CASE REPORT: This article describes a case of a 41-year-old male, a chronic smoker with an actively bleeding, ulcerated, solitary, firm lesion on the lateral border of the tongue which had bled thrice before. A differential diagnosis of pyogenic granuloma, haemangioma, fibroma, nerve sheath tumour, salivary gland tumour and malignancy was made and surgically excised. Histopathology of the excised specimen revealed a well-circumscribed lesion with spindle-shaped cells arranged in interlacing fascicles and with the help of immunohistochemical markers confirmed it to be a PEN.
DISCUSSION: To our knowledge, this is the first description of an ulcerated PEN presented with an active bleed.
METHODS: An international, multidisciplinary panel of 23 experts in orbital tumor surgery was formed. A modified Delphi method was used to develop the cavernous hemangioma exclusively endonasal resection (CHEER) staging system with a total of 2 rounds being completed.
RESULTS: Tumors medial to a plane along the long axis of the optic nerve may be considered amenable for an exclusively endonasal resection. In select cases, tumors may extend inferolaterally if the tumor remains below a plane from the contralateral naris through the long axis of the optic nerve (ie, plane of resectability [POR]). This definition reached consensus with 91.3% of panelists in agreement. Five stages were designed based on increasing technical resection difficulty and potential for morbidity. Stages were based on the relationship of the tumor to the extraocular muscles, the inferomedial muscular trunk of the ophthalmic artery (IMT), and orbital foramina. Staging by anatomic location also reached consensus with 87.0% of panelists in agreement. Size was not included in the staging system due to the lack of agreement on the contribution of size to resection difficulty.
CONCLUSION: Endoscopic orbital tumor surgery is a nascent field with a growing, yet heterogeneous, body of literature. The CHEER staging system is designed to facilitate international, high-quality, standardized studies establishing the safety, efficacy, and outcomes of endonasal resection of OCH.
DESIGN: Retrospective interventional case series.
PARTICIPANTS: Five patients with visual disturbances resulting from a benign, well-circumscribed orbital apex tumor (3 cases of cavernous hemangioma and 2 cases of schwannoma).
METHODS: Each patient treated with GKRS with a total radiation dose of 20 Gy in 4 sessions (5 Gy in each session with an isodose line of 50%) delivered to the tumor margin.
MAIN OUTCOME MEASURES: Best-corrected visual acuity, visual field changes, orbital imaging, tumor growth control, and side effects of radiation.
RESULTS: All patients demonstrated improvement in visual acuity, pupillary responses, color vision, and visual field. Tumor shrinkage was observed in all patients and remained stable until the last follow-up. No adverse events were noted during or after the radiosurgery. None of the patients experienced any radiation-related ocular morbidity.
CONCLUSIONS: From this experience, multisession GKRS seems to be an effective management strategy to treat solitary, benign, well-circumscribed orbital apex tumors.