Primary vascular bone tumors in the spine: a challenge for pathologists and spine oncology surgeons

Abstract

Purpose Primary vascular bone tumors of the spine represent a challenge for oncology surgeons, due to the need of planning a surgical strategy appropriate to the tumor behavior. But these tumors represent a challenge also for pathologists, as immunohistochemical and molecular analyses have recently refined the terminology. Methods A cohort of 81 cases was retrospectively reviewed, targeting the evolution of diagnoses and the treatment-related outcome. Sixty-six cases (including one case originally diagnosed as chordoma) were diagnosed before 2011, and 15 were diagnosed and treated in the period 2011-2017. Fully documented outcome studies are available for 46 patients whose immunohistochemical and molecular analyses were available. The follow-up ranges from 6 months of the early patient death to 300 months of the longest disease-free survival. Results The outcome was related not only to the treatment performed, but also to the evolution of diagnoses. The term Hemangioendothelioma that defined a benign aggressive (Enneking stage 3) tumor is now obsolete and replaced by the diagnosis of Epithelioid Hemangioma for benign aggressive tumor and Epithelioid Hemangioendothelioma for low-grade malignant tumor. En bloc resection was appropriate for local control, but 2 out of 7 cases of epithelioid Hemangioendotheliomas died due to the spread of the disease None of the Hemangiomas recurred after intralesional excision with or without radiotherapy, or after vertebroplasty. Both cases of Angiosarcoma had a fast and lethal evolution. Conclusions Surgeons must be aware of the evolution of terminology to decide the most appropriate treatment options. Graphical abstractThese slides can be retrieved from Electronic Supplementary Material. Key points1. Immunohistochemical and molecular analysis have recently refined the terminology of Primary Vascular Bone Tumors, a further ado for spine oncology surgeons who must plan proper treatment over the constraints of surgical anatomy.2. Out of a cohort of 85, this study focuses on 46 cases of Primary Vascular Bone Tumors of the spine confirmed by immunohistochemical and molecular analysis.

A Clinical Model of Bone Angiosarcoma Patients: A Population‐based Analysis of Epidemiology, Prognosis, and Treatment

“…BA is a high-grade malignant tumor (Enneking Stage II, mostly B as extracompartmental), different from hemangioma and hemangioendothelioma 4 4 . Because of the histological differences, the prognosis of BA also intuitively differs from those of other types of malignant vascular bone tumors, including epithelioid hemangioendothelioma, Kaposi sarcoma, and hemangiopericytoma 4,25 . However, this speculation requires support from data from large-scale databases.…”

“…BA is a high‐grade malignant tumor (Enneking Stage II, mostly B as extracompartmental), different from hemangioma and hemangioendothelioma 4 . Hemangiomas are nearly benign tumors and most are asymptomatic.…”

“…Angiosarcoma commonly arises in soft tissue, viscera, and skin rather than bone 2 . Compared to hemangioma as a benign tumor, epithelioid hemangioendothelioma as a low‐grade malignant tumor, and hemangioendothelioma as the intermediate condition, angiosarcoma is a high‐grade malignancy 4 . Angiosarcoma patients have a poor prognosis, with a reported 5‐year survival rate of approximately 31% 5 ; the survival of angiosarcoma occurring in the bone is significantly poorer than that of general angiosarcoma, with a 20% 5‐year survival rate and approximately 10‐month survival time 6, 7 .…”

Rare Primary Malignant Bone Sarcomas

“…Data on treatment and survival of bone angiosarcoma are scars, often presented together with soft tissue lesions [65] and EHE [63] or as case reports [66,67].…”

“…Boriani et al [ 63 ] presented a cohort of 81 patients with vascular tumor of the spine including benign and malignant entities. Of the seven epithelioid hemangioendotheliomas, four were treated by en bloc resection without any other adjuvant treatments; patients presented with multiple bone and soft tissue lesions, survival patterns were very different, and successful treatment with intralesional resection with radiotherapy in some cases…”