Rare Presentation of Pediatric Multiple Enchondromatosis Limited to Single Ray or Single Nerve Distribution in the Hand: A Multicenter Case Series.

BACKGROUND: Multiple enchondromas in the pediatric hand is a relatively rare occurrence and the literature regarding its incidence and treatment is sparse. Within this rare subset of patients, we identified a unique cohort in which lesions are confined to multiple bones in a single ray or adjacent rays within a single nerve distribution. We review the clinical and pathologic characteristics and describe the indications for and outcomes of treatment in this unique subset of patients as well as offer conjectures about its occurrence. METHODS: Institutional review board (IRB)-approved retrospective multicenter study between 2010 and 2018 identified subjects with isolated multiple enchondromas and minimum 2-year follow-up. Data analyzed included demographics, lesion quantification and localization, symptoms and/or fracture(s), treatment of lesion(s), complications, recurrence, and presence of malignant transformation. RESULTS: Ten patients were evaluated with average age at presentation of 9 years (range: 4 to 16) and mean clinical follow-up of 6 years (range: 2.8 to 8.6). Five subjects had multiple ray involvement in a single nerve distribution and 5 had single ray involvement with an average of 4 lesions noted per subject (range: 2 to 8). All children in the study had histopathologic-proven enchondromas and underwent operative curettage±bone grafting. Indications for surgical intervention included persistent pain, multiple prior pathologic fractures, impending fracture and deformity. During the study period three subjects experienced pathologic fracture treated successfully with immobilization. Recurrence was noted in 40% at an average of 105 weeks postoperatively (range: 24 to 260) and appears higher than that reported in the literature. No case of malignant transformation was observed during the study period. CONCLUSIONS: A rare subset of pediatric patients with multiple enchondromas of the hand is described with lesions limited to a single ray or single nerve distribution. Further awareness of this unique subset of patients may increase our understanding of the disease and improve patient outcomes. LEVEL OF EVIDENCE: Level IV-therapeutic (case series).

Chondroma of the urinary bladder: A case report.

RATIONALE: Chondromas are benign tumors comprising cartilaginous tissue that commonly occur in the small bones of the hands and feet. Chondromas are extremely rare in extraskeletal soft tissues, and only six cases of bladder chondromas have been reported thus far. PATIENT CONCERNS: A 75-year-old woman presented with abdominal pain and urinary symptoms, including increased frequency and a weak stream. DIAGNOSIS: Cystoscopy revealed a well-defined bladder mass over the anterior bladder wall. The pathology report showed neoplastic chondrocytes within the hyalinized and focal myxoid matrix, and immunopositivity for S-100, leading to the seventh known diagnosis of bladder chondroma. INTERVENTIONS: The tumor was endoscopically resected. The postoperative stay was uneventful, and 5 days later, the patient was discharged after the removal of the urinary catheter. OUTCOMES: One month after surgery, repeated cystoscopy showed no recurrence of the bladder tumor, and the patient reported improvement in urinary symptoms and relief of lower abdominal pain. LESSONS: Chondromas of the urinary bladder can present as urinary symptoms and abdominal pain in older patients. Transurethral resection is the treatment of choice.

Incomplete Carney Triad: A Surgical Case of a Rare Syndrome.

We describe a 36-year-old woman with multiple gastric gastrointestinal stromal tumors, hepatic and lymphatic metastasis, and a mediastinal paraganglioma as a presentation of an incomplete Carney triad. We present our therapeutic approach, with emphasis on the surgical and oncologic specificities of this syndrome.

Clinical and Imaging Features of Tumors in the Scapula.

BACKGROUND: The scapula is a small irregular-shaped flat bone, which may suffer from a variety of tumors or tumor-like lesions. As the imaging manifestations are complex and changeable, correct imaging diagnosis is difficult. INTRODUCTION: At present, there are few related radiology literatures, and it is necessary to fully analyze the imaging signs of different types of benign and malignant tumors in scapula to guide clinical treatment. This study was to investigate clinical and imaging presentations of tumors and tumor- like lesions in the scapula so as to increase the diagnostic accuracy of diseases in the scapula. METHODS: Patients with scapular tumors confirmed by pathology were enrolled. The imaging and clinical data were analyzed. RESULTS: Among 108 patients, benign tumors were in 53 (49.1%) cases, intermediate in seven (6.5%), and malignant in 48 (44.4%) involving 16 diseases. Osteochondroma was the first benign tumors in 45 cases accounting for 84.9% of all benign scapular tumors, followed by chondroma in four cases (7.5%). The intermediate tumors were mainly eosinophilic granuloma in four cases. Metastatic tumors were the commonest malignant tumor (27 cases or 56.2% of all malignant tumors), followed by chondrosarcoma (in 13 cases). Except for the one case of chondroblastoma in which the lesion involved the glenoid cavity, all the other cartilaginous tumors were located in the scapular body and processes. The type of lesions in the bony processes is the same as in the scapular body, the common lesions in the central area of the body were malignant tumors, and the commonest lesions in the glenoid area were metastasis. Common imaging features of malignant scapular tumors were ill-defined margins, cortical destruction and soft tissue involvement. The imaging features of chondrosarcoma lack specificity except for calcification. Benign lesions usually had a clear boundary and marginal sclerosis. CONCLUSION: A wide variety of benign and malignant tumors may occur in the scapula with mostly cartilaginous and metastatic tumors, and the location and distribution of lesions are similar in the scapula to those in the long bones.

Evaluation of triage tool for low-grade cartilage tumors: Four-quadrant approach.

BACKGROUND AND OBJECTIVES: The "four-quadrant approach" (FQA) for triage of benign enchondromas (E) and low-grade malignant chondrosarcomas (LGC) divides patients into treatment categories based on the presence or absence of pain and observation of aggressive or benign radiographic features. This article evaluates the usefulness of the FQA in predicting E versus LGC and operative versus nonoperative outcome. METHODS: Patients had working diagnosis of E or LGC, 1-year minimum follow-up, imaging, clinical data, outcomes, and no radiographic evidence of high-grade chondrosarcoma. Statistical analysis determined whether quadrant distribution correlated to E versus LGC and operative versus nonoperative intervention. RESULTS: Of 56 lesions (49 patients), 9 were LGC and 47 E. Twenty-five lesions (all 9 LGC, 16 E) were treated operatively and 31 (all E) nonoperatively. There were statistically significant correlations between quadrant distribution and both tumor type (p = 1.9 × 10-6 ) and operative intervention (p = 6.28 × 10-6 ). CONCLUSIONS: The FQA is a promising diagnostic tool to distinguish between E and LGC hyaline cartilage tumors, along with determining operative versus nonoperative intervention. Prospective evaluation is warranted.

Benign Cartilage-forming Tumors.

Although uncommon in many pathology practices, cartilage-forming tumors represent some of the most frequent primary bone tumors. Diagnosis can be challenging given their variable histologic spectrum and the presence of overlapping morphologic, immunohistochemical, and genetic features between benign and malignant entities, particularly low-grade malignancies. Correlation with clinical findings and radiographic features is crucial for achieving an accurate diagnosis and appropriate clinical management, ranging from observation to excision. Tumors can be characterized broadly by their location in relation to the bone (surface or intramedullary). In specific instances, ancillary testing may help.

Epiphyseal enchondroma masking as osteoid osteoma: a case report.

BACKGROUND: Enchondromas originating in the epiphyses of long bones are rare and epiphyseal osteoid osteomas are also uncommon. Diagnosis can become elusive when enchondromas or osteoid osteomas occur in atypical locations and present with nonspecific clinical and imaging characteristics. CASE PRESENTATION: We report a case of epiphyseal enchondroma of the left proximal femur in a 15-year-old girl with a 2-month history of left lower extremity pain. Preoperative CT displayed thickened cortex in the anterior surface of the left proximal femur with specks of calcification and inhomogeneity of the adjacent bone marrow cavity. She was diagnosed with osteoid osteoma. Postoperative pathological examination of surgically excised specimens revealed a diagnosis of enchondromas. CONCLUSIONS: Our case highlights that enchondroma should be considered in lesions of the epiphysis.

Cytopathology of chondromyxoid fibroma: a case series and review of the literature.

INTRODUCTION: Chondromyxoid fibroma is a rare bone tumor characterized by immature myxoid mesenchymal tissue showing early primitive cartilaginous differentiation. There have been limited case reports describing the cytologic features of chondromyxoid fibroma. Herein, we reported cytologic features of chondromyxoid fibroma on fine-needle aspiration (FNA). MATERIALS AND METHODS: We performed a retrospective search in our cytopathology and surgical pathology database for cases diagnosed as chondromyxoid fibroma that had corresponding cytology specimens from three medical institutions. All available cytopathology specimens were reviewed. RESULTS: Eight cases were retrieved from patients aged 16-77 years (mean, 51 years), and M:F ratio of 1.7:1. Seven tumors (88%) were primary, and most (62%) occurred in flat bones. Cytologic diagnoses were made in 6 cases with cytologic slides not available to review in 1 case. All cases showed metachromatic matrix in the background, while hyaline cartilage fragments were absent. All cases had two cell populations comprising oval to round cells and stellate to spindle cells. The spindle to stellate cells were more commonly embedded in matrix material. Moderate atypia (hyperchromasia and moderate anisonucleosis) was present in 4 cases (80%), while no mitotic figure was present in all cases. CONCLUSIONS: Our study highlights common cytologic features of chondromyxoid fibroma, including the presence of the spindle or stellate cells embedded in matrix material. Hyaline cartilage is uncommon and, if present, diagnostic considerations should include enchondroma or low-grade chondrosarcoma. A specific cytologic diagnosis primarily using FNA samples can be challenging but possible when evaluated in conjunction with clinical and radiologic data.

Calcaneal Bone Tumors.

Bone tumors of the foot are an uncommon finding. Most tumors are found incidentally on imaging and are benign. Care must be taken although due to the aggressive nature of malignant bone tumors that can occur in the calcaneus. Malignant lesions will more commonly present with symptoms of pain and swelling. Often misdiagnosed as soft tissue injuries, it is critical to be able to diagnose and treat these lesions early. Imaging plays an important role with plain films and advanced imaging. Surgical treatments can range from curettage with grafting to amputation for more aggressive lesions.

The Natural History of Benign Bone Tumors of the Extremities in Asymptomatic Children: A Longitudinal Radiographic Study.

BACKGROUND: Benign bone tumors are common incidental findings in the pediatric population during radiographic evaluation. Counseling these patients requires reassurance and raises questions about the natural history of these tumors over time. The purpose of this study was to estimate the prevalence and observe the behavior of benign childhood bone tumors in an asymptomatic population. METHODS: A historical, longitudinal radiographic collection of healthy children was reviewed, which included comprehensive left-sided radiographs of the extremities at yearly intervals. In this study, 262 subjects with 25,555 radiographs were screened for benign bone tumors at a median age of 8 years (range, 0 to 18 years). All potential tumors were reviewed by a multidisciplinary panel, which confirmed the radiographic diagnosis of each lesion, the age at which the lesion first appeared, and the age at which it had resolved. Prevalence rates were calculated using the number of distinct subjects available for each radiographic location and age. RESULTS: Thirty-five tumors were identified in 33 subjects, including 19 nonossifying fibromas, 8 enostoses, 6 osteochondromas, and 2 enchondromas. The prevalence rate for all tumors combined increased with age and was 18.9% overall. The overall prevalence rates for specific tumor types were 7.5% for nonossifying fibromas, 5.2% for enostoses, 4.5% for osteochondromas, and 1.8% for enchondromas. Nonossifying fibromas demonstrated a bimodal distribution of prevalence, with a peak at 5 years (10.8%) and another after skeletal maturity (13.3%). The median age at the first appearance for all tumors combined was 9 years (range, 2 to 15 years), but varied by tumor type. Nonossifying fibromas often resolved (7 [37%] of 19), with further resolution possible beyond the last available radiograph. Enostoses, osteochondromas, and enchondromas persisted until the last available radiographs in all subjects. CONCLUSIONS: The prevalence of benign childhood bone tumors of the extremities was 18.9% in a historical asymptomatic population. Longitudinal radiographs allowed observation of the timing of the first appearance and the potential for resolution for each tumor type. These findings provide unique evidence to answer many commonly encountered questions when counseling patients and their families on benign bone tumors. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Hand tumors: A review of 186 patients at a single institute.

PURPOSE: The spectrum of diagnoses and clinical features of hand tumors differ from those of tumors in other body parts. However, only a few reports have comprehensively referenced the diagnosis and clinical features of hand tumors. This study aimed to elucidate the diagnostic distribution and the clinical features of hand tumors undergone surgery in our institute. PATIENTS AND METHODS: A total of 235 lesions in 186 patients diagnosed with hand tumors between 1978 and 2020 were reviewed. Age at surgery, gender, chief complaint, tumor location, and pathological diagnosis were analyzed. RESULTS: There were 121 benign bone tumors, 98 benign soft tissue tumors, and 16 malignant tumors. Chondroma and tenosynovial giant cell tumor were common benign bone and soft tissue tumors at the proximal phalanx of the ring finger and the palm, respectively. Meanwhile, chondrosarcoma and synovial sarcoma were common malignant tumors at the dorsal part of the hand. Local pain and painless mass were the chief complaints in patients with benign bone and soft tissue tumors, respectively. Most patients with malignant tumors were referred after unplanned resection. When patients were classified into two categories by tumor size according to maximal diameter, tumors larger than 19 mm had a significantly higher risk of malignant (p = 0.031) despite being smaller than other tumors in different body parts. CONCLUSION: When a tumor malignancy is suspected, the patient should be referred to a specialist to avoid unplanned resection or delayed diagnosis due to misdiagnosis. Knowing the distribution and clinical features should help in diagnosing hand tumors.

Can MRI differentiate between atypical cartilaginous tumors and high-grade chondrosarcoma? A systematic review.

Background and purpose - Adequate staging of chondroid tumors at diagnosis is important as it determines both treatment and outcome. This systematic review provides an overview of MRI criteria used to differentiate between atypical cartilaginous tumors (ACT) and high-grade chondrosarcoma (HGCS).Patients and methods - For this systematic review PubMed and Embase were searched, from inception of the databases to July 12, 2018. All original articles describing MRI characteristics of pathologically proven primary central chondrosarcoma and ACT were included. A quality appraisal of the included papers was performed. Data on MRI characteristics and histological grade were extracted by 2 reviewers. Meta-analysis was performed if possible. The study is registered with PROSPERO, CRD42018067959.Results - Our search identified 2,132 unique records, of which 14 studies were included. 239 ACT and 140 HGCS were identified. The quality assessment showed great variability in consensus criteria used for both pathologic and radiologic diagnosis. Due to substantial heterogeneity we refrained from pooling the results in a meta-analysis and reported non-statistical syntheses. Loss of entrapped fatty marrow, cortical breakthrough, and extraosseous soft tissue expansion appeared to be present more often in HGCS compared with ACT.Interpretation - This systematic review provides an overview of MRI characteristics used to differentiate between ACT and HGCS. Future studies are needed to develop and assess more reliable imaging methods and/or features to differentiate ACT from HGCS.

[Hamartochondroma: An unusual cause of endobronchial obstruction in smoking patients]. / L'hamartochondrome : cause inhabituelle d'obstacle endobronchique chez des patients fumeurs.

Endobronchial hamartochondroma is a rare benign tumor which differs from the parenchymal form in its symptomatology and also by its treatment which should be as conservative as possible. The endobronchial location is exceptional. Here we present the cases of two patients with endobronchial hamartochondroma associated with clinical manifestation, chest pain and repeated pulmonary infections, respectively. The diagnosis was made after performing a CT-scan, a PET-SCAN and histological analysis. After discussion in a multidisciplinary staff meeting, conservative treatment was chosen in both cases.

FDP Avulsion Injury Due to Enchondroma: A Unique Surgical Approach.

Flexor Digitorum Profundus avulsion injury associated with an enchondroma at the level of the distal phalanx is extremely rare. There have been few cases reported to date and most have been surgically managed using a screw and/or Bunnell pull-out wire technique with or without bone grafting. We describe the first case using a simple interosseus fixation technique for the reattachment of FDP tendon without bone grafting. The patient made an excellent post-operative recovery. This technique is a simple, effective, patient-friendly and internalised solution which, in addition, may prevent the need for bone grafting.

Differential Diagnosis of Cartilaginous Lesions of Bone.

CONTEXT.­: Cartilaginous tumors represent one of the most common tumors of bone. Management of these tumors includes observation, curettage, and surgical excision or resection, depending on their locations and whether they are benign or malignant. They can be diagnostically challenging, particularly in small biopsies. In rare cases, benign tumors may undergo malignant transformation. OBJECTIVE.­: To review common cartilaginous tumors, including in patients with multiple hereditary exostosis, Ollier disease, and Maffucci syndrome, and to discuss problems in the interpretation of well-differentiated cartilaginous neoplasms of bone. Additionally, the concept of atypical cartilaginous tumor/chondrosarcoma grade 1 will be discussed and its use clarified. DATA SOURCES.­: PubMed (US National Library of Medicine, Bethesda, Maryland) literature review, case review of archival cases at the Massachusetts General Hospital, and personal experience of the authors. CONCLUSIONS.­: This review has examined primary well-differentiated cartilaginous lesions of bone, including their differential diagnosis and approach to management. Because of the frequent overlap in histologic features, particularly between low-grade chondrosarcoma and enchondroma, evaluation of well-differentiated cartilaginous lesions should be undertaken in conjunction with thorough review of the imaging studies.