Spindle cell hemangioendothelioma: A neoplasm associated with Maffucci's syndrome

Abstract

A case of a 21-year-old male with Maffucci's syndrome is presented in which the patient developed multiple cutaneous spindle cell hemangioendotheliomas (SHE). Recent reports suggest an association between spindle cell hemangioendotheliomas (SHE) and Maffucci's syndrome. Our case supports the interpretation that SHE is associated with Maffucci's syndrome.

Spindle Cell Hemangioma

“…Probably, some cases of hemangiomas described in the dermatologic literature under the designation of zonal or dermatomal hemangiomas are examples of SCH [7, 8]. SCH can be associated with Maffucci’s syndrome, Klippel-Trenaunay syndrome, early-onset varicosities and congenital lymphedema [4, 5, 6, 9, 10, 11]. …”

Maffucci’s syndrome associated with spindle cell hemangioendothelioma

“…Spindle cell hemangioendothelioma is a recently described rare vascular tumor of borderline malignancy, showing a histologic picture of both cavernous hemangioma and Kaposi's sarcoma-like areas [3]. To our knowledge, there have been only 12 cases of multiple skeletal enchondromatosis with spindle cell hemangioendothelioma reported in the literature [3][4][5][6][7][8][9][10][11][12]. We report here an additional example of this peculiar vascular tumor occurring in a patient with multiple enchondromatosis.…”

“…A case of Maffucci's syndrome associated with spindle cell hemangioendothelioma was initially described by Weiss and Enzinger. We could find 11 other cases with this association of multiple enchondromatosis in the literature, some of which have been described repeatedly [4][5][6][7][8][9][10][11][12]. Scott and Rosai reported a case of such association that had developed in a 19-year-old woman, initially diagnosed as having acute myelomonocytic leukemia [4].…”

“…Furthermore, some of the vascular lesions in patients with spindle cell hemangioendothelioma have been reported to be present either at or shortly after birth [3,13]. Enchondromatosis is usually discovered during youth or young adulthood, and can also arise during the first decade of life [4][5][6][7]9]. Thus, the combination of this vascular lesion and multiple enchondromatosis may be explained as a form of congenital mesodermal dysplasia.…”

Spindle Cell Hemangioma Reoccurrence in the Hand: Case Report

“…Of the 34 cases in the literature involving the hand, the vast majority present as multiple lesions [9,13,20,34]. Multifocal SCH's have been associated with Maffucci's syndrome [13,17,32,46], Ollier disease [27], lymphadema [42,47], Klippel Trenaunay syndrome, and early onset varicose veins [13]. Of these, Maffucci's syndrome is the most common, and many advocate that a patient with multifocal SCH should be evaluated for enchondromas.…”