Spindle Cell Hemangioma

Abstract

A 27-year-old woman presented with multiple nodules closely grouped on her right upper distal extremity. The lesions, dating from childhood, increased slowly in time. Microscopic examination of one nodule showed the histologic features of spindle cell hemangioendothelioma (SCH). At the periphery of the nodule there were also some features of the so-called sinusoidal hemangioma. Clinically, SCH can present as a solitary lesion or as multiple lesions in zonal distribution. When the lesions are multiple, the diagnosis of Maffucci’s syndrome should be considered. SCH may be interpreted as a reactive process secondary to thrombosis and recanalization occurring in angiomatous lesions with different clinical presentations. Spindle cells are probably mesenchymal cells modified by blood pressure. For this entity the term hemangioma seems to be preferable to that of hemangioendothelioma.

Multiple mucinous and lipomatous variant of eccrine angiomatous hamartoma associated with spindle cell hemangioma: a novel collision tumor?

“…Individual lesions are asymptomatic or painful, smooth‐surfaced, skin‐colored to bluish, firm nodules, and show the potential for local or regional recurrence and multifocal slow growth 7,16 . SCH shows histopathologic features of dermal or subcutaneous nodules composed of thin‐walled dilated vascular structures containing inconsistently organized thrombi and phleboliths in their lumina, and more solid, cellular zones with fascicles of spindle cells of bland, mitotically inactive cells with tapering or rounded vesicular nuclei, admixed with small aggregates of plump, epithelioid cells, some of which with intracytoplasmic vacuoles 5,7 . Immunohistochemically, endothelial cells lining vascular spaces and epithelioid cells are positive for factor‐VIII‐related antigen, CD31, CD34, and Ulex europaeus I lectin, whereas the spindle cells are negative for these markers 5,16 …”

“…Although SCH initially presents as a distinctive low‐grade variant of angiosarcoma and can be classified into vascular tumors of intermediate or low‐grade malignant potential, it has been suggested that spindle cell hemangioendothelioma may be a non‐neoplastic, reactive process arising in association with a local congenital or acquired vascular malformation 4,7,16–18 . SCH can be associated with epithelioid hemangioendothelioma, congenital lymphedema, Maffucci's syndrome, Klippel–Trénaunay syndrome, early onset varicosities, Kasabach–Merritt syndrome, and arteriovenous fistula 4–7,19–21 . As we had evaluated bony abnormalities of our patient using MRI, possibility of Maffucci's syndrome can be ruled out.…”

“…Spindle cell hemangioma (SCH) was first described as spindle cell hemangioendothelioma, a distinctive low‐grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma 4 . Although originally believed to be a tumor with limited metastatic potential, it is regarded as a benign hemangioma with reactive process, frequently showing multifocal lesions 4,5 . Clinically, SCH presents as multiple nodules on the distal extremities and is occasionally associated with other conditions such as Maffucci's syndrome or Klippel–Trénaunay syndrome 4,6 .…”

The Dąbska Tumor: A Thirty-Year Retrospect

“…A number of neoplasms display intravascular proliferations [10, 11, 20, 21, 22, 23, 24, 25, 26, 27]. These include reactive angioendotheliomatosis, benign intravascular endothelial hyperplasia, angiosarcoma and retiform hemangioendothelioma.…”

Spindle Cell Hemangioma

“…Clinically, the lesions may occur solitary or multiple in zonal distribution. Most frequently, the single lesions are small, usually 0.5 to 2.0 cm, asymptomatic, bluish nodules with a livedoid pattern and of a firm consistence 1,3,6 . A case of multiple SCH extending over the entire body was described by Fletcher et al 7 Cases of multiple lesions have been diagnosed in association with Maffucci's syndrome 8,9 .…”