scholarly journals Paget ‘s Disease: Not So Typical for Atypical Femur Fracture

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A209-A210
Author(s):  
Ashima Mittal ◽  
Murray B Gordon
Keyword(s):  
Task Force ◽  
Paget’S Disease ◽  
Distal Tibia ◽  
Elevated Serum ◽  
Hip Osteoarthritis ◽  
Paget's Disease ◽  
Cortical Thickening ◽  
Task Force Report ◽  
Femur Fractures ◽  
History Of

Abstract Introduction: Atypical femur fractures (AFF) are reported in patients taking prolonged bisphosphonate therapy, but Paget’s disease (PD) has been rarely reported as a cause of AFF. Case: 71-year-old female with past medical history of right hip osteoarthritis, seizure disorder, hypertension, and Hashimoto’s thyroiditis presented with persistent right hip pain. There was no history of trauma or fall. She had otosclerosis with bilateral hearing loss and bilateral stapedectomies. Her medications were primidone, levothyroxine, lisinopril-hydrochlorothiazide, and vitamin D. She did not smoke tobacco or drink alcohol. She had elevated serum alkaline phosphatase of 300 U/L (35–104). The X-ray of the skull was negative for any cortical thickening. CT of right femur revealed cortical thickening and coarsening of trabeculae of the proximal right femur consistent with PD and incomplete atypical subtrochanteric proximal fracture. Urine NTx 303 BCE/mM Cr (<89). Bone scan showed uptake in the R proximal femur, L distal tibia, and L3-L4 vertebral bodies suspicious of PD. DXA showed osteopenia. She was given zoledronic acid 5 mg IV. Discussion: PD leads to an increased incidence of fractures particularly of the lower extremities with most fractures transverse in nature. Non-union is not uncommon1. Stress fractures in PD are caused by disorganized bone remodeling due to excessive breakdown and formation of bone. Our patient met the major criteria for AFF as per the ASBMR 2010 task force report but there was no exposure to bisphosphonates2. The ASBMR task force recommended that bisphosphonates should be discontinued in patients with bisphosphonate-associated AFF due to their severely suppressed bone turnover status. On the other hand, the AFF in patients with PD may heal in response to bisphosphonate treatment. References: 1. Singer FR. Bone Quality in Paget’s Disease of Bone. Curr Osteoporos Rep. 2016;14(2):39–42. DOI:10.1007/s11914-016-0303-62. Shane E, Burr D, Ebeling PR, Abrahamsen B, Adler RA et.al. Atypical subtrochanteric and diaphyseal femoral fractures: report of a task force of the American Society for Bone and Mineral Research. J Bone Miner Res. 2010; 25:2267–2294.

scholarly journals A rare case of Paget’s disease affecting the cervical spine

2019 ◽  
Vol 101 (2) ◽  
pp. e38-e42
Author(s):  
J Holton ◽  
M Jones ◽  
Z Klezl ◽  
M Czyz ◽  
M Grainger ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Independent Living ◽  
Paget’S Disease ◽  
Posterior Part ◽  
Cord Compression ◽  
Bone Cell ◽  
Paget's Disease ◽  
History Of ◽  
Posterior Instrumented Fusion ◽  
Degenerative Cervical Spine

We present the case of a 75-year-old man with a rapidly progressive cervical myelopathy on a background of a 3-year history of neck pain and a severely degenerative cervical spine. The patient developed progressive myelopathy over a six-month period and suffered from worsening kyphosis. Suspicion of an underlying oncological process prompted transfer to our tertiary referral unit. Biopsy was consistent for Paget’s disease, an extremely rare diagnosis of the cervical spine. Magnetic resonance imaging revealed cord compression between C4 and C6 with associated cord signal change indicative of myelopathy. A three-level corpectomy and posterior instrumented fusion was performed. There was significant blood loss (3.5l) intraoperatively, consistent with a diagnosis of Paget’s disease of the bone. Cell salvage was used, as was neuromonitoring for both the anterior and posterior part of the procedure. Postoperatively, neurological function improved slightly and the patient required community neurorehabilitation to allow independent living.

scholarly journals Extramammary Paget's Disease: A Case Report

2020 ◽  
Vol 8 (1) ◽  
pp. 83-85
Author(s):  
Anita Shah ◽  
Yogesh Poudel ◽  
Anuj Poudel
Keyword(s):  
Paget’S Disease ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Erythematous Plaque ◽  
Eosinophilic Cytoplasm ◽  
Topical Glucocorticoids ◽  
Atypical Cells ◽  
History Of ◽  
White Scale ◽  
Extramammary Paget's Disease

A 60- year-old man presented with four year history of a non- healing rash. The rash was pruritic and painless. He had previously been treated empirically with oral and topical antibiotics, topical antifungal agents, and topical glucocorticoids without improvement. The physical examination revealed an erythematous plaque with superficial white scale affecting the skin of the groin and scrotum. Skin biopsy and histopathology revealed epidermal infiltration by large atypical cells that had eosinophilic cytoplasm and prominent nucleoli, findings were consistent with Pagets disease. Extramammary Paget's disease (EMPD) is an intraepithelial adenocarcinoma that is sometimes misdiagnosed as dermatitis clinically. It most commonly involves the vulva and in perianal skin, scrotum, penis and axilla. The patient was kept on regular surveillance. The patient has increased intensity of itching and increase in size of lesion after six months of diagnosis.

scholarly journals Molecular insights into an ancient form of Paget’s disease of bone

2019 ◽  
Vol 116 (21) ◽  
pp. 10463-10472 ◽  
Author(s):  
Barry Shaw ◽  
Carla L. Burrell ◽  
Darrell Green ◽  
Ana Navarro-Martinez ◽  
Daniel Scott ◽  
...  
Keyword(s):  
Direct Sequencing ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Paget’S Disease Of Bone ◽  
Paget's Disease Of Bone ◽  
North West ◽  
Sequestosome 1 ◽  
The North ◽  
History Of ◽  
Skeletal Samples

Paget’s disease of bone (PDB) is a chronic skeletal disorder that can affect one or several bones in individuals older than 55 y of age. PDB-like changes have been reported in archaeological remains as old as Roman, although accurate diagnosis and natural history of the disease is lacking. Six skeletons from a collection of 130 excavated at Norton Priory in the North West of England, which dates to medieval times, show atypical and extensive pathological changes resembling contemporary PDB affecting as many as 75% of individual skeletons. Disease prevalence in the remaining collection is high, at least 16% of adults, with age at death estimations as low as 35 y. Despite these atypical features, paleoproteomic analysis identified sequestosome 1 (SQSTM1) or p62, a protein central to the pathological milieu of PDB, as one of the few noncollagenous human sequences preserved in skeletal samples. Targeted proteomic analysis detected >60% of the ancient p62 primary sequence, with Western blotting indicating p62 abnormalities, including in dentition. Direct sequencing of ancient DNA excluded contemporary PDB-associated SQSTM1 mutations. Our observations indicate that the ancient p62 protein is likely modified within its C-terminal ubiquitin-associated domain. Ancient miRNAs were remarkably preserved in an osteosarcoma from a skeleton with extensive disease, with miR-16 expression consistent with that reported in contemporary PDB-associated bone tumors. Our work displays the use of proteomics to inform diagnosis of ancient diseases such as atypical PDB, which has unusual features presumably potentiated by yet-unidentified environmental or genetic factors.

scholarly journals Statin Use and Its Effects on Skeletal Related Events in Patients with Multiple Myeloma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5774-5774 ◽  
Author(s):  
Fernando M Vargas Madueno ◽  
Amin Benyounes ◽  
Gentry King ◽  
Kuan-Hsiang Gary Huang ◽  
Gabor Varadi
Keyword(s):  
Multiple Myeloma ◽  
Vitamin D ◽  
Alcohol Abuse ◽  
Statin Therapy ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Exact Test ◽  
History Of ◽  
Skeletal Related Events ◽  
Statin Use

Abstract Introduction: Skeletal related events (SRE) in multiple myeloma (MM) patients are associated with significant morbidity and mortality. Studies assessing the anti myeloma effect of statins in conjunction with chemotherapy have shown conflicting results with regards to overall survival or disease response. To our knowledge there have been no studies evaluating the effect of statins on SRE. Here we sought to assess the relationship between statin use and the presence of SRE. Methods: We retrospectively reviewed 101 patients seen at our institution between the years 2007-2012 who had a diagnosis of MM separating them in 2 groups, patients on statin therapy (n=50) and those without statin therapy (n=51). Statin use was considered if present prior to the occurrence of a SRE. SRE were defined as pathologic fractures, necessity for orthopedic intervention, radiation therapy or spinal cord compression. MM stage as per the International Staging System (ISS), as well as history of osteoporosis, malignancy, hypothyroidism, Paget's disease of the bone, alcohol abuse, smoking status, calcium-vitamin D, and bisphosphonate use were also recorded. Results: In our cohorts the prevalence of SRE was significantly lower in patients on statin medication when compared to statin naive patients, (36% vs. 58.8% respectively, p = 0.029, Fisher's exact test, Figure 1). No significant differences were noted between statin treated group and statin naive group in the following variable subgroups (Fisher's exact test): the history of smoking or alcohol abuse, the documented diagnosis of osteoporosis, coexistent malignancy, hypothyroidism, or Paget's disease of the bone, and the use of other medications including bisphosphonates or calcium - vitamin D supplementation. There were no significant differences in distribution of cancer staging (according to ISS) when we compared between statin use status (chi-square test for trend). Conclusion: The use of statins was associated with a decrease in the prevalence of SRE in patients with MM. This was found independent of disease stage, history of osteoporosis or second malignancy, and bisphosphonate use. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Hydrocephalus and Headaches in Paget’s Disease of the Skull: Complete Relief by Ventriculo-Atrial Shunt

1984 ◽  
Vol 11 (1) ◽  
pp. 69-72 ◽  
Author(s):  
Chantal Hausser ◽  
Georges Elie Ouaknine ◽  
Jacques Sylvestre
Keyword(s):  
Paget’S Disease ◽  
Paget's Disease ◽  
Neurological Dysfunction ◽  
Complete Relief ◽  
Communicating Hydrocephalus ◽  
Intellectual Function ◽  
History Of ◽  
Atrial Shunt ◽  
First Time ◽  
Shunting Procedure

SUMMARYA 76 year old patient with a long history of headaches was found to have Paget’s disease and communicating hydrocephalus. There were (otherwise) no neurological or musculo-skeletal manifestations of Paget’s disease, but moderate impairment of intellectual function was present. Treatment with disphosphonates did not bring any significant improvement, but three days following a ventriculo-atrial shunting procedure, the patient became headache-free for the first time in several years.In the literature, patients with hydrocephalus have been shown to respond quite unevenly to atrio-ventricular shunting, but in most instances the descriptions concerned advanced cases with well-established symptoms of dementia, ataxia and incontinence. Our case is reported to stress the importance of early diagnosis and management of hydrocephalus in Paget’s disease for the prevention of widespread neurological dysfunction.

scholarly journals Recent advances in understanding and managing Paget’s disease

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1485
Author(s):  
Ian R Reid
Keyword(s):  
Disease Progression ◽  
Paget’S Disease ◽  
Cost Effective ◽  
Paget's Disease ◽  
Symptomatic Disease ◽  
Joint Replacement Surgery ◽  
Replacement Surgery ◽  
Primary Endpoints ◽  
History Of ◽  
Biochemical Indices

Paget’s disease is a condition which continues to challenge and surprise. The dramatic fall in its incidence over the last three decades has been an enormous surprise, as is the capacity of a single infusion of the potent bisphosphonate, zoledronate, to produce biochemical remission in 90% of patients, remissions which usually persist for many years and raise the possibility of a cure in some patients. However, challenges in its management remain. The trials carried out in Paget’s disease have almost always had biochemical indices as their primary endpoints. From these studies, we also know that bone pain is relieved, quality of life improved, bone histology normalised, and radiological lesions healed. Thus, disease progression is halted. Studies have not been powered to assess whether clinically important endpoints such as fracture and the need for joint replacement surgery are diminished, although these complications are well established as part of the natural history of the condition. Since disease progression is prevented by potent bisphosphonates, it is likely that disease complications will also be prevented. Zoledronate also reduces the frequency of follow-up needed and therefore provides a very cost-effective intervention in those who have symptomatic disease or are at risk of complications.

scholarly journals MON-371 The Case of Dueling Femurs

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Richa Patel ◽  
Ana Ramirez Berlioz ◽  
Bhavana Chinnakotla ◽  
Lilamani Romayne Goonetilleke Kurukulasuriya
Keyword(s):  
Alkaline Phosphatase ◽  
Lumbar Spine ◽  
Bone Resorption ◽  
Bone Turnover ◽  
Bone Turnover Markers ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
X Ray ◽  
History Of ◽  
Turnover Markers

Abstract Introduction: Paget’s disease of the bone is characterized by excessive osteoclastic bone resorption followed by formation of disorganized bone; which is often focal. Bone pain and deformities are common features and it often leads to complications such as pathological fractures, deafness or neurologic deficits. Elevated bone turnover markers and alkaline phosphatase reflect ongoing exaggerated bone resorption and osteoblastic activity. We present an unusual scenario of post-menopausal osteoporosis and Paget’s disease occurring in the same patient. Clinical Case: 86-year-old female with history of Type 2 Diabetes Mellitus, Hypertension, Hypothyroidism, degenerative joint disease of lumbar spine with prior interbody fusion and laminectomy was referred to our clinic by Orthopedics for evaluation of newly diagnosed Paget’s disease. 2 months ago, she noticed severe right hip pain limiting daily activities. She denied any history of falls, fractures or family history of Paget’s. Physical exam was notable for tenderness to right sacro-iliac joint and right femoral trochanteric region. Work up included MRI of Lumbar spine and Pelvis, Pelvis X-ray, DEXA scan and routine blood work. Interestingly, her DEXA scan showed T score of +2.9 in Right hip and -3.1 in Left hip. On Pelvis X-ray cortical thickening, coarse trabecula and osteoarthritic changes were noted in right femur and hip, consistent with Paget’s disease. Left femur showed strikingly thinner cortices compared to the right, due to underlying osteoporosis. MRI of lumbar spine and pelvis was consistent with polyostotic Paget’s involving L3-L5, Sacrum and Right femur. Nuclear bone scan showed areas of uptake including anterior calvarium, lumbar spine, right hip, right femur, 8th rib, left mid tibia and 1st metatarsal of left foot. Since the distribution of uptake seemed atypical for Paget’s, a skeletal survey was obtained which was negative for bone lesions suggestive of malignancy. Laboratory testing revealed serum calcium 9.8mg/dL(8.4–10.2), 25-Hydroxy Vitamin D 30ng/dL(20–30), PTH 45.6pg/mL (15–65), Alkaline Phosphatase 370U/L (35–104), Procollagen I intact N-terminal 516mcg/L (16–96) and N-Terminal Telopeptide (NTX) 126.4 nM BCE (6.2–19). Patient received one dose of IV Zoledronic acid with modest improvement in hip and lower back pain. She continues to take Calcium carbonate 600mg twice daily and vitamin D3 1000IU once daily. We plan to see her in follow up in 3 months with repeat levels bone turnover markers. Conclusion: This is a unique case of Paget’s disease and osteoporosis, two very different diseases of metabolic bone disorder spectrum found in one patient. Treatment of Paget’s disease is indicated for pain reduction, prevention of fractures and deformities and to prevent disease progression in weight bearing areas. Bisphosphonates can target pathology of both diseases by reducing osteoclastic bone resorption.

Invasive cervical adenocarcinoma arising from extension of recurrent vulval Paget’s disease

2020 ◽  
Vol 13 (6) ◽  
pp. e232424
Author(s):  
Liesel Elisabeth Hardy ◽  
Lachlan Baxter ◽  
King Wan ◽  
Chloe Ayres
Keyword(s):  
Radical Hysterectomy ◽  
External Beam Radiotherapy ◽  
Paget’S Disease ◽  
Figo Stage ◽  
Paget's Disease ◽  
Cervical Adenocarcinoma ◽  
External Beam ◽  
Invasive Adenocarcinoma ◽  
Laparoscopic Radical Hysterectomy ◽  
History Of

An 83-year-old woman with a long-standing history of both invasive and vulval extramammary Paget’s disease (EMPD) was referred to a tertiary gynaecological oncology service for suspicion of contiguous extension to the vagina and cervix. Vaginal biopsies confirmed EMPD; however, a loop excision of the cervix demonstrated invasive adenocarcinoma arising from Paget’s disease. The patient subsequently underwent laparoscopic radical hysterectomy, bilateral salpingo-oophorectomy and radical upper vaginectomy, confirming FIGO stage 1B1 cervical adenocarcinoma. She was recommended but declined to have adjuvant pelvic external beam radiotherapy. To our knowledge this is the second case of invasive cervical adenocarcinoma arising from vulval Paget’s disease in the literature.

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