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Hypercalcemia in PDB is rare; its occurrence requires thorough investigations as it may reveal several diseases, such as primary hyperparathyroidism, malignant transformation, metastases, or myeloma.

Paget's disease is characterized by disorganized bony tissue due to altered equilibrium of bone formation and resorption. Its malignant transformation is well known and is usually described to an osteosarcoma, often with bad prognosis. In this paper, we present a case clinic of an angiosarcoma from a Paget’s disease of a pelvic bone. The patient underwent a surgical procedure of tumor resection with wide margins (Enneking zone 2-3). The follow-up of 5 years in this case is unique in the literature.

ZusammenfassungDer klassische M. Paget der Mamille ist histologisch charakterisiert durch eine intraepidermale Tumorinfiltration, die von einem intraduktalen oder invasiven Mammakarzinom ausgeht, immunhistologisch durch eine regelhafte Überexpression von HER2 und klinisch durch ekzemartige Veränderungen von Mamille und Areola. Zu den Varianten mit davon abweichenden histologischen, immunhistologischen und klinischen Erscheinungsformen gehören der isolierte M. Paget der Mamille, der anaplastische M. Paget, der M. Paget mit Invasion und der pigmentierte M. Paget der Mamille. Differenzialdiagnostisch ist der M. Paget der Mamille abzugrenzen gegenüber benignen Veränderungen, insbesondere der Toker-Zell-Hyperplasie, dem Mamillenekzem und seltenen Dermatosen.

Teaching point: When the joints between bones are ankylosed, such as in ankylosing spondylitis, Paget’s disease can involve multiple contiguous bones leading to pagetic vertebral ankylosis.

Poorly controlled Paget's disease leads to excessive blood loss following total hip arthroplasty. The effect in shoulder arthroplasty is unknown. The authors reviewed 3 patients with Paget's disease involving the proximal humerus, comparing them with 17 patients with Paget's disease but no humeral involvement. The 3 patients had an estimated blood loss of 1400 mL, 1100 mL, and 350 mL, compared with an average of 280 mL in the control group. The first 2 cases required 4 units of packed red blood cells intraoperatively, and both were not managed with bisphosphonates. Paget's disease of the humerus leads to more intraoperative blood loss and higher blood transfusion requirements, particularly in cases not managed with bisphosphonates. [ Orthopedics . 2021;44(4):e614–e619.]

Paget's disease is a chronic benign bone disease characterized by excessive and abnormal bone remodeling. Monostotic Paget's disease accounts for only 20% of the cases, and the monostotic form involving the vertebra with the Mickey Mouse sign is very rare. Herein, we report a case of suspected bony metastasis in the second lumbar vertebra that was diagnosed as Paget's disease because of the Mickey Mouse sign on bone scintigraphy, and the diagnosis was confirmed by biopsy. Therefore, bone scintigraphy may provide a positive contribution to the diagnosis, and may help to avoid unnecessary biopsy in cases with specific signs and patterns.Paget, aşırı ve anormal kemik yapımı ve yıkımı ile karakterize kronik, iyi huylu bir kemik hastalığıdır. Monostotik Paget hastalığı, sadece olguların %20’sini oluşturmakla kalmaz; Mickey Mouse bulgusu olan, vertebra tutulumu ile giden monostotik formu daha da nadirdir. Sunduğumuz olguda, ikinci lumbar vertebrada metastaz şüphesi ile araştırılan hastada; kemik sintigrafisinde Mickey Mouse işareti görülmesi üzerine Paget hastalığı tanısı kondu ve tanı biyopsiyle doğrulandı. Bu olguda görülmektedir ki; kemik sintigrafisi tanıya olumlu katkıda bulunur ve belirli belirti ve paternler görülmesi sayesinde gereksiz biyopsileri önleyebilir.

Paget’s disease of bone is characterized by a chronic focal bone remodelling disorder, which is usually detected incidentally during routine imagining examinations. Although the measurement of total serum alkaline phosphatase usually provides a general indication of bone turnover in Paget’s disease, about 10-15% of patients present with alkaline phosphatase levels within the normal range. The treatment of symptomatic forms relies on bisphosphonates that are efficient in reducing the bone turnover. Here, I present the case of an 80-year-old man suffering from chronic low back pain, and diagnosed as Paget’s disease, with normal serum alkaline phosphatase and bone-specific alkaline phosphatase levels.

Tc-99m methylene diphosphonate bone scintigraphy (bone scan) is a highly sensitive technique for the evaluation of osseous pathology. However, the nonspecificity of the method can present diagnostic challenges in differentiating benign from malignant processes. Often, Paget's disease and osseous metastasis can coexist in elderly patients. Therefore, it is important to accurately distinguish the two pathologies, as each has a different prognosis and impacts clinical management. Obtaining the appropriate clinical diagnosis often involves a combination of laboratory, radiographic, and clinical data. We present a case of newly diagnosed prostatic carcinoma presenting with synchronous osseous metastasis, degenerative changes, and incidental multifocal Paget's disease.

Dermatoses of the nipple and areola are rare. The Paget's disease is a common dermatosis, which is presented in the form of a well-demarcated erythematous area, sometimes erosive, oozing or hyperkeratotic. Because the condition may be confused with benign diseases of the nipple, treatment is frequently delayed. The study aimed to highlight the incidence and make a clinical assessment of patients with Paget’s disease of the breast in the eastern part of Libya. The Medical records and histopathological reports of all patients attended the breast clinic in 7th October Hospital in the period from June 1990 till December 2008 were collected and reviewed retrospectively. Demography, clinical features, and biopsy results were noted. The results: The total number of patients who had breast cancer was 897, from which Paget’s disease was diagnosed in 19 patients (2.1%). Mean age at presentation was 57.3 years, and all of them were Women. Eczema of the nipple-areola complex and palpable mass occurred in (63.1% and 42.1%) respectively. The metastatic axillary lymphadenopathy at time of presentation were found in 36.8%. We conclude that Paget’s disease is serious but commonly misdiagnosed. A thorough history and physical examination are important for every patient who is presented with skin and/or nipple changes of the breast, and physicians should maintain a high index of suspicion for Paget’s disease of the breast.

Objectives: To study computed tomography findings in Paget's disease of temporal bone (PDTB) and analyze the relations between otic capsule bone mineral density values measured in Hounsfield Units (HU) and hearing loss (HL). Study Design: Observational case-control study. Setting: Tertiary referral center. Patients: Radiographically confirmed PDTB cases and control group. Intervention: Diagnostic. Main Outcome Measures: Hearing thresholds and computed tomography bone density values. Results: Twenty-three ears in the case group (PDTB) and 27 control ears were included. In the PDTB group, HL was found in 87% of the ears (43% mixed) and an air-conduction threshold of 50.7 dB (SD = 19.8). In the control group, 48% of the ears showed HL (7% mixed) and an air-conduction threshold of 34.5 dB (SD = 20.6) was found; the difference was statistically significant (p < 0.05). Measurements of bone density (HU) in the otic capsule (regions of interest [ROI] 1 and 2) and in the petrous bone (ROI 3) were significantly lower (p < 0.05) in the PDTB group than in controls. The PDTB group presented a significant association between otic capsule bone density in ROI 1 and mean otic capsule density with air and bone-conduction thresholds (p < 0.05). In controls, no association was observed between any density value and audiometric thresholds. Conclusion: PDTB patients showed more frequent HL, lower thresholds, and a higher proportion of mixed HL than controls. Bone density (HU) was decreased in all ROIs in PDTB patients in comparison with controls. Bone density in the otic capsule was associated with HL in PDTB patients, but no association was observed between bone density and HL in controls.

Paget’s disease of bone is very common in older people, secondary to an altered remodeling, deforming bone structure. X-rays are characteristic. Most cases are asymptomatic, but can attain pathological fractures and long term infrequent cases of secondary sarcomas.

Highlights • A rare case of Vulval Paget’s Disease (VPD) treated with primary Radiotherapy(RT) • Till date only 14 cases of VPD have been treated with primary RT in literature. • This case is probably the 15th case to be reported. • Invasive VPD in perimenopausal age group is very rare as was in this case. • Role of radiotherapy for treating invasive VPD is still not established in literature., 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.

“TWEETABLE” ABSTRACT: Extramammary Paget’s treatment with imiquimod and photodynamic therapy in combination can be easily tolerated and used safely and effectively.

Pamidronate [3-amino-1-hydroxypropylidene-1,1-bisphosphonate (APD)] was the first nitrogen-containing bisphosphonate (N-BP) investigated in clinical studies. In contrast to other clinically used bisphosphonates, pamidronate was discovered and its properties were initially studied in an Academic Institution. On the occasion of the 50th Anniversary of the first publications on the biological effects of bisphosphonates, I review in this article the contribution of Leiden investigators to the development of pamidronate that led to the recognition of the significance of the Nitrogen atom in the side chain of bisphosphonates for their action on bone resorption and to the formulation of principles for the use of N-BPs in the management of patients with different skeletal disorders.

Background: Primary Hyperparathyroidism (PHPT) and Paget’s Disease of the Bone (PDB) are two distinct bone mineral disorders which rarely co-exist. It has been very scarcely reported in the elderly but almost unheard of in young adults. Clinical case A middle-aged Indian lady presents with multiple and recurrent facial and jaw bony swellings, since the age of 12. She had normal developmental as a child, without visual, auditory deficits or limb deformities. No family history of hypercalcemia or endocrine disorders was elicited. At initial diagnosis, biochemical parameters were consistent with PHPT, with mild to moderate hypercalcemia (2.6-3.0 mmol) and concomitant hypophosphatemia (0.6-0.8 mmol/L), elevated intact PTH 10.6pmol/L and ALP persistently more than 350 U/L. She had undergone multiple surgeries to remove the bony swellings, presumed to be brown tumours. She developed medullary nephrocalcinosis approximately 30 years after onset of illness. Over a span of three decades, numerous modalities were employed in attempt to localise the parathyroid lesion, including imaging (ultrasonography, sestamibi with SPECT CT, FDG PET),angiography, venous sampling and exploratory neck surgeries which were unsuccessful. Due to failure in localising the offending parathyroid lesion, it was conceded to maintain her on medical therapy.She was initially started on oral phosphate therapy, intranasal calcitonin and subsequently calcimimetics. Aside from rising ALP levels, her other biochemical parameters remained static. Her diagnosis was re-evaluated when she presented with osteomyelitis of the right mandible requiring surgery. Pre -operative CT imaging showed mixed lytic and sclerotic lesions at the mandible, maxilla, facial bones and skull. It also revealed diffuse thickening of the calvarium with narrowing of the posterior fossa resulting in hydrocephalus, requiring a ventriculoperitoneal shunt insertion. Histopathological examination of the mandible showed a background of osteosclerosis. These findings were inconsistent with PHPT and was most often described in PDB. On the other hand, a repeated skeletal survey showed generalised osteopenia in the small bones of the hand with subperiosteal and terminal phalanges reabsorption which was in keeping with PHPT. Her pelvis, spine and long bones remained normal and bone densitometry was preserved. It was concluded that this patient was suffering from dual pathology, PHPT and PDB and was commenced on bisphosphonates. Conclusion: This lady presented with two diseases which simulate each other despite not being etiologically linked. The aberrancy in the age of onset and the overlapping clinical features of the dual pathology imposed a significant challenge in making the correct diagnosis. Retrospectively, histopathological examination of the bone at an earlier stage of disease may have been the key to solving this perplexing case.

Background: Inclusion body myopathy associated with Paget disease of bone (PDB) and/or frontotemporal dementia (IBMPFD) is a rare, autosomal dominant condition, characterized by adult-onset muscle weakness, early-onset PDB, and premature frontotemporal dementia. Paget’s disease is a chronic disorder of bone resulting in increased bone resorption, followed by a disorganized and excessive formation of bone. Clinical Case: A 43 year old gentleman was referred to neurology services with foot drop, limb weakness and cognitive impairment. Following a prolonged period of diagnostic evaluation a mutation in valosin-containing protein (VCP) gene was uncovered IBMPFD has a variable phenotype which may include PDB. This gentleman denied bony pain and had an alkaline phosphatase within reference range. Plain film radiographs at multiple sights demonstrated no signs of PDB. An MRI whole body was performed which reported coarse trabecular markings in L2 vertebral body and multilevel degenerative changes with bridging osteophytes in the lumbar spine consistent with PDB. At initial review in endocrine clinic, he denied fractures or bone pain. He had no signs of increased cardiac output or cranial nerve deficits. A radionuclide bone scan identified intense radiopharmaceutical accumulation in L2 vertebral body consistent with MRI findings and also curvilinear increased activity in the left occipital bone and low-grade activity in the left hip, thus confirming polyostotic Paget’s disease. He received a 5mg IV infusion of zolendronate, side effects of which included myalgia, pyrexia and lethargy. Six months following the zoledronate infusion a repeat bone scan demonstrated that the extent of uptake at affected sites had decreased significantly. He subsequently fractured his left femur following a mechanical fall. PDB can affect up to 50% of patients with IBMFTD. PDB can lead to complications such as bone pain, localized pain and deformity of the long bones, pathologic fractures and deafness. This case highlights the association of Paget’s disease with IBMFTD and as it can be asymptomatic, as in our case, radiological imaging is required for diagnosis. It also reminds us that Paget’s disease can be due to genetic causes. Understanding the role of VCP in the cell cycle may help in further understanding bone physiology.

Background The death rate of metastatic breast cancer in patients with mammary Paget's disease and underlying cancer is 61.3%, with a 10-year cumulative survival rate of 33%. The aim of this investigation was to develop a reliable nomogram to predict Female Paget’s Disease patient, thus helping clinical diagnosis and treatment. Methods Date of patients diagnosed with Paget’s Disease between 2004 to 2014 were downloaded from the Surveillance, Epidemiology, and End Results (SEER)database. Univariate and multivariate cox analysis were used to identify independent prognostic factors, which were used to construct nomograms to predict the probabilities of OS. The performance of the nomogram was assessed by C-indexes, receiver operating characteristic (ROC) curves and calibration curve. Decision curve analysis (DCA) was used to compare clinical usage between the nomogram and Adjusted AJCC staging system.Results Based on the univariate and multivariate cox analysis, features that correlated with survival outcomes were used to establish nomograms for OS prediction. The nomograms showed favorable sensitivity at predicting 1-,3-, and 5-year OS, with a C-index of 0.795(95% confidence interval (CI) 0.773-0.818) for OS. Calibration curves and ROC curves revealed excellent predictive accuracy. Finally, the 3- and 5-year DCA curves yielded larger net benefits than the Adjusted AJCC stage. Conclusions In conclusion, we have successfully established an effective nomogram to predict OS in Paget’s Disease patient, which can help clinicians in determining the appropriate therapy strategies for individual PD patients.

Paget’s disease of bone was first described by Sir James Paget as early as in the year 1877

Paget’s disease of bone is a localized disorder of bone remodeling. These functional and structural alterations, interacting with the specific characteristics of the site of involvement, account for most of the complications of the disease. Bilateral progressive hearing loss is the most frequently encountered complication of Paget’s disease, because of the involvement of the temporal bones. There may be difficulty in distinguishing patients with presbycusis from those with Paget’s disease-related hearing loss. Pagetic hearing loss seems to be stabilized by effective medical treatment. For patients with severe hearing loss, cochlear implantation may be recommended.

Male breast cancer accounts for less than 0.5%of all breast cancer, and Paget's disease of the breast in males is extremely rare. Here, we report 2 cases of Paget's disease of the nipple areola complex with invasive ductal carcinoma as typical examples of male PDB. Case 1 was a 64-year-old man with an altered appearance of the left nipple, itching and redness, without a palpable breast mass at first. Case 2 was a 55-year-old man with a palpable mass in the left breast and histologically confirmed Paget's disease of the nipple with invasive ductal carcinoma. Both patients underwent modified radical mastectomy but with different adjuvant therapies and remained well during follow-up with no recurrence. Furthermore, we reviewed all the sporadic cases of male PDB from the literature. This may help contribute to the development of diagnostic strategies and appropriate interventions for male Paget's disease of the breast. Key words: Paget's disease; male; clinical manifestations; treatment; prognosis

Context.— Paget disease of the breast, in most cases, represents intraepidermal spread of ductal carcinoma in situ. Molecular subtypes of invasive carcinoma of the breast have prognostic and therapeutic significance and show characteristic distribution. Little is known about the distribution of molecular subtypes in Paget disease of the breast. Objectives.— To examine the distribution of molecular subtypes in Paget disease of the breast and to compare them to concurrent invasive carcinoma of the breast, if present. Design.— We examined 48 cases of Paget disease of the breast with immunohistochemistry and antibodies against estrogen and progesterone receptors, human epidermal growth factor receptor 2 (HER2), and Ki-67, as well as HER2 chromogenic in situ hybridization, to classify the cases into molecular subtypes. Then, we compared the results to the molecular subtypes of associated invasive carcinoma of the breast, if present. Results.— The HER2 subtype was the most common found in Paget disease of the breast, followed by the luminal B subtype and 2 cases of the triple-negative subtype. The associated invasive carcinoma cases were most often of the luminal B subtype, followed by the HER2 subtype and the triple-negative subtype. The molecular subtype of Paget disease and invasive carcinoma was congruent in most of the cases. Conclusions.— Molecular subtypes of invasive carcinoma of the breast can already be detected in Paget disease. The distribution of molecular subtypes of Paget disease and of Paget disease–associated invasive carcinoma differs from invasive carcinoma without associated Paget disease, with the HER2 subtype overrepresented in Paget disease and associated invasive carcinoma and the luminal and triple-negative subtypes underrepresented.

Introduction. Paget’s disease of the bone is a skeleton condition affecting bone remodelling of unknown cause. Bone formation and resorption, as parts of bone turnover which are normally coupled in healthy skeleton, in this condition are disconnected. We aim to introduce an adult male case with the condition which is also diagnosed with a multinodular goitre. This is a case report. This is a 60-year old male who is a former smoker and recently accused intermittent mild loss of hearing at left ear. Whole body bone scintigram (20mCi, Tc99m) showed increased uptake of the radiotracer with high bone affinity at parietal and occipital parts of the skull. Accidental multinodular goitre is diagnosed with a large cystic macronodule. Fine needle aspiration showed BETHESDA 2 with modest risk of haemorrhage and indication of surgery. 6 months after a dose of 5 mg zolendronic acid the bone markers were not increased and subsequent follow-up is necessary. Conclusion. Early diagnosis of Paget’s bone disease improves the potential risk of complications; however, distant potential complications need lifelong periodic check-up. The synchronous diagnosis of multinodular goitre with dominant cystic macronodule seems accidental in this male case.

Paget's disease of the breast is a rare type of cancer of the nipple–areola complex and that is often associated with an underlying in situ or invasive carcinoma. Diagnosis and treatment of Paget's disease is controversial. Expert oncologists discuss on the update on the approaches of Paget's disease diagnosis and its treatment options. This expert group used data from published literature, practical experience and opinion of a large group of academic oncologists to arrive at this practical consensus recommendations for the benefit of community oncologists.

Paget's disease of bone (PDB) is a focal remodeling disorder of unknown origin. Head bones are frequently involved and patients may suffer from deformities and specific symptoms, mainly headache, or neurological deficits, such as deafness. Several pathophysiological entities can give rise to headache in PDB patients. Clinical, laboratory, radiological and scintigraphic findings contribute to distinguish headache related to PDB from other causes of headache. Up to the present, treatment with intravenous zolendronic acid has been reported to improve symptoms of PDB and induce long-standing biochemical and scintigraphic remission. In this review, the main strands of literature about epidemiology, pathophysiology, clinical characteristics and treatment of PDB-related headache are drawn together.

Paget’s disease of the breast is an eczematous skin disease that is usually associated with an underlying breast cancer. Male breast cancer accounts for only 1-3% of all breast malignancies. Paget’s disease in men is a very rare condition of the male breast. There have been only nine reported cases of Paget’s disease of the male breast between 1980 and 2015. We described a breast invasive carcinoma of a 72-year-old man who was previously diagnosed with Paget’s disease.

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Made available in DSpace on 2019-10-06T15:31:40Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-01-01. Added 1 bitstream(s) on 2021-07-15T15:05:10Z : No. of bitstreams: 1 S1413-78522019000100031.pdf: 146919 bytes, checksum: 023880c08e4c8a62bfbf93112a1a04c9 (MD5) A doença de Paget óssea é uma afecção crônica, caracterizada por distúrbio focal da absorção e formação ósseas, podendo levar a deformidades anatômicas, dor, fraturas e malignização das lesões. é frequente no Reino Unido, Austrália, Nova Zelândia e América do Norte. Mantém forte relação de incidência com parentes de primeiro a terceiro graus. Sua etiologia ainda permanece incerta e o tratamento se baseia no controle da doença, com uso de bisfosfonados, visando melhora dos sintomas e das alterações laboratoriais. O tratamento cirúrgico também pode ser necessário, para correção de deformidades ou para tratamento de fraturas. Este estudo compilou oito pacientes em acompanhamento pela doença de Paget óssea, no Ambulatório de Ortopedia deste hospital, acerca do tratamento realizado e como evoluíram. Dentre os pacientes acompanhados, houve um caso de carcinoma de próstata concomitante ao Paget, chamando atenção para outras patologias ósseas que são diagnósticos diferenciais ou coexistem com a doença. Nível de evidência IV, Tipo de Estudo: Série de Casos. Paget's disease of bone is a chronic condition characterized by focal abnormalities of absorption and formation of bone, and it may lead to anatomical deformities, pain, fractures, and malignant transformation. It is common in the UK, Australia, New Zealand, and North America and has a strong hereditary component, affecting first- to third-degree relatives. The etiology remains unclear and treatment is based on control of the disease with bisphosphonates, with the aim of relieving symptoms and correcting laboratory abnormalities. Surgical treatment may also be necessary to correct deformities or treat pathological fractures. This study evaluated the management and course of 8 patients with Paget's disease of bone, followed in the Orthopedic Clinic of this hospital. Among these patients, 1 had concomitant advanced prostate carcinoma, highlighting the association between Paget's disease and secondary bone diseases that can affect the differential diagnosis. Level of evidence IV, Study type: Case Series. Universidade Estadual de São Paulo Júlio de Mesquita Filho (UNESP) Faculdade de Medicina de Botucatu Universidade Estadual de São Paulo Júlio de Mesquita Filho (UNESP) Faculdade de Medicina de Botucatu

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