Bone Tumours Research Articles

A Case of Aneurysmal Bone Cyst of Fourth Metacarpal Bone: A Rare Entity at Unusual Location

An Aneurysmal Bone Cyst (ABC) is a locally aggressive, rapidly growing, rare benign bone tumour that typically occurs in children and early adulthood. It usually occurs in the first and second decades of life, with a female predominance. The long bones of the lower extremities and spine are common sites for ABC, with rare involvement of the small bones of the hand. An 18-year-old female presented to the hospital with progressive swelling over her left hand for 4 to 5 months, associated with pain. Local examination elicits pain and tenderness over the 4th metacarpal bone with restricted movement at the Metacarpophalangeal (MCP) joint. A histopathological diagnosis of ABC was made due to the presence of osteoclastic giant cells lining a cyst, along with areas showing a pool of haemorrhage and stromal cells. The postoperative period remained uneventful, with no complaints of pain and good healing of the operation site noted during follow-up. Metacarpal bones are rare sites for ABCs.

Current Concepts in the Surgical Management of Metastases to the Spinal Column

Abstract Spinal metastasis is a common malignant bone tumour that often causes compression of the spinal cord and nerve roots. It has a high mortality rate and is primarily treated palliatively. A multidisciplinary approach is recommended. The treatment methods include surgery, minimally invasive treatment and radiotherapy, each having its own clinical effects, advantages and limitations.

CircRNA TUBA1C promotes proliferation and glucose metabolism, and blocks apoptosis ofosteosarcoma cells through sponging miR-143-3p.

Osteosarcoma (OS) is amalignant bone tumour that commonly occurs in paediatric and adolescent patients. Currently, effective therapy for OS remains elusive due to poor patient survival rates. In this study, we observed significantly elevated expressions ofcircTUBA1C in OS tumours and cells. Silencing circTUBA1C effectively suppressed proliferation and glucose metabolism, and promoted apoptosis ofOS cells. Furthermore, we discovered that miR-143-3p played areverse role to circTUBA1C in OS cells. Bioinformatics analysis, RNA pull-down assay, and luciferase assay demonstrated that circTUBA1C acted as asponge for miR-143-3p, blocking its expression in OS cells. Finally, rescue experiments showed that inhibition ofmiR-143-3p in circTUBA1C-silenced OS cells significantly overrode thelow-circTUBA1C-mediated miR-143-3p upregulation and OS cell progression in vitro and in vivo . Our results demonstrate thecritical roles and molecular targets ofcircTUBA1C in modulating OS progression, suggesting that circTUBA1C inhibition could serve as anew therapeutic strategy for treating OS.

Multimodal layer-by-layer nanoparticles: a breakthrough in gene and drug delivery for osteosarcoma.

Osteosarcoma is one of the most common primary malignant bone tumours in children and adolescents, frequently arising from mesenchymal tissue in the distal femur. It is highly aggressive, often metastasising to the lungs. Current treatments, which include surgery combined with neoadjuvant chemotherapy and radiotherapy, are often unsatisfactory due to the inability of surgery to control metastasis and the side effects and drug resistance associated with chemotherapy. Thus, there is an urgent need for new treatment technologies. This study explored the use of nanoparticles for gene and drug delivery in osteosarcoma treatment. The nanoparticles were composed of biodegradable and biocompatible polymers, chitosan and PLGA, and were loaded with miRNA-34a, a short RNA molecule that functions as a tumour suppressor by inducing cell cycle arrest and apoptosis in osteosarcoma cells. Recognising that the co-delivery of multiple drugs can enhance treatment efficacy while reducing systemic toxicity and drug resistance, three additional classes of nanoparticles were developed by adding doxorubicin and resveratrol to the chitosan-PLGA-miRNA-34a core. A layer-by-layer technique was employed to create a bilayer nanocoating using pectin and chitosan as polyelectrolytes, for encapsulating the therapeutic payloads. The manufactured nanoparticles were tested on U2OS and Saos-2 cells to assess cell viability, metabolic activity, and morphology before and after treatment. Cells were treated in both two-dimensional cultures and three-dimensional osteosarcoma spheroids, creating a biomimetic cellular model. Increased apoptotic activity and disruption of cellular functions were primarily observed with nanoparticles co-delivering miRNA-34a and drugs, particularly those functionalised with the LbL nanocoating, as confirmed by PCR analysis.

Cytodiagnosis of Metastatic Thyroid Follicular Carcinoma in Left Iliac Bone with Occult Primary: A Case Report

Bone metastasis of Follicular Thyroid Carcinoma (FTC) is an aggressive complication. Typically, follicular carcinoma remains confined to the thyroid gland, with distant metastasis occurring in 4-15% of cases. The lungs are the most frequent site of metastasis, followed by bone (2-13%). A peculiar clinical manifestation of FTC observed is the metastasis with the primary still remaining clinically occult. Solitary bone metastases are exceedingly rare and can easily be misdiagnosed as primary bone tumours on clinicoradiological assessment. This causes delays in the diagnosis and management. In these cases, the cytomorphology of cells on diagnostic Fine Needle Aspiration (FNA) was found to be useful in suggesting the primary origin of FTC. Distant bone metastases from follicular carcinoma on histopathological examination from biopsy specimens have been reported in the literature. The cytodiagnosis and cytomorphology of metastatic FTC by Fine Needle Aspiration Cytology (FNAC) are seldomly described in the literature. Involvement of the iliac bone in the metastasis of FTC is uncommon. The FNAC of such a bony lesion helps immensely not only in the diagnosis of the metastasis but also in suggesting the primary malignancy. Authors, hereby report a clinicopathological profile of one such rare case of a 74-year-old female with cytodiagnosis of FTC with no clinical thyroid swelling. The cytodiagnosis made from the primary lytic lesion involving the left iliac bone led to the retrospective radiological and histopathological work-up of the thyroid gland, confirming the occult primary carcinoma of the thyroid gland. The present case highlights the rare presentation and cytomorphological details of metastatic thyroid follicular carcinoma.

Shared decision making in primary malignant bone tumour surgery in children and young adults

BackgroundChildren and young adults needing surgery for a primary malignant bone tumour around the knee face a difficult, life changing decision. This study describes the level of shared decision making (SDM) experienced and preferred by these patients, parents and physicians in surgical consultations, and its relation to experienced decisional conflict and decisional regret. MethodsMulticentre, cross-sectional cohort study. All patients who underwent surgery for a primary bone tumour around the knee in the Dutch designated orthopaedic oncological centres between 2012 and 2015, and their parents, were invited to complete the SDM-patient-Questionnaire (SDM-Q-9), Decisional Conflict Scale (DCS), Decisional Regret Scale (DRS) and Control Preferences Scale (CPS). Physicians completed the SDM- physician-Questionnaire (SDM-Q-Doc) and CPS. ResultsTwenty-four patients >16 years with twenty-two parents, and ten parents of patients between 5 and 16 years old, completed the questionnaires. Patients’ median SDM-Q-9 score was 60 (8.9–97.8), parents’ 77.8 (8.9–100) and physicians’ 82.2 (66.7–97.8). The SDM-Q-9 scores of patients (rs=−0.753, p < 0.01) and parents (rs=−0.850, p < 0.01) correlated with their DCS scores. DCS scores were correlated with decisional regret in patients (rs=0.701 p < 0.01) and parents (rs=0.405, p < 0.05). Fourteen patients (78%), twenty-eight parents (96%) and twenty-three physicians (92%) preferred a shared relationship in decision making on type of surgery. ConclusionsPatients, parents and physicians agree on sharing responsibility choosing a surgical option. Patients and parents who reported more involvement in the decision-making process experienced less decisional conflict; less decisional conflict was associated with less decisional regret. These findings show the importance of SDM in these life changing surgeries.

Is perioperative chemotherapy effective in patients with localized myxoid liposarcoma?

This study aimed to compare the local recurrence, distant metastasis and disease-specific survival rates of patients with localized myxoid liposarcoma in the surgery and adjuvant chemotherapy group versus the surgery alone group. A total of 456 patients in the Japanese National Bone and Soft Tissue Tumour Registry database who had localized myxoid liposarcoma and underwent surgery and adjuvant chemotherapy or surgery alone between 2001 and 2019 were included in this retrospective study. The study adjusted for background differences between patients who underwent surgery and adjuvant chemotherapy (n=228) or surgery alone (n=228) using propensity score matching. Univariate analysis showed no significant difference in local recurrence rate between the two groups (5-year local recurrence-free survival: 98.6% [95% confidence interval: 95.9-99.6] vs. 94.0% [95% confidence interval: 89.7-96.6], P=0.052). Univariate analysis showed no difference in the incidence of distant metastases between the two groups (5-year distant metastasis-free survival: 80.5% [95% confidence interval: 73.9-85.8] vs. 75.1% [95% confidence interval: 67.7-81.2], P=0.508). Univariate analysis showed no difference in disease-specific survival between the two groups (5-year disease-specific survival: 92.6% [95% confidence interval: 86.1-96.2] vs. 93.2% [95% confidence interval: 87.6-96.4], P=0.804). In the high-risk group (n=203) with high-grade tumours and tumour size ≥10cm, there were no significant differences in the local recurrence, distant metastasis and disease-specific survival rates between the surgery and adjuvant chemotherapy group and the surgery alone group. The effect of adjuvant chemotherapy on localized myxoid liposarcoma appears to be limited.

Narlumosbart: First Approval.

Narlumosbart () is a recombinant, fully human, anti-receptor activator of nuclear factor kappa-Β ligand (RANKL) IgG4 monoclonal antibody being developed by CSPC Pharmaceutical and its wholly owned subsidiary Shanghai Jinmante Biotechnology for the treatment of giant cell tumour of bone (GCTB), bone metastases from solid tumours and osteoporosis. The RANK/RANKL signalling pathway plays a pivotal role in osteoclastogenesis and in the pathogenesis of GCTB. Narlumosbart specifically binds to RANKL and blocks the interaction of RANKL with RANK, thus inhibiting osteoclastogenesis and bone resorption by osteoclasts. In September 2023, narlumosbart received conditional first approval in China for the treatment of adults with GCTB that is unresectable or when surgical resection would result in severe functional disability. Clinical studies of narlumosbart for bone metastases, postmenopausal osteoporosis and glucocorticoid-induced osteoporosis are underway in China. This article summarizes the milestones in the development of narlumosbart leading to this first approval for the treatment of adults with GCTB.

A rare case of chondromyxoid fibroma of distal phalanx of great toe

Chondromyxoid fibroma (CMF) is rare benign tumour of bone which are due to incomplete cartilage differentiation and first described by Jaffe and Lichtensein in 1948. CMF’s constitutes for less than 1% of all bone tumors. Histopathology is diagnostic and CMF show lobulated areas of spindle shaped or stellate cells with hypocellular center of chondroid or myxoid material and periphery is hypercellular with abundant fibromyxoid tissue and few multinucleated osteoclasts like giant cell. CMFs are commonly seen in metaphysis of long bones including proximal tibia or distal femur. Short tubular bones of hand and feet are uncommon sites and toes constitutes less than 5% of the tumors. We present a case of chondromyxoid fibroma of distal phalanx of left great toe in 35 years old male patient.

Perioperative hemorheological status of oncological patients with primary knee replacement

Introduction. Endoprosthesis of large joints is associated with high risk of venous thromboembolism. Researchers focus on biochemical characteristics of homeostasis, blood clotting in particular, as the main reason for abnormal blood flow. At the same time, biophysical condition of homeostasis, namely decreased blood fluidity, is rarely considered. Study of rheological behavior of blood after primary oncological endoprosthesis of large joints in patients can potentially affect the selection of perioperative thromboprophylaxis and patient rehabilitation approach.Aim. To study the indicators of the hemorheological status of patients with tumours of long bones in the preand postoperative periods of primary oncological knee replacement.Materials and methods. The study included 36 patients (26 men and 10 women, mean age 46 years) without cardiovascular diseases and pathology of the veins of the lower limb admitted to the Russian Scientific Center of Roentgen Radiology for oncological endoprosthesis of the knee joint. All patients were prescribed: an antiplatelet agent (acetylsalicylic acid) and an anticoagulant (enoxaparin sodium 40 mg daily). Distal resection of the femur (on average 14 cm) was performed in 28 patients, and proximal resection of the tibia (on average 12 cm) in 8 patients, followed by knee replacement. Before the operation, on the 1st and 6th days after the operation, the hemorheological status and the state of the hemostasis system were evaluated in all patients. To determine the reference values, 24 healthy people were examined, comparable in gender and age. Statistical analysis using Statistica 10.0.Results. During the preoperative examination, integral assessment showed the differences in the severity of hemorheological disorders between patients, and two groups were identified: Group 1 – 21 (61 %) patients whose hemorheological disorders were classified as severity degree I, and Group 2 – 14 (39 %) patients with severity degree II d. On day 1 after the surgery, both patient groups showed negative dynamics: hemorheological disorders in group 1 worsened from grade I to II d., and in group 2 to III d. (critical) (p &lt;0.05). On day 6, the parameters did not change significantly in all patients. Similar dynamic changes were observed in the haemostasis system.Conclusion. Activation of the blood coagulation system and deterioration of hemorheology occurred after surgery during administration of anticoagulant and antiplatelet therapy. None of the patients had venous thromboembolic complications, however the risk of their development remained high. A differential model describing hemorheological status can be useful for selection of thromboprophylaxis and rehabilitation methods which include physical factors that improve the hemorheological profile: low-frequency magnetic and/or laser therapy in patients.

Three-Dimensional Multimodality Image Reconstruction as Teaching Tool for Case-based learning among medical postgraduates: a focus on primary pelvic bone Tumour Education

BackgroundPostgraduate medical education in oncology orthopedics confronts obstacles when instructing on pelvic tumors, primarily due to their intricate anatomy and the limitations of conventional teaching techniques. The employment of Three-dimensional multimodality imaging (3DMMI) can be considered a valuable teaching tool, as it gracefully elucidates the intricacies of pelvic anatomical structures and the interactions between tumors and surrounding tissues through three-dimensional imaging, thereby providing a comprehensive and nuanced perspective. This study aimed to assess the feasibility and effectiveness of incorporating 3DMMI in combination with a Case-Based Learning (CBL) approach for postgraduate education.MethodsThe study encompassed a 10-week course involving 90 surgical postgraduates, focusing on common pelvic tumor diseases. Students were assigned representative clinical cases, and each group created a PowerPoint presentation based on these cases. The core educational content included fundamental knowledge of pelvic anatomy, as well as clinical presentations, radiological features, and treatment principles of common pelvic tumor diseases. The research compared two groups: a traditional CBL group (n = 45) and a 3DMMI-CBL group (n = 45). The 3DMMI-CBL group had access to advanced imaging technology for better visualization. Various evaluations, including image interpretation, theoretical knowledge, and questionnaires, were used to assess the learning outcomes.ResultsThe 3DMMI-CBL group outperformed the CBL group not only in the imaging diagnosis of common pelvic diseases but also in their mastery of the related theoretical knowledge. Student questionnaires indicated higher scores for the 3DMMI-CBL group in basic pelvic anatomy knowledge (8.08 vs. 6.62, p < 0.01), image interpretation (8.15 vs. 6.69, p < 0.01), learning efficiency (8.07 vs. 7.00, p < 0.01), clinical reasoning (7.57 vs. 6.77, p < 0.01), and learning interest (8.46 vs. 7.00, p < 0.01). Teacher questionnaires revealed that 3DMMI technology enhanced teachers’ clinical knowledge, facilitated instruction, and increased overall satisfaction and interest in teaching.ConclusionOur study introduced an enhancement to the conventional Case-Based Learning (CBL) model by incorporating 3DMMI technology for visualizing pelvic anatomy. In contrast to pure CBL, this adaptation improved teacher instruction, substantially heightened student engagement, ignited greater interest in learning, and boosted overall efficiency, ultimately leading to positive learning outcomes. Consequently, our study demonstrated the potential feasibility and acceptability of the 3DMMI-CBL teaching method for postgraduates in pelvic bone tumor education.

Solitary Osteochondroma of Scapula: A Common Entity at an Uncommon Location

Introduction: Introduction: Osteochondroma are benign bone tumours that often affect the metaphysical region of long bones; the scapula is rarely affected. Owing to its bulk effect, scapular osteochondroma may frequently present with symptoms. These tumours typically damage the skeleton's developing ends and stop becoming larger once the skeleton reaches maturity.Suspicion of a cancer should be raised by any increase in size. Case Report: A female patient, age 25, complained of a hard lump on her shoulder posteriorly on further imaging it was confirmed as an osteochondroma of scapula. Discussion: Osteochondromas must exhibit continuity with the underlying parent bone cortex and medullary canal. They are made up of cortical and medullary bone with an overlaying hyaline cartilage cap. They have a very minimal potential for malignancy and are often asymptomatic. Conclusion: The cases of osteochondromas in the scapula are relatively rare, it is crucial to remain vigilant and consider the possibility of malignancy if there are concerning changes in symptoms or size.

Imaging features at the time of diagnosis of osteosarcoma and Ewing sarcoma in children

IntroductionOsteosarcoma and Ewing sarcoma are the most frequent malignant bone tumours in children. The aim of this study is to characterize clinical and radiological features at presentation of a large cohort of children with these diseases, radiological findings useful to differentiate them and the main prognostic factors. Material and methodsRetrospective analysis of clinical and imaging findings of 83 children diagnosed and treated of Ewing sarcoma and osteosarcoma in a paediatric hospital during a period of 10 years. ResultsBoth tumours showed aggressive radiological features such as permeative or moth-eaten margins, cortical disruption, discontinuous periosteal reaction, intense contrast uptake, tumoral necrosis and soft-tissue component. They differed in their location, osseous matrix and gender predilection. Osteosarcoma occurred more frequently in the metaphysis of long bones (62%) with a blastic appearance (53%). Ewing sarcoma showed a predilection for male patients (71%), occurred in flat bones (42%) and in the diaphysis of long bones (58%) with a lytic appearance (82%). 29% of children presented with primary metastasis, most frequently located in the lungs. Survival rates were 78% in OS and 76% in Ewing sarcoma. Metastatic disease, aggressive radiological features and low percentage of tumoral necrosis after neoadjuvant chemotherapy were associated with poor prognosis (p < 0.05). ConclusionsImaging can confidently diagnose malignant paediatric bone tumours in children and may differentiate Ewing sarcoma from osteosarcoma, based on gender, location and appearance of the neoplasm. Metastatic disease, presence of aggressive radiological features and low percentage of tumoral necrosis after neoadjuvant chemotherapy were associated with poor prognosis.

Current indications for denosumab in benign bone tumours.

Denosumab is a fully humanised monoclonal antibody to RANK ligand, inhibiting the RANK-RANKL pathway. It promotes the apoptosis of osteoclast-like giant cells, a secondary ossification and connective tissue formation. Given its high efficacy, denosumab is the standard treatment of unresectable or metastatic giant cell tumour of bone (GCTB) requiring morbid surgery. Neoadjuvant administration of denosumab may be justified to enable the resection of the tumour in certain cases; it should be considered, however, with caution for joint-saving surgery due to high local recurrence rates. In cases of unresectable or metastatic GCTB, however, denosumab treatment should be administered for years or even as a lifelong therapy. This poses many yet unanswered questions concerning the frequency of denosumab treatment as well as the ratio of the adverse events in the following years. Denosumab suppresses, not directly targets, the neoplastic stromal cells of GCTB. Ongoing in vitro studies suggest that other drugs alone or in combination (e.g. sunitinib) with denosumab may target both the neoplastic and the giant cells. Promising results have been reported regarding the off-label use of denosumab in other giant cell-rich tumours/tumour-like lesions, i.e. aneurysmal bone cysts and central giant cell granulomas. Data are derived, however, mostly from case reports and case series. Large prospective clinical trials are needed to evaluate the role and also the side effects of denosumab in the treatment of these rare diseases.

Oncological and functional outcomes after resection of malignant tumours of the scapula.

The scapula is a rare site for a primary bone tumour. Only a small number of series have studied patient outcomes after treatment. Previous studies have shown a high rate of recurrence, with functional outcomes determined by the preservation of the glenohumeral joint and deltoid. The purpose of the current study was to report the outcome of patients who had undergone tumour resection that included the scapula. We reviewed 61 patients (37 male, 24 female; mean age 42 years (SD 19)) who had undergone resection of the scapula. The most common resection was type 2 (n = 34) according to the Tikhoff-Linberg classification, or type S1A (n = 35) on the Enneking classification. The ten-year disease-specific survival was 76%. High tumour grade (hazard ratio (HR) 4.27; p = 0.016) and a total resection of the scapula (HR 3.84; p = 0.015) were associated with worse survival. The ten-year metastasis-free and local recurrence-free survivals were 82% and 86%, respectively. Total scapular resection (HR 6.29; p = 0.004) was associated with metastatic disease and positive margins were associated with local recurrence (HR 12.86; p = 0.001). At final follow-up, the mean shoulder forward elevation and external rotation were 79° (SD 62°) and 27° (SD 25°), respectively. The most recent functional outcomes evaluated included the mean Musculoskeletal Tumor Society Score (76% (SD 17%)), the American Shoulder and Elbow Score (73% (SD 20%)), and the Simple Shoulder Test (7 (SD 3)). Preservation of the glenoid (p = 0.001) and scapular spine (p < 0.001) improved clinical outcomes; interestingly, preservation of the scapular spine without the glenoid improved outcomes (p < 0.001) compared to preservation of the glenoid alone (p = 0.05). Resection of the scapula is a major undertaking with an oncological outcome related to tumour grade, and a functional outcome associated with the status of the scapular spine and glenoid. Positive resection margins are associated with local recurrence.

Children’s orthopaedics

The December 2023 Children’s orthopaedics Roundup360 looks at: A comprehensive nonoperative treatment protocol for developmental dysplasia of the hip in infants; How common are refractures in childhood?; Femoral nailing for paediatric femoral shaft fracture in children aged eight to ten years; Who benefits from allowing the physis to grow in slipped capital femoral epiphysis?; Paediatric patients with an extremity bone tumour: a secondary analysis of the PARITY trial data; Split tibial tendon transfers in cerebral palsy equinovarus foot deformities; Liposomal bupivacaine nerve block: an answer to opioid use?; Correction with distal femoral transphyseal screws in hemiepiphysiodesis for coronal-plane knee deformity.

SOLITARY DIAPHYSEAL OSTEOCHONDROMA OF DISTAL FEMUR - A CASE REPORT

Osteochondromas are among the most common benign bone tumours with a male predilection which are usually incidentally detected. They are usually found in the metaphyseal region of long bones.This case is reported in view of the unique origin of the tumour in the diaphyseal region in a female. A woman came to the outpatient department with complaints of a painless, non mobile swelling over left distal femur for past 4 years.The swelling initially small gradually increased in size over 4 years but was not associated with pain or restriction of movements. Clinical examination revealed bony hard swelling in the anteromedial aspect of left distal femur not involving the knee joint. X ray showed a cauliflower shaped, pedunculated bony mass arising from the diaphyseal region of distal femur. MRI done was suggestive of osteochondroma. In order to confirm the diagnosis and to rule out malignant transformation,surgical excision and biopsy of the bony mass was performed. Histopathological reports confirmed the diagnosis of osteochondroma.

Clinical characteristics and treatment outcomes of angiosarcoma of the head and neck: A 17-year single-centre experience

Angiosarcomas in the head and neck region are aggressive tumours associated with high local recurrence and metastatic rates. We present our 17-year experience at the North of England Bone and Soft Tissue Tumour Service. A retrospective review of our prospectively maintained database was undertaken, looking for patients diagnosed with angiosarcomas affecting the head and neck. Data were gathered using a pre-defined proforma to include demographics, histological characteristics, treatment modalities, adjuvant therapies, local recurrence, distant spread, and disease-specific survival. A total of 23 patients (17 males, 6 females) were identified, with a mean age of 76 years at presentation. Fourteen presented with scalp lesions, whereas the remainder arose on the face. Eighteen patients underwent resection with curative intent, whereas three received palliative radiotherapy and two received comfort-based care only. Of the patients undergoing surgery, 12 had local flap reconstruction and 6 underwent free tissue transfer. Clear resection margins were obtained in nine cases (50%). Fourteen patients (78%) presented with local recurrence after surgery, and 11 (61%) developed distant metastases. The median disease-specific survival time for patients treated with curative intent was 38 months. Eight patients had mapping biopsies ahead of their resection; however, complete resection was achieved in only two cases. Angiosarcomas of the head and neck are associated with a poor prognosis, with most patients rapidly developing local recurrence, resulting in adverse clinical outcomes. Mapping biopsies do not demonstrate a clear advantage for achieving complete surgical resection. A radical surgical approach is warranted, given the aggressiveness of the pathology. However, there remains no consensus on optimal surgical management; we recommend further synthesising studies to determine the most appropriate treatment pathway.

Middle ear neuroendocrine tumour-case report of a rare tumour of the temporal bone

Several conditions are known to present as masses in the middle ear medial to an intact tympanic membrane. Reddish masses that blanch on seigelization are usually paragangliomas. Sometimes, a high rising jugular bulb or an aberrant carotid artery can also be seen. Whitish masses are usually congenital cholesteatomas, tympanosclerosis, cartilage grafts that have been surgically placed, and rarely middle ear adenomas (MEA). An elderly male presented with decreased hearing, which was revealed to be secondary to a pale mass in the middle ear, displacing the intact tympanic membrane laterally. He underwent biopsy followed by complete excision of the mass, with preservation of the intact ossicular chain. There no evidence of recurrence on follow-up. Histopathology revealed the mass to be a MEA, also known as middle ear neuroendocrine tumour (MeNET). Middle ear neuroendocrine tumours are rare tumours. We describe the clinical presentation and management of this tumour.

Telengiectatic osteosarcoma of mandible with special emphasis on immunohistochemical and molecular characteristics: A case report with review of literature

AbstractBackgroundTelangiectatic osteosarcoma (TOS) is an uncommon pathology accounting for about 0.4%–12% of all osteosarcomas with predilection for younger adults. It is most frequently seen in the metaphysis of long bones, appearing as painful osteolytic mass that histopathologically characterized by dilated, blood‐filled vascular spaces lined by malignant osteoblasts along with osteoid formation. It has exchangeable clinical, radiological and pathological features with benign lesions like aneurysmal bone cyst and giant cell tumour of bone as well. Thus, delineating this malignancy from its benign counterparts is a crucial part in the therapeutic management.AimTo report a case of telengiectatic osteosarcoma of jaw in a 17 year old female patient with special highlights on molecular diagnostics and therapeutic modules.Materials and MethodsA panel of immunohistochemical markers comprising of SATB2, CDK4, MDM2, panCK, CD31, desmin, NKX2.2 and TLE1 were employed in combination with fluorescent in‐situ hybridization to detect the prevalence of rearrangement in CDK4 and MDM2 genes.ResultsOn immunohistochemistry, the neoplastic cells were strongly positive for SATB2 and negative for CDK4, MDM2, panCK, CD31, desmin, NKX2.2 and TLE1. Fluorescent in‐situ hybridization of CDK4 and MDM2 genes was also negative.ConclusionConsidering its rarity in jaws combined with clinicopathological overlap with other benign pathologies, diagnosis of this entity is crucial in treatment planning and prognostic significance. Moreover, only five cases of TOS have been reported so far in the craniofacial region. Here, we have reported the sixth case of its kind with detailed clarification pertaining to its immunohistochemical and molecular aspects.