Ocular PEComas are frequently melanotic and TFE3-translocated: report of two cases including the first description of PRCC-TFE3 fusion in PEComa.

Ocular perivascular epithelioid cell tumor (PEComa) is exceedingly rare. We reported two examples involving the choroid and subconjunctival tissue, respectively, in patients aged 17 and 20 years. Both tumors comprised packets and sheets of large polygonal cells with moderately pleomorphic nuclei and prominent nucleoli, traversed by delicate fibrovascular septa. Melanin pigmentation was present in one case. The tumors showed HMB45 and TFE3 immunoreactivity. TFE3 gene translocation was confirmed by FISH break-apart probes. RNA seq revealed PRCC-TFE3 and NONO-TFE3 fusions, with the former representing the first description of PRCC-TFE3 in PEComa. Critical reappraisal of the reported cases showed that ocular PEComa frequently affected young patents with melanin pigmentation, frequent TFE3 protein expression, and/or TFE3 gene translocation. No recurrence or metastasis was reported after complete excision despite the presence of cytologic atypia.

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa.

Peripheral nerve sheath tumors (PNSTs) are known to occur in the orbit and comprise 4% of all orbital tumors, but have not been well studied in contemporary literature. Ninety specimens involving the eye and ocular adnexa (1979-2015) from 67 patients were studied. The mean age was 32.5years. Locations included orbit (58.9%), eyelid (60.0%), and other ocular adnexa. Most specimens were neurofibromas (70.0%), followed by schwannomas (11.1%), neuromas (11.1%), granular cell tumors (n=4), nerve sheath myxomas (n=2), and malignant PNST (n=1). Fifty-six (88.9%) neurofibroma cases were neurofibromatosis 1 associated. Among neurofibromas, 31.7% were localized, 38.1% were plexiform, 25.4% were diffuse, and 4.8% were diffuse and plexiform. These tumors involved skin (31.7%), soft tissue (11.1%), skeletal muscle (22.2%), peripheral nerve (63.0%), lacrimal gland (20.6%), and choroid (n=1). Other histologic findings included pseudo-Meissner corpuscles (27%), Schwann cell nodules (4.8%), prominent myxoid component (7.9%), melanin-like pigment (3.2%), and inflammation (14.3%). Available immunostains included S100 (+ in 15/15 cases), EMA (+ in 2/4 cases), CD34 (+ in 4/4 cases), and Ki-67 (<1% in 4/4 cases). Among 10 schwannomas, 8 were conventional and 2 were plexiform. Observed features included capsule (n=5), hyalinized vessels (n=5), Verocay bodies (n=7), and Antoni B pattern (n=5). Immunostaining included S100+ in 4 of 4 cases, and collagen IV+ and Ki-67 <1% in 3 of 3 cases. Neurofibromas are the most common PNST involving the eye and ocular adnexa, and the majority are associated with neurofibromatosis 1. Plexiform and diffuse patterns and the presence of pseudo-Meissner corpuscles are relatively frequent in this area.

Primary bilateral corneal nerve sheath neoplasm in a dog.

A 15-year-old, neutered male, Shih Tzu cross developed progressive corneal stromal thickening and vascularization of the right eye, and 5 months later, of the left eye. Both eyes became blind due to extensive corneal opacification and were enucleated. Light microscopic examination revealed a diffuse corneal infiltrate of neoplastic mesenchymal cells, and immunohistochemistry revealed diffuse cytoplasmic vimentin immunoreactivity and variable cytoplasmic and nuclear immunoreactivity for S100 in the neoplastic cells. Transmission electron microscopy revealed desmosomes between contiguous cells, thread-like cytoplasmic processes coated with basement membrane, extracellular bundles of collagen, and axonal degeneration consistent with features of a nerve sheath neoplasm. This is the first report of primary, bilateral corneal nerve sheath sarcoma in a canine.

Molecular diagnosis in patients with retinoblastoma: Report of a series of cases. / Diagnóstico molecular en pacientes con retinoblastoma: reporte de una serie de casos.

OBJECTIVES: To report the benefits of genetic diagnosis in patients with retinoblastoma. METHOD: Observational study. Patients with retinoblastoma and their families were included. Demographic and clinical data were recorded. Blood and tumour samples were obtained. Next generation sequencing was performed on the samples. When deletion 13 q syndrome was suspected, cytogenetics microarray was performed (Cytoscan® HD, Affymetrix, Santa Clara, CA, USA), with a high density chip of 1.9 million of non-polymorphic probes and 750 thousand SNP probes. RESULTS: Of the 7 cases were analysed 4 were male. The mean age at diagnosis was 21 months (range 5-36). Three cases had bilateral retinoblastoma, and 4 unilateral. None had family history. In all patients, blood was analysed, and a study was performed on the tissue from 2 unilateral enucleated tumours, in which 6 mutations were identified, all de novo. Just one was novel (c.164delC; case 1). One case of unilateral tumour revealed blood mosaicism, showing that his condition was inheritable, and that there is a high risk of developing retinoblastoma in the unaffected eye. The patient also has an increased risk of presenting with other primary tumours. CONCLUSION: Molecular diagnosis of RB1 in patients with retinoblastoma impacts on the decision process, costs, treatment, and prognosis of patients, as well as their families.

Genetic analysis in retinoblastoma and peripheral blood correlation.

OBJECTIVE: To determine the importance of intratumoral genetic analysis in the diagnosis of germ-line mutations in patients with retinoblastoma. To underline the importance of performing these genetic tests in every case of retinoblastoma. METHOD: Intratumoral genetic analysis of RB1 mutation was performed on 17 enucleated eyes that were non-responsive to conservative treatment. Patients had no family history of retinoblastoma, and lesions were always single. The identified mutations were then also studied in peripheral blood analysis. RESULTS: There were 12 (70.6%) cases with positive results in intratumoral analysis. In 8 cases (47.1%) mutation of both RB1 alelli were detected, and in 4 (23.5%) cases only one allele was found mutated. In 5 patients (29.4%) no mutation was identified. In the first hit, mutations comprised 7 frameshift or nonsense and 2 splice, whereas in the second hit, one splice mutation, 2 nonsense and 8 loss of heterozygosity were identified. Among 6 patients where intratumoral analysis detected a single mutation associated with a loss of heterozygosity, the peripheral blood analysis was able to detect the same mutation in 3 cases (50%). CONCLUSIONS: Intratumoral genetic analysis of sporadic retinoblastoma can detect germ-line mutations. These patients are at higher risk of bilateralization and development of second tumors or trilateral retinoblastoma. Genetic screening is recommended in every patient diagnosed with retinoblastoma.

HOW EXPRESSIONS OF CLAUDIN-1 AND MMP-2 IN RETINOBLASTOMA CORRELATE WITH HISTOLOGICAL DIFFERENTIATION AND OPTIC NERVE INVASION.

Retinoblastoma is a commonly seen and dangerous intraocular malignant tumor in infants. Studies have found that Claudin-1 and MMP-2, whose expressions may be connected, play roles in tissues of retinoblastoma. In this study we analyze and discuss changes of Claudin-1 and MMP-2 expressions, and the correlation between the expressions and retinoblastoma histological differentiation and optic nerve invasion. MaxVisionTM was applied to detect expressions of Claudin-1 and MMP-2 in 45 samples of retinoblastoma and 15 paraffin-embedded samples of normal retina. The correlation between Claudin-1 expression and MMP-2 expression was analyzed based on chi-squared test and Spearman’s correlation test. Positive expressions of Claudin-1 in retinoblastoma were fewer than those in retina; higher positive expressions were found in differentiated tissues than in undifferentiated tissues; while compared to expressions in invasive optic nerves, Claudin-1 expressed more positively in optic nerves without invasion. As for MMP-2, its expressions were higher in retinoblastoma than in normal retina; undifferentiated tissues had higher positive expressions than differentiated tissues, which were not statistically significant; higher positive expressions were detected in invasive optic nerves. Thus, it could be concluded that the correlation between Claudin-1 expression and MMP-2 expression in retinoblastoma was negative. Expressions of Claudin-1 were positively related to histological differentiation and optic nerve invasion of retinoblastoma; while MMp-2 expression had negative correlation with histological differentiation and optic nerve invasion of retinoblastoma. Claudin-1 and MMP-2 played a negative role in the optic nerve invasion and tumor development of retinoblastoma.

Detection of MYB Alterations and Other Immunohistochemical Markers in Primary Cutaneous Adenoid Cystic Carcinoma.

Adenoid cystic carcinoma (ACC) can arise in several organs, and prognosis is highly dependent on the primary tumor site. Primary cutaneous ACC has an excellent prognosis compared with salivary or lacrimal ACC. Activation of MYB by gene fusion or other mechanisms has been found in salivary, breast, and lacrimal ACCs but has not been described in cutaneous ACC. We analyzed the histopathologic and immunohistochemical features of 19 primary cutaneous ACCs, 2 periorbital ACCs, and 12 salivary gland ACCs and assessed for MYB activation in primary cutaneous ACC by immunohistochemistry and molecular methods. The presence of perineural invasion differed significantly among ACCs of various sites (83% salivary, 50% eyelid, 11% skin, P=0.0002). Over 90% of all ACCs were grade 1 or 2 and exhibited diffuse (>50%) positivity with CD117, SOX-10, and smooth muscle actin immunostains. CK15 and vimentin showed diffuse positivity in 36% and 57% of cutaneous ACCs, respectively, and were negative or only focally positive in all salivary ACCs (P=0.04 and 0.002). Six of the 11 cutaneous and periorbital ACCs tested with reverse transcriptase polymerase chain reaction and/or fluorescence in situ hybridization had MYB rearrangements including 2 cases that expressed MYB-NFIB fusion transcripts. Diffuse expression of MYB protein assessed by immunostaining was present in 8 of 9 cutaneous ACCs, including cases both with and without MYB rearrangements. These results indicate that cutaneous ACCs possess the same types of MYB alterations as ACCs of other anatomic sites. Vimentin and CK15 appear to have some discriminatory value in differentiating between primary cutaneous and salivary gland ACCs.

Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma.

Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. We are reporting 7 cases of an unusual clinical variant of HVL with primarily periorbital edema. All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. Most cases were initially misinterpreted as inflammatory processes including cellulitis, arthropod bite reactions, and periorbital lupus erythematosus. The biopsy of these lesions revealed an atypical lymphocytic infiltrate predominantly distributed in the deep dermis and in subcutaneous fat. Immunohistochemistry studies revealed a cytotoxic T-cell (CD8) profile. All cases were associated with Epstein-Barr virus infection. Our study presents a rare clinical variant of HVL with predominant periorbital edema. This variant could potentially be overlooked and misdiagnosed as an inflammatory condition; thus, it needs to be included in the differential diagnosis of periorbital edema in young patients.

Extranodal marginal zone lymphoma from ocular adnexae with subcutaneous involvement.

Extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue usually originates from cutaneous or mucosal surfaces. A rare site of involvement is the subcutaneous tissue of any location. Here, we describe a 58-year-old man who presented with bilateral extranodal MZL of mucosa-associated lymphoid tissue from ocular adnexae that involved subcutaneous tissue and subsequently extended to multiple anatomical locations in the head and neck, upper back, and arm. The neoplastic cells expressed B-cell markers, and the plasma cells expressed IgG4. The unusual pattern of infiltration of this extranodal MZL and the possible significance of IgG4 expression in this case are discussed.

A skin lesion that catches the eye.

Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is rare and only represents 1% of all cutaneous T-cell lymphomas. To our knowledge, only 40 cases have been described. It often presents with generalised skin lesions, preferentially affecting the extremities. There is a well-documented association with haemophagocytic syndrome. Treatment is difficult since PCGD-TCL is often resistant to chemotherapy and radiotherapy. Most case reports describe an aggressive clinical course with an estimated mean survival of 15 months. We present a 72-year-old female patient with stage IV primary cutaneous gamma-delta T-cell lymphoma. Our patient presented with fever, night sweats and multiple skin lesions (figure 1). Computed axial tomography of chest and abdomen revealed multiple solid nodular lesions in both kidneys. During admission a subconjunctival lesion appeared and progressed rapidly (figure 2). Histopathological examination of skin biopsy revealed infiltration of atypical lymphocytes with hyperchromatic irregular nuclei. Immunophenotyping pattern of skin biopsy was compatible with PCGD-TLC. Clonal gamma-delta T-cells were also detected by immunohistochemical analysis of peripheral blood and bone marrow. Polymerase chain reaction amplification revealed clonal rearrangement of the T-cell receptor gamma chain gene. These findings together were consistent with stage IV primary cutaneous gamma-delta T-cell lymphoma. The rapid progression of the subconjunctival extra-nodal manifestation is characteristic for the aggressive course of this lymphoma. Our patient was treated with two cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone). However, her clinical condition deteriorated rapidly. She declined further therapy and died within three months of initial presentation.

Primary ductal adenocarcinoma of the lacrimal sac: the first reported case.

Primary lacrimal sac tumor is extremely rare, and moreover, glandular tumor is exceptional. Herein, we described the first documented case of primary ductal adenocarcinoma of the lacrimal sac. A 79-year-old Japanese female presented with persistent swelling of her left lower eyelid. Computed tomography demonstrated an irregular-shaped tumor involving the left lacrimal sac, lower eyelid, sinonasal tract, and internal side of the left orbit. Biopsy from the eyelid revealed a poorly differentiated adenocarcinoma. Histopathological study of the resected lacrimal sac tumor revealed an infiltrative neoplastic growth that was composed of cribriform structures with comedonecrosis. The neoplastic cells had relatively rich granular eosinophilic cytoplasm and large round to oval nuclei containing conspicuous nucleoli. The left cervical lymph nodes had metastatic carcinoma. Immunohistochemically, the neoplastic cells were diffusely positive for gross cystic disease fluid protein-15 and androgen receptor. Moreover, mammalian target of rapamycin (mTOR), 4E-BP1, and p4E-BP1 were expressed. According to these results, an ultimate diagnosis of primary ductal adenocarcinoma of the lacrimal sac was made. Only 9 cases of primary lacrimal sac adenocarcinoma have been reported, and this is the first reported case of ductal adenocarcinoma of the lacrimal sac. Ductal adenocarcinoma of the salivary gland shows an aggressive clinical course, and the present case had multiple cervical lymph node metastases. This report is the first to demonstrate that mTOR pathway proteins, which are central proteins involved in carcinogenesis, are activated in ductal adenocarcinoma. Therefore, mTOR inhibitor may be a potential candidate for treatment of this highly aggressive carcinoma.

Vitreoretinal presentation of secondary large B-cell lymphoma in patients with systemic lymphoma.

IMPORTANCE: Intraocular lymphomas represent a diverse group of hematologic malignant neoplasms involving different tissues within the eye. Predominant involvement of the retina and vitreous without uveal infiltration in systemic lymphoma, mimicking a primary vitreoretinal lymphoma, is extremely rare. Our study emphasizes the importance of systemic evaluation in addition to central nervous system evaluation in all patients with newly diagnosed vitreoretinal lymphoma. OBJECTIVE: To determine the incidence of secondary intraocular lymphoma presenting as vitreoretinal infiltrates without central nervous system involvement, mimicking primary vitreoretinal lymphoma in patients with systemic lymphoma. DESIGN Retrospective review of all vitreous aspiration biopsy samples acquired because malignant neoplasm was suspected clinically that were obtained at our institution from January 1, 2000, through December 31, 2010. Review included patient clinical history, radiographic study findings, cytologic and/or histologic preparations, immunophenotypic study findings, treatment, and outcomes. SETTING Mayo Clinic pathology database. PARTICIPANTS: Fifty-five patients with vitreous specimens available for review. EXPOSURES: Vitreous aspiration biopsy. MAIN OUTCOME AND MEASURE: Confirmation of the diagnosis of diffuse large B-cell lymphoma (DLBCL). RESULTS: Of the 55 patients with vitreous specimens available for review, 3 (5%) had a DLBCL infiltration in the vitreous without any central nervous system involvement as a manifestation of systemic lymphoma. All 3 patients were men, aged 54, 66, and 73 years, and had blurred vision and floaters for several weeks before undergoing diagnostic vitrectomy. Ophthalmic examinations revealed clumps of vitreous cells but no choroidal involvement. One patient had no history of lymphoma; the diagnosis of vitreoretinal lymphoma was followed by DLBCL after a lymph node biopsy. The other 2 patients had low-grade B-cell lymphoma and chronic lymphocytic leukemia for 29 and 7 months before large-cell transformation in the eye. Patients were treated with systemic chemotherapy plus intraocular injections of rituximab or methotrexate sodium. CONCLUSIONS AND RELEVANCE: Vitreoretinal symptoms of DLBCL in patients with systemic lymphoma may be more frequent than previously thought (5% in our study). Not all lymphomas with vitreoretinal involvement represent primary intraocular lymphomas; thorough ophthalmologic evaluation in patients with visual symptoms and complete staging in patients with documented ocular lymphoma are of utmost importance.

Epitheloid hemangioendothelioma of the palpebral lobe of the lacrimal gland.

PURPOSE: To describe a case of an epitheloid hemangioendothelioma which is a tumor of endothelial origin with borderline malignancy between hemangioma and angiosarcoma and which is very rare in the orbit. METHODS: Clinical features, results of imaging and histopathological studies and postoperative clinical course of a 22-year-old female patient who presented with a gradual swelling of the left upper eyelid were reviewed. RESULTS: Magnetic resonance imaging studies suggested a solid lesion with moderate contrast enhancement in the palpebral lobe of the lacrimal gland. A short course of systemic corticosteroid therapy failed to resolve the lesion. Excisional biopsy of the tumor yielded the diagnosis of epitheloid hemangioendothelioma of the lacrimal gland. The tumor was composed of nests of epitheloid cells, some forming CD31 positive intracytoplasmic vascular channels containing erythrocytes. CD34 and EMA were also positive and desmin, SMA, p63, S100, Factor VIII and HHV-8 were negative on immunohistochemical studies. During 44 months of follow-up, there has been no recurrence or systemic metastasis. CONCLUSION: Epitheloid hemangioendothelioma can occur in the palpebral lobe of the lacrimal gland as a hard, painless, immobile mass. Simple excisional biopsy of the tumor, assumed to have an intermediate malignancy grade, without further local or systemic therapy provided a disease-free 3-year survival.

Isolated diffuse episcleral neurofibroma.

AIMS: To report a rare case of isolated diffuse episcleral neurofibroma and to discuss possible differential diagnoses. CASE REPORT: A 37 year old Caucasian female was found to have an epibulbar tumour arising from the superior aspect of her left eye. Clinical examination revealed a 12 mm × 8 mm "salmon pink" coloured lesion. RESULTS: A biopsy was performed and histopathologic examination and subsequent systemic evaluation showed it to be a rare case of isolated diffuse episcleral neurofibroma. CONCLUSION: There are many differential diagnoses for an epibulbar lesion and neurofibroma should be added to these. Even though a small risk of malignant transformation exists, we recommend a conservative approach for slow growing lesions.

Diffuse large B-cell lymphoma of the ocular adnexal region: a nation-based study.

PURPOSE: To characterize the clinicopathological features of diffuse large B-cell lymphoma (DLBCL) of the ocular adnexal region. METHODS: The present series of orbital and adnexal DLBCLs were found by searching the Danish Registry of Pathology between 1980 and 2009. Histological specimens were re-evaluated using a panel of monoclonal antibodies. Clinical files from all patients with confirmed DLBCL were collected. RESULTS: A total of 34 patients with DLBCL of the ocular adnexal region were identified. Eighteen of the patients were men. The patients had a median age of 78 years (range 35-97 years). Ninety-seven per cent of the patients had unilateral ocular adnexal region involvement, and the orbit (76%) was the most frequently affected site. Nineteen patients (56%) presented with Stage I lymphoma. Of these, 18 were diagnosed with primary lymphoma. Four patients (12%) had Stage II, one patient (3%) had Stage III and ten patients (29%) presented with Stage IV lymphoma. The 5-year overall survival (OS) rate for the whole study group was 20%. The patients with Stage I lymphoma had a significantly better 5-year OS rate (28%) than patients in Stage II-IV (5-year OS rate, 9%). In Cox regression analysis, concordant bone marrow involvement and the International Prognostic Index (IPI) score were prognostic factors for OS. CONCLUSIONS: Diffuse large B-cell lymphoma of the ocular adnexal region is mainly prevalent in elderly patients. Most patients had unilateral orbital involvement. The overall prognosis is poor. Concordant bone marrow involvement and the IPI score were independent prognostic factors for mortality.

Basal cell cystadenoma of the lacrimal gland: diagnostic pitfalls of a basaloid pattern in lacrimal tumours.

An 85-year-old male experienced a painless swelling along the left lateral orbit for one year. A computed tomography scan demonstrated a cystic mass in the orbit adjacent to the lacrimal gland. There was a concern for malignancy considering the large size and the patient's age, so the tumour was excised. Histopathology of the tumour showed nests with basaloid patterns, but a definitive diagnosis was not rendered. The uncertainty of tissue diagnosis coupled with the basaloid pattern, which carries a grim prognosis in some salivary gland tumours, led us to refer this case to an authority on lacrimal gland pathology, who suggested that this tumour be called a basal cell cystadenoma. To the best of our knowledge, a basal cell cystadenoma of the lacrimal gland has not been reported in the literature. We present histopathological features that distinguish this tumour from malignant tumours with a basaloid pattern. We also discuss the management differences associated with basaloid patterns in lacrimal tumours.

Evidence for a stem-cell lineage in corneal squamous cell carcinoma using synchrotron-based Fourier-transform infrared microspectroscopy and multivariate analysis.

The cornea is one of the few human tissues where the in situ locations of stem cells (SCs), transient-amplifying (TA) cells and terminally-differentiated (TD) cells have been relatively well localised and characterised. Mid-infrared (IR) (4000-400 cm(-1)) is absorbed by biological molecules and facilitates the acquisition in the biochemical-cell fingerprint region (1800-900 cm(-1)) of spectra representative of structure and function. Human cornea derived from normal or squamous cell carcinoma (SCC) samples were acquired, cryosectioned (10 µm), floated onto BaF(2) windows and interrogated using synchrotron-based radiation (SRS) Fourier-transform IR (FTIR) microspectroscopy. Spectra were analysed using principal component analysis (PCA) with or without linear discriminant analysis (LDA) to allow cluster analysis of the cell categories. A clear cell lineage emanating from SCs to TA cells to TD cells was noted in normal samples. Within the SCC samples, a small sub-population of the cell-derived spectra pointed to a SC-like phenotype with the vast majority pointing to a TA cell-like character; these cells would tend to be the most proliferative within a tissue. Our findings suggest that SRS FTIR microspectroscopy has the potential to identify and characterise cancer SCs.

Recurrent blue nevus of the corneoscleral limbus.

PURPOSE: To describe an unusual corneoscleral blue nevus. METHODS: Review of clinical and histopathological characteristics and previous relevant literature supplemented with immunohistochemical studies. RESULTS: A 13-year-old Pakistani girl presented with a 5 x 2-mm, superotemporal, nonmovable, recurrent, black, limbal nodule. The lesion had been incompletely removed two and a half years earlier. Nonfascicular, variably pigmented, banal, spindle melanocytes with bland nuclei were discovered histopathologically in the conjunctival substantia propria and superficial sclera in the original and recurrent specimens. HMB-45+ and MART-1+ immunostainings established that all the variably pigmented spindle cells were melanocytes. Dispersed plump melanophages displayed clumped melanin granules and were shown to have small nuclei after decolorization. The Ki-67 proliferation index was very low (0.05%), supporting a benign diagnosis. No residual pigmentation after excision and cryotherapy was observed in follow-up examinations during a year and a half. CONCLUSIONS: Careful histopathological and immunohistochemical evaluation of recurrent, incompletely excised, blue nevi can determine if they remain benign. Adjunctive cryotherapy was performed to prevent further recurrence, based on results from published data.

Schwannomas of the lacrimal gland fossa.

PURPOSE: Schwannomas are rare benign nerve sheath tumours that account for 1% of orbital tumours and, although sometimes associated with neurofibromatosis, most occur as isolated lesions and arise from branches of the ciliary or trigeminal nerves. METHODS: Two rare cases of Schwannoma arising within the lacrimal gland fossa are presented, these tumours being managed with intact surgical excision. RESULTS: A 66-year-old Caucasian female was referred with 6 months of progressive left painless proptosis and intermittent diplopia. Restriction of left upgaze was associated with 3 mm relative proptosis. On a presumptive preoperative diagnosis of pleomorphic adenoma, the patient underwent a bone-swinging lateral orbitotomy with intact excision of a moderately-firm, lobulated lesion which proved to be a Schwannoma. A 30-year-old White female was referred with a 4-year history of slowly progressive right supratemporal periocular lump. There was 2 mm of right relative proptosis and slight restriction of right extraocular movements. Although lacrimal gland remnants were present at the edge of both specimens, at surgery it was evident that the tumours completely occupied the lacrimal gland fossa and any normal orbital lobe of the lacrimal gland had undergone atrophy. CONCLUSIONS: Two very rare cases of Schwannoma arising within the lacrimal gland fossa are presented, the tumours causing complete atrophy of the normal orbital lobe of the lacrimal gland.