Principles of Management of Extremity Skeletal Metastasis.

Understanding the epidemiology of extremity skeletal metastasis and the factors deciding the treatment decision-making are essential in developing a diagnostic and treatment strategy. This leads to optimum care and reduces disease-related burden. With the evolution of medical, radiation therapy, and surgical methods, cancer care has improved the quality of life for patients with improved survival and functional status in patients with skeletal metastasis. Based on the currently available literature, we have described a step-wise evaluation and management strategy of metastatic extremity bone disease. The present review article addresses various aspects and related controversies related to evaluation, staging, and treatment options in the management of extremity bone metastasis. This article also highlights the role of multidisciplinary involvement in management of extremity skeletal metastasis.

A Case Report of Giant Aneurysmal Bone Cyst of Distal Tibia with a Non-healing Fungating Mass and its Management in a 30-year-old Male.

Introduction: Aneurysmal bone cyst (ABC) is a benign, expansible, non-neoplastic tumor of usually long bones and is identified by blood vessels and spaces that are most often differentiated by fibrous septae. It is challenging to treat these rare, giant ABCs as they have a damaging effect on the bones and compress the nearby structures, especially in load-bearing bones of the body. Case Report: We report a case of a giant ABC in the distal tibia one-third with soft tissue component of a 30-year-old male. The patient presented to our outpatient department with complaints of pain and swelling over left ankle for 1 year. The size of the swelling was 15 cm × 10 cm × 10 cm over medial aspect of ankle with 3 discharging sinuses which present over swelling. His blood parameters were suggestive of low hemoglobin count. X-rays showed cystic lesions over medial aspect of left ankle. Computed tomography scan and magnetic resonance imaging reports were suggestive of ABC. Conclusion: Our case report is unique as it reminds us that when presented with a case of ABC, excision of fungating soft tissue with curettage followed by cementing can be a preferable and better treatment option. ABC was extensively curetted out, the formed cavity was packed with bone cement, and fixation with 3 cortico cancellous screws was carried out. At 4-month follow-up, the lesion had receded, and the patient was walking without pain and any deformity. We suggest that this method of treatment is beneficial for ABC at this site and at this age.

Recurrent Giant Cell Tumor of the Distal End of the Femur.

Introduction: The aim of the study was a case report of a right-sided recurrent giant cell tumor of the distal end of the femur. Methodology: A case of a 25-year-old male patient with a history of recurrent giant cell tumor of the right distal femur who presented with chief complaints of pain over right distal femur and stiffness in the right knee for 2 years with restricted knee movement and is unable to walk. He was diagnosed with recurrent giant cell tumor of the right of the distal femur and was treated with wide excision with mega prosthesis reconstruction. Result: Wide excision with mega prosthesis reconstruction showed a good functional range of motion with early rehabilitation, stability, and mobility of joints. Conclusion: We recommend wide excision and reconstruction with mega prosthesis is an effective method compared to sandwich technique and nailing and can be successfully done in the case of recurrent giant cell tumor of the distal femur with a good outcome, functional range of motion, stability, and mobility of the joint with early rehabilitation though it is a technically demanding surgery. The knee joint could have been salvaged and the need for more extensive surgery could have been prevented, had the diagnosis of recurrent giant cell tumor was made earlier.

Upper Cervical Leiomyoma in an Adolescent Male.

Introduction: Leiomyomas are benign epithelial tumors with a female preponderance usually in the uterus, gastro-intestinal tract and skin. They are well-capsulated tumors with no mitotic activity and little pleomorphism. Primary leiomyoma in the upper cervical spine region is rare and the occurrence of these lesions in young immunocompetent males is extremely rare. Case Report: A 15-year-old male had swelling over the nape of the neck for 4 years with slight difficulty in neck movements for a few months. Asymptomatic 4 years back be developed a painless swelling at the nape of the neck on the right side initially the size of a pea which gradually increased size. A 10x8cm firm, on-tender and non-pulsatile swelling at the nape of the neck on the right extending from the occipital nuchal line and crossing the midline up to the posterior border of the sternomastoid. The skin over the swelling wasn't adherent and the swelling did not reduce on neck movements. Neck movements were terminally restricted with restriction on rotation towards the right. Routine X-ray's and magnetic resonance imaging were suggestive of a soft tissue mass in the inter-muscular plane on the posterior aspect more on the right side with a cystic component and causing thinning of the C2 lamina with no intraspinal extension. Biopsy was done. Findings were suggestive of a spindle cell benign tumor. Posterior en bloc excision was planned and the lesion blog with the entire capsule was excised and sent for histopathology which revealed the lesion was a leiomyoma. The patient has shown no clinical or radiological evidence of recurrence at 4 year fol-low-up. Conclusion: Leiomyomas in the upper cervical spine is extremely rare in adolescent immunocom-petent male.

Primary aneurysmal bone cysts.

AIMS: The aim of this study was to analyze the complications and outcomes of treatment in a series of previously untreated patients with a primary aneurysmal bone cyst (ABC) who had been treated by percutaneous sclerosant therapy using polidocanol. METHODS: Between January 2010 and December 2016, 56 patients were treated primarily with serial intralesional sclerosant injections. Their mean age was 20 years (1 to 54). The sites involved were clavicle (n = 3), humeri (n = 11), radius (n = 1), ulna (n = 3), hand (n = 2), pelvis (n = 12), femur (n = 7), tibia (n = 13), fibula (n = 3), and foot (n = 1). After histopathological confirmation of the diagnosis, 3% polidocanol (hydroxypolyaethoxydodecan) was injected into the lesion under image intensifier guidance. Patients were evaluated clinically and radiologically every six to eight weeks. In the absence of clinical and/or radiological response, a repeat sclerosant injection was given after eight to 12 weeks and repeated at similar intervals if necessary. RESULTS: There were no complications of treatment. One patient was lost to follow-up. Overall, 46/55 (84%) of lesions healed after one or more injections of polidocanol: 24/55 (44%) patients healed with a single injection, and 43/55 (78%) within two injections. Of these 46, four (9%) patients developed local recurrence, two of whom healed with a repeat sclerosant injection. Thus, 44/55 (80%) patients of primary ABC healed with sclerotherapy. The mean follow-up was 62 months (20 to 111). The local recurrence free survival (LRFS) with percutaneous sclerosant therapy with polidocanol was 100%, 98% (95% confidence interval (CI) 85 to 100) and 93% (95% CI 78 to 98) at two, three, and five years, respectively. CONCLUSION: Percutaneous sclerotherapy using polidocanol is a safe, effective, minimally invasive and inexpensive method of treating a primary ABC of the limbs or pelvis. Cite this article: Bone Joint J 2020;102-B(2):186-190.

Case of Atypical Adenoid Hyperplasia of Lung with Polyostotic Fibrous Dysplasia and Diabetes Mellitus: A Random Coincidence or a Possible Unrecognized Syndromic Association?

INTRODUCTION: Fibrous dysplasia (FD) is a benign intramedullary pathologic condition that is characterized by the replacement of bone with fibrous tissue. FD may be monostotic or polyostotic with the craniofacial bones, and the proximal femur is most commonly involved. CASE REPORT: A 39-year-old lady presented to our hospital, a tertiary care center with asymmetric swelling of her arms, for over 20 years. Radiographs revealed gross enlargement with marrow expansion of the right humerus, scapula with ground-glass matrix, along with a multiseptated cystic appearance. Positron emission tomography-computed tomography screening for the activity of these lesions incidentally demonstrated a few lung nodules, which on biopsy was found to be atypical adenomatous hyperplasia. The lady also had endocrine dysfunction in the form of diabetes mellitus, for which she was on treatment. CONCLUSION: In this article, we briefly review the available literature to decipher if any of the associated syndromes with polyostotic FD (being the dominant clinical feature) are known to have associations which explain the above findings. There is a need to recognize the underlying pattern so that appropriate genetic counseling, if any, can be provided to such patients.