Paget's Disease
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Thomas Vestermark Thomsen ◽  
Ahmad Makki ◽  
Emir Hasanbegovic ◽  
Mikkel Børsen Rindom ◽  
Lars Bjørn Stolle

2021 ◽  
Vol 22 (18) ◽  
pp. 10158
Marco Paoletta ◽  
Antimo Moretti ◽  
Sara Liguori ◽  
Alessandra Di Paola ◽  
Chiara Tortora ◽  

The role of the endocannabinoid/endovanilloid (EC/EV) system in bone metabolism has recently received attention. Current literature evidences the modulation of osteoclasts and osteoblasts through the activation or inhibition of cannabinoid receptors in various pathological conditions with secondary involvement of bone tissue. However, this role is still unclear in primary bone diseases. Paget’s disease of the bone (PDB) could be considered a disease model for analyzing the role of the EC/EV system on osteoclasts (OCs), speculating the potential use of specific agents targeting this system for managing metabolic bone disorders. The aim of the study is to analyze OCs expression of EC/EV system in patients with PDB and to compare OCs activity between this population and healthy people. Finally, we investigate whether specific agents targeting EC/EV systems are able to modulate OCs activity in this metabolic bone disorder. We found a significant increase in cannabinoid receptor type 2 (CB2) protein expression in patients with PDB, compared to healthy controls. Moreover, we found a significant reduction in multi-nucleated tartrate-resistant acid phosphatase (TRAP)–positive OCs and resorption areas after treatment with JWH-133. CB2 could be a molecular target for reducing the activity of OCs in PDB, opening new therapeutic scenarios for the management of this condition.

2021 ◽  
pp. 1328-1332
Akihiro Kato ◽  
Hiroshi Kato ◽  
Satoshi Komori ◽  
Satsuki Nakano ◽  
Takayuki Murase ◽  

Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget’s disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget’s disease with a literature review.

2021 ◽  
pp. 1242-1247
Wataru Goto ◽  
Shinichiro Kashiwagi ◽  
Yuko Kawano ◽  
Asuka Komoda ◽  
Sae Ishihara ◽  

Mammary Paget’s disease is a rare malignancy. Mastectomy or breast-conserving surgery has been considered as the standard treatment, while there have been few reports of neoadjuvant chemotherapy (NAC). A 53-year-old woman with erythema and skin ulceration of the left breast was admitted to our institution. Breast examinations revealed left invasive ductal carcinoma (cT1bN0M0, cStage I), and a punch biopsy of the left mammary erythema indicated Paget’s disease (cTisN0M0, cStage0). The patient received NAC because of multiple lesions. Consequently, the breast tumor clinically disappeared, and the erythema improved. These outcomes made it easier to perform surgery (left mastectomy and sentinel node biopsy). Histopathological examination revealed no residual cancer cells in either the mammary gland or breast skin, and no metastasis was found in the sentinel lymph node. Therefore, NAC may be a useful treatment for mammary Paget’s disease.

2021 ◽  
Vol 28 (4) ◽  
pp. 2969-2986
Shoichiro Ishizuki ◽  
Yoshiyuki Nakamura

Extramammary Paget’s disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.

2021 ◽  
Vol 162 ◽  
pp. S192
Cheng-I Liao ◽  
Ashley Moon ◽  
Hsiu-Min Chen ◽  
Ravali Reddy ◽  
Amandeep Mann ◽  

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