Malignant Vascular Tumors of Temporal Bone: Review Article

Objective: The objective of this study was to present a review article about temporal bone vascular tumors. Data Sources: Published English-language literatures in PubMed and Google scholar. Review methods: PubMed and Google scholar were systematically searched using search terms: angisarcoma, temporal and bone, hemangioendothelioma, temporal and bone. Kaposi, sarcoma, temporal and bone, hemangiopericytoma, temporal and bone. Study Selection: We included studies about vascular tumors of temporal bone. Results: Fortyfive studies were included in this study.The results showed the most patients were male, mass was the common symptom, external ear was the most common site and surgery was the essential treatment. Conclusion: malignant vascular tumors of temporal bone are rare malignant sarcomas that should be considered in the differential diagnosis of temporal bone mass. Glob J Otolaryngol 2(3): GJO.MS.ID.555589 (2016) 00


Introduction
Malignant vascular tumors are extremely rare in children and adults. Vascular neoplasms are the most common subcutaneous deep soft tissue sarcoma and in pediatric age. Malignancy is only seen in 2% of blood vessel tumors. They include angiosarcoma, hemangioendothelioma, Kaposi sarcoma and hemangiopericytoma A review by the University of Minnesota revealed only four case of malignant vascular tumor among 228 vascular tumor (3 angiosarcoma and one Kaposi cancer). A review by the Memorial Sloan-Kettering Cancer Center included six patients less than 21 years diagnosed between 1970 and 1995: four were angiosarcomas and two malignant hemangioendotheliomas. Farr et al. [1,2] reported 21 cases of head and neck malignant vascular tumor, ten of them were angiosarcomas and the other eleven cases were hemangiopericytomas .The absolute and net five year cure rate was 25 % and was achieved by radical surgery alone.

Material and Methods
Literature review was conducted using PubMed (MEDLINE) and Google Scholar for English articles. The following keywords were used: angisarcoma, temporal and bone; hemangioendothelioma, temporal and bone; Kaposi, sarcoma, temporal and bone; hemangiopericytoma, temporal and bone.  female. There were 11 children and 36 adults. Angiosarcoma and hemangiendothelioma are seen more in child and young patients, while hemangiopercytoma and Kaposi sarcoma are seen more in adults (Figures 1-3) (Table 3).

D. Clinical course:
Kaposi sarcoma of temporal bone usually appear as mass in external ear, hemangioperacytoma appear like a glmous tumor or mass, angiosarcoma appear as CSOM, while there is no specific form for angiosarcoma ( Figure 6).

E.
Treatment: Surgery is the essential treatment for malignant vascular tumors, radiotherapy used for high degree tumors or close margins, chemotherapy used for angisarcoma and multiple lesions of Kaposi sarcoma. Interferon or cortisonmay be used for hemangioendotheloma. AIDS should be treated in positive patients with sarcoma Kaposi (Figures 7 & 8).

F.
Recurrence: There were 6 cases of recurrence 12% (one case of angiosarcoma, one case of hemangiopercytoma, one case of hemangioendothelioma and 3 cases of Kaposi sarcoma).

Angiography
Middle meningeal artery was the most common artery giving blood supply to tumors followed by posterior auricle artery (Figure 7).

Discussion
Hemangioendotheliomas are neoplastic proliferations of blood vessel endothelial cells, forming thin-walled blood vessels and sheets of neoplastic cells. These lesions have variable malignant behavior, depending on their microscopic features. Hemangioendotheliomas usually show poor histologic differentiation, nuclear a typia and the existence of mitotic figures. These tumors may be divided into three groups: grade I, grade II, and grade III. The diagnostic grades I through III [3][4][5][6][7] are based on the presence of abundant vascular channels lined by only mildly atypical neoplastic endothelial cells and rare mitoses (in grade I). Grade III lesions show the most anaplastic features.
The radiographic findings of hemangioendothelium usually demonstrate an osteolytic sharply demarcated lesion. Surgical resection is the treatment of choice for hemangioendothelioma, radiation is advised for high grade lesions and non-operable cases. Chemotherapy has no significant role in the treatment.

Global Journal of Otolaryngology
Some authors reported successful treatment with a interferon in two infants with hemangioendotheliomas [3].
KS is a vascular tumor that originates from vascular and lymphatic endothelium, it is characterized by a multifocal angiogenic process, frequently presenting as multiple vascular cutaneous and mucosal nodules. Nodal and visceral manifestations are usually observed in case of severe immuno suppression. Risk factors for KS development may include e diabetes, advanced age and the use of corticosteroid medication KS is also associated with viral infection such AIDS and HHV8. Typical lesions are reddish-purplish papules, maculae, or nodules. KS mostly occurs in elderly men as multiple bilateral cutaneous lesions of the lower extremities. The involvements of the head and neck area have been observed in only 14% of cases of KS with Only a few cases of ear involvement. A standard therapeutic guideline is not available; the treatment is depending on the subtype and stage of the neoplasm as well as on the immune status of the patient [15]. Pre-operative embolization may help to obtain a better surgical result. Surgical excision is the treatment of choice. Radiotherapy may be helpful after incomplete resection of the tumor. The role of chemotherapy in the treatment of the haemangiopericytoma is still unclear. The recurrence rate of the tumour is relatively high [31].
Angiosarcoma is a rare malignant sarcoma with endothelial differentiation. They represent only 0.5-1% of malignant primary bone neoplasia. Bone angiosarcom as usually occur in the limbs and less likely in the pelvis, ribs, and vertebra. The skull is an unusual site of angiosarcom with only few cases reported in the literature. Parietal and frontal bones are more commonly involved than the sphenoid, occipitaland temporal bones. Radiation therapy, arsenic exposure and previous trauma have not reported to be predisposing factors for skull angiosarcoma. Cranial angiosarcomas are often unicentric lesions. Most cases of skull angiosarcoma occurred in the third decade with a marked male predominance. Skull angiosarcoma often occur as growing mass or swelling, while the manifestations of temporal bone angiosarcoma are hearing loss, tinnitus, and otalgia with a mass in the temporal area. On CT scan, skull angiosarcoma usually appear as a well-demarcated lytic, hypervascularized and hemorrhagic mass. Angiosarcomas have variable malignant behavior related to their degree of vascular differentiation and microscopic appearance. Immunohistochemistry is necessary in poorly differentiated tumors for the identification of an endothelial lineage. Skull angiosarcomas have a rapid onset of symptoms and high percentage of local recurrence or metastasis. The prognosis of skull angiosarcoma is a poor due secondarily involvement of the meninges and the brain and the impossibility to perform a complete surgical resection. Extensive staging must be conducted in all cases at the time of diagnosis and the follow-up should include regular bone CT scan, cerebral imaging and chest radiography. Preoperative embolization may help to achieve a total excision of the tumor with minimal blood loss. The complete surgical removal of the tumor is the most effective treatment, but adequate margins are often difficult to achieve. Radiotherapy may be given as an adjunct to surgical therapy or as palliative treatment in non-operable cases of skull angiosarcoma. Adjuvant chemotherapy with paclitaxel may help the condition of patients with soft-tissue sarcoma of the face or scalp [41].

Conclusion
Malignant vascular tumors of temporal bone are very rare, mostly seen in adult male except hemangioendothelioma which is seen more in pediatrics, Kaposi sarcoma is the most commonly type of theses tumors, it is usually seen as multiple lesions associated with HIV or HSV * virus.. Mass and hearing loss are the most common symptoms. Middle meningeal artery was the most common artery giving blood supply. Surgery is the essential treatment with adjuvant radiotherapy and chemotherapy for selected cases. There is a local recurrence in 12% of cases (Table  4).