Patients who have Paget\'s Disease more frequently require total hip arthroplasty (THA) and total knee arthroplasty (TKA) than matched controls. However, controversy remains regarding their outcome. We aimed to evaluate the literature regarding outcomes following THA and TKA in patients who have Paget\'s Disease.
MEDLINE, EMBASE and Cochrane databases were searched for all articles evaluating outcomes following THA and TKA in patients who have Paget\'s Disease. Quality of included studies was assessed using the Newcastle-Ottawa Scale.
A total of 19 articles (published between 1976 and 2022) were included, comprising 58,695 patients (48,766 controls and 10,018 patients who have Pagets Disease), from 209 potentially relevant titles. Patients with Paget\'s Disease have a pooled mortality of 32.5% at a mean of 7.8 years (range, 0.1 to 20) following THA and 31.0% at a mean of 8.5 years (range, 2 to 20) following TKA, with a pooled revision rate of 4.4% at 7.2 years (range, 0 to 20) following THA and 2.2% at 7.4 years (range, 2 to 20) following TKA. Renal and respiratory complications, as well as heterotopic ossification and surgical-site infection were the most common post-operative complications.
There is marked heterogeneity in outcome reporting of studies assessing arthroplasty in patients who have Paget\'s Disease, with studies of low to moderate quality. Patients with Paget\'s Disease undergoing THA and TKA appear to have similar implant longevity as their unaffected counterparts. However, they appear to have an increased risk of medical and surgical complications and may have a higher mortality risk from their procedure.

Extramammary Vulval Paget\'s Disease (VPD) is rare neoplasm of post-menopausal women. In relatively young perimenopausal patients, it can cause both diagnostic dilemma and therapeutic challenges. Majority of VPD is of non-invasive variety. Among invasive Paget\'s disease, only 20% cases show invasion more than 1 mm. The present report describes a unique case of an invasive extra-mammary VPD with depth of invasion more than 4 mm presenting at a relatively young perimenopausal lady. Only 14 cases of VPD has been treated with primary radiotherapy in literature till date. We report this case to be the 15th case where radiotherapy was solely used to treat an invasive VPD.

Paget\'s disease is a monostotic or polyostotic progressive skeletal disease with a genetic predisposition. The affected bone areas show osseous swelling and often grotesque deformation, chronic pain and fractures. Many cases are asymptomatic for a long time resulting in a late diagnosis. The pathogenesis is still unknown. In addition to a genetic predisposition, viral factors are also discussed. Laboratory tests and imaging are used for diagnosis. The effective principle of medicinal bisphosphonate treatment is to inhibit osteoclastic bone resorption and should be initiated early to prevent secondary complications. This article presents the current knowledge about this rare osteological disease.
UNASSIGNED: Beim Morbus Paget liegt eine mon- oder polyostotische, progrediente Skeletterkrankung mit genetischer Prädisposition vor. In den betroffenen Knochenarealen kommt es zur ossären Auftreibung und oft grotesken Verformung, zu chronischen Schmerzen und Frakturen. Viele Fälle sind lange Zeit asymptomatisch bis oligosymptomatisch, sodass sie erst spät diagnostiziert werden. Die Pathogenese ist weiterhin ungeklärt, neben einer genetischen Prädisposition werden auch virale Faktoren diskutiert. Zur Diagnosestellung dienen Labor und Bildgebung. Das wirksame Prinzip der medikamentösen Bisphosphonat-Therapie liegt in der Hemmung der osteoklastären Knochenresorption und sollte frühzeitig erfolgen, um Sekundärkomplikationen zu verhindern. Der vorliegende Artikel präsentiert die aktuellen Erkenntnisse zu dieser seltenen osteologischen Erkrankung.

OBJECTIVE: To determine the functional outcomes, complications and revision rates following total knee arthroplasty (TKA) in patients with Paget\'s disease of bone (PDB).
METHODS: A systematic review of the literature was performed. Four studies with a total of 54 TKAs were included for analysis. Functional outcomes, pain scores, complications and revision rates were assessed. The mean age was 72.0 years and the mean follow-up was 7.5 years.
RESULTS: All studies reported significant improvement in knee function and pain scores following TKA. There were 2 cases of aseptic loosening, with one patient requiring revision of the femoral component 10 years after the index procedure. Malalignment, bone loss, soft tissue contractures were the most commonly reported intra-operative challenges. There were five cases (9%) that were complicated by intra-operative patellar tendon avulsion.
CONCLUSIONS: The findings support the use of TKA in patients with PDB. The post-operative functional outcomes are largely similar to other patients, however there are specific perioperative challenges that have been highlighted, in particular the high risk for patellar tendon avulsion.

OBJECTIVE: To investigate the clinical and functional outcomes following total hip arthroplasty (THA) in patients with Paget\'s disease.
METHODS: We carried out a systematic review of the literature to determine the functional outcome, complications and revision rates of THA in patients with Paget\'s disease. Eight studies involving 358 hips were reviewed. The mean age was 70.4 years and follow-up was 8.3 years. There were 247 cemented THAs (69%), 105 uncemented THAs (29%) and 6 hybrid THAs (2%).
RESULTS: All studies reported significant improvement in hip function following THA. There were 19 cases of aseptic loosening (5%) at a mean of 8.6 years. Three cases occurred in the uncemented cohort (3%) at a mean of 15.3 years and 16 cases developed in the cemented group (6%) at a mean of 7.5 years (P = 0.2052). There were 27 revisions in the 358 cases (8%) occurring at a mean of 7 years. Six revisions occurred in the uncemented cohort (6%) at a mean of 8.6 years and 21 in the cemented cohort (9%) at a mean of 6.5 years (P = 0.5117).
CONCLUSIONS: The findings support the use of THA in patients with Paget\'s disease hip arthropathy. The post-operative functional outcome is largely similar to other patients; however, the revision rate is higher with aseptic loosening being the most common reason for revision. Uncemented implants appear to be associated with a lower failure rate, however, there were no modern stem designs fixed using current generation cementing techniques used in the reported studies, and as such, caution is advised when drawing any conclusions.

BACKGROUND: Paget\'s disease of bone (PDB) is a disease of elderly characterized by disorganized bone remodeling. Development of secondary neoplasm in PDB is a known but rare phenomenon. Development of giant cell tumor in PDB (GCT-PDB) is extremely rare, and little is known about its etiopathogenesis and management. We present a case report of such a development with a review of the literature and the role of various new modalities of treatment available in the management of this rare condition.
METHODS: A 40-year-old gentleman presented with back pain and on evaluation was diagnosed as a case of polyostotic PDB. He was treated with intravenous bisphosphonates, calcium, and vitamin D supplements. After an asymptomatic period of 3-year, he presented with a gluteal mass involving ilium and sacrum which was confirmed as GCT on biopsy. Serial angioembolization was attempted but mass progressed, so surgery performed with excision and curettage of the lesion. He presented with a local recurrence 2 years later with a large soft tissue component. He was started on denosumab, RANKL inhibitor, with the aim to downstage the lesion. The patient showed a good response after 6 doses with reduction in soft tissue mass followed by which he underwent surgery with partial T-1 internal hemipelvectomy and curettage of sacrum. Currently, the patient is asymptomatic at a follow-up of 15 months.
CONCLUSIONS: GCT-PDB is a rare phenomenon occurring mainly in polyostotic PDB and is associated with more severe manifestations of the disease. The management is challenging and requires multimodality management. Pharmacological agents include use of bisphosphonates and RANK ligand inhibitor - denosumab. Although surgery is the mainstay of treatment for GCT, other modalities of treatment such as RANK ligand inhibitors (denosumab), selective arterial embolization, or radiation therapy has to be used for inoperable cases or where surgery would be functionally too morbid, especially in cases of GCT-PDB where the disease affects more commonly the axial skeleton.

Paget\'s disease is a condition involving focal overactivity of bone cells (osteoblasts and osteoclasts), which can result in significant skeletal morbidity. It is unclear in which bone cells the causative lesion resides. It is managed effectively with potent bisphosphonates, but treatment is difficult if these drugs are contraindicated. We describe a 75-year-old woman with Paget\'s disease involving the skull who was intolerant of bisphosphonates, so was treated with denosumab. This intervention normalized serum alkaline phosphatase for 4-8 months after each injection and led to some symptomatic improvement. Scintigraphic activity in the lesion was improved but not normalized. We conclude that reduction in RANKL activity by denosumab only partially corrects pagetic activity, indicating that the osteoclast overactivity of Paget\'s disease is not wholly mediated by RANKL. Denosumab has some clinical utility in Paget\'s disease and may become a second-line agent in those with contraindications to intravenous bisphosphonates.

Synchronous bilateral primary breast malignant phyllodes tumor or/and carcinoma of the breast with Paget\'s disease is rare. In the article, we present a case of bilateral carcinoma of the breast with Paget\'s disease of the right breast and malignant phyllodes tumor of the left breast. A 44-years-old Chinese woman presented with a 1 month history of the right breast nipple with eczema and fester and growing and palpable mass of left breast. Molybdenum target X-ray revealed microcalcification in the right breast, which was highly suspected of malignant tumor, and round-like mass with smooth surface and homogeneous density in the left breast. Color ultrasound showed a lobulated lump which circumferential blood flows around in the left breast, and which did not show any mass in the right breast. The patient was undertaken the bilateral modified radical mastectomy. The histological diagnosis was Paget\'s disease associated with infiltrating ductal carcinoma in the right breast and malignant phyllodes tumor the left breast. The patient also received 6 cycles of the postoperative adjuvant chemotherapy by using T.T. regimen comprised docetaxel (100 mg) and pirarubicin (60 mg).

Paget\'s disease of bone (PDB) is a disease characterized by a disorder in the bone metabolism. The spine is the second region affected after the pelvis. Surgical treatment is reserved for cases refractory to medical treatment. We performed a systematic review of patients with Paget disease of bone affecting the spine, treated surgically in the last 30 years. The main objective of the review is to find out indications for surgery, outcomes of these patients and also the standard perioperative management.