Retroperitoneal sarcomas (RPSs) are a heterogeneous group of mesenchymal tumors. The mainstay of treatment of RPS is curative surgical resection, which often involves a multivisceral resection. The predominant pattern of failure in RPS is local recurrence, which poses significant management challenges and limits survival. There is a paucity of high-level evidence to guide the management of primary and recurrent RPS, mainly due to the rarity of the disease. This review highlights the challenges and decision making in the diagnosis and management of recurrent RPS. 

Key words: chemotherapy, distant recurrence, leiomyosarcoma, liposarcoma, local recurrence, radiation therapy, retroperitoneal sarcoma, surgical resection 

Approximately 12,310 soft tissue sarcomas (STSs) were diagnosed in 2016. These tumors originate from mesenchymal cells throughout the body and account for less than 1% of all newly diagnosed cancers in adults. STSs comprise a heterogeneous group of tumors, with over 50 different histologic subtypes categorized based on the presumptive tissue of origin, each with varying individual characteristics and varying patterns of behavior. Most commonly, they originate in the extremity followed by the trunk as the second most common site of primary lesions. The likelihood of metastatic spread is highly variable among histologic subtype and primary disease site. Distant hematogenous metastases occur in up to 50% of patients with high-grade sarcomas but in only 5% of patients with low-grade sarcomas. Most metastases occur within 2 years following initial diagnosis. The risk of metastasis is dependent on several prognostic factors, including tumor depth, tumor size, and mitotic rate. The role of surgery in the management of metastatic disease is governed by careful patient selection in the context of a multidisciplinary plan including systemic treatment. Selection of patients for resection of sarcoma metastases is most appropriately based on consideration of factors associated with long-term survival. We review the role of surgery in the management of STS metastases, rational selection of patients for resection, and strategies for integrating surgery into multidisciplinary treatment planning for patients with metastatic STS. 

This review contains 3 figures, 3 tables and 60 references

Key words: gastrointestinal stromal tumor, hepatic sarcoma metastases, metastatic sarcoma, pulmonary sarcoma metastases, retroperitoneal sarcoma, sarcoma, soft tissue sarcoma, surgical resection of sarcoma metastases

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.

This review contains 5 figures, 4 tables and 28 references

Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision

Soft tissue sarcomas are rare tumors and are mostly managed in tertiary care centers. They are broadly classified based on their differentiation into multiple different types. They affect patients of all ages and mostly occur in the extremities. Pathologic examination of the biopsy or resection of the tumor is an extremely important aspect in the diagnosis of these tumors and appropriately guiding the clinical team in the management of these rare tumors. This review focuses on the aspects of pathologic evaluation of these tumors and discusses the morphologic aspects of the predominant soft tissue sarcomas based on differentiation. The review also highlights the latest molecular diagnostic studies that are used in the accurate subtyping of these tumors. 

This review contains 12 figures, 2 tables, and 28 references.

Key words: angiosarcoma, fibromatosis, fibrous, liposarcoma, myxoid, nerve sheath tumor, sarcoma, synovial sarcoma

Soft tissue sarcomas are a heterogeneous group of neoplasms that arise throughout the body, but most commonly in the extremity and trunk. A multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. For patients with localized soft tissue sarcoma, limb-sparing surgical resection is the mainstay of therapy, with radiation therapy and chemotherapy used as adjuncts to reduce local and distant recurrences. Amputation is reserved for patients with primary or recurrent tumors that cannot be grossly resected with a limb-sparing procedure and preservation of function, which should occur in less than 5% of patients. Most patients who die of soft tissue sarcoma die of metastatic disease, which becomes evident within 2 to 3 years of initial diagnosis in 80% of cases, highlighting the need for improved systemic therapy options and optimal treatment at the time of diagnosis.

This review contains 13 figures, 4 tables and 53 references

Key words: amputation, multimodality, radiation therapy, soft tissue sarcoma, staging, surgery, treatment, wide resection

Reconstruction of sarcoma defects of the trunk or extremity requires detailed understanding of a variety of techniques for wound closure. Advances in reconstruction and oncologic treatment have led to higher rates of limb salvage in patients who would have previously undergone amputation. Such defects often require consideration of the reconstructive ladder, moving from the most simplistic closure to free flaps. As previous surgeries or radiation therapy can create a challenging wound environment with scar and fibrosed tissues, at times the more complex up-front option will be a better long-term solution for a patient following sarcoma resection. A multidisciplinary approach to the initial management, resection, and reconstruction is certainly warranted to attain the best overall outcome for the patient from the oncologic and aesthetic standpoint. This review provides an overview of the reconstructive techniques employed in addressing defects of the trunk and extremities following sarcoma resection.

This review contains 20 Figures and 30 References. 

Key words: abdominal wall reconstruction, chest wall reconstruction, free flap, lower extremity reconstruction, pedicled flap, propeller flap, reconstructive elevator, reconstructive ladder, upper extremity reconstruction 

In this review, we cover the clinical use of radiation therapy in the treatment of soft tissue extremity and truncal sarcomas. After a brief overview of the seminal trials establishing the efficacy of radiation therapy as a component of limb-preserving therapy, we cover current indications for use and discuss when radiation therapy can be withheld without compromising oncologic outcomes. Controversies in treatment sequencing with surgery are fleshed out with pros and cons for both pre- and postoperative treatment. Finally, different modalities of radiation therapy are discussed and common toxicities are covered.

This review contains 3 figures, 1 table and 41 references

Key words:brachytherapy, limb preservation, neoadjuvant therapy, radiation therapy, soft tissue sarcoma

Extremity sarcomas are a heterogeneous group of malignant tumors with a varied propensity for local recurrence. This review focuses on factors associated with local recurrence and survival, diagnostic workup, management, outcomes, and surveillance. Local recurrence is more common in patients with previous local recurrence, positive margins, high-grade histology, and deep tumors. In the absence of metastases, the mainstay of treatment is limb-sparing surgery, with radiation to improve local control. Modalities such as brachytherapy or proton therapy may be valuable in the setting of previous irradiation. Systemic chemotherapy is typically limited to the treatment of distant disease, although chemotherapy can be delivered locally via limb perfusion or infusion for locally advanced or recurrent disease. Amputation is used if local control cannot be achieved while preserving adequate limb function or as a palliative option for pain, bleeding, or fungating tumors. Prognostic factors associated with poor survival include tumors that recur with high-grade histology, with a large size (> 5 cm), or within a short interval (< 16 months). Reports of 5-year overall survival in patients with locally recurrent sarcomas vary from 36 to 65%. Surveillance includes physical examination, cross-sectional imaging, and chest x-ray. Genetic profiling and intratumoral injections provide novel therapeutic targets.

This review contains 1 figure, 4 tables and 40 references. 

Key words: chemotherapy, hyperthermic isolated limb perfusion, intratumoral injection, isolated limb perfusion, local recurrence, margin status, radiation, soft tissue sarcoma, wide local excision 

Leiomyosarcoma of soft tissue is a rare tumor affecting one new patient in every 100,000 people yearly. The most frequent sites of origin are the retroperitoneum and abdomen, followed by the limbs and trunk, skin, major vessels, and, rarely, organs. Increasing evidence suggests that all of these tumors originate from the smooth cell muscles of blood vessels. Surgery is the main treatment for localized disease. The risk of disease progression at distant sites outweighs that of local recurrence, suggesting that extensive resections are not recommended, particularly for retroperitoneal tumors. Moreover, leiomyosarcomas are sensitive to various chemotherapy regimens. The eighth edition of the AJCC TNM staging system introduced new accurate nomograms for predicting patient survival that are improving prognostic stratification for identifying those patients at higher risk for disease progression who are likely to benefit from perioperative treatments. More research for this rare tumor is needed to identify innovative predicting and prognostic biomarkers and new effective treatment strategies.

This review contains 14 figures, 3  tables and 56 references

Key words: inferior vena cava leiomyosarcoma, leiomyosarcoma, retroperitoneal sarcoma

Proper workup of intra-abdominal and retroperitoneal sarcomas cannot be overstated in the management of these rare tumors. In particular, evaluation should begin with a thorough history and physical examination, including careful consideration of genetic syndromes. Next, computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast should be performed to determine whether a tumor is completely resectable as complete resection is the mainstay of sarcoma management. Completion staging studies with CT of the chest should also be performed. Following imaging, a core-needle biopsy, usually an interventional radiology–guided biopsy, is performed to obtain a tissue diagnosis. In certain large, intermediate- to high-grade tumors involving other vital organs, neoadjuvant chemotherapy or neoadjuvant radiation is considered to try to decrease the tumor in the hope of improving the chance of obtaining an R0 resection and to limit the extent of surgery. Overall, it is important to remember that prior to the initiation of any therapy, all patients should be evaluated and managed by a multidisciplinary team with expertise and experience in sarcoma.

This review contains 3 figures, 3 tables and 39 references

Key words: biopsy, genetic testing, intra-abdominal and retroperitoneal sarcoma, neoadjuvant chemotherapy, neoadjuvant radiotherapy

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T₂ hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials.

This review contains 4 figures, 4 tables and 54 references

Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T₂ hyperintense tails, undifferentiated pleomorphic sarcoma

Angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE) are rare but clinically disparate vascular sarcomas. Common AS scenarios include the head and neck/scalp region in older patients or in association with chronic lymphedema or radiation therapy (often in the setting of breast cancer treatment). AS behaves like a high-grade tumor with a propensity for multifocality and hematogenous metastases. The potentially curative treatment is negative margin wide resection. Adjuvant radiation may be considered to does not reduce the risk of local recurrence. Unresectable localized tumor can be treated with definitive radiation therapy. Although data are limited, neoadjuvant and adjuvant chemotherapy are occasionally used. Extensive primary tumor or metastatic disease is treated with systemic therapy. EHE can arise in the liver, bone, lung, pleura, or skin/soft tissue and can also metastasize hematogenously. The tumor is frequently asymptomatic and can be clinically indolent. Therefore, specific therapy is reserved for documented progression. Limited primary tumor is treated with surgical resection and occasionally adjuvant radiation. There is no proven benefit to adjuvant chemotherapy. Liver-directed therapy and occasionally transplantation are used for unresectable hepatic disease. Metastatic disease is treated with systemic therapy, but only in the setting of clinical progression.

Key words: angiosarcoma, breast, cutaneous, epithelioid hemangioendothelioma, lymphedema, neoadjuvant chemotherapy, radiation associated, visceral

Soft tissue sarcoma (STS) represents a diverse group of malignant tumors derived from mesenchymal elements. Evaluation of a soft tissue mass of the trunk or extremity starts with a detailed history and physical examination, which will help guide further diagnostic evaluation.  Imaging should then be obtained for an STS, with MRI being the preferred modality for the trunk or extremities. Imaging can assist with diagnosis and delineate the extent of the primary lesion, including the relationship to contiguous structures, for treatment planning and assessing resectability. Pathology diagnosis is most commonly performed via examination of a specimen obtained through image-guided percutaneous core-needle biopsy. This requires multidisciplinary planning between Surgery and Radiology to place the needle within the anticipated definitive surgical incision to allow for en bloc resection of the needle tract. Once the diagnosis of an STS is made, appropriate staging studies should be performed based on the history and physical examination and tumor histology, grade, and size. Clinical and pathology data are used to stage STS patients using the American Joint Committee on Cancer staging system, which incorporates the STS site, size and grade of the primary tumor, and absence or presence of nodal and distant metastatic disease.

This review contains 6 figures, 6 tables and 50 references

Key words: core-needle biopsy, extremity, histology, imaging, risk factors, soft tissue mass, soft tissue sarcoma, staging evaluation, trunk, tumor grade

Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation.

This review contains 3 figures, 10 tables and 50 references

Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)

This review discusses the clinical presentation, histopathologic characteristics, and diagnostic and treatment algorithms for several distinct fibroblastic and myofibroblastic tumor subtypes. Low-grade fibromyxoid sarcomas typically arise in the deep soft tissues of the proximal extremities or trunk in young adults. They tend to have a high local recurrence rate, although some may have distant metastases. Histologically, they consist of bland fibroblasts with a whorled or linear arrangement alternating with hypocellular myxoid stroma. Sclerosing epithelioid fibrosarcomas typically arise in the deep soft tissues of the lower extremities of middle-aged adults. They tend to be slow growing but clinically aggressive, with a high propensity for local recurrence and distant metastases. Classic histologic features include the presence of small, uniform, ovoid epithelioid cells with sparse cytoplasm, arranged in cords within a sclerotic fibrous stroma. Myxoinflammatory fibroblastic sarcomas mainly affect the hands and feet. They are prone to local recurrence, with a very low likelihood of distant metastasis. Histologically, they have nodular architecture and highly cellular areas with an abundance of inflammatory cells, among which characteristic large Reed-Sternberg–like and lipoblast-like cells and hypocellular areas rich in myxoid stroma can be seen. Inflammatory myofibroblastic tumors typically occur in children and young adults, with common anatomic locations being the omentum, mesentery, retroperitoneum, and lung. Histologically, they are composed of myofibroblastic spindle cells accompanied by a lymphoplasmacytic inflammatory infiltrate. Surgical resection is the mainstay of treatment for all of the above tumor types. Radiation therapy may be used as adjuvant therapy for tumors with a high risk of local recurrence, in the setting of positive margins, or for unresectable primary or metastatic disease. There is no definite role for standard chemotherapy, but in certain cases, targeted therapy may be of use, such as ALK inhibitors in ALK-positive inflammatory myofibroblastic tumors. 

Key words: inflammatory myofibroblastic sarcoma, low-grade fibromyxoid sarcoma, myxoinflammatory fibroblastic sarcoma, sclerosing epithelioid fibrosarcoma

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma.

This review contains 14 figures, 5 tables and 50 references. 

Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 

Medical imaging plays a pivotal role in the detection, diagnosis, and clinical management of primary soft tissue tumors. Various imaging modalities have been used, and each modality offers unique advantages in the workup of soft tissue tumors by localizing the lesions in different compartments of the body and characterizing macroscopic tissue composition of the lesions in a noninvasive and safe manner. We review the clinical role and technical aspects of the frequently used imaging modalities, including magnetic resonance imaging (MRI), computed tomography, ultrasonography, positron emission tomography, and plain radiography with an emphasis on MRI as a mainstay imaging modality and a brief discussion of advanced techniques. We also review imaging features of common soft tissue tumors that can be detected and characterized using current imaging techniques.

Key words: computed tomography, diffusion-weighted imaging, imaging, magnetic resonance imaging, positron emission tomography, soft tissue, technique, tumor, ultrasonography

Solitary fibrous tumors (SFTs) are a subtype of soft tissue sarcoma with fibroblastic differentiation. SFTs typically occur in patients between ages 50 and 70 and most commonly develop in the pleural space or abdomen, although they have been described in virtually every body site. Cross-sectional imaging (CT or MRI) is useful to help characterize the tumor, and core-needle biopsy is critical to obtain tissue for diagnosis. Definitive diagnosis is now made by immunohistochemical expression of STAT6, which results from a key genetic aberration (NAB2-STAT6 fusion) recently identified in SFTs. For patients with SFT, surgical resection is the mainstay of treatment. Current data suggest some benefit with adjuvant radiation therapy and systemic therapy; however, further studies are needed. After treatment, surveillance in SFT patients is important, and late recurrences have been reported. Although these tumors are generally indolent, with low metastatic potential, a subset of cases will demonstrate aggressive behavior. Simple risk assessment models have been developed to help predict clinical behavior. Novel predictive biomarkers and disease biology–driven targeted therapy options are needed.

This review contains 4 figures, 2 tables and 37 references

Key words: cellular angiofibroma, characteristic chromosomal translocation, dermatofibrosarcoma protuberans, Doege-Potter syndrome, hemangiopericytoma, perineuroma, preoperative angioembolization, schwannoma, solitary fibrous tumors

Several regional therapies are used for the local treatment of patients with soft tissue sarcomas (STS), especially for tumors at high risk for local recurrence. Surgery with negative tumor resection margins is the main treatment for primary STS. External-beam radiation therapy is considered for deeply seated, large, and high-grade disease to lower the risk of local recurrence. A combination of preoperative chemo-radiation is associated with improved local control. TNF-α-based isolated limb perfusion is another regional chemotherapy strategy available at specialized surgical oncology units for unresectable STS. Other strategies suitable for management of advanced STS include cryoablation and radiofrequency. This review discusses these and other current regional treatment strategies. 

This review contains 10 figures, 6 tables and 64 references

Key words: cryoablation, extremity, hyperthermia, isolated limb perfusion, limb infusion, radiotherapy, regional therapy, sarcoma

Liposarcoma is a mesenchymal malignancy that predominantly arises in the retroperitoneum, trunk, and extremities. Histologic subtypes of liposarcoma include well-differentiated/dedifferentiated, myxoid/round cell, and pleomorphic liposarcoma. Although liposarcoma is rare and can occur in almost any anatomic location, it is the most common soft tissue sarcoma in the retroperitoneum, and surgery remains the only potentially curative therapy. This review discusses liposarcoma pathogenesis, classification, clinical-diagnostic evaluation, treatment, and surveillance. The cornerstone of treatment is surgery, with the main therapeutic goals of improving survival, limiting local recurrence, maximizing function, and minimizing morbidity. Perioperative radiation therapy may be indicated in patients with large high-grade liposarcomas. Systemic treatment—whether traditional chemotherapy or molecularly targeted therapy—may benefit patients depending on their histologic subtype, location, and extent of disease. For the majority of patients, however, systemic treatment is not curative. Controversial issues in the management of liposarcoma, such as surgical margins and extent of resection, are also discussed.   

This review contains 2 figures, 5 tables and 39 references

Key words: atypical lipomatous liposarcoma, liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, retroperitoneal sarcoma, soft tissue sarcoma, well-differentiated/dedifferentiated liposarcoma

Although neoadjuvant chemotherapy has been an established component of multimodality cancer care for patients with pediatric sarcomas for the past 25 years, the role of adjuvant or neoadjuvant chemotherapy in the management of adult patients with soft tissue sarcoma (STS) amenable to treatment with curative intent remains controversial. Overall, meta-analyses have revealed modest improvements in survival outcomes with the use of adjuvant or neoadjuvant chemotherapy, but individual trials have demonstrated inconsistent results leading some to question the robustness and external validity of the results. A recent randomized trial using anthracycline- and ifosfamide-based chemotherapy has provided further positive evidence in support of neoadjuvant chemotherapy for adult STS patients, but concerns persist regarding the risks of chemotherapy-related toxicities and the generalizability of the findings. Given the substantial risk of distant recurrence and disease-specific death for adult STS patients with tumors greater than 10 cm, especially those with synovial sarcoma and myxoid or round liposarcoma histologies, these patients should be strongly considered for neoadjuvant chemotherapy as part of a combined modality approach. The impact of recent level I data on the broader implementation of adjuvant or neoadjuvant chemotherapy in adult STS remains to be seen.

 This review contains 5 figures and 34 references

Key Words: chemotherapy, limb salvage, myxoid/round cell liposarcoma, multimodality therapy, soft tissue sarcoma, surgery, survival, synovial sarcoma, undifferentiated pleomorphic sarcoma

Peripheral nerve sheath tumors represent a spectrum of entities ranging from benign tumors, such as schwannoma, to high-grade malignant tumors termed malignant peripheral nerve sheath tumors. In this chapter, we discuss current concepts and problematic areas in the diagnosis, treatment, and management of peripheral nerve sheath tumors. We discuss the major categories of nerve sheath tumors including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. The different benign variants and their propensity for malignant transformation are highlighted. We suggest useful guidelines for the surgical management of these challenging cases. Finally, we discuss some of the advances in molecular biology that have provided new insights into the nature of the various peripheral nerve sheath tumors and suggest potential novel targeted therapeutic strategies for these tumors.

This review contains 11 figures, 5 tables and 66 references.

Key Words: malignant peripheral nerve sheath tumors, nerve sheath tumor, neurofibroma, neurofibromatosis, schwannoma, schwannomatosis, NF1, NF2

Desmoplastic small round cell tumor (DSRCT) is a relatively recently identified, rare, aggressive cancer that arises from the peritoneal lining of the abdominal cavity and predominantly affects male adolescents and young adults. DSRCT is included in the Ewing family of tumors and harbors a unique translocation between EWSR and WT1 genes. Patients characteristically present with advanced disease, including widespread involvement of the abdominal cavity and pelvis, with frequent extraperitoneal metastases involving liver, spleen, and thoracic lymph nodes. Five-year overall survival remains approximately 15 to 20% in recent series despite aggressive multimodal therapy. Neoadjuvant chemotherapy, complete resection, defined as less than 1 cm³ of residual tumor, and consolidative whole abdominopelvic radiotherapy (WAP-RT) appear to play important role in curative treatment. The rarity of DSRCT makes large randomized trials difficult and consensus statements about clinical approach impossible. However, this chapter summarizes the best available data on the pathogenesis, diagnosis, and treatment of DSRCT; highlights the major advances made in these areas; and discusses the addition of local therapy to the treatment paradigm.

This review contains 3 figures, 1 table, and 51 references.  

Key Words: DSRCT, peritoneal malignancy, desmoplastic round cell tumor, surface malignancies of the peritoneum, desmoplastic small round cell tumor of the abdomen, surgical oncology, rare malignancies, surgical therapy of abdominal malignancies

Lymphotropic sarcomas encompass a rare subset of soft tissue sarcoma histotypes known for their distinct ability to metastasize to locoregional lymph nodes. Epithelioid and clear cell sarcomas are the predominant lymphotropic subtypes and the focus of this review. The utility of lymph node assessment, whether clinical, radiographic, and/or surgical, in patients with these sarcomas is presented. The role of sentinel lymph node biopsy as well as completion lymphadenectomy in patients with these lymphotropic sarcoma histotypes is discussed. Surgery remains the only potential curative option for these tumors, and advancement in histology-specific chemotherapeutic regimens and biologic therapy options is necessary.

 This review contains 6 tables and 50 references.

Key words: clear cell sarcoma, completion lymphadenectomy, epithelioid sarcoma, EWS-ATF1, limb-sparing surgery, lymph node metastasis, sentinel lymph node biopsy, soft tissue sarcoma

Desmoid-type fibromatosis is a rare disease that does not have metastatic potential. It can be locally aggressive or regress spontaneously. For this reason, and because many of these tumors are inoperable or surgery is associated with significant morbidity, nonoperative treatment modalities are now more commonly employed to manage these lesions. Active observation, ablative therapies, and systemic treatments such as doxorubicin-based chemotherapy or sorafenib can be prescribed to minimize morbidities associated with the disease and surgical intervention. A thorough understanding regarding desmoid biology and the relative indications for multimodality therapies is essential to triage patients for appropriate interventions.

This review contains 5 figures, and 29 references.

Key Words: active observation, β-catenin, desmoid, familial adenomatous polyposis, fibromatosis, soft tissue tumors