Neoadjuvant Reirradiation for Radiation Therapy-Associated Angiosarcoma of the Breast.

PURPOSE: Radiation-associated angiosarcoma of the breast (RAASB) is a rare side effect after breast radiation and has been associated with poor outcomes. At this time, there is no consensus regarding management of RAASB, and the role of reirradiation remains controversial. We present our modern institutional outcomes in managing RAASB with incorporation of neoadjuvant hyperfractionated reirradiation. METHODS AND MATERIALS: Patients identified were treated between 2016 and 2020 with inclusion of any histologically proven RAASB without metastatic disease at diagnosis, while excluding those with a history of radiation therapy outside of the breast/chest wall or other sarcoma histologies. Major wound complications were defined as requiring wound care and/or wound vacuum or return to the operating room for wound repair at any time after surgery. RESULTS: Eight patients were identified, and the median follow-up was 34 months. Median time to RAASB development was 8 years from initial radiation therapy. With respect to RAASB management, all underwent surgery and neoadjuvant reirradiation therapy, and all but 1 patient received taxol-based chemotherapy. At last follow-up, 7 patients remained free of disease, and 1 patient died with distant disease. With respect to acute toxicity after reirradiation, all patients developed at least acute grade 2 toxicities. Five of the 8 patients developed a major wound complication. CONCLUSIONS: Our institutional analysis suggests excellent local control and survival outcomes for RAASB treated with neoadjuvant hyperfractionated reirradiation, surgery, and taxol-based chemotherapy. However, major wound complications represent a major challenge with this approach. Future studies should consider how best to improve the therapeutic ratio while maintaining high rates of local control and survival.

Stereotactic Body Radiation Therapy for Sarcoma Pulmonary Metastases.

BACKGROUND: Lung metastases are the most common form of distant failure for patients diagnosed with sarcoma with metastasectomy considered for some patients with limited metastatic disease and good performance status. Alternatives to surgery such as stereotactic body radiation therapy (SBRT) can be considered, though data are limited. We present outcomes after SBRT for sarcoma lung metastases. METHODS: Fifty sarcoma patients with 109 lung metastases were treated with SBRT between 2005 and 2021. Outcomes evaluated included local control (LC), overall survival (OS), and toxicity including lung pneumonitis/fibrosis, chest wall toxicity, dermatitis, brachial plexus, and esophageal toxicity. Systemic therapy receipt before and after SBRT was recorded. RESULTS: SBRT schedules were divided into 3 cohorts: 30 to 34 Gy/1fx (n=10 [20%]), 48 to 50 Gy/4 to 5fx (n=24[48%]), and 60 Gy/5fx (n=16[32%]). With a median follow-up of 19.5 months, 1/3-year LC rates were 96%/88% and 1/3-year OS 77%/50%, respectively. There was no differences between the 3 regimens in terms of LC, OS, or toxicity. Size >4 cm was a predictor of worse LC ( P =0.031) and worse OS ( P = 0.039) on univariate analysis. The primary pattern of failure was new metastases (64%) of which the majority were in the contralateral lung (52%). One-year chemotherapy-free survival was 85%. Overall, 76% of patients did not require chemotherapy initiation or change of chemotherapy regimen after lung SBRT. Toxicity was reported in 16% of patients overall, including 25%, 20%, and 14% in the 30 to 34 Gy/1fx, 48 to 50 Gy/4 to 5fx, and 60 Gy/5fx cohorts, respectively. CONCLUSIONS: SBRT outcomes for lung metastases from sarcoma demonstrate high rates of LC and are similar with different dose/fractionation regimens. Lung SBRT is associated with prolonged chemotherapy-free survival. Prospective validation of these results is warranted.

Update on Accelerated Whole Breast Irradiation.

Since the advent of breast conservation, adjuvant radiation therapy (RT) has been standard of care following breast conserving surgery (BCS). Radiation therapy following BCS has traditionally been whole breast irradiation (WBI); studies comparing breast conservation to mastectomy utilized standard fractionation WBI, which delivers treatment daily over 5 to 7 weeks (1.8-2 Gy/fraction) and was the standard for decades. More recently, multiple randomized trials have compared standard fractionation WBI to moderately hypofractionated WBI (2.66 Gy/fraction, 15-16 fractions), which allows for completion of treatment in 3 to 4 weeks. Results have demonstrated no difference in local control between these two approaches with comparable toxicity and cosmetic outcomes with long-term follow-up. As such, moderately hypofractionated WBI represents the standard of care approach for most patients with early-stage breast cancer following BCS at this time. In the past few years, ultra-hypofractionated WBI (5.2-5.7 Gy/fraction, 5 fractions) has emerged with promising outcomes; 5-year outcomes from the FAST-Forward randomized trial demonstrated noninferiority between ultra-hypofractionated WBI and moderately hypofractionated WBI. Moving forward, long-term outcomes from ultra-hypofractionated WBI studies are expected, as well as the potential for incorporating moderately hypofractionated regimens into patients requiring regional nodal irradiation following BCS. Finally, the advent of ultra-short regimens may allow clinicians to re-evaluate treatment de-intensification in early-stage breast cancer to consider radiation therapy alone following BCS in lieu of endocrine therapy.

Current Diagnostic Tools for Breast Cancer-Related Lymphedema.

PURPOSE OF REVIEW: Breast cancer-related lymphedema (BCRL) can have a significant impact on breast cancer survivors quality of life. The purpose of this review is to evaluate diagnostic tools for the assessment of BCRL. RECENT FINDINGS: Multiple BCRL diagnostic tools are available, though older diagnostic tools have low sensitivity, limiting the ability for sub-clinical BCRL diagnosis while BIS and perometry have increased sensitivity and the ability to diagnose BCRL sub-clinically. Prospective studies have demonstrated such an approach coupled to early intervention is associated with low rates of chronic BCRL while a recently published randomized trial demonstrated that prospective surveillance with BIS coupled with early intervention reduced rates of chronic BCRL as compared to circumference measurements with compression garments. Prospective and randomized data support the use of prospective surveillance for BCRL. The strongest data available comes from the PREVENT trial and supports prospective BCRL surveillance with bioimpedance spectroscopy coupled to early intervention with a compression sleeve.

Cost-Effectiveness Analysis of Ultra-Hypofractionated Whole Breast Radiation Therapy Alone Versus Hormone Therapy Alone or Combined Treatment for Low-Risk ER-Positive Early Stage Breast Cancer in Women Aged 65 Years and Older.

PURPOSE: The optimal management of early-stage, low-risk, hormone-positive breast cancer in older women remains controversial. Recent trials have shown that 5-fraction ultrahypofractionated whole-breast irradiation (U-WBI) has similar outcomes to longer courses, reducing the cost and inconvenience of treatment. We performed a cost-utility analysis to compare U-WBI to hormone therapy alone or their combination. METHODS AND MATERIALS: We simulated 3 different treatment approaches for women age 65 years or older with pT1-2N0 ER-positive invasive ductal carcinoma treated with lumpectomy with negative margins using a Markov microsimulation model. The strategies were U-WBI performed with a 3-dimensional conformal technique over 5 fractions without a boost ("radiation therapy [RT] alone"), adjuvant hormone therapy (anastrozole for 5 years) without RT ("aromatase-inhibitor [AI] alone"), or the combination of the 2. The combination strategy was calibrated to match trial results, and the relative effectiveness of the RT alone and AI alone strategies were inferred from previous randomized trials. The primary endpoint was the cost-effectiveness of the 3 strategies over a lifetime horizon as measured by the incremental cost-effectiveness ratio (ICER), with a value of $100,000/quality-adjusted life-year deemed "cost-effective." RESULTS: The model results compared with the prespecified target outcomes. On average, RT alone was the least expensive strategy ($14,775), with AI alone slightly more ($14,998), and combination therapy the costliest ($19,802). RT alone dominated AI alone (the incremental cost-effectiveness ratio [ICER] -$5089). Combination therapy, compared with RT alone, was slightly more expensive than our definition of cost-effective (ICER $113,468) but was cost-effective compared with AI alone (ICER $54,451). Probabilistic sensitivity analysis demonstrated RT alone to be cost-effective in 50% of trials, with combination therapy in 36% and AI alone in 14%. CONCLUSIONS: U-WBI alone appears the more cost-effective de-escalation strategy for these low-risk patients, compared with AI alone. Combining U-WBI and AI appears more costly but may be preferred by some patients.

Early outcomes of ultra-hypofractionated preoperative radiation therapy for soft tissue sarcoma followed by immediate surgical resection.

BACKGROUND: There is increasing interest in shorter courses of radiation therapy (RT) in the management of soft tissue sarcoma (STS). We report our institutional experience for patients undergoing ultra-hypofractionated preoperative RT followed by immediate resection. METHODS: An IRB approved review of patients treated with preoperative 5 fraction, once daily RT followed by immediate resection (within 7 days) for STS of the extremity or trunk was conducted. The primary endpoints are major wound complications and local control (LC). Secondary endpoints include grade ≥ 2 toxicity, metastasis free survival (MFS), and overall survival (OS). RESULTS: Twenty-two patients with a median age of 67 years (range 30-87) and median follow-up of 24.5 months (IQR 17.0-35.7) met eligibility criteria; 18/22 patients (81.8 %) had ≥ 1 year follow-up. Primary tumor location was lower extremity in 15 patients (68.2 %), upper extremity in 5 (22.7 %), and trunk in 2 (9.1 %). All patients received 30 Gy in 5 fractions. The median time to resection following RT was 1 day (range 0-5). The median time from biopsy to resection was 34 days (range 20-69). Local control was 100 %; in patients with localized disease, 2-year MFS and OS were 71.3 % and 76.9 %, respectively. Major wound complications occurred in 9 patients (40.9 %), with wound complications requiring reoperation occurring in 8 patients (36.4 %). Other acute and late grade ≥ 2 toxicities were seen in 0 and 4 patients (18.2 %), respectively. CONCLUSION: Ultra-hypofractionated preoperative RT followed by immediate resection permits expedited completion of oncologic therapy with early results demonstrating excellent local control and acceptable toxicity. Prospective data with long-term follow-up is needed.

Intraoperative radiation therapy in the management of early stage breast cancer.

Adjuvant radiotherapy (RT) following breast conserving surgery (BCS) is associated with an improvement in local control and a reduction in breast cancer mortality. While traditionally delivered with whole breast irradiation (WBI), novel approaches have looked to reduce the duration, target volume, and toxicity of adjuvant RT. One such approach is intraoperative radiation therapy (IORT), which delivers radiation at the time of surgery with 80-90% of patients not requiring additional WBI. The current review presents IORT techniques and outcomes from modern series evaluating IORT as monotherapy or as a tumor bed boost. Based on two randomized trials (TARGIT-A and ELIOT) with recent updates, concern regarding higher rates of local recurrence with IORT exist, whether using electrons or low-energy techniques. In contrast, data is promising regarding IORT used as a boost, with ongoing studies evaluating its role prospectively. With respect to toxicity, the data suggest IORT is associated with comparable to slightly lower rates of toxicity though there may be a higher risk of seroma requiring aspiration and fat necrosis with IORT. Given current data and guidelines, WBI or other partial breast techniques should remain the standard of care in early stage breast cancer patients, while IORT should not be utilized outside of prospective clinical trials at this time.

Atypical perigraft seroma masquerading as a forearm tumor in a dialysis patient.

BACKGROUND: An extremely rare manifestation of perigraft seroma (PGS), in which a dense, semisolid jelly-like mass had formed around the shunt instead of the standard fluid-like form of the usual seroma, leading to misdiagnosis with other entities, such as tumors around the synthetic arterio-venous shunt (AVS) was presented. CASE REPORT: A 64-year-old male with multiple myeloma post autologous bone marrow transplant with a renal impairment, presented with a rare form of PGS, which was noticed 2 months after placing a synthetic AVS vascular graft. The mass increased in size, and multiple attempts for excision failed due to recurrence, which led to tumor misdiagnosis. The mass reoccurrence stopped completely only after the radical shunt removal. CONCLUSION: This case report revealed a rare form of PGS, in which the seroma was represented as a firm, semisolid jelly-like mass rather than the typical fluid type transudate seroma. Despite its rarity, it was associated with a high recurrence rate because unlike the standard perishunt seroma, this semisolid jelly-like material could neither be aspirated, nor could it be resected en-bloc, leading to shunting dysfunction. Its management included advanced imaging and a high probability of shunt removal or replacement.

Salvaging the Digit in Invasive Subungual Malignancies Using a Triple Technique under Awake Local Anesthesia.

INTRODUCTION: Amputation for subungual malignancy (SUM) was thought to be the gold standard in preventing recurrence and metastasis. The rationale behind this aggressive treatment was never based on scientific evidence. Even though multiple recent studies supported more conservative management by illustrating successful results of the digit salvage technique, especially for "in situ" SUM, this salvage approach is not well supported for the more aggressive type of the "invasive" SUM; herein, we salvaged two cases of "invasive" SUM. Case Presentation. We present two cases of invasive SUM without radiographic evidence of intraosseous involvement, where we avoided digit amputation for both invasive subungual squamous cell carcinoma of the thumb and invasive subungual melanoma of the ring finger. Both were salvaged by using a triple technique under awake local anesthesia which included (I) radical excision of the nail bed unit including both eponychium and periosteum, (II) dorsal cortical bone shaving using a high-speed burr for the distal phalanx, and (III) flap coverage. Brunelli flap was used for the thumb in the first case, and V-Y plasty combined with proximal nail fold advancement flap was used for the ring finger in the second case. There was no evidence of local or distant recurrence, with a good functional outcome after 2.5 years in the first case and 2 years in the second. CONCLUSION: Ensuring complete resection with negative margins while preserving the functionality of the affected digit is considered to be the optimal challenge in treating "invasive" subungual malignancies. These two case reports contribute by reporting a successful digit salvage. The safety of this procedure could be confirmed by larger series and longer follow-up periods.

"Whoops" fixation of proximal humerus pathological fracture ended with forequarter amputation - Case report.

INTRODUCTION: Even with the advancement of limb salvage surgery techniques, forequarter amputation (FQA) is still used in orthopedic oncology. Even though it might pose catastrophic sequelae on the patient's lifestyle, debilitating one's ability to perform regular tasks, FQA is still considered as a treatment of last resort for huge fungating tumors of the upper extremity. CASE PRESENTATION: We present a case of an 18-year-old male patient, who was diagnosed in Libya with left proximal humerus fracture after a trivial trauma and underwent open reduction and internal fixation using k-wires as it was thought to be a simple fracture. Soon after, pain and swelling progressed severely and an open biopsy confirmed a diagnosis of osteosarcoma and imaging suggested metastatic disease to the lungs for which he was started on chemoradiotherapy. He was referred to our cancer center to continue his management and due to the aggressive nature of the tumor, the patient underwent palliative forequarter amputation followed by multiple lines of chemotherapy and radiotherapy, all of which failed to halt the progression of the disease. The patient was lost to follow up due to his decision to go back to Libya. CONCLUSION: "Whoops" surgeries are fixated upon repairing fractures without looking for the alarming signs on radiographs to exclude pathological entity. As in our case, the procedure done escalated the osteosarcoma into such a massive fungating tumor due to the violation of the osteosarcoma pseudo capsule, in which the only available option is to do a palliative forequarter amputation.

Fungating malignant peripheral nerve sheath tumor arising from a slow-growing mass in the forearm: a case report and review of the literature.

BACKGROUND: Malignant peripheral nerve sheath tumor is a rare and aggressive form of sarcoma that arises from a peripheral nerve, mostly in association with neurofibromatosis type 1. Half of the cases were reported in the extremities, with the lungs being the most common site of metastasis. We report a rare case of successful limb salvage surgery performed for a large exophytic malignant peripheral nerve sheath tumor of the right forearm with wide surgical margins followed by split-thickness skin graft and later a flexor carpi radialis tendon transfer to extensor digitorum communis tendon. CASE PRESENTATION: A 51-year-old Bedouin Arabic man presented to our institution with an incompletely excised, large, fungating, malignant peripheral nerve sheath tumor occupying most of his right forearm. Staging imaging showed multiple lung nodules. He underwent wide local excision followed by skin graft and tendon transfer as a palliative measure to preserve the function of his dominant limb. The operation was performed without any complications, and the patient had an excellent postoperative result. Afterward, he was started on multiple lines of chemotherapy that failed because of disease progression, and the patient died 7 months after the operation. CONCLUSION: Clinicians must consider the possibility of soft tissue sarcoma even in a patient with a small, slow-growing, superficial mass. Furthermore, a wrong open biopsy or nononcological surgical procedure may lead to possible contamination and ultimately a more radical procedure than would have originally been necessary, where this can be prevented by an early referral to a highly specialized sarcoma center.

Surgical Reconstruction Methods following Radical Excision of Distal Ulna Osteosarcoma in Both Skeletally Mature and Immature Patients.

The distal ulna has always been considered to be expendable and its removal has been advocated for a variety of post-traumatic degenerative and oncological conditions but recent studies showed that the distal radioulnar joint allows supination and protonation of the forearm and is important to one's grip strength and lifting ability. Several prosthesis models have already been made to replace the mechanical functionality of the distal radioulnar joint. We present two cases of females aged 22 and 12 years, respectively, who presented with wrist pain and swelling without any history of trauma and with terminal degree limitation in wrist movements due to tenderness and swelling. Both of them did not have any distant metastasis upon radiographic staging. The skeletally mature patient underwent radical excision of the distal ulnar osteosarcoma and received a distal radioulnar joint replacement prosthesis (Scheker prosthesis). The other skeletally immature patient underwent radical excision of the involved distal ulnar osteosarcoma with stabilization of the residual ulnar stump using the extensor carpi ulnaris sling in a modified version of the Goldner and Hayes technique. Both of our patients were treated according to the protocols of our multidisciplinary clinic sarcoma team by starting with neoadjuvant chemotherapy, followed by surgery and adjuvant chemotherapy. Both registered an almost complete restoration of the normal wrist and hand function and were in complete remission for 26 and 24 months, respectively. Based on our literature review, these are some of the extremely rare cases in which the osteosarcoma affected an unusual site (the distal ulna where they underwent a rare type of reconstruction status following radical excision of a malignant tumor).

Innovative Surgical Planning in Resecting Soft Tissue Sarcoma of the Foot Using Augmented Reality With a Smartphone.

Augmented or hybrid reality is a display technology that combines the real world with the virtual world; it permits digital images of preoperative planning information to be combined with the surgeon's view of the real world. Augmented reality (AR) can increase the surgeon's intraoperative vision by providing virtual transparency of the real patient and has been applied to a wide spectrum of orthopedic procedures, such as tumor resection, fracture fixation, arthroscopy, and component's alignment in total joint arthroplasty. We present a case of a male patient who presented with pain in the medial aspect of his left foot after he underwent an incomplete mass excision elsewhere where it turned out to be synovial sarcoma. Because the mass was small, impalpable, and deeply positioned beneath both the plantar and the medial plantar aponeuroses, it was impossible to preoperatively decide a plan for resection. We opted to use the aid of AR in the form of an application using the camera of a smartphone. We were able to excise the tumor with negative surgical margins. On 12-month follow-up, the patient is in complete remission and has optimal mobility and functionality of his foot. In conclusion, AR holds great potential for use in the future of orthopedic surgical oncology. We emphasize using it via a handheld device that we found to be optimal for planning resection of the small and relatively fixed tumor. Based on our literature review, this is the first case describing the surgical planning in resecting an impalpable synovial sarcoma of the foot using AR technology.

Novel Double Central Ray Amputation of the Third and Fourth Digits: Case Report and Literature Review.

Whenever partial hand amputations for soft tissue sarcomas are attempted, special consideration should be given to achieve a balance between complete resection associated with negative margins and preservation of functionality to the patient so that the hand can support the contralateral intact hand for bimanual activities. This difficult decision is even more challenging within the limited anatomical confines of the hand. Based on our literature review, this is the first case of double central 3rd and 4th ray amputation, as far as we know with good hand function, evaluated by the Musculoskeletal Tumor Rating Scale.

Adaptive radiotherapy in patients receiving neoadjuvant radiation for soft tissue sarcoma.

AIM: The aim of this study is to evaluate tumor volume changes during preoperative radiotherapy and to assess the role of adaptive radiation. BACKGROUND: Contemporary neoadjuvant radiotherapy utilizes image guidance for precise treatment delivery. Moreover, it may depict changes in tumor size and shape. MATERIALS AND METHODS: Between 2016 and 2018, 23 patients aged ≥18 years with soft tissue sarcoma were treated with neoadjuvant radiation followed by surgical resection. The tumor volumes (cc) were measured using the Pinnacle planning system prior to starting radiotherapy and during treatment, the changes in volume and absolute differences were estimated. Moreover, patient's position on the machine was evaluated to assess setup offsets. The triggers for plan adaptation were >1 cm expansion or unacceptable setup offsets. RESULTS: The mean tumors volume at presentation was 810 cc (range, 55-4000). At last cone beam CT the tumor volume had changed in 14 patients (61%); it was stable in nine patients (39%). Disease regression was documented in eight patients (35%), with median shrinkage of -20.5% (range, -2 to -29%), while tumor progression was observed in six cases (26%), the median change was 12.5% (range, +10 to +25%).Adaptive radiation was required in four patients (17%). For the remaining 19 cases (83%), the dose distribution was adequate to cover target volumes. CONCLUSIONS: Change in soft tissue sarcoma volume during radiation is not uncommon. Image guidance should be used to reduce setup errors and to detect differences in tumor volume. Image guidance and adaptive radiation are paramount to ensure optimal radiation delivery.

Enhancing value of quality assurance rounds in improving radiotherapy management: a retrospective analysis from King Hussein Cancer Center in Jordan.

PURPOSE: The quality assurance (QA) chart rounds are multidisciplinary meetings to review radiation therapy (RT) treatment plans. This study focus on describing the changes in RT management based on QA round reviews in a single institution. MATERIALS AND METHODS: After 9 full years of implementation, a retrospective review of all patients whose charts passed through departmental QA chart rounds from 2007 to 2015. The reviewed cases were presented for RT plan review; subcategorized based on decision in QA rounds into: approved, minor modifications or major modifications. Major modification defined as any substantial change which required patient re-simulation or re-planning prior to commencement of RT. Minor modification included treatment plan changes which didn't necessarily require RT re-planning. RESULTS: Overall 7,149 RT treatment plans for different anatomical sites were reviewed at QA rounds. From these treatment plans, 6,654 (93%) were approved, 144 (2%) required minor modifications, while 351 (5%) required major modifications. Major modification included changes in: selected RT dose (96/351, 27%), target volume definition (127/351, 36%), organs-at-risk contouring (10/351, 3%), dose volume objectives/constraints criteria (90/351, 26%), and intent of treatment (28/351, 8%). The RT plans which required major modification according to the tumor subtype were as follows: head and neck (104/904, 12%), thoracic (12/199, 6%), gastrointestinal (33/687,5%), skin (5/106, 5%), genitourinary (16/359, 4%), breast (104/2387, 4%), central nervous system (36/846, 4%), sarcoma (11/277, 4%), pediatric (7/251, 3%), lymphoma (10/423, 2%), gynecological tumors (2/359, 1%), and others (11/351, 3%). CONCLUSION: Multi-disciplinary standardized QA chart rounds provide a comprehensive and an influential method on RT plans and/ or treatment decisions.

Advanced Tracheal Adenoid Cystic Carcinoma with Thyroid Invasion Mimicking Thyroid Cancer Treated with Definitive Radiation: Case Report and Review of the Literature.

A 54-year-old female patient, a breast cancer survivor and a case of unresectable adenoid cystic carcinoma of the trachea, with thyroid invasion, presented with suprasternal neck swelling mimicking thyroid primary. A literature search was undertaken to highlight this rare presentation. There have been few reports in the literature describing tracheal adenoid cystic carcinoma involving the thyroid.