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Introduction: Excisional biopsies for soft-tissue sarcoma (STS) of the hand are commonly done outside of sarcoma centers and can compromise incorporation of the biopsy site into standard limb salvage or amputation flaps. We sought to identify risk factors for these suboptimal biopsies., Methods: We analyzed prospective data on all patients (N = 109) who underwent definitive resection of primary STS of the hand between 1995 and 2019 at our institution. Biopsies were classified by type (excisional vs. incisional/needle), whether they were done before referral, and whether the incision could be incorporated into standard limb salvage or amputation flaps (ILS biopsies) or not (NILS biopsies). Analyses examined potential predictors of NILS biopsies and whether outcomes differed by biopsy type., Results: Biopsies done before referral (N = 91) were more likely to be excisional (79% vs. 17%). Excisional biopsies were associated with smaller tumor size (median, 2.0 vs. 3.15 cm; P = 0.025) and longer time to first intervention (1.88 vs. 1.17 months; P = 0.001). Forty-eight percent of excisional and 29% of incisional biopsy sites required soft-tissue coverage at the time of definitive surgery ( P = 0.07). Biopsy type was not associated with Musculoskeletal Tumor Society score or need for amputation. Risk factors for NILS biopsies included larger tumor size, deep tumor, and excisional biopsy. High-risk areas for NILS biopsies included the carpal tunnel, volar wrist, first webspace, radial palm, and proximal thumb. NILS biopsies were associated with positive margins, need for soft-tissue coverage, and lower Musculoskeletal Tumor Society scores., Discussion: This study informs referral guidelines for patients with STS of the hand. Patients with tumors that are deep, large, or in high-risk locations should be referred to a sarcoma center before biopsy. If that is not possible, incisional biopsy in line with standard resection incisions or radiology-guided core needle biopsy is preferable to excisional biopsy., Type of Study: Prognostic study., Level of Evidence: Level II., Competing Interests: Support: This research was funded in part through the NIH/NCI Cancer Center Support Grant P30 CA008748. Jessica Lavery discloses salary support from the American Association of Cancer Research Project Genomics Evidence Neoplasia Information Exchange Biopharma Collaborative (GENIE BPC). The other authors report no conflicts of interest related to this study., (Copyright © 2023 by the American Academy of Orthopaedic Surgeons.)

Primary malignant tumors in the hand are rare as compared to benign lesions; however, it is essential for orthopeadic surgeons to be familiar with their clinical presentation. The approach to working up a mass in the hand must be methodical and thorough, and clinicians must maintain high index of suspicion when evaluating these lesions. Successful identification and treatment of malignant tumors of the hand may include referral to a sarcoma center for staging, workup, and multidisciplinary treatment. Surgical treatment of malignant tumors in the hand is uniquely complicated by the increased number of critical structures that may be in close proximity to the tumor. It is therefore imperative to understand the principles of diagnosis, staging, biopsy, resection, and amputation before treating malignant tumors of the hand., (Copyright © 2020 by the American Academy of Orthopaedic Surgeons.)

Atypical lipomatous tumor/well-differentiated liposarcoma is the most common sarcoma of soft tissue in adults. We describe the clinical, radiologic, and pathologic features of an atypical lipomatous tumor arising within the soft tissue of the left hand of a 68-year-old female that underwent transformation to dedifferentiated liposarcoma and eventually metastasized. At initial presentation, imaging demonstrated an extensively calcified fatty soft tissue mass with displacement of the digits. Following biopsy and staged debulking, the patient subsequently developed local recurrence, dedifferentiation, and widespread metastases to the lungs, pancreas, bone, and soft tissues. To our knowledge, this is the first case of a cytogenetically proven atypical lipomatous tumor of the hand that has undergone dedifferentiation with widespread metastases.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a low grade soft tissue sarcoma with a predilection for acral sites, being associated with a high rate of local recurrence but very infrequent distant metastases. Although a t(1;10) translocation resulting in TGFBR3-MGEA5 fusion has been reported as a recurrent genetic event in MIFS, this abnormality is seen only in a subset of cases. As no studies to date have investigated the spectrum of alternative genetic alterations in TGFBR3-MGEA5 fusion negative MIFS, we undertook a genetic analysis of this particular cohort for further molecular classification. Triggered by an index case occurring in the finger of a 37-year-old female and harboring a novel TOM1L2-BRAF fusion by targeted RNA sequencing we investigated potential recurrent BRAF abnormalities by screening a large group of 19 TGFBR3-MGEA5 fusion negative MIFS by fluorescence in situ hybridization. There were 6 (32%) additional MIFS with BRAF genetic abnormalities, including 5 gene rearrangements and one showing BRAF amplification. Interestingly, VGLL3 amplification, a recurrent genetic abnormality coexisting with t(1;10) in some MIFS, was also detected by fluorescence in situ hybridization in 4/6 (67%) BRAF-rearranged MIFS, but not in the BRAF-amplified case. Up-regulated VGLL3 mRNA expression was also demonstrated in the index case by RNA sequencing. The 7 BRAF-rearranged/amplified MIFS arose in the fingers (n=3), and 1 each in wrist, forearm, foot, and knee, of adult patients (36 to 74 y; M:F=4:3). The histologic spectrum ranged from predominantly solid growth of plump histiocytoid to epithelioid tumor cells with focal myxoid change to a predominantly myxoid background with scattered tumor cells. Varying degree of inflammatory infiltrates and large tumor cells with virocyte-like macronucleoli were observed in most cases. Immunohistochemical stains of phosphorylated ERK, a downstream effector of BRAF activation, were positive in all 4 cases tested (2 diffuse strong, 2 focal strong). Unlike t(1;10), BRAF rearrangements were only found in MIFS but not in 6 hemosiderotic fibrolipomatous tumor (HFLT) lacking TGFBR3-MGEA5 fusions (including 2 pure HFLT, 2 hybrid HFLT-MIFS, and 2 associated with pleomorphic hyalinizing angiectatic tumors).

Objective: To determine the imaging features of hemosiderotic fibrolipomatous tumor (HFLT), which has a propensity towards local recurrence and the potential to transform into myxoinflammatory fibroblastic sarcoma (MIFS)., Materials and Methods: The study included 8 patients with a diagnosis of HFLT and imaging at a tertiary cancer center. Imaging studies included radiographs (n = 2), ultrasound (n =  3), and MRI (n = 16). Imaging features were evaluated including location, calcification, sonographic echogenicity, vascular flow, size, border, signal characteristics, contrast enhancement, and blooming on MRI., Results: The HFLT was located in the ankle/foot in 4 out of 8 and was subcutaneous in 8 out of 8, ranging in size from 2 to 18 cm. Histology at initial diagnosis was HFLT in 5 out of 8 and HFLT with MIFS in 3 out of 8. None was calcified on radiography. On ultrasound 2 out of 3 were heterogeneously echogenic with ≥10 foci of vascular flow. Two out of 8 patients had MRI only at local recurrence. The tumor border was infiltrative in 4 out of 6 at initial diagnosis and in 2 patients with MRI at recurrence only. Fat and septae were present in 7 out of 8 at initial diagnosis and at recurrence. Signal intensity was iso-/hypointense to muscle on T1-weighted sequences in more than two thirds of the tumor in 4 out of 7 and hyperintense to muscle in at least one third of the tumor on fluid-sensitive sequences in 6 out of 8. Contrast enhancement was heterogeneous in 7 out of 7; blooming in two thirds of the tumor on gradient-echo sequence MRI indicated hemorrhage., Conclusion: The HFLT commonly presents as a mass with an infiltrative border, interspersed fat and septations at initial diagnosis and local recurrence on MRI regardless of histology of HFLT alone or with MIFS. Hemosiderin deposits may be detected as blooming on gradient-echo sequences.

Purpose: To determine whether the diagnosis of hand glomus tumors by magnetic resonance imaging (MRI) is associated with tumor size, tumor pathology, tumor location, and/or clinical suspicion., Methods: We reviewed our pathology database for patients with hand glomus tumors diagnosed between 2006 and 2013 and included those patients who had preoperative MRI at our institution. We excluded patients with recurrent and persistent tumors. Magnetic resonance imaging reports were reviewed for clinical history, tumor location, and associated bone erosion. Pathology reports were reviewed for diagnosis and tumor size. We classified MRI studies as positive (glomus tumor diagnosis), negative (no mention of glomus tumor as possible diagnosis), or indeterminate (glomus tumor mentioned as possible differential diagnosis). Fisher exact test was used to compare positive studies and those that were nondiagnostic (ie, either negative or indeterminate)., Results: Of the 46 patients who had pathologically confirmed hand glomus tumors, 38 had preoperative MRI studies. A total of 24 MRI studies were positive, 5 were indeterminate, and 7 were negative. Five patients had atypical pathology, 1 had a multifocal tumor, and 2 had extra-digital hand glomus tumors. Failure to diagnose glomus tumors on MRI was associated with atypical pathology, atypical location (ie, not located in the subungual region), absence of bone erosion, and lack of clinical suspicion. Tumor size was not associated with MRI diagnosis., Conclusions: In this series of 36 hand glomus tumors, one-third of MRI studies were nondiagnostic. Occurrence of nondiagnostic MRIs was more likely when glomus tumors were pathologically and/or anatomically atypical, without bone erosion, and with no or unrelated clinical history provided. These findings highlight the continued importance of clinical suspicion in glomus tumor diagnosis., Type of Study/level of Evidence: Diagnostic IV., (Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Purpose: With the continued advancement of limb salvage surgery, major upper-limb amputations are being performed less frequently and are generally reserved for patients with large, multifocal, or recurrent tumors for whom limb salvage is no longer an option. We conducted a retrospective review of the current indications and patient outcomes after major upper-limb amputations for malignant tumors., Methods: Using the institution surgical database, we identified 43 patients who underwent major upper-limb amputation for primary and metastatic malignant tumors from 1996 to 2008., Results: Of these 43 patients, 25 had soft tissue sarcoma, 7 had bone sarcoma, and 11 had carcinoma. Two patients had stage I, 3 had stage II, 21 had stage III, and 17 had stage IV disease. We performed 45 amputations: 5 below the elbow, 14 above the elbow, and 26 at the forequarter. Among the 45 amputations, 2 patients underwent a second more proximal amputation for local tumor recurrence. Of the 17 patients with stage IV disease, 10 underwent palliative amputation for symptom control. A total of 28 patients (65%) died. Median survival after amputation was 13 months (95% confidence interval, 8-19 mo). The 6-month cumulative incidence of local recurrence was 22%. Overall survival after forequarter amputations was 42% at 1 year., Conclusions: Survival after major upper-limb amputation is poor, especially because amputations are reserved for patients with advanced tumors. However, amputation remains an option for local tumor control and can palliate symptoms in selected patients. Improvement of survival requires more effective systemic treatment strategies., Type of Study/level of Evidence: Therapeutic IV., (Copyright © 2012 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Malignant tumors of the hand are rare, although there remain many instances in which marginal excisions are performed for unsuspected malignant hand lesions. Suboptimal biopsy incisions and inadvertent contamination during these excisions may result in larger resections or amputations being necessary to ensure complete removal of the tumor with negative margins. This article provides an update for the current management of patients with primary malignant and metastatic tumors of the hand, including the roles of adjuvant radiotherapy and chemotherapy for the more common hand tumors., (Copyright © 2010. Published by Elsevier Inc.)

Background: Partial hand amputations for malignant tumors allow tumor resection with negative resection margins, which is associated with lower local recurrence rates and improved overall survival while preserving native tissue, which improves functional outcome., Questions/purposes: We conducted this study to assess the functional outcome of double ray amputations of the hand., Methods: We retrospectively reviewed the records of five patients who underwent double ray amputations at our center over 12 years: four amputations of the fourth and fifth rays and one amputation of the second and third rays. Mean age at surgery was 34 years (range, 10-45 years), and minimum followup was 64 months (mean, 98 months; range, 64-136 months). All five patients had high-grade soft tissue sarcomas of the hand, two synovial sarcomas, two malignant peripheral nerve sheath tumors, and one undifferentiated sarcoma. No patients had detectable metastases at surgery., Results: Four of the five patients were completely disease-free at latest followup. One patient was alive with lung metastases detected 32 months after surgery. No patients developed local tumor recurrence. Functional assessment showed a mean Musculoskeletal Tumor Society score of 24 (range, 19-28) and mean grip strength 24% of the contralateral side (range, 17%-35%)., Conclusions: Although double ray amputation results in worse functional outcome than single ray, good key, tip, and tripod pinch can be preserved when the deep motor branch of the ulnar nerve is preserved, and this hand can still assist in bimanual hand activities. Our observations suggest double ray amputation is an acceptable hand-preserving procedure.

Purpose: Diagnosis of a soft tissue sarcoma of the hand is at times made only in retrospect after marginal excision of a presumed benign soft tissue mass. Magnetic resonance imaging (MRI) helps determine the presence of residual disease and the extent of contamination caused by marginal excision of unsuspected soft tissue sarcomas and assists in the planning of definitive treatment with surgery and radiotherapy when required. We sought to determine the accuracy of MRI in detecting residual sarcoma after marginal excision., Methods: We retrospectively studied case records and imaging studies for all patients with soft tissue sarcomas of the hand evaluated and treated at our institution from 1996 to 2005. We included in this study 33 patients who underwent definitive surgery at our center after prior marginal excision. MRI scans done before definitive wide resection were classified as positive or negative for residual tumor. A musculoskeletal radiologist, blinded to the sarcoma type, findings on histopathology, and surgery, reviewed 19 scans to see whether the accuracy could be improved., Results: There were 8 epithelioid sarcomas, 6 synovial sarcomas, 4 malignant fibrous histiocytomas, 2 leiomyosarcomas, 2 liposarcomas, 2 myxofibrosarcomas, and one each of 9 other diagnoses. A total of 11 were low-grade and 22 were high-grade tumors, with 4 superficial and 29 deep tumors. Pathology examination after definitive wide resection or partial hand amputation showed that 15 patients had residual tumor, 9 gross and 6 microscopic. The sensitivity of MRI in detecting residual soft tissue sarcoma of the hand was 60%, specificity was 78%, positive predictive value was 69%, and negative predictive value was 70%. The sensitivity of MRI in detecting gross residual soft tissue sarcoma of the hand was 89%, specificity was 79%, positive predictive value was 62%, and negative predictive value was 95%. Even when an experienced musculoskeletal radiologist reassessed 19 MRI scans, the accuracy did not improve., Conclusions: Magnetic resonance imaging does not reliably detect residual gross or microscopic soft tissue sarcoma after marginal excision of unsuspected soft tissue sarcomas of the hand, with residual tumor not readily distinguished from postoperative change. The absence of disease on MRI should not be used as the sole criterion in determining whether a repeat resection should be performed., Type of Study/level of Evidence: Diagnostic III., (Copyright 2010. Published by Elsevier Inc.)

Unlabelled: Single ray amputation after hand trauma or infection can result in good aesthetic and functional outcomes. The role of this procedure in the management of aggressive benign or malignant hand tumors has been described only in case reports and small case series. We retrospectively reviewed the records of all 25 patients who underwent single ray amputations at our center during a 10-year period; there were seven index, five middle, six ring, and seven small ray amputations performed. The minimum followup was 2 months (mean, 36 months; range, 2-120 months), with four patients having a followup of 1 year or less. No patients had local recurrences, although two patients had positive resection margins. One underwent repeat resection followed by radiotherapy. The other was treated with radiotherapy alone, as local tumor control would have required a hand amputation. Functional assessment based on the Musculoskeletal Tumor Society staging system showed an average of 27.5 (range, 21-30). Patients who underwent perioperative radiotherapy experienced a decrease in functional ability. Grip strength was an average of 66% (range, 38%-100%) of the contralateral side. Our study suggests single ray amputation for hand tumors has a low local recurrence rate and high functional scores. However, function can be compromised by radiotherapy and a decrease in grip strength by a mean of 34% is to be expected., Level of Evidence: Level IV, case series. See Guidelines for Authors for a complete description of levels of evidence.

Purpose: Hand amputations cause marked functional loss for patients. In patients with large soft tissue sarcomas of the hand, partial hand preservation is extremely challenging for surgeons attempting a complete resection of the tumor with negative resection margins. We conducted this review to examine the oncologic outcome, including local recurrence rate and patient overall survival, and functional outcome after resections for large soft tissue sarcomas with partial hand preservation., Methods: We performed a retrospective review of all patients with soft tissue sarcomas of the hand treated at our institution from 1995 to 2007. We identified 8 patients who had tumors at least 5 cm in maximum dimension and had tumor resection with partial hand preservation. The mean age at the time of surgery was 49 years (range, 10-80 years). Two patients had myxofibrosarcoma, 2 patients had synovial sarcoma, 2 patients had malignant fibrous histiocytoma, 1 patient had a malignant peripheral nerve sheath tumor, and 1 patient had a liposarcoma. Two patients had low-grade tumors, and 6 patients had high-grade tumors. Two patients had American Joint Committee on Cancer stage 1b tumors, and 6 patients had American Joint Committee on Cancer stage 3 tumors. No patients had distant metastases at the time of surgery. Hand function was evaluated using Musculoskeletal Tumor Society criteria., Results: Of the 8 patients, 1 died of distant metastatic disease, 1 developed local tumor recurrence and is alive with locally recurrent disease, and the other 6 patients are completely disease-free. The mean Musculoskeletal Tumor Society score was 26 (range, 19-29), with the 2 patients who had received double-ray amputations having the lower scores (19 and 24)., Conclusions: Partial hand preservation is possible in selected patients with large soft tissue sarcomas of the hand, obtaining low local recurrence rates, good overall survival, and good functional outcome. However, all effort should be made to achieve negative resection margins., Type of Study/level of Evidence: Therapeutic IV., (Copyright 2010 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Background: Previous studies highlight the risk of valgus ankle instability in children following vascularized fibular procedures. We have observed that persistent valgus instability results in valgus deformity in these ankles. The aim of this study was to explore the risk factors associated with valgus ankle deformity following vascularized fibular graft harvest., Methods: We present 31 patients with minimum follow-up of 2 years and maximum of 18 years. They underwent regular clinical evaluation of their ankles and routine radiological evaluation when valgus deformity became clinically apparent., Results: Five patients developed valgus ankle deformities. Risk factors for development of valgus deformity included age under 14 years (P = 0.02) and short [6 +/- standard deviation (SD) 1 cm] residual fibular lengths (P = 0.02). Age-residual fibula index (age in years plus residual distal fibula length in centimeters) under 16 strongly predicted the development of ankle deformity (P = 0.0008). Short residual fibular lengths were not consistently associated with valgus deformity. Children developed focal lateral tibial epiphyseal atrophy and premature antero-medial fusion of the distal fibular physis resulting in a concave-anterior bowing of the fibula. Skeletally mature patients had congruent joints and posterior rotation of the proximal fibula without bowing., Conclusions: Mechanical causes cannot solely explain valgus ankle deformity following vascularized fibula harvest. Secondary changes due to growth arrest in the ankle significantly contribute to this deformity.

Background: Although limb salvage is possible for most extremity sarcomas, amputation has often been advocated as the treatment of choice for tumors involving the digits. The thumb poses a dilemma, however, because loss of its function can severely impair the use of the hand and entire upper limb. The authors report their experience with thumb reconstruction following wide excision of bone and soft-tissue tumors of the thumb., Methods: This was a retrospective review of all patients who underwent excision of thumb or first ray tumors between 1994 and 2005 followed by reconstruction at Memorial Sloan-Kettering Cancer Center. Patient demographics, tumor pathologic findings, ablative and reconstructive operations performed, postoperative adjuvant therapy, recurrence, and survival were analyzed. Function and reconstruction outcomes were assessed., Results: Seventeen patients were identified with bone or soft-tissue sarcomas, melanoma, or squamous cell carcinoma. Median follow-up was 33 months (range, 6 to 105 months; mean, 47 months). Negative resection margins were achieved in all patients. Of the 16 patients who underwent reconstruction, three had a reconstruction using free tissue transfer, four had a pedicled flap, one had an axial based local flap, one had a fillet flap, and seven achieved wound closure directly or with the use of skin grafts. The mean American Musculoskeletal Tumor Society Score following reconstruction was 28.8 (95.8 percent)., Conclusions: Thumb reconstruction and salvage after tumor resection following wide excision is technically possible. Reconstruction following accepted reconstructive principles and techniques can result in acceptable functional outcomes with low risk of complication.

Background: Hand-sparing surgery is now the most common option for sarcomas of the hand. The importance of obtaining widely negative margins has increased the complexity of operations and the importance of soft-tissue and bony reconstruction. The authors review the experience at a single institution of hand reconstruction following sarcoma resection based on prospectively collected data., Methods: Data were collected for all patients undergoing sarcoma resection by a single surgeon. Variables evaluated included patient demographics, tumor pathologic findings, location, size, depth, grade, stage, method of resection and reconstruction, adjuvant therapies, complications, and patient outcomes., Results: Fifty-five patients (30 male and 25 female) fulfilled eligibility criteria. The median age was 36 years (range, 1 to 80 years). The most common abnormalities were epithelioid sarcoma (n = 9), soft-tissue malignant fibrous histiocytoma (n = 8), synovial sarcoma (n = 6), and fibrosarcoma (n = 4). Flap reconstructions were required in 27 patients. The most common reconstruction method was a finger fillet flap in eight patients, followed by the radial forearm flap in five patients and the gracilis flap and skin grafting in three patients each. A suboptimal biopsy before presentation was strongly predictive of the need for flap reconstruction (p < 0.001). Nine patients had complications (delayed healing or skin necrosis, fracture, nonunion, epidermolysis, and tendon adhesion). No microvascular complications occurred., Conclusions: The choice of hand reconstruction method following sarcoma resection depends on a complete knowledge of options, risks, and benefits. Extensive use of a limited number of techniques by the authors' institution appears to contribute to excellent success and complication rates, with safe, reliable, and effective soft-tissue coverage.

Objective: Low-grade myxofibrosarcoma often relentlessly recurs after surgical resection, with an unusual infiltrative growth pattern and sometimes without a discrete tumor nodule at pathologic examination. This study was undertaken to determine and show patterns of recurrent low-grade myxofibrosarcoma at CT and MRI., Conclusion: Unlike in most other histologic types of low-grade soft-tissue sarcoma, recurrent low-grade myxofibrosarcoma often is infiltrative; shows a tapering, tail-like margin and superficial spreading configuration; and metastasizes to various distant sites, including lungs, pleura, bone, adrenal gland, soft tissue, and mesentery. Knowledge of these unusual characteristics is important in assessing the presence and extent of recurrent low-grade myxofibrosarcoma before surgical reexcision.

Fibroma of tendon sheath is an uncommon benign soft tissue tumor with a predilection for the hand. A unique case involving the scapholunate and radiocarpal joints with bone erosion is reported. The patient presented with a transilluminating mass over the volar radial wrist associated with neuropathy in the superficial radial nerve distribution. Magnetic resonance imaging demonstrated a heterogeneous and lobulated mass with nonspecific signal characteristics closely associated with the scapholunate interval and the volar wrist soft tissues. Histologically it represented a fibroma of tendon sheath. The tumor is discussed and the relevant literature is reviewed.

Malignant ectomesenchymomas, rare and potentially aggressive tumors, occur in children and exhibit mesenchymal and neuroectodermal components. This report describes the first patient diagnosed with a malignant ectomesenchymoma of the hand. The patient was a 17-month-old male who developed a hypothenar mass on his left hand that was surgically excised. Microscopic evaluation revealed ganglioneuroblastic, rhabdomyosarcomatous, and chondrosarcomatous elements. Following excisional biopsy he was treated with cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide. After 3 courses of chemotherapy the patient had a wide reexcision with no residual tumor. The patient is 4 years from diagnosis, without evidence of disease.

With advances in diagnosis, staging, imaging, and adjuvant chemo- and radiotherapy,upper limb salvage surgery has become a realistic option for upper limb malignancies. Following an adequate and complete resection, reconstruction of ensuing defects consequently has become an area of increasing importance. Many options are available,including primary closure, skin grafting, local soft tissue flaps, regional pedicle and island flaps, free tissue transfer, composite free tissue transfer, allografts, endoprostheses,and tendon, nerve, or arterial grafting. Form, function, and cosmetic outcome influence surgical decision making, as do characteristics of the patient, tumor, and involved site.