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1. Diagnostic issues faced by a rare disease healthcare network during Covid-19 outbreak: data from the Campania Rare Disease Registry

Author

Limongelli G, Iucolano S, Monda E, Elefante P, De Stasio C, Lubrano I, Caiazza M, Mazzella M, Fimiani F, Galdo M, De Marchi G, Esposito M, Rubino M, Cirillo A, Fusco A, Esposito A, Trama U, Esposito S, Scarano G, Sepe J, Andria G, Orlando V, Menditto E, Chiodini P, Campania Rare Disease, Iolascon A, Franzese A, Sanduzzi Zamparelli A, Tessitore A, Romano A, Venosa A, Nunzia Olivieri A, Bianco A, La Manna A, Cerbone AM, Spasiano A, Agnese Stanziola A, Colao A, De Bellis A, Gambale A, Toriello A, Tufano A, Ciampa A, Maria Risitano A, Pisani A, Russo A, Volpe A, De Martino B, Amato B, De Fusco C, Piscopo C, Selleri C, Tucci C, Pignata C, Cioffi D, Melis D, Pasquali D, De Brasi D, Spitaleri D, Russo D, Martellotta D, De Michele E, Varricchio E, Miraglia Del Giudice E, Coscioni E, Cimino E, Pane F, Tranfa F, Pollio F, Lonardo F, Nuzzi F, Simonelli F, Trojsi F, Habetswallner F, Valentini G, Cerbone G, Parenti G, Tedeschi G, Capasso G, Battista Rossi G, Gaglione G, Sarnelli G, Argenziano G, Bellastella G, De Michele G, Fiorentino G, Spadaro G, Scala I, Santoro L, Zeppa L, Auricchio L, Elio Adinolfi L, Alessio M, Amitrano M, Savanelli MC, Russo MG, Ferrucci MG, Carbone MT, Pellecchia MT, Salerno M, Melone M, Del Donno M, Vitale M, Triggiani M, Della Monica M, Lo Presti M, Tenuta M, Mignogna MD, Schiavulli M, Zacchia M, Brunetti-Pierri N, Iovino P, Moscato P, Iandoli R, Scarpa R, Russo R, Troisi S, Sbordone S, Perrotta S, Fecarotta S, Sampaolo S, Cicalese V., Limongelli, Giuseppe, Iucolano, Stefano, Monda, Emanuele, Elefante, Pasquale, De Stasio, Chiara, Lubrano, Imma, Caiazza, Martina, Mazzella, Marialuisa, Fimiani, Fabio, Galdo, Maria, De Marchi, Giulia, Esposito, Martina, Rubino, Marta, Cirillo, Annapaola, Fusco, Adelaide, Esposito, Augusto, Trama, Ugo, Esposito, Salvatore, Scarano, Gioacchino, Sepe, Joseph, Andria, Generoso, Orlando, Valentina, Menditto, Enrica, Chiodini, Paolo, Iolascon, Achille, Franzese, Adriana, Sanduzzi Zamparelli, Alessandro, Tessitore, Alessandro, Romano, Alfonso, Venosa, Alfredo, Nunzia Olivieri, Alma, Bianco, Andrea, La Manna, Angela, Cerbone, Anna Maria, Spasiano, Anna, Agnese Stanziola, Anna, Colao, Annamaria, De Bellis, Annamaria, Gambale, Antonella, Toriello, Antonella, Tufano, Antonella, Ciampa, Antonio, Maria Risitano, Antonio, Pisani, Antonio, Russo, Antonio, Volpe, Antonio, De Martino, Bernardo, Amato, Bruno, De Fusco, Carmela, Piscopo, Carmelo, Selleri, Carmine, Tucci, Celeste, Pignata, Claudio, Cioffi, Daniela, Melis, Daniela, Pasquali, Daniela, De Brasi, Daniele, Spitaleri, Daniele, De Brasi, Davide, Russo, Domenico, Martellotta, Donata, De Michele, Elisa, Varricchio, Elziario, Miraglia Del Giudice, Emanuele, Coscioni, Enrico, Cimino, Ernesto, Pane, Fabrizio, Tranfa, Fausto, Pollio, Filiberto, Lonardo, Fortunato, Nuzzi, Francesca, Simonelli, Francesca, Trojsi, Francesca, Habetswallner, Francesco, Valentini, Gabriele, Cerbone, Gaetana, Parenti, Giancarlo, Tedeschi, Gioacchino, Capasso, Giovambattista, Battista Rossi, Giovanni, Gaglione, Giovanni, Sarnelli, Giovanni, Argenziano, Giuseppe, Bellastella, Giuseppe, De Michele, Giuseppe, Fiorentino, Giuseppe, Spadaro, Giuseppe, Scala, Iri, Santoro, Lucio, Zeppa, Lucio, Auricchio, Luigi, Elio Adinolfi, Luigi, Alessio, Maria, Amitrano, Maria, Savanelli, Maria Cristina, Russo, Maria Giovanna, Ferrucci, Maria Grazia, Carbone, Maria Teresa, Pellecchia, Maria Teresa, Salerno, Mariacarolina, Melone, Marina, Del Donno, Mario, Vitale, Mario, Triggiani, Massimo, Della Monica, Matteo, Lo Presti, Maurizio, Tenuta, Maurizio, Mignogna, Michele Davide, Schiavulli, Michele, Zacchia, Miriam, Brunetti-Pierri, Nicola, Iovino, Paola, Moscato, Paolo, Iandoli, Raffaele, Scarpa, Raffaele, Russo, Romualdo, Troisi, Salvatore, Sbordone, Sandro, Perrotta, Silverio, Fecarotta, Simona, Sampaolo, Simone, Cicalese, Virgilio, Limongelli, G, Iucolano, S, Monda, E, Elefante, P, De Stasio, C, Lubrano, I, Caiazza, M, Mazzella, M, Fimiani, F, Galdo, M, De Marchi, G, Esposito, M, Rubino, M, Cirillo, A, Fusco, A, Esposito, A, Trama, U, Esposito, S, Scarano, G, Sepe, J, Andria, G, Orlando, V, Menditto, E, Chiodini, P, Campania Rare, Disease, Iolascon, A, Franzese, A, Sanduzzi Zamparelli, A, Tessitore, A, Romano, A, Venosa, A, Nunzia Olivieri, A, Bianco, A, La Manna, A, Cerbone, Am, Spasiano, A, Agnese Stanziola, A, Colao, A, De Bellis, A, Gambale, A, Toriello, A, Tufano, A, Ciampa, A, Maria Risitano, A, Pisani, A, Russo, A, Volpe, A, De Martino, B, Amato, B, De Fusco, C, Piscopo, C, Selleri, C, Tucci, C, Pignata, C, Cioffi, D, Melis, D, Pasquali, D, De Brasi, D, Spitaleri, D, Russo, D, Martellotta, D, De Michele, E, Varricchio, E, Miraglia Del Giudice, E, Coscioni, E, Cimino, E, Pane, F, Tranfa, F, Pollio, F, Lonardo, F, Nuzzi, F, Simonelli, F, Trojsi, F, Habetswallner, F, Valentini, G, Cerbone, G, Parenti, G, Tedeschi, G, Capasso, G, Battista Rossi, G, Gaglione, G, Sarnelli, G, Argenziano, G, Bellastella, G, De Michele, G, Fiorentino, G, Spadaro, G, Scala, I, Santoro, L, Zeppa, L, Auricchio, L, Elio Adinolfi, L, Alessio, M, Amitrano, M, Savanelli, Mc, Russo, Mg, Ferrucci, Mg, Carbone, Mt, Pellecchia, Mt, Salerno, M, Melone, M, Del Donno, M, Vitale, M, Triggiani, M, Della Monica, M, Lo Presti, M, Tenuta, M, Mignogna, Md, Schiavulli, M, Zacchia, M, Brunetti-Pierri, N, Iovino, P, Moscato, P, Iandoli, R, Scarpa, R, Russo, R, Troisi, S, Sbordone, S, Perrotta, S, Fecarotta, S, Sampaolo, S, and Cicalese, V.

Subjects
Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Home therapy, rare diseases, 030204 cardiovascular system & hematology, Campania region, Covid-19, Italy, patient registry, Disease Outbreaks, 03 medical and health sciences, COVID-19 Testing, 0302 clinical medicine, Pandemic, Health care, medicine, Retrospective analysis, Humans, AcademicSubjects/MED00860, Registries, 030212 general & internal medicine, Medical diagnosis, Pandemics, Retrospective Studies, business.industry, Public Health, Environmental and Occupational Health, Outbreak, General Medicine, Original Article, business, Delivery of Health Care, Rare disease
Abstract

Background The aims of this study were: to investigate the capacity of the rare disease healthcare network in Campania to diagnose patients with rare diseases during the outbreak of Covid-19; and to shed light on problematic diagnoses during this period. Methods To describe the impact of the Covid-19 pandemic on the diagnosis of patients with rare diseases, a retrospective analysis of the Campania Region Rare Disease Registry was performed. A tailored questionnaire was sent to rare disease experts to investigate major issues during the emergency period. Results Prevalence of new diagnoses of rare disease in March and April 2020 was significantly lower than in 2019 (117 versus 317, P Conclusions This study describes the effects of the Covid-19 outbreak on the diagnosis of rare disease in a single Italian region and investigates potential issues of diagnosis and management during this period.

Published
2021

5. Retinal vascular density in multiple sclerosis: a one year follow up

Author

Lanzillo, R, Cennamo, G, Moccia, M, Criscuolo, C, Carotenuto, A, Frattaruolo, N, Sparnelli, F, MELENZANE, ANTONIETTA, Lamberti, A, Servillo, G, Tranfa, F, De Crecchio, G, Brescia Morra, V, Lanzillo, R, Cennamo, G, Moccia, M, Criscuolo, C, Carotenuto, A, Frattaruolo, N, Sparnelli, F, Melenzane, Antonietta, Lamberti, A, Servillo, G, Tranfa, F, De Crecchio, G, and Brescia Morra, V

Subjects
retina, optical coherence tomography, parafovea, multiple sclerosi, vascular density
Abstract

Vascular pathology is increasingly acknowledged as a risk factor of multiple sclerosis (MS). Vascular density (VD) is reduced in MS patients' eyes on optical coherence tomography (OCT) angiography (A).

Published
2019

6. Orbital and eyelid b-cell lymphoma: A multicenter retrospective study

Author

Savino, Gustavo, Midena, Giulia, Blasi, Maria Antonietta, Battendieri, R., Grimaldi, Gabriela, Maceroni, Martina, Tranfa, F., Napolitano, Paola, Lanni, V., Iuliano, A., Savino G. (ORCID:0000-0002-9993-5986), Midena G., Blasi M. A. (ORCID:0000-0001-7393-7644), Grimaldi G., Maceroni M., Napolitano P., Savino, Gustavo, Midena, Giulia, Blasi, Maria Antonietta, Battendieri, R., Grimaldi, Gabriela, Maceroni, Martina, Tranfa, F., Napolitano, Paola, Lanni, V., Iuliano, A., Savino G. (ORCID:0000-0002-9993-5986), Midena G., Blasi M. A. (ORCID:0000-0001-7393-7644), Grimaldi G., Maceroni M., and Napolitano P.

Abstract

Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. Results: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8–32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6–83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 (p = 0.0112) and 9.239 (p = 0.0094), respectively. Conclusions: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

Published
2020

8. Multidisciplinary treatment of primary orbital rhabdomyosarcoma: a single-institution experience

Author

Fiorillo, Amedeo, Migliorati, R., Grimaldi, M., Vassallo, P., Canale, G., Tranfa, F., Uccello, G., Fiore, M., Muto, P., Menna, G., Parasole, R., and Bonavolonta, G.

Subjects
Eye cancer -- Care and treatment, Cancer in children -- Care and treatment, Chemotherapy, Combination -- Evaluation, Rhabdomyosarcoma -- Care and treatment, Health
Abstract

Overall, rhabdomyosarcomas are not common, but they are among the most frequent tumors of muscle tissue. Among children, rhabdomyosarcomas account for four to eight percent of cancers. While many rhabdomyosarcomas have a poor prognosis, patients enjoy a far better prognosis if the disease is limited to the eye socket, or orbit. Published studies indicate a two-year survival rate of about 90 percent. In a retrospective evaluation of the treatment of 12 children, the authors indicate that the current treatment method, which combines radiotherapy with chemotherapy, can be completely effective when properly applied. Ten of the 12 patients were immediately treated with radiation and combined chemotherapy upon diagnosis, and two patients were treated with radiotherapy upon recurrence. The authors view the treatment of these two patients as corrective, in that the initial treatment was not properly applied. All 12 patients are alive without evidence of disease at follow-up periods ranging from 7 to 123 months. Eleven of the 12 patients received a radiation dose of 6,000 cGy (a Gy, or Gary, is one Joule of energy absorbed per kilogram of tissue). This level of radiation, when administered to the eye, is believed to cause cataracts. In the present series of patients, cataract formation was observed in only one patient, and lens opacities occurred in another two. An additional eight patients retain good visual acuity; the remaining patient had almost total lack of vision prior to treatment. The excellent preservation of vision is attributed to immediate treatment with radiotherapy. The ability of the eye to recover from radiation exposure may be compromised if radiotherapy is delayed until after chemotherapy. Therefore, treatment should not be delayed once a diagnosis of rhabdomyosarcoma of the orbit is confirmed. The authors suggest that the currently available treatment methods are already optimal and require only proper administration. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1991

9. Retinal vascular density in multiple sclerosis: a 1‐year follow‐up

Author

Lanzillo, R., primary, Cennamo, G., additional, Moccia, M., additional, Criscuolo, C., additional, Carotenuto, A., additional, Frattaruolo, N., additional, Sparnelli, F., additional, Melenzane, A., additional, Lamberti, A., additional, Servillo, G., additional, Tranfa, F., additional, De Crecchio, G., additional, and Brescia Morra, V., additional

Published
2018
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10. Retinal vascular density in multiple sclerosis: a 1‐year follow‐up.

Author

Lanzillo, R., Cennamo, G., Moccia, M., Criscuolo, C., Carotenuto, A., Frattaruolo, N., Sparnelli, F., Melenzane, A., Lamberti, A., Servillo, G., Tranfa, F., De Crecchio, G., and Brescia Morra, V.

Subjects
MULTIPLE sclerosis, OPTICAL coherence tomography, MULTIPLE sclerosis treatment, RETINA, ANGIOGRAPHY, HEALTH outcome assessment
Abstract

Background and purpose: Vascular pathology is increasingly acknowledged as a risk factor for multiple sclerosis (MS). Vascular density (VD) is reduced in the eyes of patients with MS on optical coherence tomography (OCT) angiography. We performed a 1‐year prospective study to estimate VD variations over time and possible clinical correlates. Methods: A total of 50 patients with MS underwent spectral domain‐OCT and OCT angiography at baseline and after 1‐year follow‐up. Mixed‐effect linear regression models were used to assess variations of each OCT measure and its relation to treatment and clinical outcomes. Results: We observed an increase in parafovea VD (coefficient, 1.147; 95% confidence interval, 0.081–2.214; P = 0.035). Reduction in parafovea VD was associated with increase in Expanded Disability Status Scale score (coefficient, −0.969; 95% confidence interval, −1.732/−0.207; P = 0.013). Conclusions: Retinal VD can improve over time in MS, particularly in patients experiencing disease stability. Longer follow‐up, inclusion of early MS cases and combination with conventional markers of MS severity (i.e. brain atrophy) are needed to better define VD as a potential new biomarker. [ABSTRACT FROM AUTHOR]

Published
2019
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14. Nuclear BCL10 expression does not relate to t(11;18)(q21;q21) and t(14;18)(q32;q21) in ocular adnexa B-cell lymphomas and identificates a group with a worse prognosis

Author

FRANCO, Renato, Camacho F, De Renzo A, De Chiara A, Botti G, Liguori G, D'Angelo M, Laise M, Merola R, Staibano S, Bonavolonta G, Tranfa F, Piris M, De Rosa G., Franco, Renato, Camacho, F, De Renzo, A, De Chiara, A, Botti, G, Liguori, G, D'Angelo, M, Laise, M, Merola, R, Staibano, S, Bonavolonta, G, Tranfa, F, Piris, M, and De Rosa, G.

Published
2005

15. Orbital rhabdomyosarcoma: relationship between DNA ploidy, p53, bcl-2, MDR-1 and Ki67 (MIB1) expression and clinical behavior

Author

STAIBANO, STEFANIA, FRANCO R, TRANFA F, MEZZA E, LO MUZIO L, STRIANESE D, ERRICO ME, BUFO P, FERRARA G, SOMMA P, MANSUETO G, GRECO I, FIORILLO A, ROSA G., BONAVOLONTA', GIULIO, Staibano, Stefania, Franco, R, Tranfa, F, Mezza, E, LO MUZIO, L, Strianese, D, Errico, Me, Bufo, P, Ferrara, G, Somma, P, Mansueto, G, Greco, I, Fiorillo, A, Bonavolonta', Giulio, De, and Rosa, G.

Published
2004

16. Bespoke/su misura

Author

Bianco, A, Gotti, M, Curzi, M, Liverani, A, Molteni, E, Pasquini, L, Tranfa, F, Rossi, D, Santagostino, G, and Margarido, M

Published
2012

19. Morphometric analysis of AgNORs in uveal malignant melanoma

Author

Staibano, S., Orabona, P., Mezza, E., Salvatore, G., Tranfa, F., Capone, D., Errico, M. E., Bonavolontà, G., Lucariello, A., Gaetano DE ROSA, Staibano, Stefania, Orabona, P, Mezza, E, Salvatore, G, Tranfa, F, Capone, D, Errico, Me, Bonavolonta', Giulio, Lucariello, A, DE ROSA, G., and DE ROSA, Gaetano

Subjects
Uveal Neoplasms, Silver Staining, Follow-Up Studies, Humans, Image Processing, Computer-Assisted, Melanoma, diagnosis/ultrastructure, Nucleolus Organizer Region, diagnosis/ultrastructure, Image Processing, Computer-Assisted, Nucleolus Organizer Region, ultrastructure, Prognosis, Silver Staining, Uveal Neoplasm, Humans, Prognosis, Melanoma, Follow-Up Studies
Abstract

To define the value of silver-stained nucleolar organizer regions (AgNOR) in the evaluation of uveal malignant melanomas (MM).Morphometric analysis of AgNORs was performed on 30 uveal MM of spindle A, B, epithelioid and mixed cell types. Findings were compared with cytotype, largest tumor dimension (LTD) and clinical outcome.AgNOR mean perimeter (MP) was 3.76 microns (SD 1.21, range 1.79-8.51 microns) in A, 5.05 microns (SD 1.96, range 1.79-16.41 microns) in B and 6.15 microns (SD 3.86, range 1.79-33.80 microns) in epithelioid MM; mean area (MA) was 0.86 micron 2 (SD 0.60, range 0.20-2.78 microns 2) in A, 1.58 microns 2 (SD 1.11, range 0.20-9.63 microns 2) in B and 2.34 microns 2 (SD 2.10, range 0.20-15.27 microns 2) in epithelioid MM; the aspect ratio (AR) was 3.10 (SD 0.19, range 2.90-3.35) in A, 5.80 (SD 2.07, range 3.77-9.32) in B and 12.22 (SD 2.84, range 8.11-15.81) in epithelioid MM. From comparing MA and RA with follow-up, it seemed that MM with a good prognosis (spindle A and a subgroup of B tumors, B1) exhibited the lowest MA and AR values, while MM with poorer clinical behavior (epithelioid and a second subgroup of spindle B MM, B2) showed the highest. No correlation was found between AgNORs and LTD.AgNORs may contribute to the prognostic evaluation of uveal MM.

Published
1998

22. An unusual orbital lesion: hepatoma metastatic to the orbit

Author

TRANFA F, CENNAMO G, ROSA N, PUBBLICAZIONI SCIENTIFICHE, BOSCAINO A, BONAVOLONTA G., DE ROSA, Giuseppe, Tranfa, F, Cennamo, G, Rosa, N, Pubblicazioni, Scientifiche, Boscaino, A, Bonavolonta, G., DE ROSA, Giuseppe, Cennamo, Giovanni, DE ROSA, Gaetano, Bonavolontà, G., De Rosa, G, and Bonavolonta', Giulio

Subjects
Male, Pathology, medicine.medical_specialty, Carcinoma, Hepatocellular, genetic structures, Vision Disorders, Computed tomography, Hepatic carcinoma, Metastasis, medicine, Exophthalmos, Humans, Aged, Ultrasonography, Aged, 80 and over, medicine.diagnostic_test, business.industry, Liver Neoplasms, General Medicine, medicine.disease, Orbital lesion, eye diseases, Sensory Systems, Ophthalmology, medicine.anatomical_structure, Clinical diagnosis, Orbital Neoplasms, sense organs, Radiology, Complication, business, Tomography, X-Ray Computed, Orbit, Orbital metastasis, Orbit (anatomy)
Abstract

The authors describe a case of histologically proven orbital metastasis from a hepatic carcinoma in an 85-year-old patient with rapid onset of proptosis and visual loss. The clinical diagnosis of orbital metastatic lesion was established by standardized echography and CT scan. To the best of our knowledge, this is only the third histologically proven case of hepatoma metastatic to the orbit.

Published
1994

30. Orbital Dirofilariasis

Author

Strianese, D., primary, Martini, A., additional, Molfino, G., additional, Falabella, L., additional, and Tranfa, F., additional

Published
1998
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40. Orbital Rhabdomyosarcoma: Relationship between DNA Ploidy, P53, Bcl-2, MDR-1 and Ki67 (MIB1) Expression and Clinical Behavior

Author

Staibano, S., Renato Franco, Tranfa, F., Mezza, E., Muzio, L. L., Strianese, D., Errico, M. E., Bufo, P., Ferrara, G., Somma, P., Mansueto, G., Immacolatagreco, Fiorillo, A., Bonavolontà, G., Rosa, G., Staibano, S, Franco, Renato, Tranfa, F, Mezza, E, Lo Muzio, L, Strianese, D, Errico, Me, Bufo, P, Ferrara, G, Somma, P, Mansueto, G, Immacolatagreco, Fiorillo, A, Bonavolonta, G, De Rosa, G., Staibano, Stefania, Franco, R, Tranfa, Fausto, Strianese, Diego, Greco, I, Bonavolonta', Giulio, DE ROSA, Gaetano, and Bonavolontà, G

Subjects
Male, biosynthesis/genetics, Alveolar, Embryonal, biosynthesis, Male, Orbital Neoplasm, biosynthesis, Ploidies, Proto-Oncogene Proteins c-bcl-2, biosynthesis, Rhabdomyosarcoma, Rhabdomyosarcoma, Biomarkers, Tumor, Humans, genetics, Female, Humans, Immunohistochemistry, Ki-67 Antigen, genetics/metabolism/pathology, Rhabdomyosarcoma, Rhabdomyosarcoma, Embryonal, ATP Binding Cassette Transporter, Subfamily B, Member 1, Rhabdomyosarcoma, Alveolar, Ploidies, biosynthesis, Tumor Suppressor Protein p53, DNA, DNA, Neoplasm, Biological, Immunohistochemistry, genetics/metabolism/pathology, Tumor Marker, Ki-67 Antigen, genetics/metabolism/pathology, P-Glycoprotein, Proto-Oncogene Proteins c-bcl-2, Neoplasm, Orbital Neoplasms, Female, Tumor Suppressor Protein p53
Abstract

BACKGROUND: As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas. MATERIALS AND METHODS: The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data. RESULTS: The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p0.05). CONCLUSION: The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.

42. Expression of cyclin-D1 in uveal malignant melanoma

Author

Errico, M. E., Staibano, S., Tranfa, F., Bonavolonta, G., Lorenzo Lo Muzio, Somma, P., Lucariello, A., Mansueto, G., D Aponte, A., Ferrara, G., Rosa, G., Errico, Me, Staibano, Stefania, Tranfa, Fausto, Bonavolonta', Giulio, Lo Muzio, L, Somma, P, Lucariello, A, Mansueto, G, D'Aponte, A, Ferrara, G, and DE ROSA, Gaetano

Subjects
Adult, Aged, 80 and over, Male, Uveal Neoplasms, metabolism/pathology, Middle Aged, Prognosis, Immunohistochemistry, Adult, Aged, Aged, metabolism/pathology, Middle Aged, Prognosis, Uveal Neoplasm, Humans, 80 and over, Cyclin D1, Cyclin D1, Female, Melanoma, biosynthesis, Female, Humans, Immunohistochemistry, Male, Melanoma, Aged
Abstract

Uveal malignant melanoma (MM) is characterized by a marked variability in biological behavior and by an unpredictable clinical course. Therefore the search for reliable prognostic parameters is active. In this study we investigated the immunohistochemical expression of cyclin D1, a cell cycle regulatory molecule, to analyze the possible significance of the protein in the prognostic evaluation of these neoplasms.Forty-five selected uveal MM were incubated with the anti-cyclin D-1 antibody; the findings were compared with the follow-up data, with the cell type and with the largest tumor dimension.The overexpression of cyclin D1 was found to be inversely related to disease-free interval (p < 0.001) and to the spindle A/B tumors with good prognosis (p = 0.05). On the other hand, a direct correlation was found with epithelioid/spindle B melanomas with poor clinical course (p = 0.05) and with sclera infiltration (p = 0.01). No statistically significant relation emerged between cyclin D1 expression and LTD.These data may suggest a possible role of the overexpression of cyclin D1 in the tumorigenesis of uveal MM and in the prognostic evaluation of these tumors, representing a useful tool to subclassify lesions with similar morphological features.

44. BCL10 expression and localization in Ocular Adnexa MALT lymphomas: A comparative cytogenetic and immunohistochemical study

Author

Margherita Cerrone, Collina, F., Chiara, A., Corazzelli, G., Pia Curcio, M., Renzo, A., Russo, F., Cantile, M., Staibano, S., Strianese, D., Tranfa, F., Botti, G., Rosa, G., Franco, R., Cerrone, Margherita, Collina, Francesca, De Chiara, Anna, Corazzelli, Gaetano, Pia Curcio, Maria, De Renzo, Amalia, Russo, Filippo, Cantile, Monica, Staibano, Stefania, Strianese, Diego, Tranfa, Fausto, Botti, Gerardo, De Rosa, Gaetano, Franco, Renato, Cerrone, M, Collina, F, De Chiara, A, Corazzelli, G, Curcio, Mp, De Renzo, A, Russo, F, Cantile, M, Strianese, D, Botti, G, De Rosa, G, and Franco, R.

Subjects
Ocular adnexal B-cell lymphoma, Histology, 6 - Ciencias aplicadas::61 - Medicina [CDU], Genetic aberration, immune system diseases, hemic and lymphatic diseases, MALT lymphoma, BCL10
Abstract

Summary. T(1;14) (p22;q32) involving BCL10 and IGH genes is a rare but recurrent chromosomal aberration in MALT-type lymphoma. It is rarely described in ocular adnexa B cell lymphomas, although nuclear BCL10 shuttling seems to be critical for disease progression in this district. We have evaluated the translocations MALT lymphoma-related in a series of 45 ocular adnexa cases, focusing in particular on their relation with BCL10 expression and its cellular topographic distribution. A prognostic tissue microarray (TMA) with ocular adnexa MALT lymphomas was designed. A study of BCL10 expression and its topographic distribution was performed through immunohistochemistry. In addition the assessment of t(14;18) (q32;q21), t(1;14) (p22;q32) and t(11;18) (q21;q21) was determined by Fluorescent In Situ Hybridization (FISH). Our series revealed t(14;18) (q32;q21) in 6/43 cases (14,3%). t(1;14) (p22;q32), never described in ocular adnexa MALT lymphomas, was observed in 3/31 (9,7%), two of which exhibited the gain of 3’ upstream BCL10 gene signal (4%), whereas no case showed t(11;18) (q21;q21). Moreover, BCL10 expression was observed in 18/45 cases. In particular its nuclear expression was revealed in 12/45 cases, cytoplasmic expression in 5/45 and both cytoplasmic and nuclear expression in 1/45. Statistical analysis demonstrated that while BCL10 cytoplasmic expression is significantly related to the presence of the investigated chromosomal aberrations, in particular with t(14;18) (q32;q21), BCL10 nuclear shuttling does not show any correlation with these translocations. Our data support that BCL10 nuclear distribution is neither related to BCL10 rearrangement nor to other known translocations.

46. Optical Coherence Tomography Angiography Analysis of Vessel Density Indices in Early Post-COVID-19 Patients

Author

Flavia, Chiosi, Giuseppe, Campagna, Michele, Rinaldi, Gianluigi, Manzi, Roberto, dell'Omo, Giuseppe, Fiorentino, Mario, Toro, Fausto, Tranfa, Luca, D'Andrea, Magdalena, Rejdak, Ciro, Costagliola, Chiosi, F, Campagna, G, Rinaldi, M, Manzi, G, Dell'Omo, R, Fiorentino, G, Toro, M, Tranfa, F, D'Andrea, L, Rejdak, M, Costagliola, C, Chiosi, F., Campagna, G., Rinaldi, M., Manzi, G., Dell'Omo, R., Fiorentino, G., Toro, M., Tranfa, F., D'Andrea, L., Rejdak, M., and Costagliola, C.

Subjects
vessel density, inflammatory biomarker, COVID-19, OCT angiography, General Medicine, retinal microvasculature, summary statement, covid-19, oct angiography
Abstract

PurposeA hypercoagulable state has been reported to cause potential sight-threatening ischemia in patients suffering from Coronavirus disease 2019 (COVID-19). This study aimed to determine whether vessel density (VD), as measured by optical coherence tomography angiography (OCT-A), has insights into retinal and choriocapillaris vascular changes in patients affected by SARS-CoV-2 infection.MethodsHundred and fifty two patients positive for SARS-CoV-2 infection were enrolled in this observational, retrospective, controlled study. A control group of 60 healthy subjects was selected for statistical comparisons. Raw OCT and OCT-A data were exported and 3D datasets were analyzed to determine VD.ResultsHundred and forty eyes (92.1%) were included for final analysis. The VD of the superficial capillary plexus (SCP) did not differ between the two groups. The mean VD of the deep capillary plexus (DCP) and the choriocapillaris (CC) was significantly lower in the foveal sector of the COVID-19 group compared to healthy controls. Within the post-COVID-19 group, the lowest DCP and CC foveal VD values were recorded in patients treated with antiviral therapy; no differences were observed among COVID-19 patients with other comorbidities (hypertension, diabetes, thyroid disease) or taking antiplatelet therapy. DCP and CC foveal VD were significantly lower in patients hospitalized in an intensive care unit (ICU) than asymptomatic patients.ConclusionFoveal vessel density at the level of DCP and CC was reduced in post-COVID-19 patients. Further studies evaluating these changes over time will be needed to corroborate the hypothesis of a microvascular retinal impairment in individuals who have recently recovered from SARS-CoV-2 infection.

Published
2022

47. Fractionated Stereotactic Radiation Therapy for Orbital Optic Nerve Sheath Meningioma - a Single Institution Experience and a Short Review of the Literature

Author

Roberto Pacelli, Marco Salvatore, Giulio Bonavolontà, Raffaele Liuzzi, A. Farella, Manuel Conson, Laura Cella, Diego Strianese, Fausto Tranfa, Raffaele Solla, Pacelli, Roberto, Cella, L., Conson, M., Tranfa, F., Strianese, Diego, Liuzzi, R., Solla, R., Farella, A., Salvatore, M., Bonavolonta, G., Cella, L, Conson, Manuel, Tranfa, F, Strianese, D, Liuzzi, R, Solla, R, Farella, A, Salvatore, Marco, Bonavolontà, G., Conson, M, and Salvatore, M

Subjects
Adult, Male, medicine.medical_specialty, Visual acuity, JOURNAL OF RADIATION RESEARCH, genetic structures, Exophthalmos, Health, Toxicology and Mutagenesis, medicine.medical_treatment, Orbital malignancy, Stereotactic radiation therapy, Radiosurgery, Fractionated Stereotactic, orbital lymphoma, Median follow-up, Medicine, Humans, Radiology, Nuclear Medicine and imaging, Radiation, business.industry, Optic Nerve Neoplasms, Dose fractionation, Radiotherapy Dosage, Middle Aged, medicine.disease, eye diseases, Surgery, Optic nerve sheath meningioma, Radiation therapy, Treatment Outcome, RADIATION, RADIOTHERAPY, Optic nerve, Female, Radiology, Dose Fractionation, Radiation, medicine.symptom, business, Meningioma
Abstract

Optic nerve sheath meningioma (ONSM) is a rare orbital tumor that generally induces a slow progressive visual loss in affected patients. Radiotherapy (RT) has currently become the first choice to treat ONSM. In this study our experience in ONSM treatment with fractionated stereotactic radiotherapy (FSRT) is reported. Five patients with diagnosis of orbital ONSM were treated between April 2007 and December 2009 at the Radiation Oncology department of our institution. All patients underwent history and physical, and ophthalmic examinations. Orbital MRI was performed before and 6 weeks after treatment; thereafter every 6 months for the first 2 years. By previous stereotactic localization of the target, RT was delivered with 28 daily fraction of 1.8 Gy by multiple non coplanar arcs dynamically conformed by a micro multileaf-collimator. At diagnosis, in all 5 patients, visual acuity limitations of different degrees were found, while exophthalmos was present in 2, diplopy in 2, orbital pain in 1, and proptosis in 1. In all patients pre-treatment MRI showed an orbital mass involving the optic nerve. After radiotherapy, previous symptoms improved in all patients. However, after RT the MRI consistently showed a stationary status compared to the MRI before RT. At a median follow up of 26 months (range 9-37) all patients had a subjective and/or objective better visual performance than before RT without any evidence of disease progression. No late side effects were recorded. Accordingly to the current literature, our experience confirms the efficacy and the safety of FSRT in patients with orbital ONSM.

Published
2011

48. Intracorneal invasion of the ciliary body by melanoma

Author

G F, Laccarino, R, Forte, F, Tranfa, G, Cennamo, N, Rosa, Iaccarino, Gennarfrancesco, Forte, R., Tranfa, F., Cennamo, G., Rosa, N., Laccarino, Gf, Forte, Raimondo, Tranfa, F, Cennamo, G, Forte, R, Tranfa, Fausto, and Cennamo, Gilda

Subjects
Uveal Neoplasms, genetic structures, Ciliary Body, Humans, Female, Neoplasm Invasiveness, sense organs, Melanoma, eye diseases, Aged, Corneal Diseases
Abstract

The authors report a case of a slow reduction of vision in the left eye of a 69-year-old woman. The slit-lamp examination of the eye showed a vascularized achromic subconjunctival mass close to the nasal limbus, invading the inferonasal corneal quadrant. At B-scan and standardized A-scan echography, the mass was shown to be localized to the ciliary body, with characteristics of a malignant melanoma. The patient did not want to undergo conservative treatment. Therefore, enucleation was performed and light microscopy revealed a pigmented malignant melanoma of the ciliary body with extrascleral growth and infiltration of the anterior third of the corneal stroma. No alteration of Bowman's membrane was present. The corneal infiltration, limited to the anterior third of the stroma, could be due to the fact that at this level the collagen fibers are disposed less regularly than those of the posterior two-thirds, with this different disposition responsible for the weak resistance opposed to the tumoral invasion.

Published
2006

49. Effect of a Fixed Combination of Curcumin, Artemisia, Bromelain, and Black Pepper Oral Administration on Optical Coherence Tomography Angiography Indices in Patients with Diabetic Macular Edema

Author

Flavia Chiosi, Michele Rinaldi, Giuseppe Campagna, Gianluigi Manzi, Vincenzo De Angelis, Francesco Calabrò, Luca D’Andrea, Fausto Tranfa, Ciro Costagliola, Chiosi, F., Rinaldi, M., Campagna, G., Manzi, G., De Angelis, V., Calabro, F., D'Andrea, L., Tranfa, F., and Costagliola, C.

Subjects
genetic structures, Administration, Oral, black pepper, Macular Edema, Retrospective Studie, Diabetes Mellitus, Humans, curcumin, Prospective Studies, Fluorescein Angiography, Retrospective Studies, diabetic retinopathy, diabetic macular edema, artemisia, bromelain, OCTA, vascular flow density, Nutrition and Dietetics, Retinal Vessels, Diabetes Mellitu, Microaneurysm, Retinal Vessel, Bromelains, Prospective Studie, Case-Control Studies, Case-Control Studie, Piper nigrum, Tomography, Optical Coherence, Food Science, Human
Abstract

Background: To investigate the effects of a fixed combination of Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) on vascular parameters in mild to moderate diabetic macular edema (DME). Design: Prospective, case-control study. Methods: Fifty-six patients affected by diabetes mellitus type II were enrolled in the study. Twenty-eight patients with DME received 2 tablets/day, before meals of a dietary complementary supplement containing in fixed combination Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) (Intravit®, OFFHEALTH Spa, Firenze, Italy) for 6 months. Twenty-eight age-matched subjects affected by diabetes mellitus type II were given placebo and served as control group. Patients underwent best correct visual acuity (BCVA), swept optical coherence tomography (OCT), and OCT-Angiography (OCTA). OCTA images of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were obtained for each eye. By the end of the follow-up patients were defined responder to the therapy when a decrease of more than 30 μm was registered in central retinal thickness (CRT) measurement, while a poor responder was determined by the absence of reduction or an increase in central retinal thickness at 6 months. We assessed the foveal avascular zone (FAZ) area, vessel density and quantified the number of microaneurysms in each layer. Results: A significant improvement of BCVA and CRT reduction was recorded at 6 months follow-up in the dietary complementary supplementation group compared to control (respectively p = 0.028 and p = 0.0003). VD of the total capillary plexus, microaneurysms count, glycaemia and HbA1c did not vary over the follow-up period between groups. Within the Intravit® group, poor responders tended to show a larger FAZ area, more microaneurysms, and a lower VD in the DCP compared to the good responders group (p < 0.0001). Conclusions: A fixed combination of Curcumin, Artemisia, Bromelain, and Black pepper oral administration may have a positive impact on central retinal thickness, visual acuity, and VD of the DCP in compensated type 2 diabetic patients with mild DME.

Published
2022
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50. Ophthalmic complications after surgery for nasal and sinus polyposis

Author

Giulio Bonavolontà, Diego Strianese, P. Vassallo, A D'Aponte, Fausto Tranfa, Raimondo Forte, Vassallo, P., Tranfa, F., Forte, R., D'Aponte, A., Strianese, Diego, Bonavolonta, G., Vassallo, P, Tranfa, F, Forte, R, D'Aponte, A, Strianese, D, Bonavolonta', Giulio, Forte, Raimondo, and Bonavolontà, G.

Subjects
Adult, medicine.medical_specialty, Visual acuity, Eye disease, Visual Acuity, Extraocular muscles, 03 medical and health sciences, Eye Injuries, Nasal Polyps, Ocular Motility Disorders, Postoperative Complications, 0302 clinical medicine, medicine, Humans, Orbital Fracture, nasal polyposi, Orbital Fractures, Sinus (anatomy), Nose, Retrospective Studies, business.industry, General Medicine, Middle Aged, medicine.disease, Surgery, Ophthalmology, medicine.anatomical_structure, Oculomotor Muscles, surgical orbital complication, 030221 ophthalmology & optometry, Female, Nasal administration, medicine.symptom, Tomography, X-Ray Computed, Complication, business, Orbit, 030217 neurology & neurosurgery
Abstract

Purpose To describe ophthalmic complications after nasal and sinus surgery. Methods Four cases with orbital complications were retrospectively selected from among more than 2000 cases of orbital pathologies. Results Motility disturbances due to extraocular muscle injury occurred in two patients after intranasal ethmoidectomy and in one patient after a Caldwell-Luc procedure. In the fourth case an orbital apex syndrome was noted after intranasal ethmoidectomies. Conclusions Ophthalmic complications may occur after nasal and sinus surgery, even using an endoscopic procedure. Successful handling of these complications could be reached by on their early recognition and treatment.

Published
2001

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