Subdural Hematoma Caused by Epithelioid Angiosarcoma Originating from the Skull

Abstract

Cancers metastatic to the skull or dura may cause subdural hematoma (SDH). However, the frequency is low, and the presence of underlying cancers has almost always been known in such situations. We report a case of skull angiosarcoma manifesting as SDH, posing a diagnostic challenge to physicians. A 75-year-old man visited our clinic with sensorimotor disturbance of gradual onset approximately 1 month after a minor head trauma. He was diagnosed with SDH after imaging studies, and underwent surgery to evacuate the hematoma. Because the hematoma was organized, surgery was switched from burr-hole drainage to craniotomy. The bone flap as well as the dura over the hematoma had grossly normal appearance, and only the hematoma itself was submitted for histological examination. Although postoperative recovery was uneventful, the patient experienced recurrence of the SDH 2 months after surgery. At the second surgery, the bone flap and dura were intermingled with tumor tissue, and histological examination revealed that an epithelioid angiosarcoma originating from the skull was responsible for the SDH. Timely diagnosis of angiosarcoma manifesting as SDH is difficult because of its rarity. In retrospect, however, the diagnosis might have been established earlier if the bone flap and/or the dura had been biopsied at the time of the first surgery. The present case gives us a lesson that SDH may be an unusual manifestation of malignant tumors of skull or dural origin, and histological examination of not only hematoma capsule but also of the surrounding tissues may provide important diagnostic clues.

Primary epithelioid angiosarcoma of the temporal bone with initial presentation of otalgia

“…To the authors' knowledge, only two cases that originated from the skull have been described in the literature. 8,10 Our case was unique in that otalgia was the sole symptom at initial presentation. Due to the indolent course and rarity of an epithelioid angiosarcoma from skull origin, it is very unusual for a clinician to list skull malignancy on the top of the differential diagnosis list.…”

“…3 The majority of epithelioid angiosarcomas occur in deep soft tissue 4 and in elderly patients, with a male predominance. However, epithelioid angiosarcoma does occasionally arise from vessel-rich organs [5][6][7][8] and large caliber vessels. 9 Epithelioid angiosarcoma arising from the skull is rarely reported in the literature ( Table 1).…”

“…Yamada et al reported one epithelioid angiosarcoma case that originated from the left temporal bone with subdural hematoma as the initial presentation without invasion into the EAC. 8 Cui et al reported one case that most likely originated from the right mastoid process and/or right external canal with right facial nerve paralysis and otalgia as the initial symptoms. 10 The typical histology of epithelioid angiosarcoma includes anastomosing vascular channels lined by large atypical malignant cells with hyperchromatic nuclei and sheets of malignant epithelioid cells with abundant eosinophilic cytoplasm and round nuclei with prominent nucleoli.…”

Primary angiosarcoma of the skull: A rare case report

“…[26] The treatment should include complete surgical excision with a wide bony margin followed by adjuvant radiotherapy, which in our case has given a good locoregional control even at the end of 2 years. However, these patients should be followed up with repeated scans yearly to rule out locoregional as well as distant recurrence.…”

“…Angiosarcoma of the skull is a rare entity with less than 20 cases reported in literature. [ 26 ] It may present either as a primary tumor or as a metastasis of a distant extra-cranial angiosarcoma. Angiosarcomas have a poor prognosis and the prognosis is worst if the location is the skull.…”