Dataset Information

Long term outcomes of transcutaneous non-image guided bleomycin sclerotherapy in orbital/adnexal lymphatic malformations: a protocol-based management in 69 eyes.

ABSTRACT:

Objectives

To study the efficacy and the long-term outcomes of treating micro and macrocystic orbital and/or adnexal lymphatic malformations (OA-LM) with protocol-based bleomycin sclerotherapy.

Methods

A retrospective interventional study of 69 eyes having OA-LM treated with non-image guided transcutaneous or transconjunctival bleomycin sclerotherapy (1IU/ml aqueous solution) between December 2014 and December 2018. Based on clinical regression, the outcomes were classified as excellent, good, fair and poor.

Results

The mean age at presentation was 20 ± 16 years (median 16; range 1 month to 70 years). The orbital-palpebral variant was the most common presentation, seen in 29 patients (42%). Microcystic morphology was seen in 34(49%), macrocystic in 22 (32%) and mixed cyst in 13 (19%) patients. Mean units of bleomycin injected per patient were 9 ± 8 IU (median 5.5 IU, range 1-38 IU). Mean number of treatment sessions required were 2 ± 1 (median 2, range 1-6). The response was excellent in 43 (62%), good in 12 (17%), fair in 9 (13%) and poor in 5 (7%) patients. These responses were comparable across the morphological subgroups (p = 0.24, chi-square test). Adverse reactions noted were inflammation in 11 eyes (16%) and peri-ocular pigmentation in 15 (22%). There was a sustained tumour regression over a mean follow-up duration of 3.5 years (median 3; range 1.5-5 years).

Conclusions

Seventy-nine percent of eyes with OA-LM showed a good outcome with transcutaneous and/or transconjunctival non-image guided bleomycin sclerotherapy with no serious adverse events. The results were promising over long-term follow-up.

SUBMITTER: Dave TV

PROVIDER: S-EPMC8956730 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature

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Publications

Long term outcomes of transcutaneous non-image guided bleomycin sclerotherapy in orbital/adnexal lymphatic malformations: a protocol-based management in 69 eyes.

Dave Tarjani Vivek TV Madhuri Bejjanki Kavya BK Laghmisetty Srujana S Tripathy Devjyoti D Kaliki Swathi S Rath Suryasnata S Mohapatra Samir S Desai Akruti A Kapoor Anasua Ganguly AG

Eye (London, England) 20210420 4

<h4>Objectives</h4>To study the efficacy and the long-term outcomes of treating micro and macrocystic orbital and/or adnexal lymphatic malformations (OA-LM) with protocol-based bleomycin sclerotherapy.<h4>Methods</h4>A retrospective interventional study of 69 eyes having OA-LM treated with non-image guided transcutaneous or transconjunctival bleomycin sclerotherapy (1IU/ml aqueous solution) between December 2014 and December 2018. Based on clinical regression, the outcomes were classified as exc ...[more]

PMID: 33879854

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Project description:PurposeConventional modalities of external-beam radiation therapy (EBRT) are associated with high incidences of severe vision-threatening ocular and orbital toxicities when used to treat orbital malignancies. We investigate toxicities associated with high-dose volumetric modulated arc therapy (VMAT), a commonly used contemporary treatment modality for these tumors.MethodsRetrospective analysis of malignant orbital tumors managed with adjuvant high-dose VMAT preceded by globe-salvaging surgical therapy (GST) or exenteration. Dosimetric quantitation of target volumes and critical structures was performed. Incidence and severity of ocular, orbital, and adnexal toxicities were evaluated and assessed with regard to conventional EBRT toxicities for orbital malignancies described in the literature.ResultsEighty-four subjects (mean age = 65.9 ± 9.7 years) were included (N = 48 and N = 36 in GST and exenteration subgroups, respectively). Mean dose was 64.8 ± 2.1 Gy to the planning target volume. Dosing to critical structures typically did not surpass known tissue tolerance limits. Median follow up was 18.3 months. Visual acuity in the GST subgroup was not significantly different after VMAT (0.25 ± 0.06) compared with baseline (0.23 ± 0.02; P = 0.302). Whereas severe toxicities reported by major systematic analyses in the literature with older EBRT modalities were relatively common-for example, retinopathy (16-40%), optic neuropathy (16%), and corneal perforation (13%)-toxicities with VMAT were typically mild and less common. The most common toxicities with VMAT were mild dry eye (81.3%; 39/48), cataract (21.1%; 8/38 phakic eyes), and periocular dermatitis (15.5%; 13/84). Vision-threatening toxicities, including severe corneal pathologies, retinopathy, or optic neuropathy, were rare. There were no contralateral ocular or adnexal toxicities.ConclusionsHigh-dose VMAT for orbital malignancies demonstrated low incidence and severity of eye-related toxicity, in contradistinction to adverse events reported from conventional forms of radiotherapy.

Project description:Lymphatic malformations (LM) are characterized by the overgrowth of lymphatic vessels during pre- and postnatal development. Macrocystic, microcystic and combined forms of LM are known. The cysts are lined by lymphatic endothelial cells (LECs). Resection and sclerotherapy are the most common treatment methods. Recent studies performed on LM specimens in the United States of America have identified activating mutations in the phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA) gene in LM. However, whole tissue but not isolated cell types were studied. Here, we studied LM tissues resected at the University Hospitals Freiburg and Regensburg, Germany. We isolated LECs and fibroblasts separately, and sequenced the commonly affected exons 8, 10, and 21 of the PIK3CA gene. We confirm typical monoallelic mutations in 4 out of 6 LM-derived LEC lines, and describe two new mutations i.) in exon 10 (c.1636C>A; p.Gln546Lys), and ii.) a 3bp in-frame deletion of GAA (Glu109del). LM-derived fibroblasts did not possess such mutations, showing cell-type specificity of the gene defect. High activity of the PIK3CA-AKT- mTOR pathway was demonstrated by hyperphosphorylation of AKT-Ser473 in all LM-derived LECs (including the ones with newly identified mutations), as compared to normal LECs. Additionally, hyperphosphorylation of ERK was seen in all LM-derived LECs, except for the one with Glu109del. In vitro, the small molecule kinase inhibitors Buparlisib/BKM-120, Wortmannin, and Ly294002, (all inhibitors of PIK3CA), CAL-101 (inhibitor of PIK3CD), MK-2206 (AKT inhibitor), Sorafenib (multiple kinases inhibitor), and rapamycin (mTOR inhibitor) significantly blocked proliferation of LM-derived LECs in a concentration-dependent manner, but also blocked proliferation of normal LECs. However, MK-2206 appeared to be more specific for mutated LECs, except in case of Glu109 deletion. In sum, children that are, or will be, treated with kinase inhibitors must be monitored closely.

Dataset Information

Long term outcomes of transcutaneous non-image guided bleomycin sclerotherapy in orbital/adnexal lymphatic malformations: a protocol-based management in 69 eyes.

Objectives

Methods

Results

Conclusions

Publications

Long term outcomes of transcutaneous non-image guided bleomycin sclerotherapy in orbital/adnexal lymphatic malformations: a protocol-based management in 69 eyes.

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