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1
Vijayakumar, S., W. A. McCarthy, C. B. Schroy, F. J. Thomas, P. Thompson, P. D. Higgins, C. F. Lanzieri, P. M. Barrett y M. Murray. "A Technique Using Electrons and Photons in the Radiotherapy of Orbital Neoplasms". Acta Oncologica 26, n.º 6 (enero de 1987): 492–93. http://dx.doi.org/10.3109/02841868709113725.
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Vijitha, Vempuluru Sai, Anasua Ganguly Kapoor, Ruchi Mittal y Ramakrishna Vangara. "Preoperative embolisation of orbital solitary fibrous tumour". BMJ Case Reports 13, n.º 12 (diciembre de 2020): e235576. http://dx.doi.org/10.1136/bcr-2020-235576.
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Solitary fibrous tumours (SFT) is an encompassing terminology comprising of tumours with proliferating CD34 positive specialised fibroblasts. Orbital SFTs are rare slowly progressive highly vascular neoplasms. Complete surgical excision is considered the mainstay treatment. Incomplete resection is a known risk factor for recurrence and malignant transformation. Recently preoperative embolisation of SFT has shown promising results in reducing the vascularity of these tumours rendering them amenable to complete surgical excision. Less than 10 cases of embolisation of orbital solitary fibrous tumours have been described in literature. Our patient underwent an attempted surgical excision elsewhere with significant intraoperative haemorrhage which precluded its complete excision. Herein, we report successful outcome in a case of hypervascular orbital SFT managed with preoperative embolisation, surgical resection and adjuvant radiotherapy along with a review of relevant literature.
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Lindeman, Pontus, Ulf Eklund y Björn Petruson. "Survival after surgical treatment in maxillary neoplasms of epithelial origin". Journal of Laryngology & Otology 101, n.º 6 (junio de 1987): 564–68. http://dx.doi.org/10.1017/s0022215100102257.
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SummaryDuring 1968–1979, 55 cases of epithelial neoplasms originating from the maxillary antrum were diagnosed and treated within the referral area of 1.5 million inhabitants.A majority of the patients were operated upon after pre-operative irradiation therapy (40–50 Gy). No significant difference in survival rate was found between patients operated on by total maxillectomy, including orbital exenteration, and patients subjected to partial maxillectomy. Patients with tumours not completely removed during primary surgery and patients with glandular involvement had a poor prognosis despite additional surgery and radiotherapy. The prognosis for the remaining patients was considerably better, with a 5-year survival rate of 60 per cent.
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Vijayakumar, S., W. McCarthy, F. Thomas, C. Schroy y P. Thompson. "Radiotherapy of orbital neoplasms: Advantages of using photons plus electrons with “anterior chamber” block". International Journal of Radiation Oncology*Biology*Physics 12 (noviembre de 1986): 101–2. http://dx.doi.org/10.1016/0360-3016(86)90524-9.
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Thomas, Giovana R., Jacinto J. Regalado y Mark McClinton. "A Rare Case of Mucoepidermoid Carcinoma of the Nasal Cavity". Ear, Nose & Throat Journal 81, n.º 8 (agosto de 2002): 519–22. http://dx.doi.org/10.1177/014556130208100811.
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Mucoepidermoid carcinoma of the nasal cavity is rare. We report the case of a 57-year-old man who was evaluated for a rapidly enlarging subcutaneous mass on the nasal bridge. The tumor was diagnosed as a mucoepidermoid carcinoma. The patient underwent extensive surgical resection andpostoperative radiotherapy, but 5 months later he required orbital exenteration for persistent disease. Despite these radical measures, the patient died with persistent disease 9 months following the initial evaluation. The aggressive behavior of this tumor highlights the need for physicians to be aware of the differential diagnosis, symptoms, and signs of neoplasms that originate in the nasal cavity so that prompt treatment can be instituted.
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Arora, Rajender Singh, Anirudh Bhattacharya, Dwarkadas Adwani y Sidak Singh Arora. "Massive Periocular Squamous Cell Carcinoma Engulfing the Globe: A Rare Case Report". Case Reports in Oncological Medicine 2014 (2014): 1–4. http://dx.doi.org/10.1155/2014/641086.
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The eyelid tumors are rare neoplasms in head and neck oncological practice. The maximum incidence is after sixty years and most of the tumors are situated in the lower eyelid and medial canthus. A wide range of clinical presentations contribute to a high risk of misdiagnosis. The factors such as very large lesions, incomplete excision, histopathologic features such as poor differentiation, multicentric origin, pagetoid spread, and delayed diagnosis are associated with poor prognosis. Because of different tissues at eyelid level, a variety of tumor types and subtypes can arise, but most of them are carcinomas. A rare case of eyelid carcinoma spreading and engulfing the whole of globe which was treated by orbital exenteration and postoperative radiotherapy is presented with a disease-free follow-up of 10 months which, considering its size, is extremely rare. The early diagnosis and proper treatment of such rare tumors still remain the mainstay to predict favourable prognosis.
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Musin, Sh I., N. А. Sharafutdinova, A. V. Sultanbaev, S. V. Osokin, B. A. Ibragimov, K. V. Menshikov y R. T. Ayupov. "Hedgehog inhibitor in the treatment of basal cell skin cancer: case report". Head and Neck Tumors (HNT) 11, n.º 1 (24 de abril de 2021): 109–14. http://dx.doi.org/10.17650/2222-1468-2021-11-1-109-114.
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Background. In the structure of malignant neoplasms, basal cell skin cancer (BCC) occupies a leading position. In the initial stages of a malignant neoplasm, the main treatments are local treatment methods, both surgical and radiation. However, in about 1.3 % of cases, BCC can become locally or metastatic in which local treatment methods are limited. With the development of targeted drugs, such as the Hedgehog pathway inhibitor, the possibilities for effective and safe treatment of this category of patients have appeared.The objective is to present the clinical case of treating a patient with BCC who is receiving systemic therapy with a Hedgehog signaling pathway inhibitor.Case report. According to the patient, a tumor in the lower eyelid of the left eye appeared in 2000, did not seek medical help. In 2011, he applied to the clinic and was diagnosed with skin cancer of the lower eyelid of the left eye T2bN0M0. The patient refused the proposed treatment. Reapplied only in June 2017 already with a locally distributed process. The case is recognized as unresectable. From June 30, 2017 to September 4, 2017, he underwent a course of a radical program of radiotherapy, with a positive effect. 07/01/2019, he relapsed with a relapse of an orbital tumor on the left. From July 16, 2019, the patient began taking the drug wismodegib 150 mg once a day. Against the background of the treatment, control CT studies on 11/24/2019 and 11/03/2020 compared with CT data from 07/09/2019 reduced the volume of the tumor by 78 and 82 %. The patient currently continues to take wismodegib.Conclusion. The clinical case demonstrates successful treatment of locally advanced basal cell skin cancer with a Hedgehog signaling pathway inhibitor.
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Porto Silva Costa, Isabela, Talita Trindade França, Ivelise Theresa Araujo Balby, Gustavo Henrique Campos de Sousa y Luis Alves de Pinho Segundo. "Diagnostic and therapeutic approach to esthesioneuroblastoma: case report". Health Residencies Journal - HRJ 2, n.º 12 (24 de agosto de 2021): 56–66. http://dx.doi.org/10.51723/hrj.v2i12.162.
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Esthesioneuroblastoma is a rare neoplasm of the nervous system that appears in the paranasal cavities derived from the olfactory neuroepithelium, located in the cribriform region of the nasal fossa, and can invade the sinuses of the face, skull base and orbital region. Kadish classification is the most widely used to stage it according to its extension. The symptomatology is unspecific, and it may present progressive unilateral nasal obstruction, epistaxis, anosmia, headache, sinusitis, diplopia, and orbital proptosis. The diagnosis is based on the lesion biopsy, immunohistochemistry, and ultrastructural findings. Excision of the tumor is described as the treatment of choice that may be associated with radiotherapy and chemotherapy. This is a case report of an olfactory esthesioneuroblastoma in a patient admitted to the hospital complaining of orbital proptosis who, after a computed tomography of the skull, showed a suspicious lesion. After the biopsy and the anatomopathological study, a protocol with neoadjuvant chemotherapy was initiated, with the subsequent excision of the tumor. Despite the lack of other symptoms, an advanced stage of the neoplasm was identified, which reflects the importance of investigating less common complaints and thus facilitating the diagnosis and early treatment.
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Fasina, Oluyemi. "Ocular surface squamous neoplasia in Ibadan, Nigeria". Asian Journal of Oncology 01, n.º 02 (julio de 2015): 101–5. http://dx.doi.org/10.4103/2454-6798.173312.
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ABSTRACT Introduction: Squamous cell carcinoma is the most common malignancy of the conjunctiva worldwide. Ocular surface squamous neoplasia (OSSN) describes the spectrum of ocular surface intraepithelial neoplasia, pre-invasive and invasive squamous cell carcinoma. Method: This nonrandomized study aims to describe the epidemiology, clinical features and evaluate the outcome of treatment in patients with histological diagnosis of OSSN managed at a single tertiary center in Ibadan, Nigeria. Result: Twenty-five patients were managed within the study period with a mean age of 42 ± 15.3 years and male: female ratio of 1:1.5. All patients presented with growth and redness, and, visual impairment was observed in seven (28%) patients. Fifteen (60%) patients were seropositive for HIV infection and one patient (4%) had xeroderma pigmentosum. The right side was involved in 11 (44%) patients and there were no bilateral lesions. Morphologically, 18 (72%) lesions were gelatinous, six (24%) were leucoplakic while one (4%) was nodular. Twenty-two (88%) patients underwent surgical excision with alcohol kerato-epitheliectomy and cryotherapy, while three (12%) patients had lid sparing orbital exenteration. The three (12%) patients with intraepithelial neoplasm, and six (24%) who had SCC but with tumor-free margins received no adjuvant treatment post-operatively, while 13 (52%) with SCC and microscopic margin involvement were treated with four courses of 0.04% topical mitomycin C (MMC) and the three (12%) patients who had orbital exenteration were referred for radiotherapy. The average follow-up period was 12 months, no patient was lost to follow-up and none has had recurrence. Conclusion: OSSN occurs in younger individuals, and is strongly associated with HIV infection in our environment. Early diagnosis and intervention can prevent severe ocular morbidity. Wide surgical excision with intra-operative cryotherapy and adjuvant treatment with topical MMC post-operatively seem to give good outcome in our patients.
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Gomes, Lilian Brito, Jonathan Dos Santos Lima, Valéria Camargo Silveira, Jacqueline Stephanie Fernandes Do Nascimento, Nicolle Dos Santos Moraes Nunes, Marco Orsini, Gilberto Canedo Martins Jr et al. "Orbital Extramedullary Plasmacytoma: Case report and literature review". Gazette of Medical Sciences 2, n.º 1 (1 de febrero de 2021): 26–29. http://dx.doi.org/10.46766/thegms.ophthal.21020101.
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Extramedullary plasmacytoma is a rare disease, characterized histopathologically by infiltration of plasma cells of different maturities and producing monoclonal immunoglobulin outside the bone marrow. J.R.G., 52 years old, male, sought medical assistance due to amaurosis and blocked eye movements. We performed Magnetic Nuclear Resonance of the skull that showed discrete intermediate signal tissue, next to the optic nerve canal and homolateral superior orbital fissure, with slight extension to the lower orbital fissure, associated with thickening of the corresponding meninges, involving the proximal portion of the optic nerve, measuring 1.3 x 1.1 x 2.5 cm. Non-specific aspect, but suggestive of lymphoproliferative lesion. Right and extraorbital tumor excision was performed, in which it was not possible to completely resect the tumor. The anatomopathological examination of the piece showed a neoplasm of plasma cells (plasmacytoma). The diagnosis is made from the exclusion of Multiple Myeloma. The treatments of choice are radiotherapy, due to the high radiosensitivity in 80–100% of cases, and surgery for localized lesions.
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Peck, Travis, Kalla A. Gervasio, Paul J. L. Zhang, Carol L. Shields, Sara E. Lally, Ralph C. Eagle Jr. y Tatyana Milman. "Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma with Myxoid Stroma in a Hereditary Retinoblastoma Survivor". Ocular Oncology and Pathology 6, n.º 2 (8 de agosto de 2019): 79–86. http://dx.doi.org/10.1159/000501523.
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Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) is an indolent, locally aggressive mesenchymal neoplasm, most often confined to the lower extremities and retroperitoneum and rarely identified in the orbit. Diagnosis of ALT/WDL can be challenging due to its frequent morphologic overlap with benign adipose lesions and other more aggressive liposarcoma subtypes, including myxoid liposarcoma. We describe a 26-year-old female with a history of hereditary retinoblastoma and external-beam radiotherapy to the orbit, who developed orbital liposarcoma. Although initial morphologic assessment raised the consideration of myxoid liposarcoma, subsequent fluorescein in situ hybridization studies demonstrated MDM2 and DDIT3 coamplification without DDIT3 rearrangement, supporting the diagnosis of ALT/WDL with myxoid stroma. The literature review of previously reported orbital myxoid liposarcomas revealed a morphologic overlap of documented tumors with ALT/WDL, dedifferentiated liposarcoma, and pleomorphic liposarcoma with myxoid stroma as well as an absence of immunohistochemical and molecular genetic data supportive of the diagnosis of myxoid liposarcoma. This case emphasizes the potential overlap of ALT/WDL with myxoid liposarcoma and the increasing importance of molecular genetic studies in the diagnosis, prognosis, and management of orbital liposarcoma.
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Ahamed, Naushad A. B., Khalid Sait, Nisreen Anfnan, Khader Farwan, S. H. M. Nizamuddin y Saleh S. Baeesa. "Gestational Choriocarcinoma Presenting with Lacrimal Gland Metastasis: A First Reported Case". Case Reports in Obstetrics and Gynecology 2015 (2015): 1–7. http://dx.doi.org/10.1155/2015/879538.
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Background. Gestational choriocarcinoma (GC) is a recognized clinicopathological subtype of gestational trophoblastic neoplasia that usually metastasizes hematogenously to highly vascular organs like the lung, liver, and brain. However, orbital metastasis to the choroid and lacrimal gland is a rare occurrence.Case Presentation. A 21-year-old female presented with headache and left orbital swelling one year after resection of a complete hydatidiform mole followed by adjuvant methotrexate chemotherapy. A metastatic imaging screening revealed multiple metastases in the lungs, brain, and adrenal gland, in addition to the choroid and lacrimal gland. Based on her modified WHO risk factors scoring she was started on chemotherapy and whole brain radiotherapy, which resulted in a complete response. At two-year follow-up, serum b-HCG level was with normal limits; imaging surveillance was uneventful.Conclusion. We present the first case of lacrimal gland metastasis in a young girl from GC relapse.
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Al-Wassia, Rolina K. "Intensity Modulated Radiotherapy is an Effective Treatment for Basosquamous Cell Cancer". Journal of King Abdulaziz University - Medical Sciences 22, n.º 1 (1 de enero de 2015): 53–58. http://dx.doi.org/10.4197/med.22-1.8.
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Basosquamous cell carcinoma is an uncommon skin cancer with an incidence rate between 1.5% and 2.7%. Surgical excision is the therapy of choice for most skin neoplasm. Depending on the tumor presentation size, location, and patient general condition, there are complementary approaches available: radiation therapy, for curative intent or used as an alternative to maximize local control and symptomatic relief. The case is a 54-year-old male with a seventeen year history of six times recurrent Basosquamous cell cancer that started as a skin nodule on the left nasolabial fold and ended with a mass penetrating into the left ethmoid sinus and orbital cavity. The patient was then treated using intensity modulated radiotherapy as the only effective second alternative modality after debulking surgery. Radiation intent was curative, aiming for eradication of the tumor, maximizing the local control and relief of symptoms. The follow-up demonstrated an eighty percent tumor shrinkage as measured on imaging studies in addition to an excellent cosmetic outcome. Intensity modulated radiotherapy proved to be an efficient treatment modality, with acceptable toxicity and excellent local control, when the patient did not prefer the surgical option for the cosmetic compromise.
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Moro, Alessandro, Paolo De Angelis, Giulio Gasparini, Sandro Pelo, Gianluigi Petrone, Emanuela Lucci Cordisco, Umberto Garagiola, Giuseppe D’Amato y Gianmarco Saponaro. "Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review". Case Reports in Oncological Medicine 2018 (2018): 1–7. http://dx.doi.org/10.1155/2018/1684763.
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Purpose. Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, and musculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoid fibromatosis. In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit is presented. The aim of the article is to describe this unusual and rare location for the desmoid fibromatosis and outline the principle phases in the decision-making process and the therapeutic alternatives for a patient affected by desmoid fibromatosis. Methods. The protocol of this review included study objectives, search strategy, and selection criteria. The primary end point of this study was to analyze the head and neck desmoid fibromatosis. The secondary end point was to identify the available therapies and assess their specific indications. Results. The mean age of patients was 18.9 years ranging from 0 to 66, and 52% were female. A bimodal age distribution was observed, and two age peaks were identified: 0–14 years (57%) and 28–42 years (18%). The most common involved areas were the mandible (25%) followed by the neck (21%). In 86% of the cases, the treatment was the surgical resection of the disease, and only in 5% of the cases, the surgical resection was followed by adjuvant radiotherapy. Conclusion. The orbital location is extremely rare, especially in the pediatric population. The management of desmoid fibromatosis is based on the function preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of the disease or risk of functional damages, the surgical approach may be considered. Therapeutic alternatives to surgical resection are radiotherapy and systemic therapy.
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Murthy, Ramesh, Himika Gupta, Rahul Krishnatry y Siddhartha Laskar. "Electron beam radiotherapy for the management of recurrent extensive ocular surface squamous neoplasia with orbital extension". Indian Journal of Ophthalmology 63, n.º 8 (2015): 672. http://dx.doi.org/10.4103/0301-4738.169798.
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Puccini, Benedetta, Luigi Rigacci, Marco Puccioni, Sofia Kovalchuk, Elisa Fabbri, Valentina Carrai, Renato Alterini y Alberto Bosi. "Rituximab and Chlorambucil As First Line Therapy of Low-Grade Ocular Adnexal Lymphomas: Long Term Follow-up Results". Blood 120, n.º 21 (16 de noviembre de 2012): 3685. http://dx.doi.org/10.1182/blood.v120.21.3685.3685.
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Abstract Abstract 3685 Background: Ocular adnexal lymphomas (OALs) represents 8% of primary extranodal lymphomas, 80% of OALs constitutes extranodal marginal zone lymphomas (EMZL). In the last years the incidence of OLAs has increased more rapidly than Non Hodgkin Lymphoma at other extranodal site. Radiotherapy is associated to high rates of local disease control, but also to the risk of relapse and immediate and delayed complications, such as xerophtalmy, corneal ulcerations, cataract and retinal damage. Surgery is a feasible option, but not always satisfactory in terms of disease control. Single-agent chemotherapy with alkylating agents is used for the treatment of low-grade lymphomas, including OALs. Rituximab, a chimeric anti-CD20 monoclonal antibody, is effective in EMZL and in OALs. Aims: We investigated the efficacy and the safety of a combination of chlorambucil and rituximab as first line treatment in patients with OALs. Methods: Patients with histologically proven low-grade OALs were enrolled in this study. Staging included CT-scan of the orbit, neck, chest and abdomen, MR of the orbit, Chlamydia psittaci (Cp) detected by PCR and bone marrow biopsy. Treatment consisted of chlorambucil (0,1 mg/Kg/die for 45 days, then on days 1 to 15 monthly for 4 months) and rituximab (375 mg/sqm weekly for 4 doses, then monthly for 4 infusions). Toxicities were reported according to WHO criteria. At the end of treatment patients were restaged clinically and with a MR of the orbit. Results: Since November 2003 to November 2010 twenty consecutive, histologically proven, low-grade OALs (19 EMZL, a grade I follicular lymphoma) have been treated according to protocol. The median interval between onset of the first symptoms and diagnosis was 13 months (range, 4 months – 3 years). Twelve pts were female (60%) and eight pts were male (40%). Median age at diagnosis was 68 years (range, 35–86 years). Disease was localized in the conjunctiva in 15 patients (75%), in the lacrimal glands in 2 patients (10%) and in other orbital sites in the last three patients (15%). Nineteen patients presented a stage I disease, one stage IV, and no patient showed B-symptoms. LDH was within normal range in 17 of 20 patients (85%), ECOG-PS was 0 and International prognostic Index (IPI) was low or low-intermediate in all patients. We evaluated PCR for Chlamydia psittaci in the first ten consecutive pts and it was negative. All patients completed the treatment without delay; there was no grade III-IV toxicities neither hospitalizations. Five patients had grade 1–2 rituximab infusion-related reactions usually during the first infusion and haematological toxicity was mild. At the end of treatment 19 patients (95%) resulted in CR, and one obtained a partial remission (5%). After a median follow-up of 56 months (range, 21–106 months) all patients are alive, 17 maintained CR and two relapsed (after 3 and 5 years), one was retreated with the same protocol and attained a new CR and the other relapsed systemically as follicular lymphoma. The patient in PR after first line obtained CR with second line therapy (rituximab fludarabine and cyclophosphamide). The median PFS was 43 months (range, 3–98 months). All patients performed ophthalmologic follow-up visits: we did not report ocular toxicities, and all patients conserved a normal visual function, including acuity. No secondary myelodisplatic syndrome or secondary neoplasms are reported. Conclusions: After a long follow-up the combination of rituximab and chlorambucil proved to be low toxic, feasible and effective therapy for primary OALs. No delayed ocular or haematological complications are reported. For this reason the combination of immunotherapy and mild chemotherapy should be considered for the treatment of this indolent lymphoma. Disclosures: No relevant conflicts of interest to declare.
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Casiano, Roy R., William A. Numa y Alberto M. Falquez. "Endoscopic Resection of Esthesioneuroblastoma". American Journal of Rhinology 15, n.º 4 (julio de 2001): 271–79. http://dx.doi.org/10.1177/194589240101500410.
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Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neoplasm originating from neuroepithelial cells. Treatment for this tumor has traditionally required a craniofacial resection through an external approach. The safety and efficacy of transnasal endoscopic resection is unknown. A series of five patients with Kadish stage A or B lesions of the anterior skull base were resected and immediately reconstructed endoscopically over a nine-year period. There were 2 males and 3 females with a mean age of 64 years. Three patients were treated primarily and two were treated secondarily for recurrences after failing a traditional external craniofacial resection and postoperative radiotherapy. All primary lesions received adjuvant radiotherapy. The mean follow-up time was 31 months. All patients were discharged within 4 days or less. Operative complications included temporary postoperative infraorbital anesthesia (1 patient), unplanned cerebrospinal fluid leak (1 patient), and an orbital hematoma (1 patient). None of these complications resulted in any permanent sequelae. Two patients had prolonged nasal crusting for up to one year due to a variety of factors; large cavities, radiation therapy, and exposed lyophilized dural graft. However, all skull base defects healed without any short- or long-term sequelae. There have been no local recurrences. Two patients (1 primary and 1 secondary) developed regional metastasis to the orbit or cervical lymph nodes managed with primary transorbital excision or modified neck dissection, respectively. All but one patient remain free of disease by clinical, endoscopic, and radiographic (CT or MRI) surveillance. The remaining patient recurred distally as an undifferentiated carcinoma in his temporal bone and lungs 12 months after the initial resection. In experienced hands select cases of esthesioneuroblastoma can be safely excised and reconstructed endoscopically with comparable degrees of tissue removal as with external approaches. Short-term oncologic results in this small series of patients appear to be comparable to traditional methods. Longer follow-up on a larger series of patients is warranted.
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Pagano, Livio, Andrea Gallamini, Giulio Trapè, Daniele Mattei, Eros Di Bona, Emilio Iannitto, Antonio Spadea et al. "T/NK “Nasal Type” Lymphomas: An Italian Cooperative Retrospective Survey." Blood 104, n.º 11 (16 de noviembre de 2004): 4578. http://dx.doi.org/10.1182/blood.v104.11.4578.4578.
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Abstract T/NK “nasal type” lymphoma is a neoplasm that shows a peculiar geographical distribution. In fact most of the reported cases come from Far Eastern countries such as Hong Kong, and Central America, particularly from Mexico; this kind of lymphoma occurrs rarely in western countries. Between 1997 and 2004, 19 new cases of T/NK nasal type lymphoma were diagnosed in 7 Italian Hematology Division. Patients characteristics were as follows: all caucasian; 14 men and 5 women; median age 53 years (range 20–80). The presentation at the onset was in the nasal cavity or adjacent structures in 15 cases, who presented symptoms like nasal obstruction, dysphagia, orbital edema; in 2 cases, skin lesions were the first sign of disease, followed by oropharingeal involvement; in 1 case, the patient was studied because of migrating bone pain, and in the last patient lymphoma presented as a right leg tumor. Considering the stage of disease, 10 patients presented a I stage (in 8 cases IA, 1 IB, and 1 IE); 3 patients were in stage II (2 IIB, 1 IIE); 6 patients presented an advanced stage disease (3 IVA, 3 IVB). Thus, early stage patients were 13, while advanced stages were 6. The median time from the onset of signs, as skin lesions, or symptoms, as nasal obstruction to diagnosis was 3 months, ranging between 1 and 24. Diagnosis was based on the finding of a T/NK phenotype by the histological examination of oropharingeal or cutaneous lesions in 17 cases, bioptical examination of the leg tumor in another patient, and by a bone marrow biopsy in the patient who presented bone pain. All patients were treated with chemotherapy alone (8 cases), or associated to radiotherapy (10 cases); 1 patient underwent chemotherapy, radiotherapy, and surgery. Chemotherapy regimens usually adopted contained anthracyclines (11 cases); in the other cases patients were treated with CVP (2 cases), low dosages of cyclophosphamide (1), cyclophosphamide-vinblastine-mithoxantrone-bleomicine (1), DHAP (3). Radiation dosages ranged between 27.5 and 47.5 Gy, with a median dosage of 40 Gy. Eight patients reached a clinical improvement, while 11 patients resulted refractory or presented a limited response to therapy The overall median survival time was 5 months (range from 1 to 48): Eleven patients died within 14 months from diagnosis, and the median survival time of this group was 6 months. At last follow-up (June 2004) 8 patients are still alive, with a time from diagnosis ranging beteween 2 and 48 months. The preliminary results of this retrospective survey confirmed that T/NK nasal type lymphoma is a very rare lymphoma in italian population, and it is characterized by a very bad prognosis. The rarity of this disease cause a lacking of a standardized therapeutic approaches. More data are needed to know the epidemiology of this kind of lymphoma in Europe.
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Mueller, Antonia M. S., Alex Kuehnemund y Monika Engelhardt. "High-Dose Chemotherapy in Small Cell Neuroendocrine Carcinoma: Favorable Long-Term Outcome for Extrapulmonary Primary Localization." Blood 108, n.º 11 (16 de noviembre de 2006): 3075. http://dx.doi.org/10.1182/blood.v108.11.3075.3075.
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Abstract Small cell neuroendocrine carcinomas (SCNC) are composed of round to spindle-shaped cells with features of both neuroendocrine and epithelial neoplasms. SCNC can be found in basically all epitheloid organs; however, the vast majority arises in the lung, while extrapulmonary (EP) localization is rare. Small cell lung cancer (SCLC) and EP SCNC are considered one histological entity and are treated similarly. Despite the high initial response to chemo- and radiotherapy, most patients relapse after short remission, and overall prognosis is dismal. Clinical trials employing high-dose chemotherapy (HDCT) followed by autologous stem cell transplantation (auto-SCT) neither demonstrated a clear benefit nor clarified its significance in SCLC. The role of intensified treatment in EP SCNC has not been specifically addressed in detail. Conversely to SCLC, some subgroup analyses indicated that patients with EP SCNC might benefit from intensive treatment. We analyzed a total of 22 patients: 8 with EP SCNC (group A; m:f 3:5; median age 36 years (y) [range 22–56]) and 14 patients with SCLC (group B; m:f 10:4; median age 55 y [40–63]), all undergoing HDCT with auto-SCT at our single center from 07/90–01/05. A control group C consisted of 30 patients with EP SCNC (m:f 21:9, median age 66 years [32–81], who received different standard treatments without auto-SCT. All group A patients had stage IV disease (liver n=5, lymph nodes n=4, pancreas n=1, orbita n=1, bone n=1, kidney n=1). Two of these patients received additional local radiotherapy. In group B, 7 patients had limited and 7 patients extensive disease, (stage I (n=1), II (n=2), III (n=10), IV (n=1). HDCT with VIC (etoposide, ifosfamide, carboplatin; n=21), or CCT (carboplatin, cyclophosphamide, thiotepa; n=1) was followed by infusion of a median 3.2x10^6 CD34+ cells. Prophylactic radiotherapy was performed in 12 patients (mediastinum n=11; brain n=10). With a median follow-up (FU) of 48 months (7–152) for group A and 85 months (0–170) for group B, 5/8 (63%) of patients with EP SCNC (group A) are alive and in complete remission (CR), compared to 5/14 (36%) SCLC patients (group B). Best response ever achieved after HDCT was a CR in 5/8 (63%), a partial remission (PR) in 2/8 (25%) and stable disease (SD) in 1/8 (12%) in group A. In group B, a CR was attained in 11/14 (79%), a PR and a SD in 1/14 (7%) patients, respectively. In the conventionally treated control group C, a transient PR was achieved in 5/30 (16%), and after a median FU of 9.7 months, only 2/30 (6.7%) EP SCNC patients are alive. Our analysis suggests that selected SCNC patients may benefit from HDCT, particularly when integrated into multimodal treatment concepts. The remarkably favorable outcome in patients with EP primary site, even when HDCT was implemented as salvage treatment warrants further studies on the role of HDCT in SCNC. Careful attention will have to be paid to prognostic clinical features, such as primary site and/or histological parameters including neuroendocrine marker profiles and mitotic indices. These may help to predict which patients will benefit from intensified treatment. In addition, further histological studies should address the identification of markers specific for lung- vs. extrapulmonary primary localization. For this purpose all available tumor tissue from our study is currently under histological re-analysis, assessing the expression of the novel tumor testis antigens.
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Guerra Soares Brandão, Antonio Adolfo, Giancarlo Fatobene, Andre Abdo, Luis Alberto de Padua Covas Lage, Israel Bendit, Luciana Nardinelli, Sheila Aparecida Coelho de Siqueira et al. "Clinical, Laboratory, and Genetic Features of Erdheim-Chester Disease Patients from Two Reference Centers in a Developing Country". Blood 136, Supplement 1 (5 de noviembre de 2020): 22–23. http://dx.doi.org/10.1182/blood-2020-142617.
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INTRODUCTION: Erdheim-Chester Disease (ECD) is a rare histiocytic neoplasm with a heterogenous clinical course with asymptomatic localized course or systemic compromise involving multiples organs causing significant morbidity and mortality. There are few cohorts published however mainly from North America and Europe. Given the scarcity of data on ECD in Latin America, we have established a local registry in the city of São Paulo to collect clinical and biological material of ECD patients. METHODS: We retrospectively collected clinical data on biopsy-proven ECD patients diagnosed and treated at two reference centers for histiocytic disorders (Hospital das Clínicas da Universidade de São Paulo e Hospital Sírio-Libanês, Brazil) from January 2006 to February 2020. RESULTS: Sixteen patients with confirmed diagnosis of ECD were included with median age of 53 years. 75% were males and a median follow-up time of 50 months (7-163). Median time from onset of symptoms to diagnosis was 13 months (0.1-142). Immunohistochemistry (IHC) findings showed positivity for CD68 in 15/16 (94%) and for S100 in 3/16 (19%) patients, no case had CD1a positivity. The most frequent organs involved were: bone (75%), skin (44%), central nervous system (CNS) (44%), lymph nodes (31%), lung (13%), liver (6%), spleen (6%), and gastrointestinal tract (6%) of cases. CNS lesions involvement occurred mostly in the pituitary gland (86%). Twelve of 16 (75%) patients presented disease in more than one organ. Xanthelasma and xanthoma were the most common skin lesions (44%). The most frequent histiocytosis-related clinical manifestations were bone pain (44%) and diabetes insipidus (38%). The most frequent radiologic findings were osteosclerosis in 12/16 (75%) patients, retroperitoneal fibrosis around the kidneys in 6/16 (38%), the coated aorta sign and orbital infiltration were found in 4/16 (25%) of cases. 18FDG/PET-CT was performed in all patients, of whom 13 (81%) had hypermetabolic lesions. BRAF status at diagnosis was available in 13/16 patients using the technique of Sanger in 5/13, pyrosequencing in 3/13, IHC in 3/13 and polymerase chain reaction (PCR) in 2/13. Mutations were detected in only 3/16 (19%) cases. All patients received treatment due to symptomatic disease with a median of two lines of therapy (1-7). Median time between diagnosis and the first treatment was one month. First-line treatments were interferon in 12/16 patients, steroids in 5/16, and each one of thalidomide, vemurafenib and tumoral resection in one patient. Beyond first-line therapy, the most conventional chemotherapy regimens used were cladribine (4/16 patients) and LCH-like etoposide-containing vinblastine, methotrexate and mercaptopurine (2/16 patients). Other treatments included radiotherapy (4/16 patients) and a single patient used cobimetinib, imatinib and infliximab. Median progression free survival (PFS) after the first line treatment was 7.5 months (95% CI 5-10), and median overall survival (OS) was not reached to this date. Time to next treatment was 9 months in patients who did not achieved at least partial response after first line, and 15 months in those who attained it. PFS at 2 years was 45% (95% CI 0.17-0.71), and OS at 2 years was 100%. One patient died due to infection complication after the first cycle of cladribine after 50 months of follow-up. CONCLUSION: To our knowledge, despite the low number of patients, this is the largest Latin American cohort of patients with ECD reported to date. Our findings resemble demographic characteristics, sites of involvement and treatment choices reported by other groups. However, it is noteworthy that the proportion of ECD patients bearing a BRAF mutation (18.8%) was pretty lower than previously reported (approximately 50%). This needs to be taken cautiously due to the small number of subjects and due to technical issues, since all samples analyzed by PCR or Sanger were negative for BRAF mutation. A national registry of histiocytosis is needed to confirm these preliminary data. Disclosures No relevant conflicts of interest to declare.
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Hussain, Ibrar, Tajamul Khan, Zaman Shah y Zulfiqar Ali. "Orbital Rhabdomyosarcoma – A Case Series of 12 Patients from North – West Pakistan". Pakistan Journal of Ophthalmology 36, n.º 4 (2 de septiembre de 2020). http://dx.doi.org/10.36351/pjo.v36i4.1075.
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Purpose: The purpose of this study is to find out demographic characteristics, clinical behavior and outcome of this neoplasm in North West Pakistan.
Study Design: Retrospective Case series.
Place and Duration of Study: Department of Ophthalmology, Khyber Teaching Hospital Peshawar Pakistan, from 2015 to 2019.
Methods: All patients with Orbital Rhabdomyosarcoma consulting our department were registered and a self-designed proformawas used to document demography, clinical features, management, and follow-up of all cases. At the end of study, the data was analyzed using SPSS version 25 and result compiled.
Results: Twelve patients with “Orbital Rhabdomyosarcoma” were included in the study with mean age of7.2 ± 1.6 years. Majority of patients presented with mass in upper lid with proptosis. On CT-Scan and/or MRI orbit proved that in seven (58.3%) cases the mass was occupying the superior orbit, in 3 (25%) cases inferior orbit and in 2 (16.6%) cases the tumor was advance enough to occupy the whole orbit.Biopsy showed embryonic type (66.7%), alveolar type (25%) and undifferentiated (8.3%). All patients received chemotherapy and radiotherapy with excision of tumor in 16.7% and exenteration in 41.7% patients. The course of follow-up extended from 7 to 24 months. Five (41.7%) patients felt well at the end of last follow-up and four (33.3%) developed recurrence. At the end of follow-up, 3 (25%) patients died of tumor.
Conclusion: In North West Pakistan patients with Orbital Rhabdomyosarcoma present late and the prognosis is poor due to not following the proper protocol for follow-up.
Key Words: Rhabdomyosarcoma, Orbital rhabdomyosarcoma, IRS Staging.
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Das, Dhivya y Sharon Jones. "P25 Erdheim-Chester disease: a rare histocytosis masked as IgG4 disease". Rheumatology 59, Supplement_2 (1 de abril de 2020). http://dx.doi.org/10.1093/rheumatology/keaa111.024.
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Abstract Background Erdheim-Chester disease (ECD) is a rare non-inherited, non-Langerhans form histiocytosis reclassified as a histiocytic neoplasm was first described in 1930 by Jakob Erdheim and William Chester. Only 500 cases were reported. Onset is usually between 50 to 70 years old. Male to female ratio is 3:1. Symptoms include long bone pain, fever, weight loss, exophthalmos, papilledema, headaches, cranial nerve palsies and sensory disturbances. Skeletal Abnormalities includes bilateral and symmetric cortical osteosclerosis of long bones. Cardiovascular involvement includes a ‘coated aorta’ which can involve renal arteries leading to reno-vascular hypertension. Pulmonary and retroperitoneal fibrosis has been reported. Methods We present a 90 year old gentleman with a complicated history of diplopia, proptosis with large retrobulbar orbital masses (confirmed on CT orbits) increasing in size over 2 years. He was symptomatic with bone pain, recurrent falls, dizziness and shortness of breath. He had a background history of pulmonary embolism, atrial fibrillation, hypertension and osteoporosis. On examination, no joint synovitis or lymphadenopathy noted. Chest examination revealed mild inspiratory crackles. Results CRP was recorded at 150mg/L. ANA, ENA, ANCA, anti-DsDNA, RF, anti-MCV antibodies were negative. IgG4 levels were normal. Pulmonary Function tests showed a restrictive Pattern - FEV1/FVC - 64%, FVC - 68%, TLC - 75%, TLCO - 68% predicted. CT Chest reported aortitis and mild undifferentiated pulmonary fibrosis. CT aorta done subsequently reported extensive soft tissue changes of the abdominal and thoracic Aorta which extended to the renal hila causing bilateral hydronephrosis. There was confirmed retroperitoneal Fibrosis and widespread para-aortic lymphadenopathy. He was stented by the urologists for hydronephrosis. Orbital biopsy of his left eye revealed xanthogranulomatous inflammation with widespread foamy histiocytes (CD68 positive). S100 and CD1a were negative. This confirms a clinical correlation of ECD. Further multidisciplinary discussions suggested that he stays on high dose prednisolone of 30mg daily. Localised radiotherapy was successful in debulking his orbital lesions. Chemotherapy was discussed but he does not wish for this. Conclusion Differential diagnoses for ECD include IgG4 disease, Granulomatosis with polyangiitis, Takayasu’s arteritis, Langherhan Cell histiocytosis, or malignancies. Tissue diagnosis describes infiltration of foamy histiocytes, lymphocytes and lipid laden macrophages. It is distinguished by immunohistological characteristics (ECD positive for CD68, negative for CD1a and S-100 protein) in 80 percent of cases. Serum Assay Samples can show high IFN-alpha, IL1, IL-6 and IL-12 titres. First line treatment is pegylated IFN-alpha. Bisphosphonates can alleviate bone pain. Cladribine has helped those with orbital involvement. Anakinra or Inflixamab improves symptoms of mild forms of ECD. Vemurafenib is used for patients with the BRAFV600 proto-oncogene mutation. PET scans are recommended for disease activity assessment. ECD has a variable prognosis but poorer in those with CNS involvement. With treatment, the mortality rate is 26% and 5-year survival is 68%. Disclosures D. Das None. S. Jones None.