IMPORTANCE: To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort. OBJECTIVE: To characterize the ...clinical features of OA-MCL. DESIGN, SETTING, AND PARTICIPANTS: This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months. MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival, and progression-free survival were the primary end points. RESULTS: Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65% at 3 years (95% CI, 52%-78%) and 34% at 5 years (95% CI, 21%-47%). Disease-specific survival after 5 years was 38% for the entire cohort (95% CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95% CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95% CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95% CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region. CONCLUSIONS AND RELEVANCE: These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.
Recent Children's Oncology Group (COG) trials tested the efficacy of reduced therapy in an effort to lessen late effects compared to the Intergroup Rhabdomyosarcoma Study (IRS) IV regimen with ...associated hematologic and hepatic toxicity, and infertility. Here, we analyze the efficacy of 45 Gray (Gy) local radiotherapy (RT) in patients with Group III orbital embryonal rhabdomyosarcoma (ERMS) enrolled on the COG low-risk study ARST0331.
Sixty-two patients with Group III orbital ERMS were treated on ARST0331 with four cycles of vincristine (VCR), dactinomycin (DACT), and cyclophosphamide (CPM; VAC, total cumulative CPM dose 4.8 g/m
) followed by four cycles of VCR and DACT over 22 weeks. Forty-five Gray of radiation was administered in 25 fractions beginning at week 13 of therapy.
Fifty-three patients were evaluable for this response analysis; seven had missing week 12 response evaluation data and two had progressive disease prior to starting RT. Median follow-up was 7.8 years. None of the 15 patients with radiographic complete response (CR) compared to 6 of the 38 patients with <CR after 12 weeks of VAC chemotherapy had local recurrences (P = 0.11). There was no difference in overall survival by response at week 12 (P = 0.52).
For patients with Group III orbital ERMS achieving a CR following VAC chemotherapy that includes modest dose CPM, 45 Gy may be sufficient for durable failure-free survival. However, for those with <CR treated with the ARST0331 systemic therapy, a different local therapy approach may be needed to achieve the control rate of IRS-IV without its toxicity.
To evaluate clinically the efficacy of 360-degree minimally invasive endoscopic surgery in patients with orbital intraconal tumors.
Retrospective interventional case series.
A series of consecutive ...patients with orbital intraconal tumors were treated with minimally invasive surgery using an endonasal endoscopic approach or an anterior endoscopic orbitotomy over a 5-year period between January 2015 and January 2020 at the National Institute of Oncology and Radiobiology of Cuba.
In 22 patients, the mean age was 52.3 ± 11 years (range 18-71 years) with a female to male ratio of 1.8:1. Visual loss was present in 3 patients and proptosis was present in 19 patients. The most common diagnosis was cavernous hemangioma (72.7%) followed by orbital sheet meningioma (9.1%). The lower-inner (type II) location was the most usual, followed by the lower-outer (type IV). A gross total resection was achieved in 95.5% of patients. In only 1 case with an orbital pseudotumor was the resection subtotal because of the fibrous consistency, irregularity, and adherences to the medial rectus muscle. All patients with visual loss improved to normal visual function after surgery at follow-up and orbital proptosis recovered in all patients with these symptoms. Both immediate and long-term morphologic and aesthetic results were good in all patients.
The minimally invasive 360-degree surgical approach with a full endoscopic visualization can be safe and efficient in patients with select orbital intraconal tumors. Optic nerve location constitutes the main aspect in the surgical approach selection. A large case series is necessary to standardize this surgical philosophy.
Dermoid cysts are one of the most common benign orbital tumours in children and usually occur unilaterally. Bilateral dermoid cysts in the orbit are rare. We report here, a case of bilateral orbital ...dermoid cysts, in a 29-month-old baby girl. The patient’s prognosis was favourable following surgical resection. Through this case report, we hope to increase the recognition and understanding of this condition.
Melanocytoma is a benign tumor with histologic similarity to oculodermal melanocytosis, which can undergo malignant transformation in rare cases. Melanocytoma more commonly involves the optic disc, ...and few cases of orbital melanocytoma have been reported. Primary orbital melanoma is a rare malignancy known to arise from pigmentary conditions, although there is little information on this tumor arising from melanocytoma. The authors present a case of malignant transformation of orbital melanocytoma in the setting of ipsilateral oculodermal melanocytosis. This case illustrates histopathologic features associated with malignant transformation and highlights the significance of GNAQ, BAP1, and specific intrachromosomal alterations occurring in oculodermal melanocytosis and melanocytoma. The molecular markers observed in this case are of interest as they have overlap with those present in uveal melanoma. This case demonstrates a timeline of genetic and molecular alterations occurring in the malignant transformation of orbital melanocytoma.
Piecemeal excision of dermoid cysts carries the risk of implanting epithelial fragments into orbital fat, which is well recognized to continue secreting oily debris, inciting chronic, often ...granulomatous inflammation. The authors present the clinical and histological details for two patients with persistent lipogranulomatous inflammation for years after piecemeal excision of deep orbital dermoid cysts, in the absence of any residual epithelium. The importance of copious saline lavage - to 'float-out" and reduce microscopic lipid droplets - is also emphasised.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Lymphoma is the commonest orbital malignancy. The typical presentation is proptosis or swelling, which warrants imaging and confirmation by tissue biopsy. Enophthalmos is a much rarer clinical sign ...and if bilateral and symmetrical can often present late. We describe a patient who presented with bilateral enophthalmos and symptomatic, secondary entropion due to bilateral non-Hodgkin's lymphoma in which orbital fat was replaced by a monoclonal proliferation of small B cells. Low-dose orbital radiotherapy and entropion surgery relieved the patient's symptoms.
Purpose:
To analyze the differences in clinical features, treatment methods, prognosis, and etiological factors between lacrimal gland, adenoid cystic carcinoma (LGACC), and orbit adenocarcinoma.
...Material and Methods:
All cases of LGACC and orbit adenocarcinoma between 1975 and 2016 were obtained from the US National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database.
Results:
The 3-, 5-, and 10-year overall survival (OS) rates for 118 LGACC cases were 82.2%, 70.2%, and 51.7%, respectively. The 3-, 5-, and 10-year OS rates for 29 orbit adenocarcinoma cases were 76.3%, 51.2%, and 51.2%, respectively. There was no statistically significant difference in the overall survival rate between LGACC and adenocarcinoma (P=0.566). Univariate analysis showed that the proportion of deaths was higher with increased age and year of diagnosis in LGACC (P=0.010 and P=0.000), while the death rate was higher for poorly differentiated adenocarcinoma (P=0.020). The survival rate for LGACC was lower in patients ⩾60 years of age than in those <60 years of age (P=0.035). There was no significant difference in survival rate between adenocarcinoma patients ⩾60 years and <60 years old (P=0.102). There was no significant correlation between the degree of tumor differentiation and the survival rate for LGACC or adenocarcinoma (P=0.401 or P=0.098, respectively).
Conclusion:
There is no significant difference in prognosis between LGACC and adenocarcinoma. The degree of tumor differentiation is not associated with prognosis for either LGACC or adenocarcinoma.
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