Practical Issues for Retroperitoneal Sarcoma.

BACKGROUND: Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. METHODS: A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed. RESULTS: Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed. CONCLUSIONS: An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma.

A Modified Lodwick-Madewell Grading System for the Evaluation of Lytic Bone Lesions.

OBJECTIVE: Lodwick's well-established grading system of lytic bone lesions has been widely used in predicting growth rate for lytic bone lesions. We applied a Modified Lodwick-Madewell Grading System as an alternative means to categorize lytic bone tumors into those with low, moderate, and high risks of malignancy. MATERIALS AND METHODS: A retrospective review of the radiographs of 183 bone lesions was performed. Cases were selected to include a broad range of benign and malignant tumors. Readers applied our Modified Lodwick-Madewell Grading System, and consensus was reached in all cases. This modified system consists of grade I, which is composed of grades IA and IB as listed in the Lodwick system; grade II, which is grade IC in the Lodwick system; and grade III, which is composed of IIIA (changing margination), IIB (moth-eaten and permeative patterns), and IIIC (radiographically occult). Grading was correlated with the final diagnosis. RESULTS: Of the 183 tumors, 81 were classified as grade I, 54 as grade II, and 48 as grade III. When correlating grade with pathology, we found that 76 of 81 (94%) grade I lesions were benign and 39 of 48 grade III lesions (81%) were malignant. A nearly equal number of grade II lesions proved to be benign (29/54; 54%) and malignant (28/54; 53%). CONCLUSION: By expanding Lodwick's grading system to include two additional patterns of disease described by Madewell and colleagues (changing margination and radiographically occult) and by reclassifying them into three distinct grades, we propose a modified system-the Modified Lodwick-Madewell Grading System. Application of this system shows correlation of tumor grade with tumor biologic activity and with risk of malignancy: Grade I lesions are usually benign, grade II lesions carry moderate risk of malignancy, and grade III lesions possess a high likelihood of malignancy.

Sarcopenia as a predictor of complications in penile cancer patients undergoing inguinal lymph node dissection.

PURPOSE: Lymphadenectomy (LND) is part of the surgical management of penile cancer but causes significant perioperative morbidity. We determined whether sarcopenia, a novel marker of nutritional status, is a predictor of postoperative complications after LND. MATERIALS AND METHODS: Seventy-nine patients underwent LND for penile cancer from 1999 to 2014, and 43 had available preoperative abdominal imaging. Skeletal muscle index (SMI) was calculated on axial computed tomography images at the third lumbar vertebrae, and an SMI of 55 cm(2)/m(2) was used to classify patients as sarcopenic versus not. This classification was then correlated with postoperative complications and survival. RESULTS: Median lumbar SMI was 54.7 cm(2)/m(2) with 22 (51.2 %) patients categorized as sarcopenic versus 21 (48.8 %) who were not. Twenty-seven postoperative complications occurred in 20 patients within 30 days, of which 11 (40.7 %) were major (Clavien score ≥IIIa) and 16 (59.3 %) were minor. The most common complications were wound dehiscence (25.9 %), wound infection (18.5 %), lymphocele (18.5 %), and flap necrosis (14.8 %). On univariate analysis, the presence of sarcopenia, nodal disease, and lymphovascular invasion were predictors of postoperative complications. On multivariate analysis, only sarcopenia was an independent predictor of 30-day complications [p = 0.038; 95 % confidence interval (CI) 1.1-21.1]. Although sarcopenia was not statistically associated with worse overall survival (OS), there was a trend toward poorer outcomes in these patients. CONCLUSIONS: Sarcopenia can be a useful prognostic tool to predict the likelihood of postoperative complications after LND for penile cancer. Preoperative nutritional supplementation may help reduce complication rates in the future.

Familial gastrointestinal stromal tumor syndrome: report of 2 cases with KIT exon 11 mutation.

BACKGROUND: As with cases of sporadic gastrointestinal stromal tumor (GIST), familial GIST syndrome arises from mutations in KIT or PDGFRA. Only a few dozen such families have been described in the literature. METHODS: Cases of 2 individuals from 2 different newly described kindreds with familial GIST syndrome were retrospectively reviewed. Pertinent immunohistochemical stains, including CD117, CD34, DOG1, desmin, and S100, were performed. Samples from each case were sent to outside facilities for molecular analysis. A review of the relevant literature was performed and the number of familial GIST syndrome cases reported was updated through July 2014. RESULTS: In case 1, a woman 40 years of age with a family history of GIST presented with abdominal pain and gastrointestinal bleeding. Biopsy of a gastric mass revealed spindle-cell type GIST. Molecular analysis revealed a heterozygous mutation of p.Asp579del in exon 11 of KIT. The patient was placed on imatinib therapy and an initial positive response was demonstrated by imaging. Disease regression was seen on computed tomography, and several GIST tumors were surgically resected. The patient has had stable disease since surgery. In case 2, an asymptomatic woman 29 years of age presented for screening due to a family history of GIST. One small nodule was noted in her stomach and another was noted in the duodenum; both were surgically resected. The patient recovered well following surgery. The GIST in this patient was noted to have similar histological, immunohistochemical, and molecular findings as case 1. CONCLUSIONS: Imatinib has often been shown to be an effective therapy in both the familial and sporadic forms of GIST. There is no standard protocol for addressing the surveillance of patients with spindle-cell type GIST seen in the setting of familial GIST syndrome and with a p.Asp579del mutation of exon 11 on KIT.

Hibernoma: comparing imaging appearance with more commonly encountered benign or low-grade lipomatous neoplasms.

OBJECTIVE: Hibernoma is a benign neoplasm comprised of brown fat and characteristic hibernoma cells often occurring in adults with proximal extremity/truncal predilection. Limited reports regarding imaging appearance and clinicopathological features of this rare tumor are available. Four histological subtypes have been described. We present nine patients with hibernoma evaluating preoperative imaging findings (eight MRI; one PET, CT), which may distinguish hibernoma from more common benign or low-grade lipomatous neoplasms and attempt to correlate imaging with histological subtype. Clinicopathological data of 14 patients are presented. MATERIALS AND METHODS: Following IRB approval, retrospective review identified 14 patients from our institution between 2000 and 2012. Preoperative imaging, pathology, and medical records were reviewed. We tested the reader's ability to distinguish hibernoma from lipoma and atypical lipomatous tumor (ALT) at preoperative MRI based on specific imaging criteria. In a separate test, another reader attempted to predict histological subtype. Diagnostic accuracy was recorded. RESULTS: Six men and eight women with a mean age of 53 years were identified. Tumors involved the thigh (4), buttock (3), paraspinal muscles (3), retroperitoneum (2), shoulder (1), and knee (1). Eleven underwent margin negative resection; three excisional biopsy. When asked to differentiate hibernoma from lipoma and ALT, readers correctly identified 80 %, 80 %, and 100 % of hibernomas. Specific histological subtype was accurately predicted in most cases. Mean follow-up was 38 months with no local recurrences or metastases. CONCLUSIONS: Hibernomas can be difficult to differentiate from other lipomatous neoplasms, but identification of certain imaging features, common location, and patient demographics can improve preoperative confidence. Given benignity, accurate prospective diagnosis may affect treatment planning and surveillance strategy.

Bone Tumor Risk Stratification and Management System: A Consensus Guideline from the ACR Bone Reporting and Data System Committee.

The assessment and subsequent management of a potentially neoplastic bone lesion seen at diagnostic radiography is often complicated by diagnostic uncertainty and inconsistent management recommendations. Appropriate clinical management should be directed by risk of malignancy. Herein, the ACR-sponsored Bone Reporting and Data System (Bone-RADS) Committee, consisting of academic leaders in the fields of musculoskeletal oncology imaging and orthopedic oncology, presents the novel Bone-RADS scoring system to aid in risk assignment and provide risk-aligned management suggestions. When viewed in the proper clinical context, a newly identified bone lesion can be risk stratified as having very low, low, intermediate, or high risk of malignancy. Radiographic features predictive of risk are reviewed include margination, pattern of periosteal reaction, depth of endosteal erosion, pathological fracture, and extra-osseous soft tissue mass. Other radiographic features predictive of histopathology are also briefly discussed. To apply the Bone-RADS scoring system to a potentially neoplastic bone lesion, radiographic features predictive of risk are each given a point value. Point values are summed to yield a point total, which can be translated to a Bone-RADS score (1-4) with corresponding risk assignment (very low, low, intermediate, high). For each score, evidence-based and best practice consensus management suggestions are outlined. Examples of each Bone-RADS scores are presented, and a standardized diagnostic radiography report template is provided.

ACR Appropriateness Criteria® Chronic Extremity Joint Pain-Suspected Inflammatory Arthritis, Crystalline Arthritis, or Erosive Osteoarthritis: 2022 Update.

Evaluation for suspected inflammatory arthritis as a cause for chronic extremity joint pain often relies on imaging. It is essential that imaging results are interpreted in the context of clinical and serologic results to add specificity because there is significant overlap of imaging findings among the various types of arthritis. This document provides recommendations for imaging evaluation of specific types of inflammatory arthritis, including rheumatoid arthritis, seronegative spondyloarthropathy, gout, calcium pyrophosphate dihydrate disease (or pseudogout), and erosive osteoarthritis. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Chronic Hip Pain: 2022 Update.

Chronic hip pain is a frequent chief complaint for adult patients who present for evaluation in a variety of clinical practice settings. Following a targeted history and physical examination, imaging plays a vital role in elucidating the etiologies of a patient's symptoms, as a wide spectrum of pathological entities may cause chronic hip pain. Radiography is usually the appropriate initial imaging test following a clinical examination. Depending on the clinical picture, advanced cross-sectional imaging may be subsequently performed for further evaluation. This documents provides best practice for the imaging workup of chronic hip pain in patients presenting with a variety of clinical scenarios. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Cystic lesions of the pancreas: radiologic-endosonographic correlation.

Cystic lesions of the pancreas are relatively common findings at cross-sectional imaging; however, classification of these lesions on the basis of imaging features alone can sometimes be difficult. Complementary evaluation with endoscopic ultrasonography and fine-needle aspiration may be helpful in the diagnosis of these lesions. Cystic lesions of the pancreas may range from benign to malignant and include both primary cystic lesions of the pancreas (including intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, serous cystadenomas, pseudocysts, and true epithelial cysts) and solid neoplasms undergoing cystic degeneration (including neuroendocrine tumors, solid pseudopapillary neoplasms, and, rarely, adenocarcinoma and its variants). Familiarity with the imaging features of these lesions and the basic treatment algorithms is essential for radiologists, as collaboration with gastroenterologists and surgeons is often necessary to obtain an early and accurate diagnosis.

ACR Appropriateness Criteria® Chronic Elbow Pain.

Chronic elbow pain can be osseous, soft tissue, cartilaginous, and nerve related in etiology. Imaging plays an important role in differentiating between these causes of chronic elbow pain. This document provides recommendations for imaging of chronic elbow pain in adult patients. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Malignant or Aggressive Primary Musculoskeletal Tumor-Staging and Surveillance: 2022 Update.

Malignant or aggressive primary musculoskeletal tumors are rare and encompass a wide variety of bone and soft tissue tumors. Given the most common site for metastasis from these primary musculoskeletal tumors is to the lung, chest imaging is integral in both staging and surveillance. Extrapulmonary metastases are rarely encountered with only a few exceptions. Following primary tumor resection, surveillance of the primary tumor site is generally recommended. Local surveillance imaging recommendations differ between primary tumors of bone origin versus soft tissue origin. This document consolidates the current evidence and expert opinion for the imaging staging and surveillance of these tumors into five clinical scenarios. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Suspected Osteomyelitis, Septic Arthritis, or Soft Tissue Infection (Excluding Spine and Diabetic Foot): 2022 Update.

Musculoskeletal infections involve bones, joints, and soft tissues. These infections are a common clinical scenario in both outpatient and emergent settings. Although radiography provides baseline findings, a multimodality approach is often implemented to provide more detailed information on the extent of infection involvement and complications. MRI with intravenous contrast is excellent for the evaluation of musculoskeletal infections and is the most sensitive for diagnosing osteomyelitis. MRI, CT, and ultrasound can be useful for joint and soft tissue infections. When MRI or CT is contraindicated, bone scans and the appropriate utilization of other nuclear medicine scans can be implemented for aiding in the diagnostic imaging of infection, especially with metal hardware and arthroplasty artifacts on MRI and CT. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Management of isolated Adrenal Lesions in Cancer Patients.

BACKGROUND: Adrenal lesions are commonly identified in patients with extra-adrenal cancer. When lesions are present, it is important to identify if the lesion is a metastasis of the primary cancer or a primary adrenal neoplasm. If primary, the adrenal lesion must be evaluated for hypersecretion and its malignant potential determined for appropriate treatment planning. METHODS: Recent literature was reviewed that focused on the normal investigation of adrenal lesions including radiographic imaging and hormonal evaluations as well as specific focused therapeutic options available for isolated metastatic adrenal lesions. RESULTS: This review presents a pathway approach in investigating these lesions and also discusses various potential treatment options. CONCLUSIONS: A proper investigative workup of an adrenal lesion in a cancer patient is critical for proper management. Isolated adrenal metastatic lesions in the cancer patient should be surgically removed when possible, but other options can be considered. In patients who do not have metastasis from extra-adrenal cancer, the decision for surgical resection is dependent on functionality of the tumor and it's potential for malignancy. Observation plays a key role in those tumors that are nonfunctioning and have a low risk of malignancy.

Unusual Presentation of Widely Metastatic Extraskeletal Osteosarcoma: Case Report.

Extraskeletal osteosarcoma is a highly aggressive malignant osteoid forming mesenchymal neoplasm arising from soft tissues which accounts for 1% of all soft tissue sarcomas. We report the case of a 46-year-old female with no significant past medical history presenting to an emergency department with a right lateral thigh mass following minor trauma. She was eventually found to have high grade extraskeletal osteosarcoma with rapid progression of disease resulting in patient demise. Differentiation of these lesions from alternative processes relies on specific imaging and pathologic features. Differential diagnoses include both benign and malignant etiologies such as myositis ossificans, soft tissue hemangiomas, and other malignant soft tissue neoplasms such as epithelial and synovial sarcoma.

Retropubic parasymphyseal cyst: A rare entity.

We report a case of a retropubic parasymphyseal cyst in a 69-year-old multiparous female with a protracted history of metastatic small bowel carcinoid (neuroendocrine) tumor. Cysts related to the pubic symphysis are uncommon, and mostly reported in subpubic location. They may be confused with primary vulvar masses, malignant bone tumors or metastatic disease. In our case, encapsulation, lack of solid components or diffusion restriction, communication with the symphysis, lack of activity on Gallium-68-Dotatate PET/CT and signal characteristics on MRI similar to those previously reported in literature for subpubic cysts all aided in eventual diagnosis. We aim to remind the reader of this rare entity and its distinguishing features on imaging.

Synovial sarcoma of bone: Sarcoma typically of soft tissues presenting as a primary bone tumor.

Synovial sarcoma typically presents as periarticular soft tissue mass in adolescent and young adult patients. Very rarely, soft tissue sarcomas may arise primarily within bone posing a significant diagnostic challenge as primary osseous malignancies such as osteosarcoma and metastatic disease are much more common. While tissue sampling with immunohistochemical and genetic testing are required for definitive diagnosis, radiologists and orthopedic oncologists should consider alternate etiologies when typical imaging features of more common bone tumors are not identified. As an example, we present a 33-year-old male referred with a pathologic hip fracture proven to represent primary synovial sarcoma of bone.

Myopericytoma/myopericytomatosis of the lower extremity in two young patients: a recently designated rare soft tissue neoplasm.

Myopericytomas are rare, slow-growing benign perivascular tumors most commonly arising within the superficial subcutaneous soft tissues of the lower extremity. They represent one of several related perivascular tumors of myoid lineage with similar morphology and shared immunohistochemical profile including positive staining for smooth muscle actin. Histologically, myopericytoma exhibit concentric, perivascular proliferation of spindled myoid cells with bland elongated nuclei and associated blood vessels. A solitary well-demarcated nodule or mass is typically referred to as myopericytoma, whereas an infiltrative multinodular lesion has more recently been termed myopericytomatosis. At magnetic resonance imaging, tumors are most commonly superficial, may be well-defined (myopericytoma) or ill-defined (myopericytomatosis), and demonstrate highly vascularized, avidly enhancing soft tissue often with areas of internal hemorrhage. We report 2 cases involving the lower extremity (1 myopericytoma and 1 myopericytomatosis) occurring in young patients, focusing on the clinical, histopathologic, and radiologic characteristics of this relatively new distinct entity.

Paratesticular Liposarcoma Masquerading as an Inguinal Hernia.

A man with left scrotal swelling felt to represent inguinal hernia underwent computed tomography scan for reported bleeding after prostate biopsy. Computed tomography scan revealed a 15-cm extratesticular left scrotal mass containing both fat and soft tissue components, raising concern for dedifferentiated liposarcoma. At surgery and pathology, the mass was separate from the left testis, epididymis, and spermatic cord. Histopathologic findings were consistent with paratesticular dedifferentiated liposarcoma.