Novel Hormone Receptors Present in Apocrine Cystadenoma of the Eyelid.

A 53-year-old woman presented with an apocrine cystadenoma of the right upper eyelid. Histologic examination revealed proliferating epithelial cells with apocrine snouts and occasional mitotic figures. Immunohistochemical analysis revealed a Ki-67 index of 15% and positive staining for synaptophysin, chromogranin, estrogen receptor, progesterone receptor, gross cystic disease fluid protein (GCDFP)-15, and mammoglobin. The complement of positive immunomarkers in this case reinforces the importance of total excision and careful histologic assessment.

Colonisation of basal cell carcinoma by lentigo maligna: a case report, review of the literature, and series follow-up.

BACKGROUND: Melanocytic tumours which colonise basal cell carcinomas (BCC) may be considered as either lentigo maligna (LM) (in situ) or invasive melanomas. OBJECTIVES: To highlight the diagnostic approach and long-term prognosis of LM which colonises BCC. MATERIALS AND METHODS: Using Satter et al.'s classification, we identified a case of BCC colonised by LM and reviewed similar cases in the literature with long-term follow-up. RESULTS: In the absence of melanocytic extension beyond the lamina propria of the BCC compartment, mixed tumours may be considered as LM colonising the BCC, allowing for less invasive surgery. The absence of long-term relapse in our short series supports this diagnosis, rather than invasive melanomas. CONCLUSION: Our case report, review of the literature, and series follow-up illustrate the most recent assessment of melanocytic/BCC tumours, and guide the physician and the pathologist in their recognition and classification, thus allowing them to make the most appropriate therapeutic decisions.

Primary Signet-Ring Cell/Histiocytoid Carcinoma of the Eyelid: A "Binocle" Presentation of the "Monocle Tumor".

Primary cutaneous signet-ring cell carcinoma is a rare and aggressive neoplasm which diffusely involves dermis and subcutis of the eyelid or axillae. Neoplastic cells show a signet-ring cell or histiocytoid morphology in variable number, and can be found intermingled among collagen bundles, sparing the epidermis. This neoplasm typically appears in the eyelids of elderly men, in the form of a painless infiltration and swelling but with no other specific clinical feature, and frequently causes diagnostic retardation and worse prognosis. Frequent involvement of both eyelids of the same eye has given it the name of monocle tumor. Only 29 cases have been described in English literature to date, of which 7 developed metastases, mainly on regional lymph nodes. The authors present a case of involvement of contralateral eyelid, which has only been described previously in 2 cases. The immunohistochemical profile of the involvement in the contralateral eye, and the absence of other metastasis, suggest that it is locally spread from the initial lesion. However the possibility of being a second primary tumor or metastasis cannot be readily ruled out.

Apocrine mixed tumor of the eyelid: a case report.

BACKGROUND: Apocrine mixed tumor is usually found in parotid glands. Its cutaneous counterpart is rare and its occurrence in the eyelids is even rarer. CASE PRESENTATION: This study reports an apocrine mixed tumor of the upper left eyelid in a 68 year-old lady with a history of breast cancer. This mass appeared about 3 years ago, as a slowly growing small nodule, and was completely excised. On microscopic examination, an encapsulated mass with epithelial and mesenchymal features was seen. The epithelial component presented tubular, cystic and infundibular structures while the mesenchymal component was fibrous in some areas and myxoid in others. Plasmacytoid hyaline cells, lipomatous change and focal calcification were appreciated focally. Immunohistochemical studies showed stromal staining for CD10, S-100, α-SMA and p63. Luminal cell layer of the epithelial component was positive for EMA, CK-7 and GCDFP-15 markers. The capsule was unbreached and no satellite lesions were appreciated. No evidence of relapse was evident after 16 months of follow-up. CONCLUSIONS: The diagnosis of eyelid tumors of adnexal origin can be challenging because they are rare and display a wide range of morphological patterns, as the tumor cells might differentiate along any line of the folliculosebaceous-apocrine system. Immunohistochemistry helps improve the accuracy of assessment.

Primary eyelid angiosarcoma in a Chinese patient.

PURPOSE: To describe a case of primary eyelid angiosarcoma and review the literature to emphasize aware of this rare disease. CASE REPORT: We report a further case which is the first Chinese primary eyelid angiosarcoma in the literature. A 76-year-old woman presented with a 6-month history of a painless lesion on her left eyelid. The patient finally proved to be angiosarcoma on histopathologic and be treated with complete surgical excision with a frozen section margin control. With a 6 months follow-up, we haven't found any symptoms of recurrence or metastasis. CONCLUSIONS: Cutaneous angiosarcoma with eyelid is a rare, soft-tissue sarcoma of endothelial cell origin that is aggressive malignancy and has a poor prognosis. In our case report, the patient was treated with complete surgical excision with a frozen section margin control, and a 6 months follow-up, we haven't found any symptoms of recurrence or metastasis.

An unusual infiltrative basal cell carcinoma with osteoclastic stromal changes mimicking carcinosarcoma: a case report.

A 91-year-old man presented with an ulcerated nodule on his left lower eyelid. The tumor showed an epithelial component composed of basaloid and clear cells and a stroma that contained many osteoclastic giant cells. Strong, diffuse expression for cytokeratin 17 and p63 was noted in the epithelial component, whereas no staining was present in the sarcomatoid stroma, suggesting that the osteoclast-rich stromal component represented an unusual benign stromal reaction to the carcinoma rather than a manifestation of carcinosarcoma. Further supporting this interpretation was the absence of mitotic figures and low Ki-67 proliferation index (of approximately 1%) in the stromal cells. We herein reported a case of unusual infiltrative basal cell carcinoma, accompanied by a clear cell carcinomatous features and concurrent benign osteoclastic stromal changes.

Sebaceous carcinoma: clinicopathologic features and diagnostic role of immunohistochemistry (including androgen receptor).

OBJECTIVE: To explicate the clinicopathologic features of periocular sebaceous carcinoma (SC) and emphasize the importance of immunohistochemical staining that includes androgen receptor (AR) in their diagnosis. DESIGN: Retrospective study. METHODS: Clinical and histopathologic features in 56 patients with periocular SC were analyzed. Immunohistochemical staining for pan-cytokeratin (CK), epithelial membrane antigen (EMA), Ber-EP4, adipophilin (ADP), and AR was performed on all cases. Immunostaining in SC was compared with that of squamous cell carcinoma (SCC; n = 25) and basal cell carcinoma (BCC; n = 18) for all antibodies. RESULTS: SGC was more frequent in females, most commonly presenting as a discrete and nodular mass. Four patients had regional lymph node metastases, whereas in 2, the tumour metastasized to the liver. Eight patients had a locoregional recurrence between the 3rd and 45th months of follow-up. Fifty patients had a moderately differentiated tumour. In 3 patients, the resected margins displayed intraepithelial tumour spread, and 2 patients died of the disease. In general, the staining pattern in SGC was CK(+), EMA(+), BerEP4(-), ADP(+), and AR(+). SCCs were CK(+), EMA(+), Ber-EP4(-), ADP(-/+), and AR(-), whereas BCC were CK(+), EMA(-), Ber-EP4(+), ADP(-), and AR(-/+). p53 expression and Ki-67 index were higher in SC than SCC or BCC. CONCLUSIONS: Immunostaining using a panel of antibodies comprising BerEP4, ADP, EMA, and AR is useful in diagnosing sebaceous carcinoma and differentiating it from SCC and BCC, which are common to the periocular location and sometimes are morphologically identical to SC.

Schwannoma em pálpebra superior esquerda em criança de 10 anos / Schwannoma of the left superior eyelid in a 10-year-old child

Schwannoma é um tumor neurogênico benigno raro, originário das células de Schwann da bainha de mielina dos nervos periféricos. Sua localização nos tecidos oculares não é comum, sendo a órbita o local afetado com maior frequência e o acometimento das pálpebras é muito raro. Há poucos relatos descritos na literatura sobre Schwannoma palpebral, apenas dois em crianças. Este é, em nosso conhecimento, o primeiro caso relatado no Brasil.

Schwannoma is a rare benign neurogenic tumor. It arises from Schwann cells located at the myelin sheath of peripheral nerves. Its incidence is frequently associated with the orbit. Ocular tissues in general and eyelids in particular are rarely affected. Very few reports can be found in the literature describing eyelid schwannomas. Amongst these, we have found only two describing it affecting children. To our knowledge, this is the first case report about eyelid schwanomma in Brazil - and it involves a child.

Nodular hidradenoma of the caruncle.

An 84-year-old man presented with a tumor of the right caruncle causing epiphora due to insufficient eyelid closure. The tumor was cystic and mucinous in appearance. MRI showed a solitary tumor of the caruncle with no adhesions to neighboring tissue. The mass was removed by carunculectomy. Histopathologic examination revealed a tumor composed of epithelial cells with intervening strands of hyalinized stroma. Solid areas were predominant but a cribriform area could also be distinguished. Periodic acid-Schiff positive, diastase-labile material (glycogen) was detected in many tumor cells. Immunohistochemical staining of the tumor cells demonstrated positive staining with antibodies against CK-CAM 5.2, BER-EP4, p63, and smooth muscle actin-positive cells in some areas. The tumor was diagnosed as a nodular hidradenoma of the caruncle, which has never been reported before and should be included in the diagnoses of caruncular tumors.

Four cases of solitary fibrous tumour of the eye and orbit: one with sarcomatous transformation after radiotherapy and one in a 5-year-old child's eyelid.

BACKGROUND: Solitary fibrous tumour (SFT) is quite a rare neoplasm involving the eye and the orbit. It is described as showing benign behaviour in adults, but malignant cases are exceptionally reported in this location. This report describes four new cases of SFT/giant cell angiofibroma (GCA) of the eyelid and orbit, one in a 5-year-old child, and one with sarcomatous dedifferentiated transformation occurring 9 years after radiotherapy. METHODS: Four cases of ocular SFT/GCA were retrieved from the database of the Pathological Anatomy Unit, University of Padova; immunohistochemistry and RT-PCR were used to identify COL1A1-PDBGF fusion gene transcripts in all cases. RESULTS: In case 1, late relapse 9 years later was characterised by abrupt transition into a high-grade component, associated with a non-distinctive high-grade sarcomatous area. The latter component was CD34, CD99 and Bcl2 negative and smooth muscle actin positive. Molecular characterisation showed the absence of COL1A1-PDGFB fusion transcripts in cases 1, 3 and 4, excluded diagnosis of giant cell fibroblastoma in all cases. Analysis could not be performed in case 2. CONCLUSIONS: An eyelid SFT/GCA in a 5-year-old child is the youngest case reported in the literature, indicating that the tumour is not exclusive to adults. The case with sarcomatous transformation, with dedifferentiated features occurring 9 years after radiotherapy, raises some questions about the choice of treatment for ocular SFT, in which excision is sometimes difficult without devastating surgery.

Diagnostic utility of adipophilin immunostain in periocular carcinomas.

PURPOSE: To determine the efficacy of adipophilin immunohistochemistry in the diagnosis of sebaceous carcinoma of the ocular adnexal region and to provide the guidelines for interpretation of this immunostain. DESIGN: Retrospective, histopathologic case series. PARTICIPANTS: A total of 25 patients with sebaceous carcinoma, 21 patients with basal cell carcinoma, 22 patients with conjunctival squamous cell carcinoma, 9 patients with cutaneous squamous cell carcinoma, and 5 patients with conjunctival mucoepidermoid carcinoma. METHODS: Immunohistochemical staining for adipophilin was performed on paraffin-embedded tissues and correlated with hematoxylin-eosin, periodic acid-Schiff (PAS), and mucicarmine-stained preparations. Immunostaining was quantified by light microscopy and with a computerized image analysis system of scanned images. Statistical analysis was performed to compare immunostaining patterns within the tumor categories by stage and grade, between the different neoplasms, and for correlation between light microscopy observations and computerized image analysis. MAIN OUTCOME MEASURES: Localization of adipophilin immunostain, intensity of immunostaining, percent of immunoreactive cells, percentages of vacuolar staining and granular staining, and vacuole size. RESULTS: Adipophilin expression was observed in 100% of sebaceous carcinomas, 100% of cutaneous squamous cell carcinomas, 95% of basal cell carcinomas, 73% of conjunctival squamous cell carcinomas, and 60% of mucoepidermoid carcinomas. Sebaceous carcinomas demonstrated significantly stronger adipophilin expression, a greater number of intracytoplasmic vacuoles, and larger vacuoles. The specificity and sensitivity of adipophilin immunostaining in the diagnosis of sebaceous carcinoma were both 100% when more than 5% of the staining occurred in vacuoles (<95% granular staining). The diagnostic sensitivity and specificity were 100% and 96%, respectively, when the staining was graded as moderately or strongly intense and were 92% and 85% when the vacuoles were greater than 1.5 µm in diameter. CONCLUSIONS: Although upregulation of neoplastic steatogenesis is observed in both sebaceous and nonsebaceous carcinomas, the pattern and intensity of adipophilin immunostaining are helpful in distinguishing sebaceous carcinoma from other neoplasms with overlapping histology.

Intraepithelial sebaceous carcinoma: a case report of an unusual occurrence.

Sebaceous carcinoma is a malignant neoplasm that occurs most commonly in the periorbital area, usually the eyelid. Its periocular location predisposes to smaller biopsies, thus complicating diagnosis. The neoplasm tends to exhibit aggressive local behavior, metastasizing to regional lymph nodes, and distant organs. Disease limited to the epithelium is rare, with less than 10 cases reported in the literature. We present the case of a 73-year-old woman who presented with left upper eyelid entropion and conjunctival erythema. Multiple punch biopsies showed an intraepithelial proliferation of atypical basaloid cells with enlarged and hyperchromatic nuclei intermingled with vacuolated cells containing bubbly cytoplasms and scalloped nuclei, consistent with a diagnosis of intraepithelial sebaceous carcinoma. After subsequent orbital exenteration and histological examination, a wide intraepithelial sebaceous component was identified; however, there was no invasive growth. This case highlights the rarity and the potential diagnostic pitfalls of intraepithelial sebaceous carcinoma.

Uncorrectable ptosis: primary cutaneous signet-ring cell carcinoma.

Primary cutaneous signet-ring cell carcinoma (PCSRCC) is a rare but aggressive tumor. Our case highlights a 60-year-old man who presented with eyelid ptosis, for which he underwent multiple surgical procedures over a 3-year period prior to referral to our clinic. These procedures were complicated by scarring, delayed healing, and poor cosmetic outcome. In addition, the patient was noted to develop progressive enophthalmos. These concerning signs led to a CT scan and subsequent eyelid biopsy, which revealed a diagnosis of PCSRCC. Further management has involved an MRI and orbitotomy with biopsy revealing widespread extension of the carcinoma. Exenteration was performed to reduce the likelihood of metastasis. There are few documented case reports of PCSRCC of the eyelid in the literature. Of the 33 published cases of PCSRCC, 27 cases involve the eyelids and the other 6 cases involve the axilla. The unique clinical features of this case will be discussed, in particular the presentation as ptosis, an otherwise commonplace complaint in the oculoplastics clinic. The surgical course and histopathologic findings will be presented. The literature regarding PCSRCC will be reviewed including demographics, management, and prognosis. Although rare, PCSRCC follows an aggressive course with characteristically delayed diagnosis. Early identification and treatment likely offer a better prognosis. Thus, description of the clinical presentation of this rare tumor may aid in recognition and earlier treatment.

Tufted angioma (Angioblastoma) of eyelid in adults-report of two cases.

Tufted angioma, first recognized in Japanese literature as "Angioblastoma of Nagakawa", is a rare benign vascular tumour with a variable clinical presentation. It commonly manifests as a macule, papule or nodule in infancy or childhood in the region of the upper trunk and neck. Here in we report two cases of this rare progressive angioma as lesions of the eyelid in adults. Tufted angioma has a classical "cannon ball" like appearance of vascular tufts on histopathology. Immunohistochemical staining with actin highlights the spindly stromal cells surrounding the capillaries. Complete physical examination and haematological work up is recommended in patients with tufted angioma to exclude rare association of port wine stain and Kasabach-Merritt syndrome with this rare entity. To the best of our knowledge, our cases illustrate the first case report of tufted angioma presenting as an eyelid lesion. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1230909536950947.

Eyelid angiosarcoma: a case report and review of the literature.

A 77-year-old woman presented with a 3-month history of a lesion on her left lower eyelid. External examination showed a tan-colored nodule with an overlying crust-covered ulcer on the left lower eyelid, nasally. The ulcer measured 12 mm × 7 mm. Complete surgical excision with a frozen section margin control was performed. Histopathological examination showed islands and sheets of spindle and epithelioid cells with little intervening stroma. The cells had copious amounts of either rounded or tapered eosinophilic cytoplasm with occasional intracytoplasmic lumina and large vesicular nuclei with prominent nucleoli. There was intense immunoreactivity for CD34, CD31, factor VIII, and Ki-67. The diagnosis was eyelid angiosarcoma. The patient refused any further therapy. At 1-year follow-up, there was no recurrence or development of metastasis. In conclusion, tan-colored eyelid nodules with overlying ulcer are usually a basal cell carcinoma; however, rarely it can be an eyelid angiosarcoma.