Sebaceous carcinoma of the eyelid. Errors in clinical and pathologic diagnosis.

Sebaceous carcinoma of the eyelid is commonly misdiagnosed because it simulates other entities both clinically and pathologically. In a review of all pathologic material removed from the eyelids of patients at the Mayo Clinic between 1905 and 1981, 43 sebaceous carcinomas were discovered. The tumors manifested most frequently on the upper eyelid; the onset of symptoms occurred in 31 women and 12 men at a mean age of 61.5 years (range, 28-82 years). Of the 43 patients, 11 had an associated second malignant tumor or exposure to irradiation. The microscopic diagnosis was based on finding a proliferation of basophilic neoplastic cells with foamy cytoplasm and a positive fat stain. Intraepithelial neoplasia in the form of pagetoid change or carcinoma in situ was found in more than 80% of cases. Histologically, the tumor tended to be moderately differentiated. A high degree of infiltration was frequently observed cases with metastases. When intraepithelial neoplasia is observed microscopically, we recommend a fat stain on a frozen section and a full-thickness eyelid biopsy to exclude sebaceous carcinoma.

Sebaceous carcinoma of the eyelid following radiation therapy.

This paper reports a case of a 12-year-old boy with fatal sebaceous carcinoma originating from the eyelid. The carcinoma developed 11 years after curative radiation therapy for bilateral retinoblastoma. Sebaceous carcinomas associated with previous irradiation are uncommon, and only five such cases have been published. Three of the patients were treated for benign disorders and the two other cases followed radiation therapy for bilateral retinoblastoma. Second malignant neoplasms in patients with bilateral retinoblastoma are thought to be related to a genetically inherited predisposition to the development of second malignant tumors. Radiation therapy is claimed to be an inducing factor in many cases but malignant neoplasms are also found in areas distant from the portals of irradiation as well as in patients not irradiated. Most of the secondary tumors were sarcomas, and usually osteogenic sarcomas.

Malignant Merkel cell neoplasm of the eyelid.

Malignant Merkel cell neoplasms of the eyelid are most commonly found in elderly patients. They occur most frequently on the upper eyelid and are seen as large, nontender , red or violaceous masses with intact overlying skin. Differential diagnosis includes lymphoma and oat cell carcinoma. Conventional light microscopic features are usually distinctive, but tissue should be saved for electron microscopy and immunocytochemical studies, since the presence of dense-core neurosecretory granules and perinuclear microfilaments on electron microscopic examination and positive staining for neuron-specific enolase, are strongly suggestive of malignant Merkel cell neoplasm. Because of the diffuse infiltrative growth pattern and early lymphatic spread of this tumor, treatment requires careful examination of regional lymph nodes and wide surgical excision of the tumor with frozen-section control of margins.

Merkel cell tumor of the eyelid. A clinicopathologic case report.

We treated a patient who had clinical and pathological findings of a primary cutaneous tumor of the eyelid with histological and ultrastructural features of a Merkel cell carcinoma. This neoplasm is composed of cells that are thought to be derived from the neural crest and are found normally in the skin. While it may be a low-grade malignant neoplasm, this tumor can grow rapidly and metastasize. Histologically, it can mimic a metastatic undifferentiated small-cell carcinoma from the lung or other primary sites. To our knowledge, this is the first reported case involving an eyelid.

Ocular adnexal lymphoid tumors. Correlative ultrastructural and immunologic marker studies.

Twenty-two ocular adnexal lymphoid infiltrates were analyzed by electron microscopy as well as immunologically and cytochemically. Five reactive polyclonal lesions were found to be preponderantly composed of small mature lymphocytes (presumably T cells) with clumped nuclear chromatin, sparse cytoplasmic organelles, and numerous monoribosomes. In 11 monoclonal B-cell lesions, both the 1-micrometer plastic sections examined by light microscopy and the electron micrographs disclosed immature cells, with more dispersed nuclear chromatin, prominent nucleoli, abundant cytoplasmic polyribosomes, and increased numbers of mitochondria and strands of endoplasmic reticulum particularly in plasmacytoid lesions). The remaining six monoclonal B-cell lesions were composed of comparatively well-differentiated cells requiring electron microscopy to show somewhat more prominent nucleoli, slightly less dense clumping of the nuclear chromatin, increased numbers of mitochondria and short segments of rough-surfaced endoplasmic reticulum, and monoribosomes rather than polyribosomes. The importance of distinguishing this group of well-differentiated monoclonal lesions from the less well-differentiated ones was underscored by the results of the follow-up examinations, in that no evidence of extraorbital disease has been discovered in the former group, while a 50% incidence occurred in the latter.

Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases.

Five patients had juvenile fibrosarcoma of the orbit and eyelid. At initial surgical intervention, the patients ranged in age from newborn to 8 years (mean, 3 years 9 months). Three patients were male and two were female. Proptosis and/or painless swelling of the eyelid were the most common signs and symptoms. Four tumors grew within a few weeks or months, and all five were poorly circumscribed. They were composed of immature, spindle-shaped fibroblastic cells in a classic herringbone pattern or in interlacing fascicles. Hypercellularity and mitotic activity were present in all lesions. The differential diagnosis included rhabdomyosarcoma, fibromatosis, and fibrous histiocytoma. Electron microscopy confirmed the fibroblastic nature of the tumor cells in three cases. Follow-up ranged from one to 32 years (median, seven years). Two of five tumors recurred locally but none metastasized. Apparently surgical excision is the treatment of choice and the tumor has a favorable prognosis.

Granular cell tumor of the orbit and ocular adnexae.

Although granular cell tumor (GCT) has been a distinct pathological entity since 1926, confusion and controversy have long existed regarding the nature and histogenesis of the tumor. Twenty-five cases of granular cell tumor involving the orbit and ocular adnexae previously reported in the ophthalmic literature, are reviewed and six additional cases are reported. The histopathological and immunohistochemical characteristics of granular cell tumors in this uncommon location are discussed and their probable origin from Schwann cells is also considered.

Primary mucinous sweat gland carcinoma of the eyelid simulating metastatic carcinoma.

A 78-year-old man suffered an unusual variant of sweat gland carcinoma of the eyelid. This disorder is seldom recognized, and is frequently confused with inflammation, benign neoplasms, and metastatic carcinomas. On the basis of clinicopathologic and ultrastructural features found by both light and electron microscopy, we diagnosed sweat gland carcinoma of the eyelid. We considered the possibility of metastasis from a primary carcinoma elsewhere in the patient.

Trichoadenoma of the eyelid.

PURPOSE: To report the unique occurrence of a trichoadenoma of the eyelid. METHODS: An 80-year-old woman underwent surgical resection of a lesion of the right lower eyelid that was suspected to be a basal cell carcinoma. The lesion was studied with light microscopy. RESULTS: Histopathology showed a lesion in the dermis that contained keratin cysts surrounded by a proliferation of eosinophilic epidermoid cells. The findings were characteristic of a trichoadenoma. CONCLUSION: Trichoadenoma, a rare, benign, cutaneous tumor that usually occurs on the face, can develop on the eyelid and simulate a basal cell carcinoma.

Merkel cell carcinoma: a malignant neuroendocrine tumour of the eyelid.

Merkel cell carcinoma is a recently described tumour which may occur on the face of the elderly. We report such a lesion which presented as a cyst on the eyelid of an 88-year-old woman. This tumour differs from other tumours of the eyelids in its propensity for local and distant spread.

Desmoplastic malignant melanoma masquerading as chalazion.

Desmoplastic malignant melanoma is a rare and highly malignant tumour, which usually occurs in the head and neck. This is demonstrated by a case history of a patient with this lesion on the lower lid, which has not been previously described in the British ophthalmological literature. The lesion often presents considerable problems of histological diagnosis.

Phakomatous choristoma of the lower eyelid. A light and ultrastructural study.

This article documents the eighth reported case (to our knowledge) of a phakomatous choristoma, a rare congenital tumor of the lower eyelid of infants. Among the eight cases, there was no instance of tumor recurrence recorded nor was there any detectable postoperative eye defect, despite incomplete resection of the tumor in two patients. While usually clinically diagnosed as a dermoid cyst, the highly characteristic histologic features of this entity should allow more frequent recognition. Ultrastructural examination in our case provided additional evidence of its lenticular origin.

Lymphosarcoma with plasmacytoid differentiation in a scarlet macaw (Ara macao).

A lymphosarcoma in a scarlet macaw (Ara macao) affecting periocular structures is described. Microscopically and ultrastructurally, many of the lymphoid cells had plasmacytoid features. Polymerase chain reaction amplification failed to detect exogenous avian retrovirus RAV-1 in the neoplastic mass.

Gastric adenocarcinoma presenting as an eyelid and conjunctival mass.

A 50-year-old white man complained of "inflammation" of his right eyelid since January 1989. In June 1990, he had undergone a gastrectomy for an adenocarcinoma of the stomach. In August 1990, his right eye showed a painless, firm infiltration of the upper and lower lid, ulceration and loss of eyelashes of the upper lid and a reddish, subepithelial thickening of the medial bulbar conjunctiva. Biopsies of the right upper lid and nasal bulbar conjunctiva disclosed a metastatic, poorly differentiated adenocarcinoma of the stomach (signet ring cell carcinoma) both in the lid and conjunctival biopsies. Carcinoembryonic antigen (CEA) was detected in the tumor cells. Electron microscopic examination revealed tumor cells with signs of secretory activity. Although metastases to the eyelids and conjunctiva are rare, they may precede the clinical manifestation of the primary tumor for months or even years. In patients with chronic, recalcitrant lesions of eyelids or conjunctiva, especially if accompanied by loss of eyelashes, a primary or secondary malignancy should be excluded by early biopsies and histopathological examination.

Light and electron microscopic studies of malignant melanoma of conjunctiva and palpebra.

The authors experienced one case of malignant melanoma of the superior palpebral conjunctiva and the inferior palpebral skin, originating from benign acquired melanosis of the bulbar conjunctiva which developed in a 48-year-old man. Orbital exenteration including superior and inferior palpebrae was performed, and the removed tissue was examined by light and electron microscopy. In the temporal bulbar conjunctiva, melanocytes containing abundant fine melanin granules proliferated over almost the whole area of the epithelial layer. Cellular atypia and karyomitosis were not observed. The basement membrane remained intact with no proliferation of melanocytes in the substantia propria. However, in the perivascular area, in addition to the moderately prominent infiltration by lymphocytes and plasma cells, infiltration by melanophages was observed relatively frequently. Based on these findings, it was confirmed that the primary lesion of the bulbar conjunctiva was an acquired melanosis of Stage IB as classified by Zimmerman. The black nodular tumors of the superior palpebral conjunctiva and inferior palpebral skin consisted of melanoma cells showing markedly prominent cellular and nuclear atypia. The nuclei showed indentations in the membrane with electron-lucent karyoplasm, each having one nucleolus. The cytoplasm contained many round or elongated melanosomes in various developmental stages, with sizes of about 0.7 micron. Scattered among melanoma cells were a number of melanophages which had phagocytized many melanosomes at various stages of maturity.(ABSTRACT TRUNCATED AT 250 WORDS)

[Light and electron microscopic findings in eyelid tumors after cryotherapy]. / Licht- und elektronenmikroskopische Befunde von Lidtumoren nach Kryotherapie.

Over the past five years 112 patients underwent cryotherapy of lid tumours. Two methods of freezing, contact freezing and spray freezing, were used. Biopsy specimens for histological studies were taken before and after cryotherapy. The results of light and electron microscopy are reported. Electron microscopic examination shows that cryonecrosis exhibits the well known general characteristics of necrosis. Cryotherapy results in extensive tumour necroses and this fact explains the success rate of 90.9% in basalioma 5 years after therapy.

Phakomatous choristoma: clinical, histopathologic, and ultrastructural findings in a 4-month-old boy.

A case of phakomatous choristoma of the lower eyelid is described. A survey of previously reported cases reveals a characteristic and consistent clinical and morphologic picture. Universally the patient presents in infancy with a rather small, firm, rubbery nodule attached to the lower edge of the inferior tarsal plate. Always present in the nasal aspect of the lower lid, the tumor is easily palpable. Recognition of this clinical picture may allow the ophthalmologist to suspect the appropriate diagnosis, but the final diagnosis should be based on the recognition of characteristic histopathology.

Atypical cases of sinus histiocytosis (Rosai-Dorfman disease) with ophthalmological manifestations.

Sinus histiocytosis with massive lymphadenopathy is a non-neoplastic disease of unknown cause observed mainly in children with markedly enlarged cervical or other lymph nodes. We have reported two very atypical cases with extranodal manifestations that required ophthalmological consultation. Neither patient had significant lymphadenopathy. One patient, who had tumors of all four eyelids as well as lesions of the nose and larynx, was 68 years old when first examined, and 70 at the time of his last surgical procedure. The other patient, a 13-month-old child, had a unique corneoscleral lesion as his only clinical manifestation. The excised tissues obtained from the eyelids and larynx in case 1 and from the corneoscleral lesion in case 2 revealed characteristic histopathologic features of sinus histiocytosis (Rosai-Dorfman disease), including strongly positive immunoperoxidase staining for S-100 protein in the cytoplasm of most of the histiocytes.

[Primary signet ring cell carcinoma of the eccrine sweat gland in the eyelid. Immunohistochemical and ultrastructural study of a case]. / Adénocarcinome sudoral primitif de la paupière de type eccrine à cellules en bague à chaton.

We report a case of a 45-year-old woman who exhibited a primitive eccrine sweat gland carcinoma of the eyelid. Histological study showed cellular proliferation with an Indian file pattern and some signet ring cells with sialomucin secretion. Immunohistochemical study demonstrated these cells to be positive with the anticytokeratin, anti-EMA, anti-HMFG, antiestrogen receptor and antiprogesterone receptor antibodies. Ultrastructural study showed intracytoplasmic vacuoles with numerous microvilli at the apical side. Differential diagnosis with a metastasis from a mammary adenocarcinoma is difficult and a complete staging is necessary to confirm the primitive origin of the tumor. The behavior of this tumor is marked by locoregional recurrence.

[Eyelid localization of a Merkel cell tumor (trabecular carcinoma)]. / Localisation palpébrale de la tumeur á cellules de Merkel (carcinome trabéculaire).

The authors present four cases of Merkel cells tumor of the eyelid. These cases conform to the standard clinical and histological criteria specified in the dermatological literature. Immuno-histochemical studies have been performed (specific neuron enolase, cytokeratin KL 1). Il the last case an ultrastructural study has made evident the characteristic neurosecretory granulations and intermediate filaments. Various hypothesis concerning the origin of this tumor are discussed in light of anatomical evidence.