Henry L. Jaffe, MD: The Foundation of Orthopedic Pathology
Tumor-Induced Osteomalacia Localized and Excised After Pregnancy [Case Report]
Objective/UNASSIGNED:Tumor-induced osteomalacia (TIO) is a rare osteomalacia characterized by paraneoplastic secretion of fibroblast growth factor 23. Concomitant occurrence of TIO during pregnancy is rarer still. Our objective was to report a young patient with debilitating fractures diagnosed with TIO who became pregnant and subsequently had her tumor localized by gallium-68 (Ga-68) DOTATATE positron emission tomography/magnetic resonance imaging (PET/MRI). Case Report/UNASSIGNED:A 28 year-old woman with a 2-year history of stress fractures was found to have the following: (1) alkaline phosphatase level, 220 (reference range, 30-95) U/L; (2) phosphorus level, 2.1 (2.5-5.0) mg/dL; (3) 1,25-dihydroxyvitamin D3 level, <8 (18-72) pg/mL; (4) 24-hour urine phosphorus level, 0.5 (0.3-1.3) g; and (5) fibroblast growth factor 23 levels, 1241 (reference range, <180) RU/mL. The patient became pregnant, and at term, a cesarean delivery was performed. Ga-68 DOTATATE PET/MRI showed a 9-mm intracortical mass in the right fibular head and right femoral and bilateral calcaneal stress fractures. The fibular lesion was resected; pathology showed a 1.5-cm lesion with positive fibroblast growth factor receptor 1 staining. Discussion/UNASSIGNED:This patient with TIO had an uneventful pregnancy and delivery. TIO is typically caused by benign mesenchymal tumors. Ga-68 DOTATATE PET/computed tomography has been used for localizing tumors causing TIO, yet MRI has superior contrast resolution over computed tomography. Therefore, it is not surprising that Ga-68 PET/MRI successfully localized this patient's tumor to the intracortical space of the fibular head and distinguished it from insufficiency fractures. Conclusion/UNASSIGNED:To our knowledge, this is the first report of phosphate treatment in a pregnant patient with TIO and the first report of a tumor-inducing TIO being localized by Ga-68 DOTATATE PET/MRI.
Modern Management of High-risk Soft Tissue Sarcoma With Neoadjuvant Chemoradiation: A Single-center Experience
OBJECTIVE:Neoadjuvant chemoradiation (NA-CRT), followed by resection of high-risk soft tissue sarcoma (STS), may offer good disease control and toxicity outcomes. We report on a single institution's modern NA-CRT experience. MATERIALS AND METHODS/METHODS:Delay to surgical resection, resection margin status, extent of necrosis, tumor cell viability, presence of hyalinization, positron emission tomography (PET)/computed tomography data, and treatment toxicities were collected. Using the Kaplan-Meier survival analysis, 5-year overall survival, disease-free survival, distant metastasis-free survival, and local control (LC) were estimated. Clinicopathologic features and PET/computed tomography avidity changes were assessed for their potential predictive impact using the log-rank test. RESULTS:From 2011 to 2018, 37 consecutive cases of localized high-risk STS were identified. Twenty-nine patients underwent ifosfamide-based NA-CRT to a median dose of 50â€‰Gy before en bloc resection. At a median follow-up of 40.3 months, estimated 5-year overall survival was 86.1%, disease-free survival 70.2%, distant metastasis-free survival 75.2%, and LC 86.7%. Following NA-CRT, a median reduction of 54.7% was observed in tumor PET avidity; once resected, median tumor necrosis of 60.0% with no viable tumor cells was detected in 13.8% of the cases. Posttreatment resection margins were negative in all patients, with 27.6% having a margin of â‰¤1â€‰mm. Delays of over 6 weeks following the end of radiation treatment to surgical resection occurred in 20.7% cases and was suggestive of inferior LC (92.8% vs. 68.6%, P=0.025). CONCLUSIONS:This single-institution series of NA-CRT demonstrates favorable disease control. Delay in surgical resection was associated with inferior LC, a finding that deserves further evaluation in a larger cohort. LEVEL OF EVIDENCE/METHODS:Level III-retrospective cohort study.
Diagnosis and management of primary malignant tumors in the upper extremity
Bone and soft tissue sarcomas of the upper extremity are relatively uncommon. In many cases, they are discovered incidentally during evaluation of traumatic injuries or common ailments such as rotator cuff tendonitis or tennis elbow. Thus, it is important for all orthopedic surgeons to understand the differential diagnosis, workup, and treatment for upper extremity lesions. An appreciation of the clinical and radiographic features of primary malignant lesions aids in identifying patients that need referral to an orthopedic oncologist and a multidisciplinary team.
Excision of Prominent Bony Mass due to Skeletal Fluorosis: A Case Report
CASE/METHODS:A 72-year-old man presented for evaluation of bony prominences over extremities. Radiographic imaging demonstrated masses of varying sizes extending from the cortical surfaces without medullary continuity. The patient had a history of Freon inhalation abuse and was diagnosed with skeletal fluorosis due to elevated serum fluoride levels. He underwent an uncomplicated excision of a left fibular mass that was threatening skin breakdown. CONCLUSIONS:This is the first reported surgical case of skeletal fluorosis demonstrating continued enlargement of bony prominences throughout the body. Skeletal fluorosis not only causes diffuse mineralization but may also lead to protruding lesions throughout the body.
Flap Reconstruction of Sarcoma Defects in the Setting of Neoadjuvant and Adjuvant Radiation
PURPOSE/OBJECTIVE:â€ƒLimb-sparing treatment of extremity soft tissue sarcomas requires wide resections and radiation therapy. The resulting complex composite defects necessitate reconstructions using either muscle or fasciocutaneous flaps, often in irradiated wound beds. METHODS:â€ƒA retrospective chart review was performed of all limb-sparing soft tissue sarcoma resections requiring immediate flap reconstruction from 2012 through 2016. RESULTS:<â€‰0.048). At time of follow-up, Musculoskeletal Tumor Society scores for fasciocutaneous and muscle-based reconstructions, with or without radiation, showed no significant differences between groups (mean [SD]: 91% [8%] vs. 89% [13%]). CONCLUSIONS:â€ƒThe similar complication rates and functional outcomes in this study support the safety and efficacy of both fasciocutaneous flaps and muscle-based flaps in reconstructing limb-sparing sarcoma resection defects, with or without radiotherapy.
Modern Management of Soft Tissue Sarcoma with Preoperative Chemoradiation: A Single Center Experience [Meeting Abstract]
Posterior "Open Book" approach for type 1 internal hemipelvectomy
INTRODUCTION:/UNASSIGNED:Internal limb-sparing hemipelvectomy is currently the preferred surgical option for resection of pelvic tumours. Obtaining an acceptable functional outcome through the standard ilioinguinal or iliofemoral incisions, only compounds the already challenging dissection, resection and reconstruction of these extensive en-bloc extirpative oncologic operations. SURGICAL TECHNIQUE:/UNASSIGNED:We describe a novel surgical approach to the lateral pelvis that minimises injury to the gluteal muscles, spares the gluteal vessels and provides a broad yet shallow operative field conducive to microsurgery with sizeable vasculature in close proximity to facilitate any microsurgical reconstruction. Access to the ilium and sacrum for Enneking Type 1 and Type 4 resections respectively is obtained by reflecting a skin and soft tissue flap anteriorly while the gluteal muscles are reflected posteriorly and inferiorly. This technique minimises the amount of tissue reflected in any particular direction, therefore, providing a broad and shallow operative field which benefits the orthopaedic oncologic surgeon and the plastic reconstructive surgeon. CONCLUSION:/UNASSIGNED:The "posterior open-book" approach offers a promising alternative to the standard ilioinguinal or iliofemoral incisions for internal limb-sparing Type 1 hemipelvectomies while also optimising the exposure for subsequent reconstructive procedures.
Limb-sparing sarcoma reconstruction with functional composite thigh flaps
INTRODUCTION: Innervated muscle transfer can improve functional outcomes after extensive limb-sparing sarcoma resections. We report our experience using composite thigh flaps for functional reconstruction of large oncologic extremity defects. PATIENTS AND METHODS: Between 2011 and 2014, four limb-sparing oncologic resections (3 lower extremities, 1 upper extremity) underwent immediate functional reconstruction with composite thigh free flaps in three males and one female. The age of the patients ranged from 36 to 73 years. There were 3 soft-tissue sarcomas and one giant cell tumor, all required resection of entire muscle compartments. Flap components included fasciocutaneous tissue with sensory nerve, plicated iliotibial band (ITB), and variable amounts of motorized vastus lateralis (VL). RESULTS: All flaps survived without complications. All patients showed VL motor innervation by six months. Follow-up ranged from 20 to 36 months. Motor strength ranged from 2 to 5 out of 5, active range of motion was 25-92% of normal, and Musculoskeletal Tumor Society (MSTS) Scores were between 22 and 29 out of 30. CONCLUSIONS: Limb-sparing techniques for upper and lower extremity sarcomas continue to evolve. Our experience has validated the composite thigh free flap as an excellent option for one-stage functional reconstruction of large limb defects.
Orthopedic Oncology Caseload Among Orthopedic Surgery Residents
Despite educational focus regarding orthopedic oncology during residency, assessment of resident orthopedic oncology caseload has not been performed. The purpose of this study was to evaluate orthopedic oncology caseload trends and variation among residents. The Accreditation Council for Graduate Medical Education case log reports for orthopedic surgery residents were reviewed for graduating years 2007 to 2013. Trends in orthopedic oncology cases and variation in the median number of cases performed by residents in the 90th, 50th, and 10th percentiles of caseload were evaluated. The proportion of orthopedic oncology caseload among all cases performed by residents increased significantly (PÂ =Â 0.005) from 2007 to 2013. Likewise, the mean number of adult (PÂ =Â 0.002), pediatric (PÂ =Â 0.003), and total orthopedic oncology cases increased significantly (PÂ =Â 0.002). On average, residents in the 90th, 50th, and 10th percentiles performed 83, 28, and 3 cases, respectively. The current study demonstrates a significant increase in adult, pediatric, and total orthopedic oncology caseload. There is also evidence of substantial caseload variation among residents. Caseload variation may influence the education and technical proficiency of orthopedic residents.