Pubic and ischial sarcoidosis: a single bone lesion

Bone sarcoidosis is very rarely indicative of the disease. When bone lesion is associated with lung and lymph node involvement, diagnosis can be made based on clinical and imaging features. When bone lesion is isolated, it is difficult to differentiate it from bone metastases because they both have similar appearance in imaging : in this case, the diagnosis is made by bone biopsy with histological study. We report the case of a 61-year-old male with a lytic lesion of the right ischio pubic ramus which appears to be aggressive whose biopsy revealed bone sarcoidosis.

POLYRADICULONEVRITE CHRONIQUE REVELANT UN PLASMOCYTOME SOLITAIRE (A PROPOS DE DEUX CAS) CHRONICPOLYRADICULONEUROPATHYREVEALING A SOLITARYPLASMACYTOMA (ABOUT TWO CASES)

The solitaryplasmocytomais a rare tumor, made of malignant plasma cellsfound on a single bone, withoutsystemicproliferation, We report two cases of chronicpolyradiculoneuropathyrevealing a solitaryplasmocytoma.

The frequent and clinically benign anomalies of chromosomes 7 and 20 in Shwachman-diamond syndrome may be subject to further clonal variations

Background An isochromosome of the long arm of chromosome 7, i(7)(q10), and an interstitial deletion of the long arm of chromosome 20, del(20)(q), are the most frequent anomalies in the bone marrow of patients with Shwachman-Diamond syndrome, which is caused in most cases by mutations of the SBDS gene. These clonal changes imply milder haematological symptoms and lower risk of myelodysplastic syndromes and acute myeloid leukaemia, thanks to already postulated rescue mechanisms. Results Bone marrow from fourteen patients exhibiting either the i(7)(q10) or the del(20)(q) and coming from two large cohorts of patients, were subjected to chromosome analyses, Fluorescent In Situ Hybridization with informative probes and array-Comparative Genomic Hybridization. One patient with the i(7)(q10) showed a subsequent clonal rearrangement of the normal chromosome 7 across years. Four patients carrying the del(20)(q) evolved further different del(20)(q) independent clones, within a single bone marrow sample, or across sequential samples. One patient with the del(20)(q), developed a parallel different clone with a duplication of chromosome 3 long arm. Eight patients bore the del(20)(q) as the sole chromosomal abnormality. An overall overview of patients with the del(20)(q), also including cases already reported, confirmed that all the deletions were interstitial. The loss of material varied from 1.7 to 26.9 Mb and resulted in the loss of the EIF6 gene in all patients. Conclusions Although the i(7)(q) and the del(20)(q) clones are frequent and clinically benign in Shwachman Diamond-syndrome, in the present work we show that they may rearrange, may be lost and then reconstructed de novo, or may evolve with independent clones across years. These findings unravel a striking selective pressure exerted by SBDS deficiency driving to karyotype instability and to specific clonal abnormalities.

A rare case of plasmacytoma of mandible revealing plasma cell myeloma

Multiple myeloma (MM) is a malignant proliferation of plasma cells with multiple foci. Plasmacytoma is a solitary plasma cell neoplasm involving a single bone. The most commonly involved bone is vertebra. Jaw bones are rarely involved as a first bone as they have lesser hematopoietic marrow. A solitary plasmacytoma may progress to multiple myeloma within few months to year. We present a case of a swelling of mandible that on further investigations confirmed the diagnosis of multiple myeloma. We have discussed the course of treatment given and its prognosis. Keywords: multiple myeloma; plasmacytoma of jaw; bence jones Protein; abnormal plasma cells; CD138.

Single-bone versus both-bone plating of unstable paediatric both-bone forearm fractures. A randomized controlled clinical trial

Purpose This clinical trial compares the functional and radiological outcomes of single-bone fixation to both-bone fixation of unstable paediatric both-bone forearm fractures. Methods This individually randomized two-group parallel clinical trial was performed following the Consolidated Standards of Reporting Trials (CONSORT) statement at a single academic tertiary medical centre with an established paediatric orthopaedics unit. All children aged between nine and 15 years who presented to the emergency department at Assiut university with unstable diaphyseal, both-bone forearm fractures requiring surgical intervention between November 1, 2018, and February 28, 2020, were screened for eligibility against the inclusion and exclusion criteria. Inclusion criteria were diaphyseal unstable fractures defined as shaft fractures between the distal and proximal metaphyses with an angulation of > 10°, and/or malrotation of > 30°, and/or displacement > 10 mm after attempted closed reduction. Exclusion criteria included open fractures, Galeazzi fractures, Monteggia fractures, radial head fractures, and associated neurovascular injuries. Patients who met the inclusion criteria were randomized to either the single-bone fixation group (intervention) or the both-bone fixation group (control). Primary outcomes were forearm range of motion and fracture union, while secondary outcomes were forearm function (price criteria), radius re-angulation, wrist and elbow range of motion, and surgical time Results A total of 50 children were included. Out of these 50 children, 25 were randomized to either arm of the study. All children in either group received the treatment assigned by randomization. Fifty (100%) children were available for final follow-up at six months post-operatively. The mean age of single-bone and both-bone fixation groups was 11.48 ± 1.93 and 13 ± 1.75 years, respectively, with a statistically significant difference (p = 0.006). There were no statistically significant differences in gender, laterality, affection of the dominant hand, or mode of trauma between single-bone and both-bone fixation groups. All patients in both groups achieved fracture union. There mean radius re-angulation of the single-bone fixation groups was 5.36 ± 4.39 (0–20) degrees, while there was no radius re-angulation in the both-bone fixation group, with a statistically significant difference (p < 0.001). The time to union in the single-bone group was 6.28 ± 1.51 weeks, while the time to union in the both-bone fixation group was 6.64 ± 1.75 weeks, with no statistically significant difference (p = 0.44). There were no infections or refractures in either group. In the single-bone fixation group, 24 (96%) patients have regained their full forearm ROM (loss of ROM < 15°), while only one (4%) patient lost between 15 and 30° of ROM. In the both-bone fixation group, 23 (92%) patients have regained their full forearm ROM (loss of ROM < 15°), while only two (8%) patients lost between 15 and 30° of ROM. There was no statistically significant difference between groups in loss of forearm ROM (p = 0.55). All patients in both groups regained full ROM of their elbow and wrist joints. On price grading, 24 (96%) and 23 (92%) patients who underwent single bone fixation and both-bone fixation scored excellent, respectively. Only one (4%) patient in the single-bone fixation group and two (8%) patients in the both-bone fixation group scored good, with no statistically significant difference in price score between groups (p = 0.49). The majority of the patients from both groups had no pain on the numerical pain scale; 22 (88%) patients in the single-bone fixation group and 21 (84%) patients in the both-bone fixation groups, with no statistically significant difference between groups (p = 0.38). The single-bone fixation group had a significantly shorter mean operative time in comparison to both-bones plating (43.60 ± 6.21 vs. 88.60 ± 10.56 (min); p < 0.001). Conclusion Single-bone ulna open reduction and plate fixation and casting are safe and had a significantly shorter operative time than both-bone fixation. However, single-bone ORIF had a higher risk radius re-angulation, alas clinically acceptable. Both groups had equally excellent functional outcomes, forearm ROM, and union rates with no complications or refractures. Long-term studies are required.

Endometrial Carcinoma Presenting with an Isolated Osseous Metastasis: A Case Report

Patients with advanced endometrial cancer had generally a poor prognostic with a median survival often less than one year. Isolated bone metastases as the first sign of the disease of endometrial cancer was a very exceptional situation rarely described in the literature. The goal of this report is to describe the management of this rare site of metastases and to reviewed Clinic pathological features and prognosis of isolated bone metastases. We present a case of a 56-year-old woman treated for endometrial cancer with isolated ribs bone metastases. She was complaining about progressive right rib pain as a first sign of the disease of endometrial cancer. She had a biopsy of the lesion in the last right thoracic rib. This has proved a metastatic adenocarcinoma compatible with an endometrial primary cancer. An endometrial biopsy showed moderately differentiated endometrioid adenocarcinoma. Imagery did not reveal any other sites of metastatic disease. The patient underwent bilateral salpingo-oophorectomy and hysterectomy, peritoneal washing, omentectomy, bilateral pelvic and par aortic lymphadenectomy. In addition, a right thoracotomy was carried out and complete surgical excision of the rib mass was successfully performed. Adjuvant chemotherapy was administrated. The patient is clinically free of disease 10 months following diagnosis. Single-bone extrauterine metastatic site and local disease limited to the uterus had better survival outcomes than Stage IVb endometrial cancer with multiple metastatic sites. Therefore, the patient treated with surgery with a clear margin continued to behave as early-stage endometrial cancer.

A Stepwise Approach to Identify the Clinical Role of FDG PET-CT in Patients with Suspicious Bone Metastasis from an Unknown Primary Site

Objectives: In patients with suspicious bone metastasis, differential diagnosis and primary lesion identification is very important in optimizing treatment planning. The study was aimed to assign a step-wise approach based on image findings to identify the precise role of PET-CT in these patients. Patients and Methods: A total of 74 patients were enrolled with suspicious bone metastasis who underwent PET-CT for the evaluation of primary focus. Patients were classified into four groups with stepwise manners, firstly based on FDG positivity of bone lesions, then on multiplicity of positive bone uptake and finally on presence of extra-skeletal uptake. We analyzed the difference of distribution of follow-up results according to each group. In addition, we also investigated whether PET-CT had a role to guide biopsy sites. Results: Except for 7 patients without bone or extra-skeletal uptake, 44 patients were categorized in multiple bone and extra-skeletal uptake group (A), 12 in multiple bone uptake without extra-skeletal uptake group (B), 3 in single bone and extra-skeletal uptake group (C) and 8 in single bone uptake without extra-skeletal uptake group (D). In group A, 42/44 patients had metastatic bone disease; where primary lesion was correlating extra-skeletal uptake in 40 (91.0%) cases. Among the 12 patients of group B, 5 (41.7%) had primary malignant bone disease, 3 (25.0%) had benign bone lesions and 4 (33.3%) had metastatic bone disease but PET-CT failed to indicate the primary site. In group C, PET-CT positive extra-skeletal uptake sites were confirmed as primary lesions in all the 3 patients. Among the 8 patients of group D, 5 (62.5%) were benign bone disease, one (12.5%) was high grade sarcoma and the remaining two (25.0%) were metastatic bone disease where PET-CT failed to identify primary site. Additionally, there was significant (P = 0.0003) difference in prevalence of biopsy sites performed according to the presence of extra-skeletal uptake in PET-CT. Conclusion: Clinical impact of PET-CT was different according to groups classified with stepwise manners, which suggested that different strategies should be taken especially when there was not extra-skeletal uptake to optimize management plan. PET-CT also had a significant role in patients with extra-skeletal uptake lesion through guidance of easily approachable biopsy sites. Bangladesh J. Nuclear Med. 22(2): 95-102, Jul 2019

Congenital anomalies of the lumbosacral spine

The aim of the study is to compare the structure of the lumbosacral transitional vertebra, which were detected by computed tomography (CT) and identified on dried human sacral, and to discuss possible pathogenetic mechanisms of this congenital malformation. The article presents 9 cases of lumbosacral transitional vertebra, including 6 cases of L5 sacralization and 3 cases of S1 lumbarization. The formation of the transitional lumbosacral vertebra is genetically determined. All types of such developmental anomaly can be detected only on CT. L5 sacralization repeats the process of fusion of the sacral vertebra into a single bone. A lack of the costotransverse bars of the first sacral vertebrae fusion results in the S1 lumbarization.