What is the recommended treatment for a primary lacrimal gland sarcoma?

Treatment of Lacrimal Gland Sarcoma

Primary lacrimal gland sarcomas should be managed according to soft tissue sarcoma protocols with surgical resection as the cornerstone of treatment, followed by adjuvant radiotherapy for most cases, and referral to a specialist sarcoma multidisciplinary team is mandatory. 1

Critical Initial Management Principles

All patients with suspected lacrimal gland sarcoma must be referred to a specialist sarcoma MDT before biopsy or surgical treatment. 1 The lacrimal gland should be considered analogous to a minor salivary gland for reporting purposes, and treatment should follow established soft tissue sarcoma guidelines rather than protocols for epithelial lacrimal gland malignancies (such as adenoid cystic carcinoma). 1

Pre-Treatment Workup

• Obtain a pre-treatment histopathological diagnosis via percutaneous core biopsy, reviewed by a specialist sarcoma pathologist for diagnostic confirmation and appropriate molecular/genomic analysis. 1
• Perform cross-sectional imaging of the primary tumor with MRI prior to definitive surgery. 1
• Obtain CT chest imaging to evaluate for lung metastases before radical treatment. 1

Surgical Management

Surgery is the standard treatment for localized lacrimal gland sarcoma and must be performed by a surgeon with appropriate sarcoma training. 1

Surgical Approach

• The primary aim is complete tumor excision with a margin of normal tissue—wide excision through normal uninvolved tissues is the procedure of choice. 1
• A rim of at least 1 cm of normal tissue around the tumor is the target, though margins can be minimal against resistant anatomic barriers such as periosteum or bone. 1
• With adjuvant radiotherapy, a close but tumor-free margin (R0) may be adequate; occasionally a planned marginal or microscopically positive margin against a critical structure plus radiotherapy is acceptable to preserve function. 1
• Orbital exenteration (removal of eye and orbital contents) may be necessary in some cases but should not be performed reflexively—eye-sparing surgery with adjuvant radiation can achieve similar survival outcomes with better functional results. 2

Management of Inadequate Initial Surgery

• If inadvertent surgery resulted in unplanned positive margins, fully stage the patient and obtain MRI of the surgical bed to assess for gross residual disease. 1
• Re-excision should be performed if adequate margins can be achieved with acceptable morbidity and no gross residual disease is present. 1
• If re-excision would cause considerable morbidity or is unlikely to achieve clearance, radiotherapy alone may be the appropriate strategy rather than further surgery. 1

Adjuvant Radiotherapy

Pre- or postoperative radiotherapy is recommended along with surgical resection for the majority of patients with intermediate and high-grade tumors. 1

Radiotherapy Dosing and Timing

• Postoperative radiotherapy should be administered at 60-66 Gy using the best available technique, with fractions of 1.8-2 Gy, possibly with boosts depending on presentation and surgical quality. 1
• Preoperative radiotherapy is typically 50 Gy and offers advantages in long-term functional outcome with equivalent disease control compared to postoperative radiotherapy, though with increased risk of acute postoperative wound complications. 1
• Radiotherapy is indicated for deep tumors >5 cm as standard treatment, and is an option for selected cases of deep lesions ≤5 cm or low-grade tumors. 1

Role of Chemotherapy

Adjuvant chemotherapy is not routinely recommended for soft tissue sarcomas but should be considered in situations where local control is compromised or prognosis is poor, particularly in chemosensitive sarcoma subtypes. 1

When to Consider Chemotherapy

• Consider neoadjuvant or adjuvant chemotherapy for high-risk patients (G2-3, deep, >5 cm tumors) using risk stratification tools like Sarculator, where 5-year predicted survival <60% may indicate potential benefit. 1
• Histological subtype should guide decision-making, as some sarcoma types are more chemosensitive (e.g., synovial sarcoma, myxoid round cell sarcoma) while others are less responsive. 1
• If chemotherapy is used, it may be administered preoperatively to facilitate surgery and assess tumor response. 1

Chemotherapy for Metastatic Disease

• For extensive disease with synchronous lung metastases without extrapulmonary disease, chemotherapy is standard treatment. 1
• Anthracycline-based chemotherapy, specifically single-agent doxorubicin (75 mg/m² every 3 weeks), is the standard first-line treatment for metastatic soft tissue sarcomas. 3
• After achieving tumor response with chemotherapy, surgery of completely resectable lung metastases may be offered. 1

Follow-Up Protocol

Patients with intermediate or high-grade sarcoma should be followed every 3-4 months for the first 2-3 years, then twice yearly for up to 5 years, and annually thereafter for a total of 8-10 years. 1

Follow-Up Components

• Standard follow-up should include clinical history, clinical examination to detect local recurrence, and chest imaging (CT scan) to detect pulmonary metastases. 1
• MRI of the primary site should be performed if local recurrence is suspected clinically. 1

Common Pitfalls to Avoid

• Do not treat lacrimal gland sarcomas with protocols designed for epithelial lacrimal gland carcinomas (adenoid cystic carcinoma)—these are fundamentally different diseases requiring different management. 1
• Do not perform orbital exenteration as routine treatment—eye-sparing surgery with adjuvant radiotherapy achieves similar survival with better functional outcomes in selected patients. 2
• Do not delay referral to a specialist sarcoma MDT—all treatment decisions regarding surgery, chemotherapy, and radiotherapy timing should be made by the sarcoma MDT. 1
• Do not combine surgery with radiotherapy unnecessarily—single-modality treatment is effective for limited-stage disease when adequate margins can be achieved. 1