Dataset Information

Alveolar soft part sarcoma of the lateral rectus muscle: Suture technique to prevent postoperative strabismus.

ABSTRACT:

Purpose

We present a rare case of intramuscular alveolar soft part sarcoma (ASPS) of the lateral rectus (LR) muscle and the surgical technique used to maintain orthotropia after complete resection of the mass.

Observations

A 5-year-old boy presented with progressive proptosis of the left eye due to an orbital tumor. The patient was previously diagnosed with ASPS of the left LR muscle from an incisional biopsy, and the tumor size increased despite 5 cycles of chemotherapy prior to presenting to our center. Magnetic resonance imaging showed a 28x19x15mm-sized contrast-enhancing intramuscular mass of the left LR muscle, and there was no evidence of nodal or distant metastasis. The mass was excised en bloc, along with the insertion and the posterior normal part of LR muscle. To maintain proper eye alignment after resecting LR muscle, a 4-0 Prolene® hang-back suture was placed between the scleral insertion and the periorbita of the posterior orbit and the left medial rectus muscle was injected with botulinum toxin. During the follow-up of 51 months after surgery, the patient had no evidence of recurrence or metastasis and remained orthotropic in primary gaze, with a good cosmetic result.

Conclusions and importance

ASPS of extraocular muscles is a rare tumor occurring mainly in children and young adults, and treatment may cause significant sequelae such as orbital exenteration, radiation-induced complications, and large-angle strabismus. Complete resection of tumor including the extraocular muscle is essential for treatment, and a subsequent reconstruction using a hang-back suture technique is useful to achieve proper eye alignment as well as a good cosmetic outcome.

SUBMITTER: Al Ghulaiga FM

PROVIDER: S-EPMC7163059 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BACKGROUND:Alveolar soft part sarcoma (ASPS) is an exceedingly rare and orphan disease, without active drugs approved in the front line. Pazopanib and trabectedin are licensed for sarcoma treatment from second-line, but very little and contradictory data are available on their activity in ASPS. Lacking ongoing and/or planned clinical trials, we conducted a multi-institutional study involving the reference sites for sarcoma in Europe, U.S., and Japan, within the World Sarcoma Network, to investigate the efficacy of pazopanib and trabectedin. MATERIALS AND METHODS:From May 2007, 14 of the 27 centers that were asked to retrospectively review their databases had identified 44 advanced ASPS patients treated with pazopanib and/or trabectedin. Response was evaluated by Response Evaluation Criteria in Solid Tumors 1.1. Progression-free survival (PFS) and overall survival (OS) were computed by Kaplan-Meier method. RESULTS:Among 30 patients who received pazopanib, 18 were pretreated (13 with other antiangiogenics). Response was evaluable in 29/30 patients. Best responses were 1 complete response, 7 partial response (PR), 17 stable disease (SD), and 4 progressions. At a 19-month median follow-up, median PFS was 13.6 months (range: 1.6-32.2+), with 59% of patients progression-free at 1 year. Median OS was not reached.Among 23 patients treated with trabectedin, all were pretreated and evaluable for response. Best responses were 1 PR, 13 SD, and 9 progressions. At a 27-month median follow-up, median PFS was 3.7 months (range: 0.7-109), with 13% of patients progression-free at 1 year. Median OS was 9.1 months. CONCLUSION:The value of pazopanib in advanced ASPS is confirmed, with durable responses, whereas the value of trabectedin appears limited. These results are relevant to defining the best approach to advanced ASPS. IMPLICATIONS FOR PRACTICE:This retrospective study, conducted among the world reference centers for treatment of sarcoma, confirms the value of pazopanib in patients with advanced alveolar soft part sarcoma (ASPS), with dimensional and durable responses, whereas trabectedin shows a limited activity. Alveolar soft part sarcoma is resistant to conventional cytotoxic chemotherapy. Pazopanib and trabectedin are licensed for treatment of sarcoma from second line; in the lack of prospective clinical trials, these results are relevant to defining ASPS best management and strongly support initiatives aimed at obtaining the approval of pazopanib in the front line of the disease.

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