Extraosseous Extension Research Articles

The Impact of Chondrosarcoma with Positive Margins and Extraosseous Extension on Patient Outcomes.

Chondrosarcoma accounts for 20% of all bony sarcomas and may present with extraosseous extension (EOE). The presence of an extraosseous component, along with positive surgical margins, has been separately associated with increased risk of local recurrence and decreased survival. This study compared the outcomes between patients with EOE, EOE and positive margins, and a control chondrosarcoma cohort with neither feature. This was a retrospective review of 91 patients over a consecutive 13-year period. Data including treatment details and outcomes were included. Thirty-two patients had EOE of their chondrosarcoma, 7 patients had positive margins and EOE, and 52 chondrosarcoma patients had neither characteristic. Tumor characteristics, patient demographics, and overall survival, recurrence rates, and metastatic rates were compared among the three groups. Patients with positive surgical margins and EOE were noted to have significantly higher resection grade, dedifferentiation on presentation, and use of adjuvant chemotherapy compared to control. Patients with EOE alone were noted to have significantly higher grade and use of adjuvant chemotherapy compared to control. Patients with positive surgical margins and EOE additionally had significantly higher rates of mortality, recurrence, and metastasis on survival analysis compared to control. However, patients with EOE alone did not have significantly different survival rates compared to control. This study is one of the first to analyze the survival impact of EOE with positive surgical margins in chondrosarcoma. The significance found in mortality, recurrence, and metastatic rates in this unique subgroup may warrant further longitudinal observation and dictate future treatment options.

Odontogenic carcinosarcoma of the mandible: A case report.

Odontogenic carcinosarcoma, a rare and challenging diagnosis, was identified in a 60-year-old male through histopathology, revealing a biphasic neoplasm with malignant epithelial and mesenchymal components. Surgical resection is crucial for management, highlighting the importance of vigilant postoperative follow-up to ensure early detection of any recurrence. One rare mixed malignant odontogenic tumor is odontogenic carcinosarcoma, which comprises malignant epithelial and mesenchymal components. Diagnosing odontogenic carcinosarcoma is challenging due to its rarity and atypical clinical presentation. This study reports a 60-year-old male patient who presented with a painless swelling on the right side of his face and experienced facial asymmetry for 6 months, ultimately diagnosed with odontogenic carcinosarcoma. A biphasic neoplasm with malignant alterations in both epithelial and mesenchymal components was identified upon histopathological examination. MRI imaging showed an expansile multilobulated lytic lesion with cortical erosion and extraosseous extension in the posterior region of the right mandibular body and ramus. Following contrast administration, homogeneous lesion enhancement was observed, with a few small non-enhancing necrotic areas in central parts. The patient subsequently underwent a right hemi-mandibulectomy with resection of adjacent soft tissues and neck dissection due to lymph node involvement. The resulting defect was reconstructed using a pectoralis major flap. No recurrence or metastasis was reported during the 6-month follow-up, reinforcing the positive results. This case highlights the importance of recognizing odontogenic carcinosarcoma and underscores the challenges in diagnosing and managing this rare tumor. Early identification and aggressive treatment can lead to positive outcomes, as evidenced by the absence of recurrence or metastasis in this patient during the follow-up period.

En bloc resection and reconstruction using a talar prosthesis for malignant talar bone tumor: a surgical technique.

En bloc resection is required for treatment of intermediate-grade talar tumors with extraosseous extension (Enneking stage 3) and malignant talar tumors without intra-articular invasion (Enneking stages IA and IIA). After resection, reconstruction options include tibiocalcaneal fusion, frozen autograft, and talar prosthesis; however, a talar prosthesis is preferable because it preserves ankle range of motion, does not cause leg length discrepancy, and is associated with good long-term outcomes. To the best of our knowledge, en bloc resection and reconstruction of a malignant talar tumor has not been previously reported in detail. We report a detailed surgical technique for en bloc resection of a malignant talar bone tumor using combined anterior and lateral approaches followed by reconstruction using a talar prosthesis.

Preserved walking function without postoperative reconstruction for pelvic Ewing’s sarcoma: a case report

BackgroundEwing’s sarcoma is a primary bone tumor predominantly observed in children and adolescents, necessitating a multidisciplinary treatment approach. While localized cases have a 5-year survival rate of 60–70%, the prognosis is significantly worse in pelvic advanced cases with metastasis. Moreover, pelvic Ewing’s sarcoma has the unique problem of leading to high rates of postoperative infection.Case presentationWe present the case of a Japanese 14-year-old boy with left iliac Ewing’s sarcoma and multiple metastases. At the initial visit, imaging revealed a large tumor in the left iliac bone with extraosseous extension and metastasis to multiple sites. Neoadjuvant chemotherapy resulted in significant tumor reduction. Surgical resection was performed without pelvic ring reconstruction to enable early postoperative chemotherapy and minimize postoperative infection risk. Despite complete abductor muscle removal, the patient achieved a stable gait postoperatively by centering the load axis.ConclusionOur case highlights the successful management of a left iliac Ewing’s sarcoma with multiple metastases, with a focus on functional preservation and infection risk reduction. Pelvic ring reconstruction was not performed to avoid postoperative complications, emphasizing the importance of early postoperative chemotherapy. The patient achieved a stable gait postoperatively, demonstrating the potential benefits of this approach in similar cases.

Aggressive Thoracic Spine Hemangioma Treated by Total En Bloc Spondylectomy: A Case Report

Introduction: Vertebral hemangioma (VH) is the most widely faced tumor in the vertebral column. It is generally asymptomatic and slowly growing. Moreover, the appearance of extraosseous extensions in vertebral hemangiomas is locally aggressive, causing neurological deficits, and should be distinguished from other vertebral hemangiomas based on its prognosis and treatment. Here we present a rare case of vertebral hemangioma caused by thoracic cord compression and healed by total en block spondylectomy.Objectives: We present a case of VH with extraskeletal expansion that caused the progressive neurologic deficit and was medicated by resection of the entire tumor.Methods: A 33-year-old female came to the Soetomo General Hospital outpatient clinic with the main complaints of back pain and being unable to move her legs since September 2021. In July 2021, the patient felt tingling in her lower extremities. The patient was still able to walk, but she felt the weakness develop. She still can urinate, but she has a defecation problem. There is no history of a palpable mass in some places. The patient is a housewife and she has no previous history of any disease.Results: The patient felt tingling in her lower limbs. She didn’t lose her to walk, but she felt the weakness develop. On the physical examination, there was a neurological deficit at the lower extremity. According to radiographic and histopathologic examination, the patient was diagnosed with VH at the T5 level. We then performed total en-block spondylectomy and posterior stabilization. As a result, the motoric part improved but sensory deficits still occurred. Neurological improvement happened during the follow-up in three months. An inevitable challenge awaits in terms of the medication of VH with extraskeletal expansion that causes neurologic deficits. Pre-operative diagnosis including radiologic and pathologic findings is essential for the strategy and decision for combative hemangioma. An unjustified diagnosis may lead to an inappropriate surgical strategy or the emergence of various complications.Conclusions: Vertebral hemangiomas with extraosseous extension causing spinal cord compression should be considered an aggressive benign tumor, and total excision that includes a tumor margin is indicated.

Stereotactic Body Radiation Therapy for Sacral Metastases: Deviation From Recommended Target Volume Delineation Increases the Risk of Local Failure

PurposeAlthough spine stereotactic body radiotherapy (SBRT) is considered a standard of care in the mobile spine, mature evidence reporting outcomes specific to sacral metastases is lacking. Furthermore, there is a need to validate the existing sacral SBRT international consensus contouring guidelines to define the optimal contouring approach. We report mature rates of local failure (LF), adverse events, and the impact of contouring deviations in the largest experience to date specific to sacrum SBRT. Methods/MaterialsConsecutive patients who underwent sacral SBRT from 2010-2021 were retrospectively reviewed. Primary endpoint was magnetic resonance imaging-based LF with a specific focus on adherence to target volume contouring recommendations. Secondary endpoints included vertebral compression fracture (VCF) and neural toxicity. ResultsOf the 215 sacrum segments treated in 112 patients, most received 30 Gy/4 fractions (51%), 24 Gy/2 fractions (31%), or 30 Gy/5 fractions (10%). Sixteen percent of segments were non-adherent to the consensus guideline with a more restricted target volume (under-contoured). The median follow-up was 21.4 months (range, 1.5-116.9). The cumulative incidence of LF at 1 and 2 years was 18.4% and 23.1%, respectively. In those with guideline adherent vs. non-adherent contours, the LF rate at 1 year was 15.1% vs. 31.4% and at 2 years 18.8% vs. 40.0% (HR=2.5, 95% CI 1.4-4.6, p=0.003), respectively. On multivariable analysis, guideline non-adherence (HR=2.4, 95% CI 1.3-4.7, p=0.008), radioresistant histology (HR=2.4, 95% CI 1.4-4.1, p<0.001), and extraosseous extension (HR=2.5, 95% CI 1.3-4.7, p=0.005) predicted for an increased risk of LF. The cumulative incidence of VCF was 7.1% at 1 year and 12.3% at 2 years. Seven patients (6.3%) developed peripheral nerve toxicity, of which 4/7 had been previously radiated. ConclusionsSacral SBRT is associated with high efficacy rates and an acceptable toxicity profile. Adhering to consensus guidelines for target volume delineation is recommended to reduce the risk of LF.

Clinical Outcomes Among Patients Treated With Stereotactic Body Radiation Therapy to Femur Metastases for Oligometastatic Disease Control or Reirradiation: Results From a Large Single-Institution Experience

There are limited data regarding outcomes after stereotactic body radiation therapy (SBRT) for femur metastases, which was an exclusion criteria for the Stereotactic Ablative Radiotherapy for the Comprehensive Treatment of Oligometastatic Cancers (SABR-COMET) trial. We aimed to characterize clinical outcomes from a large single institution experience. Forty-eight patients with 53 lesions were consecutively treated with femur SBRT from May 2017 to June 2022. The Kaplan-Meier method and Cox proportional hazard models were used to characterize time-to-event endpoints and associations between baseline factors and clinical outcomes, respectively. Local control and locoregional control were defined as the absence of tumor progression within the radiation treatment field or within the treated femur, respectively. Most patients had Eastern Cooperative Oncology Group performance status 0 to 1 (90%), prostate (52%) or breast/lung (17%) cancer, and 1 to 3 lesions (100%), including 29 proximal and 5 distal. Fifty-seven percent of the lesions were treated with concurrent systemic therapy. Median planning target volume was 49.1 cc (range, 6.6-387 cc). Planning target volume V100 (%) was 99% (range, 90-100). Fractionation included 18 to 20 Gy/1F, 27 to 30 Gy/3F, and 28.5-40 Gy/5F. Forty-two percent had Mirels score ≥7 and most (94%) did not have extraosseous extension. Acute toxicities included grade 1 fatigue (15%), pain flare (7.5%), nausea (3.8%), and decreased blood counts (1.9%). Late toxicities included fracture (1.9%) at 1.5 years and osteonecrosis (4%) from dose of 40 Gy in 5F and 30 Gy in 5F (after prior 30 Gy/10F). One patient (2%) required fixation postradiation for progressive pain. With median follow-up 19.4 months, 1- and 2-year rates of local control were 94% and 89%, locoregional control was 83% and 67%, progression-free survival were 56% and 25%, and overall survival were 91% and 73%. Fifty percent of local regional recurrence events occurred within 5 cm of gross tumor volume. Femur SBRT for oligometastatic disease control in well-selected patients was associated with good outcomes with minimal rates of acute and late toxicity. Patterns of local regional recurrence warrant consideration of larger elective volume coverage. Additional prospective study is needed.

SAT535 ANKRD26-RET Fusion Positive Metastatic Papillary Thyroid Carcinoma - Advanced Presentation of a Rare Mutation

Abstract Disclosure: H. Hashmi: None. A. Ankireddypalli: None. L. Burmeister: None. A. Nelson: None. K. Amin: None. Background: While rearranged during transfection (RET) protooncogene mutations have been commonly associated with thyroid malignancy, ankyrin repeat domain containing protein 26 (ANKRD26) has been primarily linked to hematologic malignancy. Exon 29 of ANKRD26 (Chromosome [Chr] 10p12.1) has been shown to fuse with exon 12 of RET (Chr 10q11.21), resulting in a genetic fusion mutation ANKRD26:RET. This is a rare genetic mutation in papillary thyroid cancer with only one previously reported case. We report the first case in the US of this novel genetic mutation with a unique clinical presentation. Clinical Presentation: A 48-year-old, male patient presented with lower back pain radiating to the lower extremities of one year duration. MRI lumbar spine revealed an irregular mass lesion in the L1 vertebral body with extraosseous soft tissue extension, L1 compression fracture and extension into the T12 body. CT CAP revealed a 4.8 x 3.6 cm, heterogenous right thyroid lobe with tracheal compression and sub centimeter pulmonary nodules in the left lower and right middle lobes. Biopsy of the L1 paravertebral mass was diagnostic for metastatic thyroid carcinoma of a follicular pattern. Molecular testing with a next generation sequencing fusion profile assay was positive for ANKRD26:RET gene rearrangement. Thyroid ultrasound was notable for a solid, hypoechoic, 3.2 x 3.1 x 2.9 cm right lobe nodule with echogenic foci and level 4 lymph nodes with loss of reniform shape and fatty hilum. FNA of the nodule and right level 4 lymph node was suspicious for follicular neoplasm. The patient was admitted for acute cord compression necessitating extensive laminectomy, vertebral reconstruction and resection of the L1 paraspinal tumor. Pathology was positive for metastatic, follicular variant, papillary thyroid carcinoma. Total thyroidectomy, central and right modified radical neck dissection was performed. Pathology demonstrated a 4.2 cm papillary carcinoma, follicular type with tall cell variant; confined to the thyroid. Extensive angioinvasion (≥ 4 vessels) and lymphatic invasion was present. 10/32 positive lymph nodes. TNM grading was T3aN1bM1. Summary: The only other case of ANKRD26-RET fusion positive papillary thyroid cancer in the literature was reported by Staubitz et al in Germany in 2019. They reported a case of a 78-year-old male with follicular variant papillary thyroid carcinoma (pT3,pN0,M0). To our knowledge, our case is the second worldwide and the first to be reported in the United States of this rare fusion protein positive thyroid cancer. Such an aggressive presentation with distant osseous metastases has to our knowledge not been previously described. Conclusion: The clinical and therapeutic implications of this rare genetic mutation are not currently well elucidated. We present this case with the intent to expand the existing literature and increase recognition of this rare genetic mutation. Presentation Date: Saturday, June 17, 2023

Stereotactic Body Radiotherapy (SBRT) for Sacral Metastases: Deviation from Recommended Target Volume Delineation Predicts Higher Risk of Local Failure

A international consensus recommendation was published to guide target volume delineation specific to sacral stereotactic body radiotherapy (SBRT). We report outcomes after sacrum SBRT, focusing on the impact of contouring deviation on local failure (LF) risk, with an aim to validate this guideline. All patients who underwent SBRT to any level between S1 and S5 from 2010 to 2021 were identified from a prospectively maintained institutional database. The primary outcome was magnetic resonance-based LF. Secondary outcomes included vertebral compression fracture (VCF) and overall survival (OS). Cumulative LF and VCF rates were calculated per segment using the competing risk analysis method. Kaplan Meier analysis was used to estimate OS per patient. Cox proportional hazards model was used to assess predictive factors of LF, VCF, and OS. A total of 215 treated sacral segments in 112 patients were retrospectively reviewed. The median follow-up was 13 months (range, 0.4-116.9). The median age was 64 years (range, 18-86), and 56% were male. Most patients (52%) had treatment to a single segment. The median clinical target volume (CTV) was 129.2 cc (range, 5.8-753.5). Most segments were treated with 30 Gy/4 fractions (51%), 24 Gy/2 fractions (31%), or 30 Gy/5 fractions (10%). Thirty-one percent of segments were of radioresistant histology (gastrointestinal, kidney, melanoma, sarcoma, or thyroid primary), and 51% had extraosseous disease. Sixteen percent of segments were under-contoured per consensus guidelines, with incomplete coverage of the involved sector (71%), omission of the adjacent uninvolved sector (17%), or both (11%) as the causes for deviation. The cumulative incidence of LF was 18.4% (95% CI 13.5-24.0) at 12-months and 23.1% (95% CI 17.6-29.0) at 24-months. On multivariate analysis (MVA), under-contouring (HR 2.4, 95% CI 1.3-4.7, p = 0.008), radioresistant histology (HR 2.4, 95% CI 1,4-4.1, p = 0.001), and extraosseous extension (HR 2.5, 95% CI 1.3-4.7, p = 0.005) were predictors of increased risk of LF. The LF rates at 12/24-months were 15.1%/18.8% for segments contoured per guideline versus 31.4%/40.0% for those under-contoured. The cumulative incidence of VCF was 7.1% (95% CI 4.1-11.1) at 12-months and 12.3% (95% CI 8.2-17.2) at 24-months. On MVA, female gender was the only risk factor for VCF (HR 2.3, 95% CI 1.1-5.2, p = 0.04). The median OS was 29.5 months (95% CI 17.5-59.2). On MVA, primary kidney (HR 4.7, 95% CI 1.7-12.5, p = 0.002) or lung histology (HR 3.4, 95% CI 1.3-8.5, p = 0.010), the presence of liver (HR 2.8, 95% CI 1.2-6.4, p = 0.016) or lung (HR 2.5, 95% CI 1.3-5.1, p = 0.008) metastases, ECOG performance status 2 or 3 (HR 3.3, 95% CI 1.2-8.2, p = 0.013), and the presence of sensory or motor deficit (HR 2.6, 95% CI 1.2-5.4, p = 0.012) were prognostic for worse OS. Sacral SBRT is associated with high rates of efficacy and an acceptable VCF risk. Adherence to target volume delineation consensus guidelines reduces the risk of LF.

Outcomes of Stereotactic Body Radiation Therapy (SBRT) for Femur Oligometastases

Stereotactic body radiation therapy (SBRT) is increasingly used for oligometastatic bone disease, but there is limited data regarding the clinical outcomes of utilizing SBRT in treatment for femur metastases, which was excluded from SABR-COMET. We aimed to identify patient or treatment factors associated with clinical outcomes among patients treated with SBRT to femur metastases for oligometastatic disease control or re-irradiation. We identified 50 patients with 56 femur lesions consecutively treated with SBRT at a single institution May 2017-June 2022. The Kaplan-Meier method was used to characterize time-to-event endpoints and Cox proportional hazards models were performed to evaluate the associations between baseline factors and clinical outcomes. Local control was defined as the absence of regional tumor progression at treated area or need for surgical fixation post radiation. Most patients had ECOG 0-1 (90%), prostate (50%) or breast/lung (16%) cancer, and 1-3 lesions (100%), including 30 proximal and 5 distal. 55% of lesions received concurrent systemic therapy, including ADT (n = 18) or immunotherapy (n = 6). Median PTV volume was 54.7cc (range, 6.6 to 387cc). PTV V100(%) was 99% (range 71.5-100). Fractionation included 18-20 Gy/1F, 27-30 Gy/3F, 25-40 Gy/5F, and 50 Gy/10F. 43% of lesions had Mirel's score ≥ 7 and 91% of lesions did not have extraosseous bone extension on diagnostic CT and/or MRI. Acute toxicities included grade 1 fatigue (14.3%), pain flare (7.1%), and decreased blood counts (1.8%). Late toxicities included fracture (1.8%) at 1.5 years and 2 patients with radiation-induced osteonecrosis (3.6%) from dose of 40 Gy in 5F and 30 Gy in 5F (after prior 30 Gy/10F). One patient (n = 2%) required fixation post-radiation due to progression of disease or symptoms. With median follow up 19.4 months, 1 and 2-year rates of local control were 84% and 69%, progression-free survival were 55% and 27%, and overall survival were 91% and 74%. There was no significant association between patient or treatment characteristics and local control outcomes. Femur SBRT for oligometastatic disease control in well-selected patients was associated with good outcomes with minimal rates of acute and late toxicity. Further prospective study is warranted.

STEREOTACTIC BODY RADIOTHERAPY (SBRT) FOR SACRAL METASTASES: DEVIATION FROM RECOMMENDED TARGET VOLUME DELINEATION PREDICTS HIGHER RISK OF LOCAL FAILURE

Abstract Report outcomes after sacrum SBRT, focusing on the impact of contouring deviation on local failure (LF) risk, with an aim to validate the contouring consensus guidelines. METHODS/MATERIALS: Patients who underwent sacral SBRT from 2010- 2021 were identified from a prospectively maintained institutional database. Primary outcomes were magnetic resonance-based LF and vertebral compression fracture (VCF). RESULTS: A total of 215 treated sacral segments in 112 patients were reviewed. Most segments were treated with 30 Gy/4 fractions (51%), 24 Gy/2 fractions (31%), or 30 Gy/5 fractions (10%). Thirty-one percent of segments were of radioresistant histology (gastrointestinal, kidney, melanoma, sarcoma, or thyroid primary), and 51% had extraosseous disease. Sixteen percent of segments were under-contoured per consensus guidelines. The cumulative incidence of LF was 18.4% (95% CI 13.5-24.0) at 12-months and 23.1% (95% CI 17.6-29.0) at 24-months. On multivariate analysis (MVA), under- contouring (HR 2.4, 95% CI 1.3-4.7, p=0.008), radioresistant histology (HR 2.4, 95% CI 1,4-4.1, p=0.001), and extraosseous extension (HR 2.5, 95% CI 1.3-4.7, p=0.005) were predictors of increased risk of LF. The LF rates at 12/24-months were 15.1%/18.8% for segments contoured per guideline versus 31.4%/40.0% for those under-contoured. The cumulative incidence of VCF was 7.1% (95% CI 4.1-11.1) at 12-months and 12.3% (95% CI 8.2-17.2) at 24-months. On MVA, female gender predicted increased VCF risk (HR 2.3, 95% CI 1.1-5.2, p=0.04). CONCLUSIONS: Sacral SBRT is associated with high rates of efficacy and an acceptable VCF risk. Adherence to target volume delineation consensus guidelines reduces the risk of LF.

Imaging Features of Intraosseous Schwannoma: A Case Series and Review of the Literature.

To characterize the imaging features of patients with pathologically confirmed intraosseous schwannoma (IOS), institutional pathology and imaging databases were searched for IOS cases over a period of 17 years. A musculoskeletal radiologist evaluated all imaging studies. Additionally, a literature search was performed to identify IOS cases that had imaging findings of at least two modalities. Six patients (one female, five males, mean age of 50 ± 14 years) with IOS were identified, with all lesions localized to the lumbosacral region. Radiographic imaging was available in four patients, while all patients underwent CT and MR imaging. Radiographs depicted lytic lesions, and CT depicted heterogeneous expansile lesions with centrally hypodense areas and peripheral sclerosis. All cases involved extra-osseous extension, producing a mass effect on adjacent soft tissues and nerve roots. On MRI, the neoplasms displayed iso- to- slightly- low signal intensity on T1-weighted images and hyperintense signal intensity on T2-weighted images with heterogeneous enhancement. The literature review resulted in 102 IOS cases, which to the best of our knowledge, is the largest review on IOS, and the imaging findings of the previously published cases were the same as our cases. IOSs are rare benign neoplasms that should be considered in the differential diagnosis of well-defined expansile lytic lesions with sclerotic borders. This is particularly important in middle-aged adults with mandibular, sacral, or vertebral body mass.

Clinical and Pathological Profiles of Vertebral Bone Metastases from Endometrial Cancers: Evidence from a Twenty-Year Case Series.

Patients with endometrial cancer (EC) frequently have metastases to lungs, extra-pelvic nodes, and liver. Although an uncommon occurrence, cases of EC metastasis to bone, prevalently in vertebral bone, have also been reported. The objective of this study was to analyze clinical and pathological profiles of patients with EC metastatic to vertebral bone. We carried out a retrospective case series on surgically treated patients for this pathology. From 2001 to 2021, out of 775 patients with bone metastasis, 1.6% had bone metastasis from EC. The median time between the diagnosis of primary tumor and that of bone metastases was 31.5 months. Solitary bone lesion was present in 7 patients and lumbar vertebrae were the segments most affected. Pathological fractures in 46.2% of patients and spinal pain in all were present. In terms of location, 46.2% of bone metastases resided within the anterior section of the vertebra, while the remaining presented an extension within the anterior and posterior sections, with 46.1% of cases showing an extradural extra-osseous extension and paraspinous envelope. Median survival after diagnosis of bone metastasis was 11.5 months. Vertebral bone metastasis in EC is a rare phenomenon, with severe prognosis. An in-depth understanding of this topic may guide future management and treatment decisions, thus improving life expectancy and quality.

Hepar-1 Positive Prostatic Adenocarcinoma: A Case Report and Review of Literature

Abstract Introduction/Objective Poorly differentiated metastatic malignancy in patients with a history of more than one known primary tumor, such as a history of both hepatocellular and prostatic carcinoma as seen in our case, can be challenging. Both tumors are CK7/CK20 positive, and both can be reactive for hepar-1, as has been demonstrated in the current case, and rarely described in the literature. Methods/Case Report Herein we present a sixty-three-year-old male who presented for follow-up of his lower back pain in the setting of hepatocellular carcinoma. MRI of the spine was suggestive of an expansile metastatic lesion in the C2 vertebral body with extraosseous soft tissue extension resulting in moderate to severe central canal and left foraminal narrowing at C2-3. Multiple additional metastatic lesions were visualized throughout the cervical upper thoracic spine as detailed above. Vertebral body biopsy showed metastatic adenocarcinoma with sheets of neoplastic cells infiltrating the medullary spaces. The patient’s history of hepatocellular carcinoma was noted, and immunohistochemical staining for HepPar1 with appropriate control was performed and found to be diffusely reactive in the tumor cells. However, tumor cells were also positive for PSA, PSAP, and NKX3.1 (Fig-1-A-D, H&amp;E, Hepar-1, PSAP, PAP). This immunoprofile is consistent with a prostatic origin of the tumor cells, and a diagnosis of metastatic prostatic adenocarcinoma was given. Results (if a Case Study enter NA) NA. Conclusion Hepar-1 and even arginase-1 have been observed in rare subsets of prostatic acinar adenocarcinoma. Even in the setting of PSA and PSAP negative metastatic disease in patients with a history of hepatocellular carcinoma, we suggest utilizing additional stains suggestive of a prostatic origin, such as NKX3.1, and even molecular studies. This could be useful diagnostic approach to help differentiate between the two neoplasms in such patients.

Case report on aggressive Hemangioma

Hemangioma is a benign tumor characterized by the proliferation of blood vessels. A few hemangiomas are aggressive, characterized by bone expansion and extra-osseous extension. The vertebral hemangiomas are most often found in the thoracic and lumbar spine. The aggressive type refers to the presence of extension beyond the vertebral body, destruction of the cortex and invasion of the epidural and paravertebral spaces. An aggressive hemangioma can occur at any age with peak prevalence in young adults. We hereby present a case of an Aggressive Hemangioma in a young patient with no co-morbidities exhibiting symptoms of low backache and progressive lower limb weakness and urinary symptoms.

A Rare Case of Epithelioid Hemangioma Presenting as an Isolated Sacral Mass

Epithelioid hemangioma (EH) is an uncommon benign vascular tumor of mesenchymal origin. It mainly presents as expanding nodules around the ear, the forehead, and long bones. Only a handful of cases have been found in cervical, thoracic, lumbar, and sacral vertebrae as lytic lesions with pain and neurological impairment. We present the case of a 36-year-old female with an incidental finding of a sacral mass along with inguinal lymphadenopathy on imaging. Initially, there were no symptoms. The mass gradually progressed and later showed an extraosseous extension with involvement of sacral neural foramina and nerve roots causing severe low back pain and weakness of the left lower extremity. Differential diagnoses initially included secondary metastases and chordoma. However, the biopsy of the mass revealed findings consistent with an EH. To our knowledge, this is the first case of EH presenting as an isolated mass in the sacrum and the third case of EH involving the sacrum in continuation with other vertebrae.EH should be in our differential diagnoses when there is a sacral mass.

Stereotactic Body Radiation Therapy for Metastases in Long Bones

To evaluate the cumulative incidence of fracture and local failure and associated risk factors after stereotactic body radiation therapy (SBRT) for long bone metastases. Data from 111 patients with 114 metastases in the femur, humerus, and tibia treated with SBRT in 7 international centers between October 2011 and February 2021 were retrospectively reviewed and analyzed using a competing risk regression model. The median follow-up was 21 months (range, 6-91 months). All but 1 patient had a Karnofsky performance status ≥70. There were 84 femur (73.7%), 26 humerus (22.8%), and 4 tibia (3.5%) metastases from prostate (45 [39.5%]), breast (22 [19.3%]), lung (15 [13.2%]), kidney (13 [11.4%]), and other (19 [16.6%]) malignancies. Oligometastases accounted for 74.8% of metastases and 28.1% were osteolytic. The most common total doses were 30 to 50 Gy in 5 daily fractions (50.9%). Eight fractures (5 in the femur, 2 in the tibia, and 1 in the humerus) were observed with a median time to fracture of 12 months (range, 0.8-33 months). In 6 out of 8 patients, fracture was not associated with local failure. The cumulative incidence of fracture was 3.5%, 6.1%, and 9.8% at 1, 2, and 3 years, respectively. The cumulative incidence of local failure (9/110 metastases with imaging follow-up) was 5.7%, 7.2%, and 13.5% at 1, 2, and 3 years, respectively. On multivariate analysis, extraosseous disease extension was significantly associated with fracture (P=.001; subhazard ratio, 10.8; 95% confidence interval, 2.8-41.9) and local failure (P=.02; subhazard ratio, 7.9; 95% confidence interval, 1.4-44.7). SBRT for metastases in long bones achieved high rates of durable local metastasis control without an increased risk of fracture. Similar to spine SBRT, patients with extraosseous disease extension are at higher risk of local failure and fracture.

Carbon ion radiotherapy for unresectable primary undifferentiated pleomorphic sarcoma of the 11th thoracic spine: a case report.

Objective: Primary undifferentiated pleomorphic sarcoma (UPS) of the bone is rare. However, the common sites are the knee and proximal femur and humerus, while spinal involvement is rare. We report a case of primary UPS of the 11th thoracic vertebra, where corpectomy would have been difficult and extensive, treated with carbon ion radiotherapy.Case report: A 76-year-old man presented with an osteolytic tumor of the 11th thoracic vertebra on plain computed tomography (CT). The spinal cord was compressed and displaced posteriorly by the tumor on magnetic resonance imaging (MRI), and extraosseous extension was observed. An incisional biopsy was performed, and primary UPS of the 11th thoracic vertebra was diagnosed pathologically. Total en bloc spondylectomy was considered to be challenging because of the extraosseous extension and the patient’s age; thus, carbon ion radiotherapy (70.4 GyE / 32 fraction) was performed. Denosumab (120 mg) was administered subcutaneously every four weeks. No adjuvant chemotherapy was administered. Four years post-treatment, imaging revealed a compression fracture of the 11th thoracic vertebra, but there was no recurrence.Conclusion: Despite a poor prognosis and an aggressive course of UPS of the spine, the tumor continues to be controlled without local recurrence four years after carbon ion radiotherapy.

A RARE CASE OF MYOCARDIAL INFARCTION DUE TO INTIMAL SARCOMA OF THE AORTIC ARCH

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Intimial sarcoma (IS) of the large systemic arteries are an extremely rare class of tumors with only 81 reported cases in literature, most commonly arising in the abdominal aorta (1). Due to its nonspecific clinical presentation, IS is frequently diagnosed late in the disease process when metastasis has already developed, specifically to the lungs, abdominal organs, vertebral bone, and skin (2). Accurate diagnosis is made with immunohistochemical (IHC) staining positive for CD31, Fli-1, and ERG1. We present a case of an IS of the aortic arch resulting in a fatal tumor embolism to the left coronary artery (LCA), as diagnosed on autopsy. CASE PRESENTATION: A healthy 51-year-old male presented with severe sharp pain in the thoracic spine. Physical exam was positive for tenderness to palpation in the mid-thoracic spine with bilateral lower extremity (LE) neurological deficits. Magnetic resonance imaging (MRI) of the spine revealed bone lytic lesions in the thoracic spine from T5 to T7 with a T6 compression fracture. A computed tomography (CT) scan of the chest, abdomen, and pelvis showed no evidence of the primary tumor site. Biopsy revealed metastatic bone sarcoma of unknown origin. The patient underwent palliative radiation therapy for two weeks. One month after completion of treatment, the patient was re-admitted with worsening back pain. Repeat CT scan showed worsening compression fractures and extraosseous tumor extension into the prevertebral space. Subsequently, the patient underwent spinal cord decompression surgery. On post-operative day 4, he developed severe chest pain with refractory ventricular fibrillation leading to his death. Autopsy revealed an IS extending from the aortic arch to the aortic valve with metastasis to the thoracic vertebrae and thyroid gland. Ultimately, the cause of death was due to a tumor embolus that occluded his LCA. DISCUSSION: Aortic IS is an extremely rare neoplasm that typically presents late in the disease process. The initial manifestation of aortic IS in our case was metastatic vertebral bone lesions with no other evidence of the primary site on radiologic modalities, despite retrospective imaging analysis. It is important that aortic IS be included on the differential diagnosis for sarcoma of unknown origin in order to better understand the disease course. To our knowledge, this is the first reported case of aortic IS that presented as a sarcoma of unknown origin with bone metastases and resulted in a myocardial infarction with findings of metastasis to the thyroid gland. CONCLUSIONS: Aortic IS can be classified as: Angiosarcoma, expressing endothelial-specific antigens, and Myofibroblast, expressing mesenchymal-specific antigens. Diagnosis is tricky as IS are easily missed on CT and MRI. Definitive diagnosis requires IHC analysis. Prognosis for aortic IS is quite poor, with a mean survival period of one year. REFERENCE #1: Sebenik, M., et al. (2005). Undifferentiated Intimal Sarcoma of Large Systemic Blood Vessels. American Journal of Surgical Pathology, 29(9), 1184-1193. REFERENCE #2: Staats, P., et al. (2014). Intimal sarcomas of the aorta and iliofemoral arteries: A clinicopathological study of 26 cases. Pathology,46(7), 596-603 DISCLOSURES: No relevant relationships by Daniel Rushing, source=Web Response No relevant relationships by Amjed Zidan, source=Web Response

Diagnostic performance of tomosynthesis for evaluation of bone tumors and tumor-like lesions: a comparison with radiography.

Even though radiologic diagnosis of bone tumors and tumor-like lesions is usually based on radiographs, radiographically faint imaging features sometimes remain challenging due to overlapping anatomical structures. To compare tomosynthesis with radiography for the evaluation of bone tumors and tumor-like lesions. Forty-seven bone tumors and tumor-like lesions were assessed with radiographs and tomosynthesis images. Two radiologists independently analyzed imaging features of lesions, including margin, periosteal reaction, cortical thinning, matrix mineralization, cortical destruction (such as pathologic fracture), and extraosseous soft-tissue extension. Computed tomography (CT) imaging was used as a reference method. Diagnostic performances of radiography and tomosynthesis were analyzed and compared based on sensitivity, specificity, accuracy, positive predictive value, and negative predictive value. Effective radiation dose was compared among the three imaging modalities by phantom studies. Inter-observer variability (kappa value) for imaging features was slight to moderate on radiography (0.167-0.588), whereas it was nearly perfect on tomosynthesis (0.898-1.000) except for extraosseous soft-tissue extension (0.647 vs. 0.647). Tomosynthesis showed significantly higher sensitivity than radiography in evaluating the margin for bone tumors or tumor-like lesions (1.00 vs. 0.85; P = 0.016), and significantly higher accuracy than radiography in evaluating the margin and matrix mineralization for those (1.00 vs. 0.85; P = 0.016 and 0.91 vs.0.77; P = 0.023, respectively). In phantom studies, mean effective radiation doses were highest in order of CT, tomography, and radiography. Tomosynthesis increases sensitivity and accuracy of the margin as well as accuracy of the matrix mineralization of bone tumors and tumor-like lesions compared to radiography.