Epithelioid Hemangioma Of Bone Research Articles

Epithelioid hemangioma of the acromion causing shoulder pain: a case report.

Epithelioid hemangioma of bone is a rare benign vascular tumor. Because of the locally destructive clinical presentation and the presence of atypical histologic features with increased mitotic activity and necrosis, it is often misdiagnosed as low-grade malignant epithelioid hemangioendothelioma or high-grade malignant epithelioid angiosarcoma. Correct diagnosis through imaging studies and histopathological examination is mandatory to determine the appropriate course of treatment, as the prognosis differs from that of other malignant hemangioma tumors. A 69-year-old male who presented with intractable shoulder pain caused by epithelioid hemangioma in the acromion of the scapula was treated with tumor curettage. This paper reports a good result with a review of the relevant literature.

Epithelioid haemangioma of bone: A series of four cases with a revision of this contentious entity.

Epithelioid hemangioma of bone is a rare and locally aggressive vascular neoplasm of bone associated with a good prognosis. Because of its worrisome histomorphologic features and aggressive clinicoradiologic findings, at times with multifocal presentation, they tend to simulate malignant tumors. We report a series of four cases of epithelioid hemangioma of bone with their clinicopathologic characteristics. All had adjacent soft tissue involvement and two had multifocal bone disease. Microscopically, all cases had a tumor in lobular configuration, composed of epithelioid endothelial cells with the formation of well-formed vessels or grew in solid sheets. The tumor cells lacked significant cytologic atypia, necrosis, and increased mitosis. All cases were immunohistochemically positive for vascular markers CD34, CD31, ERG1, whereas negative for CK. Two of the cases were treated with excision, and the other two underwent curettage. None had local recurrence or metastasis on follow-up. This study highlights the importance of recognizing histomorphological and clinicoradiological features for distinguishing epithelioid hemangiomas from malignant vascular neoplasms of bone because of their distinct therapeutic implications and clinical outcomes.

Epithelioid Hemangioma of Bone: A Rare Vascular Neoplasm. A Case Report and Literature Review

Epithelioid hemangioma (EH) of bone is a rare benign, albeit locally aggressive vascular neoplasm. It is usually solitary and involves the metaphysis or diaphysis of long tubular bones, especially in the lower extremities. Rarely it may present as multifocal lesions. The differential diagnosis includes malignant vascular bone tumors such as epithelioid hemangioendothelioma and epithelioid angiosarcoma. Clinical presentation and radiographic and histological findings are not specific and diagnosis is based mostly on immunohistochemical and molecular studies. There is no consensus regarding the optimal treatment. Curettage and bone grafting or en bloc resection are the current treatment options, however local recurrence have been reported. We present a case of multifocal EH of the distal tibia, distal fibula and hindfoot in a 38 year-old male managed with curettage, radiofrequency ablation and cement osteoplasty. The imaging features, histological findings and treatment options of this rare vascular tumor are discussed.

Fusion events lead to truncation of FOS in epithelioid hemangioma of bone.

Epithelioid hemangioma of bone is a locally aggressive vascular neoplasm. It can be challenging to diagnose because of the wide histological spectrum, which can make it difficult to differentiate from other vascular neoplasms such as epithelioid hemangioendothelioma or epithelioid angiosarcoma. COBRA-FISH karyotyping identified a balanced t(3;14) translocation. Transcriptome sequencing of the index case and two other epithelioid hemangiomas revealed a recurrent translocation breakpoint involving the FOS gene, which was fused to different partners in all three cases. The break was observed in exon 4 of the FOS gene and the fusion event led to the introduction of a stop codon. In all instances, the truncation of the FOS gene would result in the loss of the transactivation domain (TAD). Using FISH probes we found a break in the FOS gene in two additional cases, in none of these cases a recurrent fusion partner could be identified. In total, FOS was split in 5/7 evaluable samples. We did not observe point mutations leading to early stop codons in any of the 10 cases where RNA was available. Detection of FOS rearrangement may be a useful diagnostic tool to assist in the often difficult differential diagnosis of vascular tumors of bone. Our data suggest that the translocation causes truncation of the FOS protein, with loss of the TAD, which is thereby a novel mechanism involved in tumorigenesis.

Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

The controversy surrounding diagnosis of an epithelioid hemangioma (EH), particularly when arising in skeletal locations, stems not only from its overlapping features with other malignant vascular neoplasms, but also from its somewhat aggressive clinical characteristics, including multifocal presentation and occasional lymph node involvement. Specifically, the distinction from epithelioid hemangioendothelioma (EHE) has been controversial. The recurrent t(1;3)(p36;q25) chromosomal translocation, resulting in WWTR1-CAMTA1 fusion, recently identified in EHE of various anatomic sites, but not in EH or other epithelioid vascular neoplasms, suggests distinct pathogeneses. We investigated the clinicopathologic and radiologic characteristics of bone and soft tissue EHs in patients treated at our institution with available tissue for molecular testing. Seventeen patients were selected after confirming the pathologic diagnosis and fluorescence in situ hybridization analysis for the WWTR1 and/or CAMTA1 rearrangements. Four patients had multifocal presentation. Most patients with EH of bone were treated by intralesional curettage. None of the patients died of disease and only four patients had a local recurrence. Our results, using molecular testing to support the pathologic diagnosis of EH, reinforce prior data that EH is a benign lesion characterized by an indolent clinical course with an occasional multifocal presentation and rare metastatic potential to locoregional lymph nodes. These findings highlight the importance of distinguishing EH from other malignant epithelioid vascular tumors as a result of differences in their management and clinical outcome. Level IV, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Multifocal Epithelioid Hemangioma with Reactive Bone Formation

A healthy 16-year-old male patient presented with several weeks of right ankle pain on weight-bearing, worsened with sporting activities, and relieved with rest. Radiographs demonstrated multiple well-marginated lytic lesions in the right distal tibia, and talar head and neck, with a further small intracortical lesion in the mid-to-distal anterior tibia. MRI showed multiple rounded low T1 and high T2 signal intensity lesions within the distal tibia and talus, with marked associated bone marrow edema. The radiologic diagnoses included multifocal hemangioendothelioma, multifocal hemangioma, angiosarcoma, atypical infection, Langerhans cell histiocytosis, or osteoblastomatosis. Histology showed sheets of epithelioid cells lining vascular structures in a hobnailed “tombstone-like” arrangement, with additional areas containing trabeculae of osteoid and woven bone, with prominent osteoblastic rimming. The differential diagnosis at this point was either multifocal epithelioid hemangioma of bone or osteoblastomatosis. Immunohistochemistry showed strong expression of the vascular marker CD31, supporting a vascular neoplasm. The final diagnosis was multifocal epithelioid hemangioma with prominent reactive bone formation, an extremely unusual tumor with only one other similar case of multifocal disease reported in the literature. The patient was treated with curettage, cryosurgery, and bioceramic replacement and is currently doing well.

Epithelioid Hemangioma of Bone Revisited

The clinical and pathologic features of 50 epithelioid hemangiomas of bone are analyzed. There were 29 males and 21 females who ranged in age from 10 to 75 (mean 35) years. The tumors arose in long tubular bones (40%), short tubular bones of the distal lower extremity (18%), flat bones (18%), vertebrae (16%), and small bones of the hands (8%). Nine patients (18%) had involvement of more than 1 bone. Radiographically, the lesions were lucent and well marginated. Microscopically, the neoplasms had a lobular architecture and were composed of epithelioid endothelial cells that formed obvious vascular lumina or grew in solid sheets. No hyalinized or solid appearing extracellular myxoid matrix was present. Thirty-five patients were treated with curettage, 13 patients had a local resection and 2 patients only had a biopsy. One patient had local lymph node involvement. Three patients were treated with surgery and radiation therapy. Follow-up information revealed that 4 patients experienced a local recurrence; and 1 patient developed limited involvement of a regional lymph node. Epithelioid hemangioma of bone is a benign lesion that may be multifocal and affect separate tissue and is successfully treated with curettage or marginal en bloc excision.