"Slipped capital femoral epiphysis in a 25-year-old hypogonadic man with a large cranial chondroma: causality or coincidence? ".

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is a hip disorder frequently occurring in adolescence. In adults it is rare and so far very few cases have been documented. CASE PRESENTATION: This report presents a 25-year-old patient diagnosed with an anterior fossa giant chondroma, hypogonadotropic hypogonadism, and SCFE. The patient underwent surgical and hormonal therapy. His symptoms revealed, and he became a father. CONCLUSIONS: Every patient diagnosed with SCFE in adulthood should undergo endocrinological assessment based on physical examination and laboratory tests.

Outcome of conservative and surgical treatment of enchondromas and atypical cartilaginous tumors of the long bones: retrospective analysis of 228 patients.

BACKGROUND: Sufficient data on outcome of patients with clinically and radiologically aggressive enchondromas and atypical cartilaginous tumors (ACT) is lacking. We therefore analyzed both conservatively and surgically treated patients with lesions, which were not distinguishable between benign enchondroma and low-grade malignant ACT based upon clinical and radiologic appearance. METHODS: The series included 228 consecutive cases with a follow-up > 24 months to assess radiological, histological, and clinical outcome including recurrences and complications. Pain, satisfaction, functional limitations, and the musculoskeletal tumor society (MSTS) score were evaluated to judge both function and emotional acceptance at final follow-up. RESULTS: Follow-up took place at a mean of 82 (median 75) months. The 228 patients all had comparable clinical and radiological findings. Of these, 153 patients were treated conservatively, while the other 75 patients underwent intralesional curettage. Besides clinical and radiological aggressiveness, most lesions were histologically judged as benign enchondromas. 9 cases were determined to be ACT, while the remaining 7 cases had indeterminate histology. After surgery, three patients developed a recurrence, and a further seven had complications of which six were related to osteosynthesis. Both groups had excellent and almost equal MSTS scores of 96 and 97%, respectively, but significantly less functional limitations were found in the non-surgery group. Further sub-analyses were performed to reduce selection bias. Sub-analysis of histologically diagnosed enchondromas in the surgery group found more pain, less function, and worse MSTS score compared to the non-surgery group. Sub-analysis of smaller lesions (< 4.4 cm) did not show significant differences. In contrast, larger lesions displayed significantly worse results after surgery compared to conservative treatment (enchondromas > 4.4 cm: MSTS score: 94.0% versus 97.3%, p = 0.007; pain 2.3 versus 0.8, p = 0.001). The majority of lesions treated surgically was filled with polymethylmethacrylate bone-cement, while the remainder was filled with cancellous-bone, without significant difference in clinical outcome. CONCLUSION: Feasibility of intralesional curettage strategies for symptomatic benign to low-grade malignant chondrogenic tumors was supported. Surgery, however, did not prove superior compared to conservative clinical and radiological observation. Due to the low risk of transformation into higher-grade tumors and better functional results, more lesions might just be observed if continuous follow-up is assured.

Differing characteristics of cartilaginous lesions of the larynx.

INTRODUCTION: The tissues of the laryngeal region only rarely harbor primary cartilaginous lesions, and squamous cell carcinoma remains the most frequently encountered malignant tumor in this area. MATERIALS AND METHODS: We reviewed the salient histological features of cartilaginous laryngeal lesions to provide differential diagnostics and guidelines for distinguishing the benign from the malignant ones. RESULTS: Cartilaginous neoplasms of the larynx include chondroma and chondrosarcoma. Among the overarching group of all forms of laryngeal sarcoma, chondrosarcoma forms the most common entity in the larynx, followed by rhabdomyosarcoma. Cartilaginous tumors comprise about 0.1%-1% of all laryngeal neoplasms with chondrosarcomas being more frequently encountered than chondromas. Several neoplasms earlier reported as giant-cell tumors of the larynx would most likely, using current terminology, be classified as cases of undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma, giant-cell variant) or aneurysmal bone cyst. CONCLUSION: When true laryngeal sarcomas do exist, they may prove to be challenging lesions both for the pathologist and the treating clinician. The diagnostic problems are mainly a result of the infrequent exposure of clinicians and diagnosticians to these lesions.

Cystic bone tumors of the foot and ankle.

Bone tumors are relatively rare in the foot and ankle region. Many of them present as cystic lesions on plain films. Due to the relative rarity of these lesions and the complex anatomy of the foot and ankle region, identification of such lesions is often delayed or they get misdiagnosed and mismanaged. This review discusses the most common cystic tumors of the foot and ankle including their radiographic features and principles of management.

Nonsurgical Treatment as Alternative to Surgical Treatment in Enchondromas of the Distal Phalanx. Analysis of a Series of 11 Cases.

PURPOSE: To describe the results of nonsurgical and surgical treatment of enchondromas of the distal phalanx of the hand. METHODS: Eleven enchondromas of the distal phalanx were retrospectively reviewed. Five patients underwent surgery (curettage and autogenous cancellous bone graft from the iliac crest) and 6 patients were treated nonsurgically. Clinical records and radiographs were reviewed for each patient. We recorded complications and cosmetic and functional results. Radiographic healing in surgical patients was scored according to the classification of Tordai and to the criteria of Wilhelm and Feldmeier. The average follow-up of the nonsurgical and surgical cases was 45 and 62 months, respectively. RESULTS: Eight patients were women. Average age at diagnosis was 40 years. Nine patients presented with a pathological fracture. The demographic characteristics of the surgical group and nonsurgical group were similar. Among the cases treated nonsurgically (6), 1 had 2 pathological fractures after diagnosis. At final follow-up, the average pain on a visual analog scale was 2.8. With the exception of 1 patient, the range of motion of the fingers was normal or minimally reduced. Among the surgical cases (5), there were no complications in the bone graft donor site, 2 patients developed infections in the operated finger, and no postoperative pathological fractures were found. At final follow-up, the average pain was 3.2. Joint mobility was normal in 3 patients. Postoperative radiological examination revealed complete bone healing in all patients (grade I in the Tordai classification). No local recurrence was seen. According to the criteria of Wilhelm and Feldmeier, there were 3 excellent, 1 good, and 1 satisfactory results in the surgical group, and 2 excellent, 3 good, and 1 satisfactory results in the nonsurgical group. CONCLUSIONS: Surgical and nonsurgical treatment in distal phalanx enchondromas appear to be associated with satisfactory results, although each has their own advantages and disadvantages. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Function after injection of benign bone lesions with a bioceramic.

BACKGROUND: A novel calcium sulfate-calcium phosphate composite injectable bone graft substitute has been approved by the FDA for filling bone defects in a nonweightbearing application based on preclinical studies. Its utility has not been documented in the literature. QUESTIONS/PURPOSES: We therefore determined postoperative function and complications in patients with benign bone lesions treated with this bioceramic. METHODS: We retrospectively reviewed all 56 patients with benign bone lesions treated with the bioceramic from 2006 to 2008. There were 29 male and 27 female patients with an average age of 17.6 years (range, 4-63 years). They were treated for the following diagnoses: unicameral bone cyst (13), aneurysmal bone cyst (10), nonossifying fibroma (eight), fibrous dysplasia (five), enchondroma (four), chondroblastoma (four), and other (12). We obtained a Musculoskeletal Tumor Society (MSTS) functional evaluation on all patients. The minimum followup was 26 months (average, 42 months; range, 26-57 months). RESULTS: The average MSTS score was 29 (range, 20-30). Most patients returned to normal function. There were three local recurrences, all of which were treated with repeat injection or curettage. Two patients had postoperative fractures treated in a closed manner. Two patients had wound complications, neither of which required removal of the graft material. CONCLUSION: Patients treated with this material reported high MSTS functional scores more than 24 months after operative intervention and experienced low complication rates. We believe the novel bioceramic to be a reasonable treatment option for benign bone lesions.

Angiogenesis in hand chondroma: an immunohistochemical study.

Hand chondroma is a particular cartilagineous tumour, being clinically benign, but morphologically malignant. This study investigates the expression of VEGF together with other growth factors and proliferation markers such as TGFß2, Ki-67, TNF, FGF1, P53 in 8 cases of hand chondroma treated with courettage, in order to define the ethiopathogenesis of this tumour and the clinical significance of the resulting immunohistochemical profile, with particular respect to angiogenesis. VEGF was expressed in all cases; 5 cases were positive for TFGß2 and 3 for PDGF. None of the other factors was expressed. On the basis of histologic results a specific model of tumour progression based on the indicators of angiogenesis could be related to hand tumours, in which VEGF expression should be the first stadium of the tumour aggressiveness, and the following PDGF, TGF 2 expression should be accompanied with a morphological outline worsening. Nevertheless the non constant expression of these indicators and the absent expression of proliferated indicators can explain the scant tendency to the relapse in presence of accurate curettage. It is important to remember that the cellular polymorphism typical of the cartilaginous tumours does not allow the application of an only oncogenesis model.

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.

Chordoma of the sacrum and vertebral bodies.

Chordomas are relatively rare tumors of bone. These primary malignant lesions occur throughout the spinal column and often show advanced growth at the time of diagnosis. Because such tumors are minimally responsive to radiation and chemotherapy, surgical resection is the mainstay of treatment. Patient survival and local control are associated with the ability to achieve wide surgical margins during excision. However, surgical morbidity may be substantial given the propensity for chordomas to abut or surround neural, vascular, and visceral structures. Thus, early recognition is essential, and treatment by a multidisciplinary team is ideal.

[A painful ring finger following minimal trauma]. / Een pijnlijke ringvinger na minimaal trauma.

We present the case of a 24-year-old man with a painful ring finger following minimal trauma during a handball game. The X-ray showed a pathological fracture of the proximal phalanx due to an enchondroma. Conservative treatment was initiated, consisting of cast immobilisation for 6 weeks. After 8 months of follow-up, the X-ray revealed that the bone marrow cavity was almost completely filled with bone tissue.

Chordoma of the anterior cranial fossa and ethmoids with orbital involvement.

PURPOSE: To report a case of orbital chordoma, emphasizing the clinical, operative, and histopathologic findings, and to review similar English-language reports. METHODS: This is a single case report with histopathologic correlation. Search of the English-language literature and review of referenced citations was performed. RESULTS: After treatment with resection and proton beam radiation, our patient is alive, without recurrence at 3-year follow-up. Biopsy of the recurrent tumor was consistent with chordoma. The original biopsy had S100 and pancytokeratin-positive tumor cells, with abundant clear to eosinophilic cytoplasm. Focal EMA positivity was present. Literature review identified 14 additional cases. CONCLUSION: Orbital chordoma is rare. Extraocular motility disturbances occur solely with intracranial lesions as well as those extending into the orbit, but globe displacement is the most common sign of orbital involvement. This tumor often recurs in the path of previous resection. Diagnosis is confirmed by distinctive histopathologic features and positive staining for S100, pancytokeratin, and EMA. Treatment and outcome analysis of orbital chordoma is difficult due to its rarity and lack of reported follow-up and may need to be extrapolated from reported skull base cases.

Treatment of cryotherapy and orthotopic transplantation following chondromyxoid fibroma of zygomatic bone: A case report.

INTRODUCTION: Chondromyxoid fibrotherma (CMF) is a rare benign cartilage tumor that occurs more frequently in young males at the age of 20 to 30. It occurs more frequently on long bones, but rarely involves craniofacial bones. CASE PRESENTATION: This study mainly introduced a 30-year-old male with CMF of zygomatic bone. Single tumor excochleation was conducted initially. However, CMF reoccurred, and then the following steps were adopted: firstly, the tumor was extensively excised; secondly, in vitro tumor excochleation was conducted; thirdly, the excised tumor bone was placed in liquid nitrogen for 3 cycles of cryoablation; finally, the orthotopic transplantation was performed to reconstruct the zygomatic appearance, with satisfactory follow-up efficacy obtained. CONCLUSIONS: Orthotopic transplantation after tumorectomy and cryopreservation of tumor bone in liquid nitrogen could lead to excellent therapeutic efficacy and deserves to be widely applied in clinical practice in the treatment of a male patient with CMF of zygomatic bone, because it not only radically eliminates the tumor and kills tumor cells, but also provides bony skeleton for the growth of new bone, thus greatly promoting postoperative aesthetic degree and reducing the occurrence rates of complications.

Chondroma of the Bladder: A Case Report and Review of the Literature.

Chondromas are benign tumors composed of mature hyaline cartilage (Bahnassy & Abdil-Khalik, 2009). Extraskeletal presentation of chondromas is extremely rare and mostly occurs in the soft tissues of the extremities, mainly the hands and feet (Bahnassy & Abdil-Khalik, 2009; Rapini et al, 2007). There are currently only 3 case reports in the literature of chondroma of the urinary bladder (Cho & Horvai, 2015; Reichard et al, 2014; Sloan & Rapoport, 1985). Here we present a case of urinary bladder chondroma with low proliferative potential managed by transurethral resection.

Intra-articular benign bone lesions treated with Magnetic Resonance-guided Focused Ultrasound (MRgFUS): imaging follow-up and clinical results.

Purpose of this study was to evaluate the employment of MRI-guided Focused Ultrasound (MRgFUS) for treatment of intra-articular benign bone lesions as alternative to surgery, and to monitor the success of the treatment on CT and MRI images. From March 2011 to August 2013, 14 intra-articular benign bone lesions were treated with MRgFUS. All patients were studied by CT and MR imaging. Pain was measured using the visual analogue scale (VAS) before and after treatment (6 and 12 months). All patients in our series demonstrated regression in painful symptomatology during screening. A significant drop in the mean VAS pain score (from 7.8 to 0.6) was observed at 12-month follow-up, and pain medication was no longer needed after treatment. No complications were observed. Three diagnostic imaging signs were found suggesting absence of biological activity and confirming the clinical findings: calcification of the treated lesion, lack of contrast enhancement and disappearance of bone oedema around the lesions. CONCLUSION: the employment of MRgFUS is safe and effective in the treatment of intra-articular benign bone lesions. The clinical outcome is satisfactory, and the success of the treatment is confirmed by diagnostic imaging.

Primary Tumors of the Osseous Chest Wall and Their Management.

Primary osseous tumors of the chest wall are uncommon neoplasms. They occur in a wide variety of pathologic forms, most of which can be distinguished by unique radiologic appearance. Management of these tumors depends on the diagnosis and stage. Adequate surgical resection is critical in achieving the best outcomes for most of these tumors. Chemotherapy and radiation may have an adjuvant role. Surgeons considering resection of any chest wall tumor should have a sound knowledge of the principles of resection and reconstruction.

Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report.

Carney triad is a rare syndrome, with only 20 complete cases reported. We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma. Immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma. Ultrastructural studies demonstrated skeinoid fibers in the GIST. To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.

Current management of hand enchondroma: a review.

Enchondromas are benign bone tumours originating from cartilages. It is mainly discovered incidentally in radiographs or due to symptoms like pathological fracture or pain. Conservative treatment through regular check-up and surgical excision using curettage are the two major treatment methods for enchondromas. This review concludes that small localized asymptomatic lesions can be treated conservatively while most expanding or symptomatic lesions should be treated with simple curettage. Adjuvant treatments like high-speed burring or alcohol instillation are not recommended.

Extensive curettage using a high-speed burr versus dehydrated alcohol instillation for the treatment of enchondroma of the hand.

The purpose of this retrospective study was to compare the clinical and radiological outcomes of patients treated with different adjuvant methods after curettage for enchondromas of the hand. Sixty-two patients with enchondroma were treated with high-speed burring (29 patients) or alcohol instillation (33 patients) after curettage. The mean follow-up was 40.8 months. No significant differences in the visual analogue scale, Disabilities of the Arm, Shoulder, and Hand scores, total range of active motion, grip strength, and complete healing time were observed between the groups. The distribution of the results of the formula by Wilhelm and Feldmeier were not significantly different between the groups. No surgery-related complications, postoperative pathological fractures, or recurrence was found in either group. For the treatment of enchondroma in the metacarpal and proximal phalanx, alcohol instillation immediately after curettage was as effective as extensive curettage using a high-speed burr.