sirolimus and Orbital-Neoplasms

Currently, there is no ideal management for orbital lymphatic malformations. Significant advances have been made since the discovery of new agents in the treatment. The purpose of this manuscript is to review the recent evidence on new sclerotherapy agents and systemic medications.. Traditional sclerosants are OK-432, sodium tetradecyl sulphate and ethanol. More recent developments are the use of doxycycline, bleomycin, and pingyangmycin. Sirolimus as a systemic medication has revolutionized the medical management of lymphatic malformations. Other oral drugs such as propranolol and sildenafil are controversial. Future treatment involves targeting lymphangiogenic pathways including inhibition of vascular endothelial growth factors and the phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit.. The development of new agents allows multimodal management either as monotherapy or combined therapy to achieve better outcomes in this difficult to manage disease.

Topics: Angiogenesis Inhibitors; Antineoplastic Agents; Bevacizumab; Bleomycin; Doxycycline; Humans; Immunosuppressive Agents; Lymphangioma; Orbital Neoplasms; Picibanil; Sclerosing Solutions; Sclerotherapy; Sirolimus

We report the case of a 7-year-old boy who presented with a swollen right eye. Magnetic resonance imaging revealed a right intraconal orbital mass with intense contrast enhancement. Incisional biopsy led to a diagnosis of perivascular epithelioid cell tumor (PEComa). Sirolimus was initiated but discontinued at the third week of treatment because the tumor had progressed. A minor regression of the tumor was seen after six cycles of systemic chemotherapy. Previously reported cases of PEComa were benign in nature, and full remission was achieved with surgical excision. In the present case the tumor was malignant and responded only slightly to systemic chemotherapy.

Topics: Antibiotics, Antineoplastic; Child; Humans; Magnetic Resonance Imaging; Male; Orbit; Orbital Neoplasms; Perivascular Epithelioid Cell Neoplasms; Sirolimus; Treatment Outcome

Microcystic lymphatic malformations are difficult to treat surgically, especially when located in the orbital apex. Recently, pharmacologic inhibition of the mTOR pathway by sirolimus was reported as a safe and efficacious treatment option for lymphatic malformations (also known as lymphangiomas). We report the case of a young male patient in which a unilateral, retrobulbar lymphatic malformation regressed to a large extent under treatment with 1 mg sirolimus given orally twice a day over a period of six months.

Topics: Administration, Oral; Antibiotics, Antineoplastic; Humans; Lymphangioma; Magnetic Resonance Imaging; Male; Orbital Neoplasms; Sirolimus; Treatment Outcome; Young Adult