenocarcinoma of the periocular region treated at 2 centers. RESULTS: Two patients with mucinous eccrine adenocarcinoma of the upper eyelid, 1 with mucinous eccrine adenocarcinoma of the lower eyelid, and 1 with mucinous eccrine adenocarcinoma of the eyebrow were included in this report. The lower eyelid lesion and the eyebrow lesion recurred after Mohs micrographic surgery. Both recurrent lesions were treated with Mohs micrographic surgery and postoperative adjuvant external-beam radiation therapy. The other 2 primary tumors were treated with wide local excision with frozen section control of margins and did not recur. The follow-up time after the most recent surgical procedure ranged from 8 to 17 months (median, 12 months). CONCLUSIONS: Mucinous eccrine adenocarcinoma is a rare ocular adnexal neoplasm that can involve the eyelid and periocular skin, can be locally invasive, and has a high risk of local recurrence despite Mohs surgery.

Sebaceous Tumours

Sebaceous Adenoma

Sebaceous tumors of the eyelids in a patient with Muir-Torre syndrome]
J Fr Ophtalmol. 2006 Jun;29(6):654-8.

Muir-Torre syndrome is an autosomal dominant hereditary condition predisposing to cancer. It is characterized by cutaneous tumors (such as sebaceous adenomas, epitheliomas, or carcinoma, and/or keratoacanthomas) and internal malignancies. A 47-year-old male patient with cancer antecedents consulted for two tumors of the eyelid. Histological study of the exeresis biopsies of the eyelid lesions showed a sebaceous adenoma and an epidermoid carcinoma with sebaceous differentiation. With the suspicion of Muir-Torre syndrome, a genetic consultation was requested. The geneticist found a mutation of the MSH2 gene, which was not classified as pathological. Cancer screening was begun for the patient and his descendants. The clinical outcome was death from urothelial carcinoma. Eyelid sebaceous tumors require complete medical check-up in search of carcinoma. Demonstrating Muir-Torre syndrome allows the medical team to propose early cutaneous and visceral carcinoma screening for patients, their collaterals, and their descendants.

 Sebaceous Carcinoma Sebaceous gland adenomas and adenocarcinomas may from the glands of Zeis or the meibomian glands.

Intraepithelial sebaceous carcinoma of the eyelid misdiagnosed as Bowen's disease. Cutan Pathol. 2006 Apr;33(4):303-8.

Outcome of patients with periocular sebaceous gland carcinoma with and without conjunctival intraepithelial invasion. Opthalmology 2001 Oct;108(10):1877-83

PURPOSE: To evaluate the clinical findings, histopathologic features, methods of management, and outcomes in 25 patients with eyelid sebaceous gland carcinoma with and without conjunctival intraepithelial (pagetoid) invasion. PARTICIPANTS: Twenty-five patients with sebaceous gland carcinoma of the periocular region. DESIGN: Retrospective, observational case series. METHODS: The patients were divided into two groups: group A included those with sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion, and group B included those with sebaceous gland carcinoma without conjunctival intraepithelial invasion. The demographic, clinical, and histopathologic features of sebaceous gland carcinoma were reviewed separately for each group. Ocular management results and systemic prognosis were reviewed. RESULTS: Of the 25 patients, 18 (72%) were females and 7 (28%) were males. The mean age at diagnosis was 65 years (range, 17-90 years). There were 11 patients (44%) in group A, and 14 (56%) in group B. The most common symptom in group A was ocular irritation (45%), and in group B the most common symptom was eyelid lump (86%). The most common sign in group A was diffuse eyelid thickening (72%), and in group B the most common sign was a distinct eyelid mass (43%). Six patients (55%) in group A, compared with one patient (7%) in group B, had both upper and lower eyelid involvement. The most frequent location of tumor epicenter was the outer third of the eyelid in both groups: 37% in group A and 57% in group B. Histopathologic origin of tumor was multicentric in four patients (36%) of group A and six patients (50%) of group B. Before referral, local recurrence had been found in 4 of 12 patients (33%) who underwent surgical treatment elsewhere. After referral, local recurrence was detected in 1 patient (9%) in group A and in 2 patients (14%) in group B. Orbital exenteration was necessary in 4 patients (36%) in group A and in one patient (7%) in group B. Tumor-related metastases occurred in two patients (18%) in group A and in two patients (14%) in group B. CONCLUSIONS: Sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion has different clinical features compared with that without pagetoid invasion. Conjunctival intraepithelial (pagetoid) invasion carries a higher risk for orbital exenteration, but the risk of tumor-related metastases is similar in both groups.

Sebaceous carcinoma of the eyelids: personal experience with 60 cases.  Opthalmology. 2004 Dec;111(12):2151-7

OBJECTIVE: To describe clinical features, management, and prognosis of sebaceous carcinoma of the eyelid and adjacent structures. DESIGN: Single-center retrospective interventional case series. PARTICIPANTS: Sixty consecutive patients with sebaceous carcinoma of the eyelid and adjacent structures. METHODS: Retrospective chart review and literature review. MAIN OUTCOME MEASURES: Presenting features, sites of origin, location, growth patterns, management, histopathologic findings, incidence of recurrence, metastasis, and mortality. RESULTS: The median age at referral was 72 years, with 73% female. Four patients had prior irradiation to the area where the sebaceous carcinoma developed. Initial clinical diagnoses elsewhere were sebaceous carcinoma (32%), blepharoconjunctivitis (25%), chalazion (20%), basal cell carcinoma (13%), and squamous cell carcinoma (10%). Initial histopathologic diagnoses elsewhere were sebaceous carcinoma (50%), squamous cell carcinoma (18%), basal cell carcinoma (8%), and others or not available (24%). Initial anatomic sites were upper eyelid (75%), lower eyelid (22%), caruncle (2%), and bulbar conjunctiva (2% [1 case]). Orbital exenteration was necessary in 13%. Recently introduced techniques of posterior lamellar resection of the eyelids with reconstruction (7%) hopefully will decrease this incidence in the future. Pathologically, 47% showed intraepithelial (pagetoid) involvement, 27% of sebaceous carcinomas arose from the meibomian glands, and 18% arose from both meibomian and Zeis glands. Local recurrence developed in 18%, metastasis in 8%, and death from metastasis in 6%. CONCLUSIONS: Despite the fact that the clinical features of sebaceous carcinoma have been widely reported, the diagnosis was suspected initially in only 32% of patients at first examination elsewhere and in only 50% at histopathologic examination elsewhere. Orbital exenteration was necessary in 13%, mostly patients seen in the earlier years of the study. With more recently employed treatment methods, there is a tendency to avoid exenteration and to use more conservative methods of treatment. It is hoped that these modern therapeutic approaches will result in fewer cases of recurrence and metastasis.

             

Tumours of the Hair Follicle

 Trichilemmoma

Trichilemmoma of eyelid and eyebrow. A clinicopathologic study of 31 cases. Arch Ophthalmol.1980 May;98(5):844-7

Thirty-one cases of trichilemmoma of the eyelid and eyebrow were studied. All lesions were solitary, small, and asymptomatic. Unlike basal cell carcinoma of the eyelid, trichilemmoma rarely involves the lid margin or the inner canthus. The age range of our patients was from 22 to 88 years old (mean, 56 years). There were 19 men and 12 women. The most frequent clinical diagnoses were verruca vulgaris and cutaneous horn. Typically, the tumor displayed a lobular pattern and was composed mostly of glycogen-rich clear cells. The periphery of the lobules showed palisading of columnar cells and a distinct basement membrane. Histologically, the tumor was frequently misinterpreted as basal cell carcinoma. Review of the literature, including our own series, discloses that the eyelid is the second most common site for trichilemmoma after the nose.

Desmoplastic trichilemmoma: a rare tumor of the eyelid.Eur J Ophthalmol. 2004 Nov-Dec;14(6):562-4.

PURPOSE: To report an upper eyelid mass which proved to be a desmoplastic trichilemmoma. METHODS: A 60-year-old man had a slowly enlarging upper eyelid mass. The tumor was excised. The pathologic evaluation of the tumor was centered on the differential diagnosis. RESULTS: The clinical appearance of this lesion is nonspecific and can simulate a verruca, follicular keratosis, or basal cell carcinoma. Central desmoplasia, outer root sheath differentiation of the tumor cells, and CD34 positivity are the main characteristics that allow differentiation from basal cell carcinoma. CONCLUSIONS: Proper recognition of a benign neoplasm that may be misdiagnosed as basal cell cancer can prevent aggressive surgical treatment.

Trichilemmal Carcinoma

Trichilemmal carcinoma of the upper eyelid.Acta Ophthalmol Scand. 2003 Oct;81(5):536-8.

INTRODUCTION: Trichilemmal carcinoma (TLC) is a rare adnexal tumour related to the external hair sheath. We describe the first case of TLC on the upper eyelid to be treated with Mohs micrographic surgery. CASE REPORT: A 65-year-old man presented with a 2-month history of a firm 4 mm left upper eyelid nodule. An incisional biopsy revealed an infiltrative, lobulated tumour composed of large, polygonal, clear cells. A diagnosis of trichilemmal carcinoma was made and the patient underwent Mohs micrographic surgery. At follow-up 2 years later, there was no evidence of recurrence. CONCLUSION: The pathogenesis of TLC remains unclear, with actinic damage, longterm low dose irradiation and transformation from benign trichilemmoma having been postulated. Trichilemmal carcinomas must be differentiated from other malignant clear cell tumours of the eyelid and the correct diagnosis made promptly as TLC takes an indolent clinical course, in which metastasis is a rare event, despite the tumour's cytologically malignant appearance. We recommend Mohs micrographic surgery as a treatment modality for this tumour, due to its potential for locally aggressive growth and local recurrence.

Pilomatrixoma

Pilomatrixoma of the eyelids--clinical differential diagnosis and follow-up. Report of 17 patients.  Ophthalmologe.1993 Dec; 90(6):746-9

Pilomatrixomas (benign epithelioma of Malherbe) are often located in the eyelid or eyebrow. Clinically the diagnosis is rarely made. Histologically, especially in partial biopsies, they can be confused with a basal cell carcinoma. We evaluated 17 tumors in 17 patients (19 months to 80 years old). The interval between the diagnosis and total excision of the mass ranged from 4 weeks to 2 years. The clinical misdiagnosis appeared to be related to the patient's age: A cystic dermoid (4x) was considered most frequently in children and sebaceous cyst or atheroma (5x) in patients of middle age. Further diagnoses were as follows: Chalazion, basal cell carcinoma or keratoacanthoma, epidermoid cyst, abscess, papilloma. Nine tumors showed calcifications that might be visible by X-ray examination. After a mean follow-up of 7.6 years no recurrence was observed. Pilomatrixoma should be included in the clinical differential diagnosis of subepidermal solid or cystic tumors, especially in the upper eyelid and eyebrow.

Large, rapidly growing pilomatrixoma of the upper eyelid. Ophthal Plast Reconstr Surg 2006 Sep-Oct; 22(5):401-3

A 41-year-old woman presented with a rapidly growing mass on her upper left eyelid that had first appeared 3 months earlier. The mass was dark red in color, alternating with whitish pseudocyst formations, and measured 3 cm long with a 1-cm base presenting. The rapid growth had caused a mechanical ptosis and bleeding as the result of erosion of the skin covering the tumor. The initial clinical diagnosis suggested a malignant lesion or vascular tumor; excisional biopsy was performed. The eyelid crease was approached for incision and resection as for a blepharoplasty. Pathologic examination yielded a diagnosis of giant pilomatrixoma. Pilomatrixomas are rare in adults and rarely attain such a large size. After 1 year of follow-up, no recurrence of the tumor has been observed.

Trichoepithelioma 

Eyelid trichoepithelioma--report of 2 cases.Arq Bras Oftalmol. 2005 Jan-Feb;68(1):136-9. Epub 2005 Mar 30.

Trichoepithelioma is a benign skin tumor and is most commonly found on the face, however, there are few reports about its occurrence on the eyelids. It is not usually recognized because of its rarity, origin, biological potential, and controversial classification. The purpose of this paper is to report two cases of solitary trichoepithelioma on the eyelid, their histopathology and treatment. This lesion should be considered when a single solid nodule on the face appears, and can be differentiated from basal-cell carcinoma. Histopathologic confirmation is essential.

Trichofolliculoma

Recurrent trichofolliculoma of the upper eyelid margin.Ophthal Plast Reconstr Surg. 1997 Dec;13(4):287-8.

We report a woman who had a recurrent trichofolliculoma on the upper eyelid margin. Only three cases of this benign tumor on the eyelid have been reported, and no recurrence in this location had been noted in the literature. The lesion, present for 6 years, had been excised twice previously (3 and 4 years before), recurred, and had been injected with a steroid preparation 2 years earlier. Lashes, both normal-looking and immature, arose from the center area of this lesion, and telangiectatic vessels were on its surface. Full-thickness wedge resection was used to excise the lesion completely. Complete primary excision of trichofolliculoma is important, and local steroid preparations should not be used.

February 2007
Pathology of the Eyelid

Normal Anatomy and histology of Eye ;

Dermoid cyst of eyelid

Inflammatory diseases of the Eyelid

Chalazion;

Retinoblastoma

Tumour and tumour-like lesions of the Eyelid

Epidermal tumours of the Eyelid ;

Melanocytic tumours of the Eyelid;

Mesenchymal Tumours of the Eyelid ;

Xanthelasma

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Skin Adnexal (Appendage) Tumours

Benign sweat gland tumours

Cysts:

Hydrocystoma -Eccrine/Apocrine

Hamartomas:

Eccrine/Apocrine naevus

Eccrine Angiomatous Hamartoma

Porokeratotic eccrine ostial naevus

Benign Neoplasms:

Syringocystadenoma Papilliferum

Hidradenoma Papilliferum

Nipple Adenoma

Syringoma

Spiradenoma

Cylindroma

Chondroid Syringoma (benign mixed tumour)

Poroma

Hidradenoma

Apocrine adenoma

Papillary eccrine adenoma

Syringofibroadenoma

Malignant sweat gland tumour 

Low grade:

Microcystic adnexal carcinoma

Eccrine epithelioma

Adenoid cystic carcinoma

Mucinous carcinoma

Primary extramammary Paget’s disease

Intermediate grade:

Porocarcinoma

Digital papillary adenocarcinoma

High grade:

Hidradenocarcinoma

Apocrine adenocarcinoma

Eccrine ductal carcinoma

Spiradenocarcinoma

Malignant cylindroma

Sebaceous tumours

Fordyce's Spots

Steatocystoma

Nevus Sebaceous

Folliculosebaceous Cystic Hamartoma

Sebaceous Hyperplasia

Sebaceoma

Sebaceous Adenoma

Sebaceous Carcinoma

 

Tumours of the Hair Follicle

Hair Germ Differentiation:

Trichoepithelioma 

Desmoplastic Trichoepithelioma

Trichofolliculoma

Trichoblastoma

Cutaneous lymphadenoma

Infundibular differentiation:              Trichoadenoma

Dilated Pore of Winer

Pilar Sheath Acanthoma

Tumour of Follicular Infundibulum

Outer root sheath differentiation:
                    
Trichilemmoma

Trichilemmal Carcinoma

Proliferating  Trichilemmal Cyst (Pilar Tumour)

Matrical differentiation:
                  
Pilomatrixoma and Pilomatrix Carcinoma

Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Lipomatous tumours

Neural tumours

Vascular tumours

Myogenic tumours

Fibroblastic/Myofibroblastic tumours

Myofibroblastic tumours

Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

 

PULMONARY PATHOLOGY

Congenital Cystic Adenomatoid  Malformation

Acute Respiratory Distress Syndrome

Emphysema

Bronchial Asthma

Pulmonary Alveolar Proteinosis

Pulmonary edema

Chronic Bronchitis

Pulmonary Infection

Pneumococcal Pneumonia

Mycoplasma Pneumonia

Pneumocystis Pneumonia

Legionellosis

Localized Fibrous Tumour of the Pleura

Biphasic Epithelial/Mesenchymal Lung Tumours

Pulmonary Carcinosarcoma

Pulmonary Blastoma

Large Cell Neuroendocrine tumour

Benign ocular adnexal tumours of apocrine, eccrine or hair follicle origin. Can J Ophthalmol. 2003 Aug;38(5):357-63.

BACKGROUND: The differential diagnosis of malignant eyelid tumours, particularly basal cell carcinoma (BCC), includes tumours of skin appendages. The incidence of these adnexal tumours has not been well established. This study aimed to determine the incidence and review the main clinicopathological features of benign tumours arising from skin appendages of the eyelid with apocrine, eccrine or hair follicle differentiation. METHODS: The histopathological diagnoses of 7751 ophthalmic specimens retrieved from 6967 patients between September 1993 and March 2002 at the Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, McGill University, Montreal, were retrospectively reviewed. Clinical data and histopathological diagnoses were obtained for 228 benign adnexal tumours of apocrine, eccrine or hair follicle origin. New histopathological slides were made from the paraffin-embedded specimens and stained with hematoxylin-eosin and periodic acid-Schiffs reagent. RESULTS: Of the 228 benign adnexal tumours, 182 were diagnosed as apocrine or eccrine hydrocystoma (79.8%), 12 pilomatrixoma (5.3%), 12 syringoma (5.3%), 11 trichilemmoma (4.8%), 5 syringocystadenoma papilliferum (2.2%), 3 trichoepithelioma (1.3%) and 3 trichofolliculoma (1.3%). Discrepancies between clinical and histopathological diagnoses were noted in 22 cases (9.6%). INTERPRETATION: Benign tumours originating from skin appendages of the eyelid are rare and frequently have apocrine or eccrine differentiation. These tumours, particularly those originating from the hair follicle, should be considered in the differential diagnosis of BCC of the eyelid.

Benign hair-follicle derived eyelids tumors in adults.Arch Soc Esp Oftalmol. 2002 Nov;77(11):605-10

PURPOSE: Benign eyelid neoplasms derived from hair follicles are frequently misdiagnosed, including pilomatricoma, trichoepithelioma, trichilemmoma, trichoblastoma and inverted follicular keratosis. METHODS: We reviewed 17 tumors seen in the last 9 years, between 1992 and 2002. Excisional biopsy constituted the treatment in all cases. RESULTS: The mean age was 44.5 years (range 13-80 years). Ten were women and 7 men. Pilomatricoma and trichoepithelioma accounted for 76.5% of all neoplasms. All trichoepitheliomas were found as solitary tumors and no patient had a history of familial trichoepithelioma. The remaining neoplasms (23.5%) were trichilemmomas, trichoblastoma and inverted follicular keratosis. No patient recurred locally and there were no malignant transformations after a mean follow-up of 3.1 years. The most frequent hair follicle neoplasms in our series was pilomatricoma (9 cases). CONCLUSIONS: Benign hair follicle neoplasms are rare in the eyelid. Most can simulate a basal cell carcinoma when evaluation is based on clinical evidence alone, and only the pilomatricoma may resemble a benign neoplasm. Thereby they must be excised with free surgical margins. All excised eyelid lesions should be sumitted for histopathologic examination because it is not possible to obtain 100% accuracy in diagnosing eyelid tumors.

Benign hair-follicle derived tumours in the differential diagnosis of basal-cell carcinoma of the eyelids: a clinicopathological comparison. Br J Ophthalmol. 1989 May;73(5):347-53

Benign eyelid tumours derived from hair follicles are rare and frequently misdiagnosed as basal-cell carcinoma when evaluation is based on clinical evidence alone. They include trichoepithelioma, trichofolliculoma, trichilemmoma, and pilomatrixoma. We reviewed 117 such tumours received in the Department of Pathology, Institute of Ophthalmology, London, in the last 30 years, a number which compared with 2447 basal-cell carcinomas seen over the same period. The hair follicle tumours may be safely excised with a narrow margin of clearance, whereas a macroscopic clearance of 3 to 5 mm or surgery with frozen section histological control is desirable for excision of basal-cell carcinoma. Therefore confirmation by incisional biopsy of the nature of any large lesion suspected of being a basal-cell carcinoma is essential in order to avoid performing an unnecessarily extensive or time consuming excision.

Eccrine acrospiroma of the eyelid with oncocytic, apocrine and sebaceous differentiation. Further evidence for pluripotentiality of the adnexal epithelia.
Eur J Ophthalmol. 1991 Oct-Dec;1(4):187-93.

A wide variety of sweat gland tumors occur in the eyelids. We used light and electron microscopy to investigate an eccrine acrospiroma showing areas of oncocytic, apocrine and sebaceous differentiation. To our knowledge this is the first report of an oncocytic lesion arising from an eccrine sweat gland, and only the second report of such a lesion arising from the skin. Furthermore, apocrine and sebaceous differentiation are atypical in lesions originating from sweat glands. The wide spectrum of differentiation in this adenoma lends further support to the concept of pluripotentiality of the adnexal glandular epithelia.