Primary vertebral epithelioid angiossarcoma—a rare case report and systematic literature review (original) (raw)

Multicentric epithelioid angiosarcoma of the spine: a case report of a rare bone tumor

Spine Journal, 2007

BACKGROUND CONTEXT: Epithelioid angiosarcoma (EA) is a high-grade sarcoma of vascular origin. EA is a rare variant of angiosarcoma. It is characterized by large cells with an epethelioid morphology. EA has been reported in sites like skin thyroid, adrenal gland, soft tissue, and rarely in bone. PURPOSE: The current article describes a case of multicentric EA of the spine that presented simultaneously at T4 spine and the posterior one third of the left fourth rib. STUDY DESIGN/SETTINGS: This study included a patient with a rare spinal tumor, which presented insidiously. Radiologically, the lesions were osteolytic with erosion of cortices. The tumor was composed of nests and cords of malignant cells with epithelioid morphology with areas of vascular differentiation, necrosis, and hemorrhage. Anastomosing vascular spaces lined by epithelioid endothelial cells suggested focal endotheliod differentiation. METHODS: Histopathological evaluation of the tumor obtained from en bloc resection of the fourth rib and specimen from decompression of the fourth thoracic vertebra was done. Immunohistochemistry showed positivity for endothelioid cell markers like CD31 and factor VIII related antigen. The authors point out the need for immunohistochemical evaluation after careful histological analysis for vascular differentiation for an accurate diagnosis of vascular bone tumors with epithelioid features so that an erroneous diagnosis of metastatic carcinoma can be avoided. EA occurs rarely in bone. EA is marked by the presence of large polygonal epithelioid malignant cells with marked cellular atypia and pleomorphism. The arrangement of the cells may mimic epithelial neoplasm. These types of lesions are a definite diagnostic challenge in bone biopsy. The tumor was treated with en bloc resection of the rib and decompression of the body of T4 spine, followed by megavoltage radiotherapy. Follow-up X-rays showed regression of the tumor at 2 years. CD31 is the most sensitive marker for EA. Even in the absence of obvious vascular differentiation, abundant intratumoral hemorrhage and intratumoral neutrophils are definite morphologic changes that should suggest a vascular origin. RESULTS: The patient was followed up for 2 years. A conservative surgical approach with a radical course of megavoltage radiotherapy could yield a good result in EA of bone. At the time of writing this report, there is no evidence of tumor recurrence. CONCLUSION: It is important to differentiate EA from other tumors because the clinical course and treatment are specific for these conditions. Careful histologic and immunohistochemical analysis will clinch the diagnosis. Even though rare, we stress the importance to be aware of the existence of this tumor, which is essential for correct diagnosis. Ó

Primary malignant tumors of the spine

The Orthopedic clinics of North America, 2009

Primary malignant tumors of the spine account for less than 5% of primary bone tumors. Data from the SEER program suggest that the most common bone sarcomas are osteosarcoma, chondrosarcoma, Ewing's sarcoma, chordoma, and malignant fibrous histiocytoma/fibrosarcoma. During the last two decades, tremendous progress has been made in clinical aspects, surgical approaches, and reconstruction with instrumentation at all levels of the spine. Stabilization procedures, including vertebroplasty and kyphoplasty, have further allowed palliation of pain and symptom relief from compression fractures. Improved radiation techniques have offered the potential for improved local control. This article reviews the changes in surgical philosophy in the management of malignant spinal tumors during the past two decades.

open-vs-MIS-spine tumors 2015.pdf

neurosurgical focus Neurosurg Focus 39 (2):E11, 2015 AbbreviAtioNs ASIA = American Spinal Injury Association; EBL = estimated blood loss; GTR = gross-total resection; ID-EM = intradural-extramedullary; MIS = minimally invasive surgery. obJect Patients with symptomatic intradural-extramedullary (ID-EM) tumors may be successfully treated with resection of the lesion and decompression of associated neural structures. Studies of patients undergoing open resection of these tumors have reported high rates of gross-total resection (GTR) with minimal long-term neurological deficit. Case reports and small case series have suggested that these patients may be successfully treated with minimally invasive surgery (MIS). These studies have been limited by small patient populations. Moreover, there are no studies directly comparing perioperative outcomes between patients treated with open resection and MIS. The objective of this study was to compare perioperative outcomes in patients with ID-EM tumors treated using open resection or MIS. methods A retrospective review was performed using data collected from 45 consecutive patients treated by open resection or MIS for ID-EM spine tumors. These patients were treated over a 9-year period between April 2003 and October 2012 at Northwestern University and the University of Chicago. Statistical analysis was performed to compare perioperative outcomes between the two groups. results Of the 45 patients in the study, 27 were treated with the MIS approach and 18 were treated with the open approach. Operative time was similar between the two groups: 256.3 minutes in the MIS group versus 241.1 minutes in the open group (p = 0.55). Estimated blood loss was significantly lower in the MIS group (133.7 ml) compared with the open group (558.8 ml) (p < 0.01). A GTR was achieved in 94.4% of the open cases and 92.6% of the MIS cases (p = 0.81).

Aggressive “Benign” Primary Spine Neoplasms

Spine, 2009

Study Design. Clinically based systematic review. Objective. To define optimal clinical care for primary spinal aggressive "benign" osseous neoplasms using a systematic review with expert opinion.

Primary spinal column sarcomas

Acta Neurochirurgica, 1996

Five cases of primary spinal column sarcomas are presented. Sarcomas primarily originating from paravertebral soft tissues were excluded. Patients' age ranged from 1 to 14 years (mean 8.4 years). The male : female ratio was 2 : 3. Two patients had Ewing's sarcoma (ES) originating from L5-S1 and L4-5 pedicles, respectively; two patients had mesenchymal chondrosarcoma (MCS) originating from L 1-2 pedicles and L5 body, respectively; and one patient had osteogenic sarcoma (OS) of C4 body. All patients clinically presented with pain and progressive weakness of the extremities. The time that elapsed between the onset of symptoms and diagnoses ranged from one to five months. All cases were treated with chemotherapy, radiotherapy and subtotal tumour resection with spinal canal decompression. Two cases received posterior spinal fusion operations. Three patients were alive 10 to 98 months following diagnosis. Only the case with ES of L5-S1 pedicles was in complete remission and off therapy at the 98th postoperative month. The two MCS cases were in partial remission, and were receiving chemoterapy at the time of analysis. These tumours caused similar clinical findings and prognoses, and required combined treatment, which consisted of surgery, radiotherapy and chemotherapy; histologically three different types of malignant tumours are presented in the same category. We preferred surgical decompression and stabilization procedures especially for neurologically symptomatic patients, even if they had extensive tumours with high grades. By spinal canal decompression and stabilisation, we did not intend to cure the disease; however, we intended to provide neurological improvement, spinal stabilisation, improved quality of life, early mobilisation of the patient, and cytoreduction by means of surgical tumour ablation, which could render the chemotherapy more effective.

Vertebral Tumors -Diagnostic & Treatent Protocols

Vertebral bony tumors are notorious for their ability to mimic each other. With ever changing treatment protocols and newer adjuvant therapies introduced at regular intervals of time, we considered a small but concise update on management of these entities with our own diagnostic algorhythm, along with brief details on management, and a quick access table for radiological diagnosis. We hope that this effort, prepared with an extensive literature review supplemented with images and tables will assist in updating spine surgeons of all hues and thus assist in treating their patients.

Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report

Journal of Clinical Medicine

Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. Primary epithelioid sarcoma arising from the spine is extremely rare, with limited data in the literature. We review the existing literature regarding spinal epithelioid sarcoma and report a case of epithelioid sarcoma arising from the spinal cord. A 54 year old male presented with a 1-month history of progressive left upper-limb weakness and numbness. Magnetic resonance imaging (MRI) of the spine showed an enhancing intramedullary mass at the level of T1 also involving the left T1 nerve root. Systemic radiological examination revealed no other lesion at presentation. Surgical excision of the mass was performed, and histology was consistent with epithelioid sarcoma of the spine. Despite adjuvant radiotherapy, there was aggressive loca...