Surgical management of central giant cell granuloma of mandible and prosthetic rehabilitation in a nine year girl: A case report

Central giant cell granuloma (CGCG) is an uncommon, benign, idiopathic, osteolytic lesion of jaws, histologically characterized by multinucleated giant cells distributed in fibrovascular connective tissue stroma. Accurate diagnosis of the lesion is essential for the successful management and the prognosis of this locally destructive lesion. In this paper, a rare case of large destructive CGCG involving anterior region of mandible, causing expansion of labial cortical plate and mobility of teeth in a nine-year girl is presented. It was treated successfully by enucleation and curettage with satisfactory preservation of the continuity of mandible. Nine months post operatively, the child was rehabilitated with a temporary partial denture to improve esthetics, phonetics and function. One year clinical and radiographic follow up showed new bone formation and no evidence of recurrence.

Desmoplastic fibroma of bone: A morphological and immunohistochemical characterization

Background: Desmoplastic Fibroma (DF) of bone is a locally aggressive and infrequent benign neoplasm. Recently was described a role of vascular endothelial growth factor in the interstitial fibrotic processes. Case presentation: A 13-year-old female presented with pain, swelling and limitation of movements in right forearm. An osteolytic lesion at the distal end of the right radius was shown, with pathologic concentration of Technetium 99 and slight enhancement of soft tissue lesion employing computerized axial tomography. The surgical biopsy showed nodular formations of hyalinized collagen fibers arranged in thick bands with few well-differentiated interstitial fibroblasts / myofibroblasts, focally expressing VEGF-A. Conclusion: The intramedullary neoplastic proliferation is limited by the cortical bone, provoking compression of the intratumorally micro-vessels, favoring both, the extracellular matrix and VEGF-A synthesis. Future research should include therapeutic intervention with anti-CD117 and anti-VEGF-A drugs, with the aim of limiting tumor growth, facilitating the complete surgical excision of the neoplasm. Keywords: desmoplastic fibroma; vascular endothelial growth factor; hyalinization; neoplasm progression.

Osteolytic Bone Lesions in Patients with Primary Myelofibrosis: A Systematic Review

Background: Philadelphia negative Myeloproliferative neoplasms classically characterized by excess production of terminal myeloid cells in the peripheral blood. Among this group, primary myelofibrosis is the least common and usually carries the worst prognosis. Bone involvement in primary myelofibrosis has many forms and tend to manifest as osteosclerotic lesions in vast majority of cases, however osteolytic lesions are reported in exceptional occasions. In this review, we tried to shed the light on this rare association. Methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the English literature (Google scholar, PubMed, and SCOPUS) for studies, reviews, case series, and case reports about patient with myelofibrosis who develop lytic bone lesion. We used the terms in combination: "Myelofibrosis'" or "Primary myelofibrosis" OR "chronic idiopathic myelofibrosis" OR "agnogenic myeloid metaplasia" and "Osteolytic bone lesion", "Osteolytic lesion", "lytic bone lesion". The review included patients with primary myelofibrosis confirmed by biopsy. The reference lists of the included studies were scanned for any additional articles. The search included all articles published up to 10th April 2021. Two independent reviewers screened the titles and abstracts of the records independently and papers unrelated to our inclusion criteria were excluded. A total of 13 articles were included in the review. Results : Total of 13 patients were included in the review. 7 patients were males, male to female ratio almost of 1:1. The mean age at time of diagnosis was 57.69 year, only two cases were diagnosed at young age, however the majority have osteolytic bone lesion at age above 50 years (12/13) of cases. The mean time between the diagnosis of primary myelofibrosis until the osteolytic bone lesion capturing was approximately 8.8 years. 9 out of 13 patients have painful bone lesion, others were incidental finding during a scan for other reasons. All patients have significant splenomegaly. All patients had the lytic lesion detected on x ray, and 2 patients had confirmed findings on magnetic resonance imaging (MRI). The most common affected bones were the vertebrae, pelvis, ribs, humerus then the scapula, femur and skull and less frequently wrist bones and calcaneus. Only one case has reported involvement of the tibia and fibula. The shape, the extension and the numbers of lesion were variable, some showed cortical sparing and others come with cortical destruction. 10 out of 13 cases have confirmed the nature of the osteolytic lesion containing hematopoietic stem cells with or without fibrosis, 2 cases were positive for JAK2 mutation. 2 patients have received ruxolitinib, one of them preceded with bone marrow transplant, others received nonspecific therapies. Discussion: The hyperdynamic ineffective bone marrow can have a negative impact on the bone structure resulting in different types of bone pathology including lytic and sclerotic lesions. The exact mechanism beyond developing lytic lesions is not fully studied, observations revealed two possible causes: systemic inflammation and direct mechanical compression from para-osseus lymph nodes. Lesions prevalence was equal in both genders which can be attributable to a small sample size, in addition, most of the patients were in advanced stages when the lytic lesions discovered and this observation can be explained by the needed time to generate extramedullary hematopoiesis and its subsequent effect on bone structure. The variation in time between the diagnosis of PMF and development of osteolytic bone lesions could be due to the indolent phase of the disease, in which patients can survive for decades without symptoms. Until recently the treatment of myelofibrosis was supportive, but after establishing the JAK2-stat pathway role in myeloproliferative disorders the FDA approved ruxolitinib a JAK2 inhibitor which shows not only survival benefit but also has a significant impact on the resolution of the lytic bone lesions as well. conclusion Osteolytic bone lesions in patients with primary myelofibrosis is extremely rare finding, and noticed shortly after diagnosis in elderly and after longer duration in young patients. The lytic lesion seems to have a bad prognostic value as we can notice 11 out of 13 patients died within one year of detection. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Non secretory myeloma in young man mimicking the Gorham disease: case report and the literature review

Multiple myeloma is a neoplasm of plasma cells affecting mostly the elderly with incidence peaks between 60 and 70 years. This disease is exceedingly rare in younger people, especially in adults under 30-year-old. Non-secretory multiple myeloma accounts for 1–5% of all cases of multiple myeloma. It is also a rare condition in young adult patients, and only six cases have been reported [1]. We herein describe a rare case of non-secretory myeloma in a 22- year-old male, explaining from chest wall pain, without general manifestation. Plain radiography and CT scans revealed diffuse osteolytic lesion mimicking the Gorham disease. A bone marrow biopsy was conducted, revealing the diagnosis of myeloma.

Primary Rosai-Dorfman Disease in 39-Year-Old Woman With Osseous Tibial Lesion Manifestion: A Case Report and Literature Review

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), is a very rare and typically benign disorder of unknown etiology with <10% bone involvement. The report is of a case seen at the authors’ hospital of a patient presenting with several months’ onset unspecified nontraumatic ankle pain. There was no physical mass or lymphadenopathy appreciated on examination. Plain radiographs and magnetic resonance images demonstrated an osteolytic lesion at the medial malleolus. Biopsy revealed the diagnosis of intraosseous manifestation of Rosai-Dorfman disease.

Bilateral simple bone cyst in mandible: Rare case report

The simple bone cyst is a benign osteolytic lesion, uncommon in the maxillomandibular region. Its etiology remains unknown, although traumatic injuries are often associated with this injury. Young patients, men and women alike are the most affected. It is asymptomatic in most cases, being diagnosed on routine radiographs. The radiolucent image, uni or multilocular, is well defined, with irregular margins, mainly in the posterior region of the mandible. Bilateral presentation is unusual. The final diagnosis is made during surgical procedures when an empty bone cavity is detected. Simple curettage is the treatment of choice and it consists of causing bleeding and filling the cavity with a blood clot. Bone repair will be observed in the follow-up procedures. The aim of this study was to present a rare case of bilateral simple bone cyst in a young male, treated with curettage of the bone walls.

Synovial Sarcoma of the Cervical Spine: Case Report

PurposeTo describe a very rare case of Synovial Sarcoma affecting cervical spine vertebra.SS is a rare malignant and aggressive soft tissue tumour arising from mesenchymal cells. Primary bone origin SS is a much rarer entity that affects more commonly long bones. Ideal therapeutic strategy is yet to be defined due to very small number of cases reported so far.Case reportA 55-year-old male, construction worker, with no other relevant medical history presented with a progressive tetraparesis after recurring several times during a 4-week period to assistant physician and emergency department complaining about bilateral shoulder pain. Image studies revealed an osteolytic lesion centred on C4 vertebra with intracanalar and intraforaminal extension, causing neurologic compression. Patient was submitted to urgent surgical decompression intervention. C3 and C4 corpectomy and excisional biopsy followed by stabilization with C2-C5 arthrodesis.OutcomesNeurological deficits did not improve after surgery. Histopathological and immunohistochemical analysis revealed phenotypical characteristics of a biphasic Synovial Sarcoma. Patient died 4 weeks after surgery due to a respiratory tract infection.DiscussionSS is a malignant rare and aggressive soft tissue tumour that usually affects young adults. Very few cases of primary bone SS affecting the spine are described in literature. Imaging studies may suggest the diagnosis of synovial sarcoma but definitive diagnosis can only be confirmed by histological and immunohistochemical analysis.The rarity of these lesions demands high clinical suspicion for the diagnosis and due to the low number of cases reported ideal therapeutic strategy is yet to be defined.

Giant Cell Tumor of Intermediate Cuneiform Evolving into Aneurysmal Bone Cyst: A Rare Case Report

Introduction: Giant cell tumor (GCT) most commonly involves distal femoral condyles, distal end of radius, proximal tibial plateau, and proximal humerus. GCT is uncommon to occur in small bones of hand and feet. 2% of GCT occur in hand. The incidence of GCT in foot is 1.2–1.8%. Only a few cases have been reported in literature worldwide. GCT is the most common cause of secondary ABC. We report a case of GCT of intermediate cuneiform in a 25-year-old female evolving into aneurysmal bone cyst (ABC). Case Report: A 25-year-old female presented to us with complaints of pain and swelling over the dorsum of right foot for a period of 1 year. On examination, there was a localized ovoid-shaped swelling of 2 by 2 cm over the dorsum of right foot. Radiographs revealed a well-defined osteolytic lesion in the intermediate cuneiform. T2 MRI showed hyper-intense lesion in intermediate cuneiform. The patient was taken up for surgery, and the intermediate cuneiform was excised completely. Removed bone was sent for histopathological examination which confirmed it to be GCT evolving into secondary ABC. The patient was followed for 1 year and had no complaints. The patient was able to weight bear and walk without any difficulty. There was no recurrence of lesion. Conclusion: GCT of the cuneiform evolving into ABC is a very rare presentation. The treatment of choice is excision of the tumor with or without bone grafting. Any osteolytic lesion in the small bones must be evaluated and should be intervened in the early stage. Keywords: Giant cell tumor, osteolytic lesion, surgical resection, intermediate cuneiform.

Intraosseous schwannoma of the humerus: a rarity yet warrants consideration

Intraosseous schwannoma is extremely rare that it is not often considered among differential diagnosis for an osteolytic lesion, especially in long bones of the extremities. Amounting to less than 0.2% of all primary bone tumours and less than 200 cases reported so far, with only 3 cases involving the humerus, we hereby report the fourth case. In addition to its rarity, this was the only case of an intraosseous schwannoma involving the humerus bone which presented with a pathological fracture in a 45-year-old woman after sustaining a trivial trauma. Radiological examination revealed a geographic type of osteolytic lesion in distal shaft region of the left humerus. Only a histopathological examination helped in revealing and confirming the diagnosis of an intraosseous schwannoma. Treatment of the tumour with complete excision with bone graft reconstruction and osteosynthesis yields good results with very low risk of recurrence.

Peritrochanteric Unicameral Bone Cyst in A 9-year-old Girl: A Case Report and Review of Literature

Introduction: The Unicameral Bone Cyst (UBC) is a benign osteolytic lesion primarily found in the metaphyseal part of long bones in children. It is important as it can involve growth plate involvement, cause pathological fractures and deformities of the affected limb. We report this case to emphasize that hip area pathologies can be represented with knee pain and discomfort. Case Presentation: The patient was a 9-year-old girl with a bone cyst in the right proximal femur, with functional knee pain and limping. The diagnosis was made after two years of pain in the knee area. As the cyst was symptomatic and the signs of impending pathological fracture were seen, the lesion was managed by curettage and fibular strut allograft and proximal humerus locking plate. Conclusions: The patient has been examined for knee joint problems for a long time, and her femur bone cyst was diagnosed after two years of pain. However, it could become a pathologic fracture or involve the growth plate and stop the limb’s growth, thus affecting the patient’s quality of life. Therefore, in children with chronic knee pain, careful assessment of the hip area is recommended.