Osseous Tumors Research Articles

Primary Osseous Tumors of the Subaxial Cervical Spine, Clinical Case Series

Background Data: Primary osseous tumors of the subaxial cervical spine are rare and can present with different clinical presentations and pathologies. Study Design: Retrospective descriptive clinical case series.Purpose: To assess the prognosis, surgical management, outcome of primary osseous tumors of the subaxial cervical spine Patients and Methods: Eight patients underwent surgical treatment for primary cervical bony tumors at Kasr Al-Aini Medical School, Cairo University, between 2007 and 2012. Mean follow-up was 24.4±6.8 months. Clinical outcome wereevaluated neurologically using Frankel grading system, while visual analog scale (VAS) has been used to evaluate neck pain. Results: There was equal sex distribution and the mean age was 38.4±14.9 years (range 17-62 years). Half of patients had benign tumors (2 aneurysmal bone cysts, one hemangioma and one osteoblastoma) while the other 4 patients had malignant tumors (giant cell tumor, chordoma, chondro-sarcoma and plasmacytoma). Neck pain was the constant complaint of all patients. In addition, 5 patients suffered from myelopathy while 2 patients had radiculopathy. Anterior corpectomy and fusion was performed in four cases, while in 2 patients, only posterior approach was adopted. In the other 2 patients, combined anterior and posterior approaches were adopted. Postoperatively, 6 patients had thesame preoperative Frankel grade (4 were grade E, 1 was D and 1 was C), while 2 patients improved (25%) (1 improved from D to E and 1 from C to D). The average VAS improved from 5±1.5 (range 3-8) preoperatively to 2.4±1.7 (range 1-5)postoperatively at last follow-up. The 4 patients with benign tumors are doing well (Frankel grade E). In contrast, 3 out of 4 patients with malignant tumors died within 12-16 m later, the survival of malignant tumors is 25% at 3 years.Conclusion: Marginal piecemeal resection and cervical instrumentation and fusion is a safe and effective method in management of benign osseous cervical tumors. However, the results are poor for malignant tumors even with adjuvanttherapy. (2013ESJ051)

Musculoskeletal lymphoma: MRI of bone or soft tissue presentations

To assess the MRI findings of musculoskeletal lymphoma primarily presenting clinically as a bone lesion or soft tissue mass. Magnetic resonance imaging of 23 cases of musculoskeletal lymphoma were retrospectively reviewed. Features assessed included tumour location, morphology, signal intensity (SI) characteristics, cortical destruction, involvement of multiple anatomic compartments, encasement of adjacent neurovascular structures/tendons and subcutaneous oedema. Osseous lesions were typically poorly defined and hypointense on T1-weighted imaging. T2-weighted SI was usually heterogeneous, with 54% of cases having a 'mosaic' pattern of marrow replacement. Ninety-two per cent of osseous tumours had cortical abnormalities, most commonly a permeative pattern. A periosseous soft tissue cuff was present in 46% of cases while 38% had a more significant extraosseous component. All cases of soft tissue lymphoma were iso-/slightly hyperintense to muscle on T1-weighted images and hyperintense on T2-weighted images. Multicompartment involvement by extraosseous tumour was present in 75% of cases, and 67% had subcutaneous oedema. Eighty-three per cent of soft tissue tumours showed encasement of adjacent structures. Multifocal lymphoma had similar morphology and SI characteristics to single-site lesions. Histopathologically, there were 21 cases of non-Hodgkin's lymphoma and two of Hodgkin's lymphoma. Magnetic resonance imaging features of primary osseous lymphoma include T2 heterogeneity, a periosseous soft tissue cuff or a more substantial mass, and cortical disruption often disproportionate to the extent of extraosseous tumour. Features characteristic of soft tissue lymphoma include relative homogeneity on T1- and T2-weighted imaging, multicompartment involvement and encasement of neurovascular structures.

관절원판의 유착을 동반한 하악과두의 골연골종의 치료: 증례보고

Osteochondroma is a benign neoplasm, osseous projection surrounded with cartilage, 35.8% of benign osseous tumor, 8.5% of whole osseous tumor and usually arises from the skeletal bone. Osteochondroma is a cartilaginous derivation and relatively uncommon in the craniofacial bone. Osteochondroma of the mandible has slow growth rates which mainly affect women around forty years of age and it can appear through the coronoid process and mandibular condyle, especially in the medial half. Clinical finding associated with osteochondroma of condyle are primarily a palpable, painless temporomandibular area mass with facial asymmetry, malocclusion and midline deviations. Sometimes pain and dysfunction like trismus often accompany the anatomic derangement. Other features include malocclusion with open-bite on the affected side and cross-bite on the contralateral side. In this study, a 45-years old female patient exhibits pain on the left temporo-mandibular joint area and malocclusion due to loss of the molar region with osteochondroma on the top left of her mandibular condyle head. The patient is able to recover gradually from the symptom through treatments on manipulation, stabilization splint, arthroscopic lavage and surgical excision, thus, this is reported as a clinical case.

Primary Osseous Tumors of the Spine

Primary osseous spinal tumors are relatively rare in comparison with metastatic disease, myeloma, and lymphoma. Despite their rarity, the interpreting radiologist must be aware of the typical imaging features to provide appropriate diagnosis for guidance of clinical management. The age of occurrence, distribution longitudinally in the spine, and distribution axially within the vertebra combined with typical imaging appearances can help indicate the correct diagnosis. This article reviews the diagnostic features of benign and malignant primary bone spinal tumors.

Benign Osteoid-Producing Bone Lesions: Update on Imaging and Treatment

Benign osteoid-producing osseous tumors are frequently encountered in a typical musculoskeletal radiology practice. Enostoses are extremely common, but osteomas, osteoid osteomas, and osteoblastomas are diagnosed less often. A thorough discussion of the typical clinical presentation, histology, imaging findings, treatment and prognosis for each tumor is provided, with particular emphasis on more current imaging techniques and treatments.

PP102: Intraosseous hemangioma of the mandible: An unusual presentation

Purpose Hemangioma is a benign vascular tissue neoplasm arising from the vascular tissue which generally occurs during infancy. Intraosseous hemangiomas are uncommon, constituting less than 1% of all osseous tumors. The most frequent sites are the calvaria and the vertebral column. Involvement of the facial bones is rare. The purpose of this presentation is to report an unusual presentation of an intraosseous hemangioma, its diagnosis and treatment outcome. Material and method A 37 year old female patient was referred to our clinic with paresthesia complaint in the lower lip. Her medical history did not indicate any systemic diseases. Intraoral examination showed no pathology. Panoramic radiography revealed a radiolucent lesion with irregular lytic borders in the right edentulous posterior region of mandible. Under local anesthesia, curettage of the lesion was performed. Results Histopathological examination showed many large and dilated endothelial sinuses which contained aggregates of erythrocytes inside connective tissue and bone marrow, indicating intraosseous hemangioma. Conclusion Intraosseous hemangiomas present difficulty in differential diagnosis. It is a bone destructive lesion which may be of varying size and appearance, but is often suggestive of a cyst. Severe haemorrhage following trauma or tooth extraction has been reported. The patient’s medical history and previous extractions in the lesion site did not show any haemorrhage indicating a lesion with a severe risk of bleeding. Also, radiographic findings did not correlate with the common signs of an intraosseous hemangioma. Treatment of intraosseous hemangioma is difficult and is done by local excision for smaller lesions and larger lesions are excised after pretreatment with sclerosing agents. Differential diagnosis and careful treatment planning is of crucial importance in hemangiomas considering the severe complications such as haemorrhage.

Incomplete Gardner’s syndrome with blepharoptosis as the first symptom

Gardner's syndrome (GS) is an autosomal dominant form of polyposis characterized by the presence of multiple polyps in the colon together with osseous tumors and soft-tissue tumors, such as epidermoid cysts and lipomas. An osteoma is a benign, osteogenic tumor and may be sporadic or related to GS. Here, we present a patient with a giant sino-orbital osteoma and blepharoptosis as the only symptom of incomplete GS. A 74-year-old woman, with no previous history of trauma or ophthalmic surgery, presented with a 2 years history of right blepharoptosis without diplopia. The results of slit-lamp and fundoscopic examination were normal. Computed tomography showed a giant sino-orbital osteoma. With suspicion for GS, we thoroughly examined the patient and found no soft-tissue tumors. Fifteen years ago, the patient, who had a family history of colonic polyposis, underwent right colectomy and chemotherapy for adenocarcinoma. We report a case of incomplete GS with blepharoptosis as the first symptom.

A dose finding, safety and pharmacokinetic study of AVE1642, an anti-insulin-like growth factor-1 receptor (IGF-1R/CD221) monoclonal antibody, administered as a single agent and in combination with docetaxel in patients with advanced solid tumours

PurposeAVE1642, a humanised mAb, binds the human IGF-1R specifically and with high affinity. This study aimed to select the dose of AVE1642 alone and then combined with docetaxel 75mg/m2 (D). Material and methodsAVE1642 was administered alone at cycle (cy) 1 and then combined with D from cy2, q3w. ResultsA total of 27 patients received a median number of 5 cy (range, 1–10). The most common tumour types were sarcoma (18.5%), osseous tumours (11.1%) and colon cancer (11.1%). Two DLTs were reported in cy1 at dose level (DL) 18mg/kg and dose escalation was stopped. No major safety issue was observed. No anti-drug antibodies were detected. The Maximal Tolerated Dose of AVE1642 was 12mg/kg. The dose selected for further combinations is 6mg/kg, based on PK/PD data. Three objective responses, (two in sarcoma and one breast cancer) were observed but only one was confirmed. Eleven patients appeared to benefit from treatment with prolonged disease stabilisation ⩾4months. ConclusionAVE1642 is well tolerated as a single agent and combined with D. The selected dose of AVE1642 combined with D is 6mg/kg. Promising activity was seen in sarcoma and breast cancer patients.

Detection of circulating tumor cells in different stages of prostate cancer

To explore circulating tumor cell (CTCs) counts in different stages of prostate cancer (PC) in association with tumor burden, metastatic pattern and conventional serum biomarkers. Overall survival (OS) analyses were conducted with respect to optimized CTC cutoff levels. Circulating tumor cell counts were assessed in healthy controls (n = 15) as well as in locally advanced high risk (LAPC, n = 20), metastatic castration resistant (mCRPC, n = 40) and taxane-refractory (mTRPC, n = 15) PC patients. CTCs were detected using the CellSearch System. In metastatic PC (mPC), CTC counts were significantly increased compared to LAPC (p < 0.001). In LAPC, CTCs were at control level (p = 0.66). Patients with both bone and visceral lesions revealed the highest median CTC count (p = 0.004), whereas patients with sole soft tissue metastases displayed CTC counts comparable to controls (p = 0.16). No correlation was observed between CTC counts and osseous tumor burden assessed by bone lesion count (p = 0.54) or bone scan index (p = 0.81). CTC counts revealed a positive correlation with alkaline phosphatase (p < 0.001) and lactate dehydrogenase (p < 0.001) as well as a negative association with hemoglobin (p = 0.004) and PSA-doubling time (p = 0.01). Kaplan-Meier analyses demonstrated a cohort adjusted cutoff level of 3 CTCs with a shorter OS in case of ≥3 CTCs compared to <3 CTCs (p = 0.001), a cutoff level applicable in mCRPC (p = 0.003) but not in mTRPC patients (p = 0.054). Circulating tumor cell counts are applicable as a prognostic molecular marker, especially in mCRPC patients harboring bone metastases with or without visceral metastases. For clinical practice, mPC patients with elevated CTC counts in combination with short PSA-DT, high alkaline phosphatase and lactate dehydrogenase levels as well as low hemoglobin levels are at high risk of disease progression and limited OS.

Editorial: Limb Preservation: Past and Present

The International Society of Limb Salvage (ISOLS) displayed the global perspective needed to bring advances in surgery, engineering, reconstruction, biology, and clinical care to the entire international community. The 16th meeting, held in 2011 in Beijing, China, marked the 30th anniversary of the organization. Starting from humble beginnings at the Mayo Clinic in 1981, Frank Sim and Ed Chao inaugurated the Society. It has grown from a meeting of 127 participants from 23 countries to 840 participants from 44 countries, reflecting the excitement of a burgeoning field and the enthusiasm that resonates among the practitioners of musculoskeletal oncology. The fundamental philosophy of the ISOLS is that all people in the world deserve to benefit from the advances in musculoskeletal cancer care achieved in the developed countries. Affording limb preservation to all seemed to be the goal. Specialty education and sharing of experience in this niche field was the method to achieve it. ISOLS pursued this egalitarian vision long before any other organization took up the mantra. It promulgated the use of standardized staging, functional outcome, and radiographic description systems. In what was perhaps an overly zealous effort, it even normalized the nonparametric functional outcome system of the Musculoskeletal Tumor Society and imposed a 100-point normative scale that encouraged researchers to present data as a percentage of normal. The result has been the relentless improvement in survival and the reduction in amputation rates around the world. ISOLS deserves a significant amount of the credit for these extraordinary advances. How did this happen? What contributed to its successes and failures? Any short answers to these questions inevitably will fail to capture the excitement of discovery or the satisfaction of sharing the hard-won knowledge. Major contributors to the effort are forgotten as new advances take priority. Nevertheless, it is worth reflecting on the contributions that created the organization and how these have created the foundation for what we do in modern musculoskeletal oncology. It is also worth thinking of what we still have to accomplish, taking the generalized findings to the public in the individualized medicine of the future. The ISOLS organizers came together to share knowledge about clinical care and prosthetic reconstruction in the new era of limb preservation. The first meeting served to introduce titanium fiber-metal, among other advances. Major advances were presented in meetings in Kyoto such as the extracorporeal radiation and reimplantation of osseous tumors and gait analysis of periacetabular resections/reconstructions. These themes were supplemented by biological and genetic information presented in Singapore and tissue regeneration prospects in New York. We have seen how these advances are now commonplace in countries worldwide such as India. The Beijing meeting allowed Dr Sim, one of the original founders, to present an awesome display of surgical virtuosity in the resection and reconstruction of sacral tumors. Quality control strategies in these developing countries were especially impressive. The durability of the original work that spawned ISOLS was glorious to behold. It was equally inspiring to learn of the innovative ways that the information is being disseminated. The papers selected for this Symposium build on the hard work of our predecessors and the creative genius of multispecialty interaction. It is a celebration of what the field of orthopaedic oncology has to offer. At the same time, it exposes the limitations of what we do and the magnitude of what still needs to be accomplished to help our tumor patients. Fig. 1 Dr. John H. Healey is shown.

Rare pediatric presentation of aneurysmal bone cyst with trabecular juvenile ossifying fibroma and ossifying fibroma

Aneurysmal bone cyst (ABC) is a benign intraosseous lesion characterized by blood filled spaces of varying sizes associated with a fibroblastic stroma containing multinucleated giant cells, osteoid and woven bone. ABC can present either singly or in association with osseous neoplasms such as ossifying fibroma (OF), giant cell granuloma etc. Juvenile ossifying fibroma has two variants: psammomatoid JOF (PJOF) and trabecular JOF (TJOF). ABC formation in TJOF is very rare in pediatric patients with only three cases reported in literature till date to the best of our knowledge. We hereby report three pediatric cases of ABC, two of which were associated with TJOF and one associated with OF.

Osteoblastoma: A Rare Cause of Stiff Elbow

Osteoblastoma is a benign, osseous tumour that is usually found in the femur, tibia, foot and posterior elements of the vertebrae but rarely around the elbow. Elbow stiffness is common after trauma, infection (in particular tuberculosis), synovitis and arthritis, but is rarely caused by a tumour. In this case, an osteoblastoma in the distal humerus was excised through an Outerbridge—Kashiwagi approach, which successfully addressed the patient's main complaint of elbow stiffness and restricted function. However, the lesion recurred a few months later and was treated successfully by computed tomography-guided thermoablation.

Giant Cell Tumour of Soft Tissue of the Finger: A Case Report

Giant cell tumour of soft tissue resembling osseous giant cell tumour is a distinct and uncommon entity. Presented is a 52-year old man with a 3-year history of swelling located on the dorsum of the right middle finger diagnosed on histology as giant cell tumour of soft tissue. The need for high index of suspicion by physicians is emphasized, as giant cell tumour of soft tissue should be considered as differential diagnosis of finger lumps.

MULTILOBULAR TUMOR OF BONE IN AN AFRICAN WILD DOG (LYCAON PICTUS)

A 13-mo-old, captive-born, female African wild dog (Lycaon pictus) was presented with a progressively enlarging, firm mass on the dorsum of the head. Due to rapid tumor growth and the development of neurologic signs, elective euthanasia and a complete postmortem were performed 2 months after initial presentation. Histologic findings were similar in both antemortem biopsy and postmortem samples from the tumor and consisted of nodules of cartilage and/or bone separated by thin interconnected trabeculae that contained spindle-shaped cells, consistent with multilobular tumor of bone. Very few reports of tumors in the endangered African wild dog have been published to date. To the authors' knowledge, this is the first report of an osseous tumor in an African wild dog and the first report of a multilobular tumor of bone in a nondomestic canid.

Malignant Osseous Tumors of the Pediatric Spine

Malignant Osseous Tumors of the Pediatric Spine

Malignant Osseous Tumors of the Pediatric Spine

In the pediatric population, malignant osseous tumors of the spine include osteosarcoma, Ewing sarcoma, lymphoma, and metastatic neuroblastoma. Although these tumors are rare, prompt diagnosis and recognition are critical to the overall prognosis. Improved understanding of the natural history of spine deformity, combined with advances in imaging, surgical technology, radiation therapy, and chemotherapeutic regimens, has improved survival rates and decreased rates of local recurrence-especially recurrence of low-grade lesions. Prognosis for patients with high-grade lesions with distant metastasis on presentation remains exceedingly poor. Recognition of these spine tumors and prompt referral to a tertiary care center that specializes in oncology can optimize patient outcomes.

Primary Osseous Tumors of the Hindfoot: Why the Delay in Diagnosis and Should We Be Concerned?

Bony tumors of the foot account for approximately 3% of all osseous tumors. Diagnosis is frequently delayed as a result of lack of clinician familiarity and as a result of their rarity. The reasons for the delays, however, are unclear. We therefore determined (1) how hindfoot tumors present and the specific reasons for delay in diagnosis; (2) whether the spectrum of disease varies between the talus and calcaneus; and (3) how these patients were treated. We retrospectively reviewed the medical notes and imaging for all patients with 34 calcaneal and 23 talar tumors recorded in the Scottish Bone Tumour Registry. Demographics, presentation, investigation, histology, management, recurrence, and mortality were recorded. Hindfoot tumors present with pain and often swelling around the heel (calcaneus) or ankle (talus), most often misdiagnosed as soft tissue injury. Calcaneal lesions were more likely to be malignant than talar lesions: 13 of 34 versus three of 23. Clinicians should be aware that hindfoot tumors can be initially misdiagnosed as soft tissue injuries and suspicion of a tumor should be raised in the absence of trauma or persistent symptoms. Lesions affecting the calcaneus are more likely to be malignant. Early diagnosis and adjuvant therapy are important. Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Ostéome ostéoïde de la phalange chez l’enfant et problème diagnostique. À propos d’un cas

Osteoid osteoma is a benign osseous tumor of unknown origin, which affects preferentially the long bones. The localization in the phalanx is unusual and difficult to diagnose. We present a case of a 13-year-old girl, presenting with a painful swelling of the first phalanx of the right middle finger of 5 months duration, not responding to anti-inflammatory drugs. The diagnosis of tuberculosis of the bones was suggested at first (tuberculosis is endemic in our country), but the biology and the bone biopsy did not confirm the diagnosis. The lesion was excised, and the histology confirmed the diagnosis of osteoid osteoma. Through our observation and after a review of the literature, we analyze the clinical and paraclinical appearance of this uncommon localization, the differential diagnosis, and finally the therapeutic possibilities.

Imaging of the Female Perineum in Adults

The female perineum is a diamond-shaped structure inferior to the pelvic diaphragm and between the symphysis pubis and coccyx. The perineum is divided into the anterior urogenital triangle and the posterior anal triangle; the vulva represents the external genitalia. A wide array of diseases affect the female perineum in adults. Vulvar trauma, infection (including Fournier gangrene), developmental lesions, and thrombophlebitis can be investigated with various imaging modalities; vulvar malignancies are best imaged with magnetic resonance (MR) imaging to identify local-regional extent of disease. MR imaging is also the modality of choice for imaging of the distal urethra, although imaging of a urethral diverticulum also includes voiding cystourethrography and ultrasonography. The distal vagina at the level of the introitus is best imaged with MR imaging for assessment of Bartholin gland cysts and malignancies. Diseases encountered in the anus include anal carcinoma, fistula-in-ano, and anovaginal fistula, which can all be imaged with various modalities offering different sensitivities and fields of view. Lastly, musculoskeletal neoplasms affecting the perineum and vulva include mesenchymal, lipomatous, nerve sheath, and osseous neoplasms. These neoplasms can be imaged with both computed tomography and MR imaging, although the latter provides higher soft-tissue contrast and greater anatomic detail for diagnosis and determination of the extent of necessary surgery. Familiarity with the anatomy of the female perineum and appropriate selection of imaging modalities facilitate prompt and accurate diagnosis and treatment.

Osteoid osteoma associated with the teeth: unusual presentation

Osteoid osteoma is a rare benign osseous neoplasm which rarely affects the jaws. It is more common in long bones which can lead to postural scoliosis. The clinical characteristic feature of this tumour is the unexplained pain that is relieved by the intake of analgesics. Histologically it is characterized by the formation of central nidus with surrounding sclerotic bone. The diagnostic dilemma in this case report is aggravated by its unusual association with the teeth. The use of CT scanning is advised to distinguish from other odontogenic tumours. Treatment for this tumour is surgical removal or excisional biopsy to avoid recurrence and progression to malignant changes.