Cystic appearance - a new feature of solid fibrous tumours in the lacrimal gland: a case report with literature review

Abstract

Background Solitary fibrous tumours (SFTs) rarely occur in the orbit, especially in the lacrimal area. These tumours are mostly solid. Cystic changes have been documented, but they remain very rare. Only three cases of primary orbital solitary fibrous tumours with cystic changes have been reported in the literature, but no cases have been reported to occur in the lacrimal gland. Solitary fibrous tumours generally follow a benign course and are treated definitively with surgical excision. Data from the literature suggest that the cystic nature of SFT presents a risk of recurrence and could be a harbinger of malignancy. Case presentation A 42-year-old woman was admitted to the endocrinology department for right unilateral exophthalmia and epiphora in the last 8 months. An ophthalmological evaluation showed exophthalmia only in the right eye (22 mm) and normal visual acuity, visual field and extraocular movements. Investigations revealed normal thyroid function. Orbital magnetic resonance imaging detected a 4 × 2,2 × 2,7 cm septate pseudocystic mass in the right lacrimal gland. Given her lacrimal gland tumour diagnosis, the patient was submitted for neurosurgical intervention with total ablation of the tumoural mass and complete right dacryoadenectomy. Although the intraoperative extemporaneous examination results were suggestive of a haemangiopericytoma, histological and immunocytochemical examination showed an extrapleural SFT. The postoperative clinical evolution was favourable, with remission of the exophthalmia. Fifteen months after surgery, no signs of recurrence were noticed. Conclusions We report the first case of an SFT with cystic changes in the lacrimal gland. Although the presence of cavitary lesions alone does not necessarily indicate aggressive behaviour, cystic changes pose a risk of recurrence and may suggest malignant transformation over time. As a result, our case requires long-term follow-up due to recurrence and malignant potential.

Giant cell-rich solitary fibrous tumour of the lacrimal gland with prominent angiomatoid cystic changes and an underlying NAB2ex3-STAT6ex18 fusion

“…Although orbital SFTs are richly vascularised, extensive cystic change is unusual and may pose a diagnostic difficulty 14–19. Rare entirely cystic examples may lead to confusion with other entities such as vascular tumours or even a hydatid cyst in one report 12 15 18.…”

Cystic Lesions of the Orbit

“…In addition to the more common cystic neoplasms, case studies have described cystic morphology in primary orbital solitary fibrous tumor, meningioma, pilomatrixoma, and synovial sarcoma, among others 127–131 . Cystic orbital metastases have been described in a diverse range of primary diseases, including melanoma, breast, neuroendocrine, bladder, renal cell, and endometrial cancers 122,132–134 …”

“…The approach to management depends upon several factors, including the type, location, and stage of disease. Management generally involves complete surgical resection of the tumor, ensuring the capsule remains intact to reduce the risk of recurrence 127,135 . In SCC and schwannoma, this may require sacrifice of the involved nerve 123 .…”

“…[124][125][126] In addition to the more common cystic neoplasms, case studies have described cystic morphology in primary orbital solitary fibrous tumor, meningioma, pilomatrixoma, and synovial sarcoma, among others. [127][128][129][130][131] Cystic orbital metastases have been described in a diverse range of primary diseases, including melanoma, breast, neuroendocrine, bladder, renal cell, and endometrial cancers. 122,[132][133][134] Though the exact pathogenesis leading to cystic degeneration is not well understood, the process is likely provoked by necrotic destruction and consequent thrombosis of tumor vasculature, resulting in intralesional hemorrhage and cyst formation.…”

“…Management generally involves complete surgical resection of the tumor, ensuring the capsule remains intact to reduce the risk of recurrence. 127,135 In SCC and schwannoma, this may require sacrifice of the involved nerve. 123 In the case of malignancy, adjuvant radiotherapy, chemotherapy, or immunotherapy may be required.…”