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PubMed1989

Optic nerve involvement in retinoblastoma.

This retrospective review of 814 retinoblastoma patients discloses 240 cases of tumor extension into the optic nerve. Optic nerve involvement was classified according to the degree of invasion; grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. The incidence of optic nerve involvement was 29.5% (240/814). The mortality rate of patients with optic nerve involvement with grade I was 10%; grade II, 29%; grade III, 42%; and grade IV, 78%. Actuarial life-table analysis shows an increased mortality rate with increasing grade of optic nerve involvement. Multivariate statistical analysis shows that the grade of optic nerve involvement and the age at diagnosis of optic nerve involvement are the factors most significantly associated with survival.

Child, Preschool↗
PubMed1990

Malignant melanoma associated with melanocytoma of the optic disc.

A 61-year-old white man underwent enucleation because of progressive growth of a pigmented epipapillary tumor that was diagnosed 9 years earlier as an optic nerve and juxtapapillary melanocytoma. Histopathologic studies showed the tumor was a malignant melanoma of the optic disc and juxtapapillary retina and choroid. Foci of typical melanocytoma cells were within the tumor. The tumor produced segmental atrophy of the optic nerve. This is a rare example of a malignant melanoma developing in conjunction with a lesion that possessed typical clinical and histopathologic features of a melanocytoma of the optic disc.

Cell Transformation, Neoplastic↗
PubMed2002

Palladium-103 plaque radiotherapy for choroidal melanoma: an 11-year study.

PURPOSE: To describe 11 years of experience with 103Pd ophthalmic plaque brachytherapy for intraocular melanoma. METHODS AND MATERIALS: Since 1990, 152 patients have been diagnosed with uveal melanoma, found to be negative for metastatic disease, and treated with 103Pd radioactive plaque radiotherapy. This study presents the first 100 patients treated with 103Pd and followed for > or = 2 years. Plaques were sewn to the episclera to cover the base of the intraocular tumor. Treatment involved delivery of a mean apical radiation dose of 80.5 Gy during 5-7 days' continuous treatment. Patients were evaluated for local tumor control, visual acuity, radiation damage (retinopathy, optic neuropathy, cataract), and metastatic disease. RESULTS: Patients in this series were followed for a mean of 4.6 years (55.4 months). 103Pd seeds were found to be equivalent to 125I with respect to plaque manufacture and ease of dosimetric calculations. We noted a local control rate of 96% and six secondary enucleations. Including the enucleated patients, the visual acuity evaluations revealed that 35% lost six or more lines of vision and 73% had vision of 20/200 or better. CONCLUSION: Long-term results now exist describing the use of 103Pd plaque radiotherapy for uveal (iris, ciliary body, and choroidal) melanoma. Compared with the results from centers using 125I, patients in this series experienced equivalent local control rates and better visual function.

Brachytherapy↗
PubMed1998

Particle beam therapy (hadrontherapy): basis for interest and clinical experience.

The particle or hadron beams deployed in radiotherapy (protons, neutrons and helium, carbon, oxygen and neon ions) have physical and radiobiological characteristics which differ from those of conventional radiotherapy beams (photons) and which offer a number of theoretical advantages over conventional radiotherapy. After briefly describing the properties of hadron beams in comparison to photons, this review discusses the indications for hadrontherapy and analyses accumulated experience on the use of this modality to treat mainly neoplastic lesions, as published by the relatively few hadrontherapy centres operating around the world. The analysis indicates that for selected patients and tumours (particularly uveal melanomas and base of skull/spinal chordomas and chondrosarcomas), hadrontherapy produces greater disease-free survival. The advantages of hadrontherapy are most promisingly realised when used in conjunction with modern patient positioning, radiation delivery and focusing techniques (e.g. on-line imaging, three-dimensional conformal radiotherapy) developed to improve the efficacy of photon therapy. Although the construction and running costs of hadrontherapy units are considerably greater than those of conventional facilities, a comprehensive analysis that considers all the costs, particularly those resulting from the failure of less effective conventional radiotherapy, might indicate that hadrontherapy could be cost effective. In conclusion, the growing interest in this form of treatment seems to be fully justified by the results obtained to date, although more efficacy and dosing studies are required.

Central Nervous System Neoplasms↗
PubMed2003

Topical alpha-interferon in recurrent conjunctival papilloma.

PURPOSE: To report the effect of topical alpha-interferon (alpha-IF) in two cases of recurrent conjunctival papillomas. METHODS: One patient with a conjunctival papilloma and one patient with a limbus-based papilloma were treated with excision and cryotherapy. Recurrences included one in situ carcinoma. Retreatment included systemic and topical alpha-IF. RESULTS: In Case 1, the papillomas regressed with systemic alpha-IF followed by topical application of alpha-IF (2.8 x 106 U) for 1 year against recurrence. In Case 2, the papillomas disappeared with topical alpha-IF over 1 year. No recurrences were seen during follow-up (84 and 91 months, respectively). The possible side effect of topical alpha-IF was superficial keratitis. Retrospective polymerase chain reaction tests for HPV were positive and showed type 6 in Case 1 and type 33 in Case 2. CONCLUSION: Topical alpha-IF can be used as adjuvant therapy in recurrent conjunctival papillomas.

Administration, Topical↗
PubMed2003

Limitations of the immunocytochemical detection of isolated tumor cells in frozen samples of bone marrow obtained from melanoma patients.

We report on a case of a 70-year-old woman with an ocular melanoma, which was diagnosed and treated 14 years ago. The patient was referred to the hospital with a suspected lymphoma. Cytological examination of bone marrow proved a marked infiltration with melanoma cells. Because detection of isolated tumor cells in the bone marrow of patients with various types of tumors was shown to be of prognostic significance and since current tumor-staging techniques are unable to detect single disseminated tumor cells or small aggregates of tumor cells, which might be the seed for subsequent metastatic relapse, we therefore evaluated the feasibility of immunocytochemical screening of bone marrow aspirates of 36 melanoma patients in different clinical stages using three monoclonal antibodies against melanoma-associated antigens in comparison with 43 non-melanoma control patients. Two of these antibodies (HMB45 and NKI-beteb) are directed against the melanoma antigen gp100/pmel17, whereas the third one (TA99) recognizes gp75/Tyrosinase-related protein 1 (TRP-1). None of the patients demonstrated a macroscopic bone marrow infiltration as was present in our patient with metastatic ocular melanoma. Seven (20.6%) of the 34 eligible melanoma patients presented with cells in the bone marrow positive for one or more of the above-mentioned melanosomal markers. Four of the positive patients were clinically free of tumors by the time of puncture, whereas the remaining 3 patients showed overt metastases in the subcutaneous fat (2 patients) and the brain (1 patient). On the other hand, 20 (66%) of the 29 patients with negative bone marrow findings also presented with clinical advanced disease with overt metastasis in the skin, lymph node, spleen, liver, lung, bone and brain. In conclusion, immunocytochemical screening of bone marrow samples is a feasible procedure that allows the detection of micrometastatic tumor cells in a subset of melanoma patients. Massive invasion of bone marrow with melanoma cells is a rare event even in far-advanced metastatic stages and no clear correlation between tumor load and bone marrow infiltration could be established.

Aged↗
PubMed1990

Peculiar ophthalmic proliferations.

Six unusual cases diagnosed by various scientific methods are described to highlight the role of the histopathologist in clinical ophthalmology. The first case shows the importance of fast tissue diagnosis to prevent possible radical treatment in a limbal pseudotumour. The second presentation is of a small conjunctival mass with orbital involvement, immunohistochemically a B-cell lymphoma and occurring after a recent testicular tumour. The third patient presented with the rarely described nodular hypersensitivity conjunctivitis (Splendore-Hoeppli reaction) and it is suggested that these ophthalmologically observed asymptomatic lesions are apparently clinically transient so reports may be few because of infrequent biopsy. Pan-uveal melanocytic tumours concurrent with thoracic or abdominal carcinoma are reviewed. DNA flow cytometry could, perhaps, assess those ocular lesions for malignancy. The fourteenth case in the literature of lacrimal sac melanoma and possibly the first by tear seedling is illustrated. Lastly, a paediatric orbital fibrous histiocytoma is a cautionary anecdote with successful outcome.

Adult↗
PubMed1991

The immunopathology of intraocular tumour rejection.

The immunopathology of intraocular tumour rejection was studied using two transplantable tumours in mice. UV5C25 fibrosarcoma and P91 mastocytoma express tumour specific antigens that elicit strong systemic T cell-mediated immune responses following intracameral transplantation. Although both tumours underwent immunological rejection, the immunopathological sequelae of tumour rejection differed markedly. P91 tumour rejection was accompanied by bulk ischaemic necrosis, microvascular damage, and extensive damage to innocent bystander cells. A large body of experimental evidence indicated that this ischaemic necrotic pattern of intraocular tumour rejection was the result of a delayed-type hypersensitivity (DTH) effector mechanism. By contrast, intraocular UV5C25 tumour rejection occurred by piecemeal necrosis and did not culminate in injury to juxtaposed normal host tissues. The results of in vitro and in vivo experiments strongly indicated a role for cytotoxic T lymphocyte (CTL) mediated mechanisms in the piecemeal necrotic pattern of tumour rejection. Collectively, the results indicated that although tumour bearing hosts possessed both CTL and DTH effector mechanisms, only one form of T cell-mediated immunity prevailed within the intraocular milieu.

Animals↗
PubMed1992

Choroid plexus papilloma. A new presentation of von Hippel-Lindau (VHL) disease.

A definite association between Von Hippel-Lindau [VHL] disease and choroid plexus tumour has not been described previously. A 24-year-old patient was found to have a choroid plexus tumour in the left cerebellopontine angle and involving the temporal bone. Examination of her fundi revealed bilateral retinal angiomatosis, thus making a diagnosis of von Hippel-Lindau disease. Later, an abdominal scan showed renal and pancreatic cysts. An important point is that molecular analysis of the choroid plexus tumour tissue showed chromosome 3 allele loss as described for other tumour types associated with von Hippel-Lindau disease.

Adult↗
PubMed2001

Clinical, histopathological, and immunogenetic analysis of ocular adnexal lymphoproliferative disorders: characterization of malt lymphoma and reactive lymphoid hyperplasia.

Malignant lymphomas and reactive lymphoid hyperplasia (RLH) in the ocular adnexa are sometimes difficult to differentiate morphologically and have often been categorized together as a lymphoproliferative disorder. Immunogenotypic characters of these diseases have not yet been well clarified. This study included 76 cases of ocular adnexal lymphoproliferative disorders. These consisted of 52 cases of malignant lymphoma (43 primary and 9 secondary), 22 of RLH, and 2 borderline cases. There were slightly more male than female subjects. Diagnoses were based on morphology and immunophenotypic characteristics. Clonalities were detected by means of polymerase chain reaction (PCR), and immunoglobulin heavy-chain variable region (VH) genes were sequenced in 10 cases of mucosa-associated lymphoid tissue (MALT) lymphoma. MALT lymphoma constituted 86% (37 cases) of the primary lymphomas. MALT lymphomas were more indolent, more rarely disseminated, and had a lower death rate than the other primary lymphomas. Two patients exhibited coexistence of MALT and diffuse large B-cell lymphoma. The average age of patients with RLH was 5.5 years younger than that of those with MALT lymphoma. One of the cases of RLH later progressed to malignant lymphoma. B-cell clonality was detected by PCR in 57%, 55%, and 0% of primary lymphomas, MALT lymphomas and RLHs, respectively. Sequencing of VH genes revealed that the VH3 family was the most commonly expressed germline VH family (70%) and that DP-63, DP-54 and DP-47 genes were frequently found in the MALT lymphomas examined. PCR analysis was useful for differentiation between MALT lymphoma and RLH. Sequence analysis of VH genes showed that an autoimmune mechanism may be involved in the lymphomagenesis of ocular adnexal MALT lymphoma.

Adult↗
PubMed2006

Imaging features of ocular adnexal lymphoproliferative disease.

PURPOSE: To evaluate the imaging characteristics of a cohort of patients with ocular adnexal lymphoproliferative disease (OALD). METHODS: A noncomparative retrospective review between 1992 and 1995 and prospective study from 1995 to 2005 of the clinical, imaging and treatment of 105 patients presenting to tertiary orbital referral centre presenting with OALD. RESULTS: One hundred and five patients (mean age 61 years, range 11-90 years) with equal gender distribution were included. Fifty-three were primary and 52 were secondary. Computed tomography (CT) usually showed a well-circumscribed lesion of greater than brain density, moulding to adjacent tissues with moderate enhancement. Aggressive histology was associated with bone destruction, while moulding was associated with indolent histology (P<0.005).MRI in OALD showed intermediate signal intensity on T1- and T2-weighted images and moderate enhancement with gadolinium. Gallium scanning sensitivity to detect ocular adnexal disease was 25 and 57% for systemic involvement. Positron emission tomography (PET) upstaged (71%) of patients with systemic lymphoproliferative involvement, having a higher sensitivity than CT in detecting distant disease (86 vs 72%). CONCLUSIONS: CT and/or MRI are essential in the evaluation of OALD and can be used to establish that an orbital lesion may be lymphoprolifetaive in nature. Further, these imaging modalities may predict the behaviour of the lymphoma in certain cases. Gallium scanning provides no additional information to CT and does not influence patient treatment. PET represents an important addition to the assessment of OALD with real impact on patient management.

Adolescent↗
PubMed1999

A case of prostatic cancer metastasizing to the orbit.

PURPOSE: We report on a case of prostatic cancer with multiple bone metastases, including right orbital involvement. METHODS/RESULTS: Hormonal treatment and local irradiation to the right orbit were performed. Subjective symptoms with exophthalmos and pain disappeared. CONCLUSIONS: We report on a case of prostatic cancer metastasizing to the right orbital bone.

Adenocarcinoma↗
PubMed2002

Chemoembolization for hepatic metastases.

Liver metastases are one of the most difficult therapeutic challenges in modern oncologic practice. Few systemic therapies are effective for solid tumors in the liver. Resection is possible in a minority of patients and usually not curative. Image-guided therapies such as chemoembolization offer substantial intensification of treatment by combining tumor ischemia, increased drug concentrations, prolonged dwell time, and reduced systemic toxicity. Although not a curative therapy, durable responses are seen in most adenocarcinomas, which may contribute to prolongation and preservation of quality of life by delaying liver failure in patients with liver-dominant disease.

Adenocarcinoma↗
PubMed2006

Reconstruction of large orbital exenteration defects after resection of periorbital tumors of advanced stage.

Orbital exenteration is a devastating procedure because of the social impact and psychological stress put upon the patient. Besides cancer ablative surgery, reconstruction after removal of the tumor constitutes a major problem and the final aesthetic result is quite important. Both obliteration of the orbital cavity and continuation of the epithelial lining are required. Free rectus abdominis muscle and musculocutaneous flaps are versatile flaps which both enable filling the cavity and reconstituting the skin defect with a cutaneous portion or with a skin graft. Both free rectus abdominis muscle and musculocutaneous flaps were used for reconstruction of orbital exenteration defects in 19 patients. All suffered partial maxillectomy as well. The flaps provided satisfactory aesthetic results in all patients.

Adult↗