Thirteen-year follow-up of parasellar intravascular papillary endothelial hyperplasia successfully treated by surgical excision: case report

Abstract

<jats:p>Intracranial intravascular papillary endothelial hyperplasia (IPEH) is very rare, and to our knowledge long-term follow-up results have not been previously published. An 11-year-old boy presented with a 6-month history of progressive visual impairment in the right eye. Magnetic resonance imaging revealed a well-enhanced, large parasellar mass involving the cavernous sinus, right frontal skull base, and ethmoid and sphenoid sinuses. Frontotemporal craniotomy and subtotal resection were performed, and the diagnosis of IPEH was confirmed. The mass increased in size during the following 3 months. A second operation was performed via frontotemporal craniotomy combined with a transsphenoidal approach, and gross-total resection of the tumor was achieved. Adjuvant radiotherapy (5040 cGy) and chemotherapy with interferon were administered. The patient's visual symptoms improved, and there was no recurrence during a 13-year follow-up period. The results of this case indicate that intracranial IPEH can recur with subtotal resection; however, optimal resection with multimodal adjuvant treatment can control the disease for many years, if not permanently.</jats:p>

Intravascular papillary endothelial hyperplasia (Masson’s tumour) arising from the superior sagittal sinus masquerading as an aggressive malignant lesion: A rare case report

“…Throughout the literature, it has been termed as benign and most of them get cured by a simple wide local excision. Till date, only around 30 cases of IPEH have been reported which are intra-cranial in location (5)(6). No case of Masson's Tumour has been reported arising from the superior sagittal sinus aggressive enough to erode the overlying parietal bone to create an elliptical defect and protruded out on to the scalp confusing the surgeon initially to be a sebaceous cyst.…”

“…Remaining cases underwent subtotal resection with a fairly significant recurrence rate. As a consequence, most authors have advocated adjuvant therapy in cases of incomplete resection, mainly in the form of radiotherapy, gamma knife surgery or even chemotherapy (6).Some researchers have also hypothesised that previous radiotherapy may lead to occurrence of IPEH due to blood flow obstruction and thrombus formation (8).…”

Co-existence of Intravascular Papillary Endothelial Hyperplasia in Superficial Skin and the Intermuscular Space

“…Such regions were designated as "intravascular papillary endothelial hyperplasia" by Clearkin & Enzinger in 1976 (4). The most common clinical symptom is a slowly-growing nodule, with bluish or reddish discoloration of the overlying skin (2,5). IPEH may occur in any blood vessel of the body, but is more prevalent on the fingers, head, and the neck region.…”

“…Intravascular papillary endothelial hyperplasia (IPEH) accounts for approximately 2-4% of all vascular tumours of the skin and soft tissue (1). It was originally considered a neoplasm, but is currently recognized as an unusual non-neoplastic, reactive endothelial proliferation associated with an organizing thrombus (2). Histologically, IPEH is characterized by the papillary proliferation of endothelial cells within a vascular lumen (3).…”

Masson’s tumor of the pineal region: case report

“…10,13,19,21,22 Intracranial IPEH has been reported in the skull base, sellar regions, and the petrous part of the temporal bone, but never the pineal region. 5,13,14,22,24 Extracranial IPEH is typically found in skin and subcutaneous tissues of the extremities; however, IPEH also presents at other locations, including the head, neck, and trunk, 20,24 and in visceral organs. 13,21,22 About 2% of all vascular tumors and subcutaneous lesions of the head, neck, and extremities are classified as IPEH.…”