Hemangiosarcoma complicating Paget's disease of the bone

An 87-year-old man with Paget's disease of the left humerus was found to have a Paget's sarcoma upon pathologic examination of the left upper limb, which was disarticulated for severe pain and recurrent fractures. The histologic type of this tumor, a hemangiosarcoma, was unique among Paget's sarcomas.     

Intraosseous Lipomata in Paget's Disease: An unusual CT appearance

A case of multiple intraosseous lumbar vertebral lipomata is reported occurring in the prescence of Paget's disease. The pathology and radiology are correlated.     

Bilateral Metachronous Paget's Disease of the Accessory Breasts in a Male

Bilateral axillary Paget''s disease in men is a rare occurrence with limited reports on its diagnosis, treatment, and prognosis. Here, we report the case of a 55-year-old Korean male, who presented with a palpable mass and eczematous skin lesion on the left axilla. An incisional biopsy and histopathologic examination indicated invasive ductal carcinoma with Paget''s disease arising in the accessory breast. Magnetic resonance imaging and positron emission tomography revealed no malignancy in the normal breast and other organs. The patient was subjected to a wide excision, wherein the left axillary lymph node was dissected, followed by the administration of adjuvant chemotherapy and radiation therapy. After 17 months of disease-free survival, the patient was diagnosed with Paget''s disease of the contralateral accessory breast. He underwent wide excision surgery along with radiation therapy. To the best of our knowledge, this is the first report of bilateral extramammary Paget''s disease in a male.     

Paget's disease of the breast.

Twenty-nine histologically verified cases of Paget's disease of the breast treated at the Hadassah University Hospital in the years 1949-1972 were followed up and analyzed. Dividing this material into two groups according to the presence or absence of a palpable breast tumor revealed significant difference in behavior and survival. Patients with a breast mass (34%) had a 50% axillary lymph node involvement and behaved as with any other ordinary breast cancer, with a 5-year survival rate of 40% and a 10-year survival rate of 33%. Patients with no palpable breast mass (66%) had only a 10.5% lymph node involvement, the 5-year survival rate being 94% and the 10-year survival rate being 91%. Delay in diagnosis seems to play no significant factor in survival rates and outcome. We believe radical mastectomy to be the treatment of choice in all cases of Paget's disease of the breast.     

Paget's disease of the male breast: a clinicopathologic study and a collective review

Two cases of Paget's disease of the male breast were detected in a retrospective histopathological review of 27 cases of male breast cancer treated during a period of 13 years from 1966 through 1978. The clinico-pathological data on these two rare cases have been compared with the 23 histologically documented cases of Paget's disease of the male breast from the literature.     

Paget's disease of the anal mucosa in association with carcinoma demonstrating mucoepidermoid features

A case of Paget's disease of the anal mucosa with an underlying carcinoma demonstrating mucoepidermoid features is reported. The histopathologic characteristics of both extramammary Paget's disease and mucoepidermoid carcinoma are noted. Special histochemical staining procedures were consistent with the diagnosis. The actual histogenesis of extramammary Paget's disease and mucoepidermoid carcinomas are controversial topics that are briefly discussed in this paper.     

Paget's disease of the breast presenting as a cutaneous horn

A 67-year-old woman developed a cutaneous horn on the nipple of her right breast. Biopsy of the skin underlying the horn disclosed Paget's disease of the breast. An intraductal adenocarcinoma of the same breast was found on mastectomy. High index of suspicion is mandatory in evaluating all nipple lesions.     

Paget's disease of the breast: What the radiologist may expect to find

Paget's disease of the nipple is characterized by the presence of Paget's cells in the epidermis of the nipple or areola. Two case reports of Paget's disease are described and used to highlight unusual features of the disease. The literature on the radiographic and pathologic findings of this disease is reviewed.     

Effect of Paget's disease of bone (osteitis deformans) on the progression of prostate cancer bone metastasis

Background:Patients with prostate cancer tend to die from bone metastases. Until now, no evidence has shown that Paget's disease of bone (PDB) affects the progression of bone metastasis or overall survival of patients with prostate cancer.Methods:We searched our patient database for men who had presented with prostate cancer and PDB between June 1993 and March 2009, and identified best-matched control patients according to stage, grade, age, date of diagnosis, treatment, and race.Results:Among 1346 consecutive patients with prostate cancer diagnosed before 2008, 15 were confirmed to have comorbid PDB. Twenty-six more were identified from the institutional billing search. Including the 41 best-matched controls, our total study population was 82 patients. In the Kaplan-Meier analysis, we estimated median times from diagnosis of prostate cancer to bone metastasis to be 21.5 years for those with PDB and 9.4 years for those without PDB (P=0.044). Median overall survival times were 11.8 and 9.2 years for the two groups, respectively (P=0.008).Conclusion:For the first time, we have obtained evidence that patients with prostate cancer and PDB have delayed time to bone metastases and improved overall survival than do patients with prostate cancer alone.     

Metastatic extramammary Paget's disease: dramatic response to combined modality treatment

A case of extramammary Paget's disease is reported. The distinguishing features of the case are: (1) localization of the primary lesion to the scrotum, (2) development of metastases in the absence of a recognizable underlying carcinoma, and (3) response of the systemic disease to a combination of radiation therapy and chemotherapy. The histogenesis, clinical behavior, and treatment of extramammary Paget's disease are reviewed.     

Oncogenic mutations in extramammary Paget's disease and their clinical relevance

Extramammary Paget's disease (EMPD) is a rare cutaneous malignant neoplasm. The genetic alterations underlying its pathogenesis have less been described. Therefore, we analyzed the possible mutations in the KRAS, HRAS, NRAS, BRAF, ARAF, RAF1, PIK3CA, AKT1, CTNNB1 and APC genes as well as methylation and expression of CDH1 in 144 EMPD cases and 42 matched normal skin tissues. A distinct mutation profile was identified in EMPDs with 27 (19%) cases mutant for RAS and RAF genes and 50 (35%) cases harboring oncogenic mutations in PIK3CA and AKT1. Moreover, a mutually exclusive pattern was observed in the genetic variants in these two signaling pathways. No mutation was detected in CTNNB1 and APC genes. High prevalence of low expression and hypermethylation of CDH1 gene was detected in 33 and 48% of the EMPD cases, respectively. Furthermore, PIK3CA and AKT1 mutations were significantly correlated with CDH1 hypermethylation which could explain why the majority of EMPD cases with mutant PIK3CA and AKT1 were invasive. Our study demonstrates that genetic variants associated with constitutive activation of RAS/RAF and PI3K/AKT pathways are involved in the pathogenesis of EMPD. This may represent novel therapeutic targets for this skin cancer.     

Atypical Case of Pott's Disease

An unusual case of Pott's Disease in an adult, Australian born, Caucasian male is reported. The typical and atypical radiological features of spinal tuberculosis are reviewed.     

Paget's disease of the bone in a Chinese woman

Paget's disease, otherwise known as osteitis deformans, is an unusual condition in the Oriental population. We report a case of Paget's disease in a Chinese woman, incidentally diagnosed on a bone scan. This was confirmed by clinical history, biochemistry and imaging findings using other modalities. Although bone scans are commonly performed to diagnose traumatic occult fractures and bone metastases, they can also be used to diagnose metabolic bone diseases, such as osteoporotic fractures, and to evaluate Paget's disease. It can also diagnose acute fractures secondary to renal osteodystrophy and osteomalacia.     

18例胸内巨大肿瘤的外科治疗分析

目的：探讨胸内巨大肿瘤的外科治疗经验。方法：回顾分析18例胸内巨大肿瘤患者的临床资料。男10例，女8例。左胸9例，右胸9例。肿瘤重2000－3800g。结果：无院内死亡。复张性肺水肿及胸腔内出血各1例。结论：麻醉后气管、大血管受压可引起窒息、休克危险。手术切口要充分显露，术中可分块切除或控制瘤蒂后整个切除，注意术中大出血，防止发生复张性肺水肿。     

Docetaxel Combined With Cisplatin for Metastatic Extramammary Paget Disease

Introduction

Metastatic extramammary Paget disease (EMPD) as a rare intraepithelial carcinoma is fatal. However, no standardized chemotherapy has been established. We provided docetaxel combined with cisplatin to EMPD patients.

累及胸壁的胸部肿瘤的外科治疗——胸壁切除与重建(附12例报告)

背景与目的胸部肿瘤累及胸壁是临床常见事件,若无远处转移,完整切除受累胸壁仍可获得良好疗效。本文结合12例肿瘤患者胸壁切除与重建(chest wall resection and reconstruction,CWRR)的经验就重建人工材料、软组织覆盖等方面作一介绍,并强调切除外科与重建外科合作的重要性。方法总结2005年10月-2011年4月北京大学肿瘤医院胸外一科和重建外科共同参与的CWRR 12例,详细复习自确诊至今的诊治全过程,包括术前治疗、手术方式、切除范围、重建方式,主要的局部及全身并发症及生存情况。结果 12例均为根治性手术,均行骨性胸壁切除,切除后骨性胸壁缺损为25 cm~2-700 cm~2,胸壁软组织缺损为56 cm~2-400 cm~2。骨性胸壁修补材料采用聚丙烯单丝网片(polypropylene mesh),软组织修复采用转移肌瓣、转移肌皮瓣及大网膜瓣。术后1例发生呼吸衰竭,呼吸机辅助通气1个月后痊愈,余例均无并发症,全组12例至今全部存活。结论只有切除外科和重建外科同时参与才能完成符合肿瘤原则的复杂CWRR。由切除外科主导、重建外科协助、了解并熟悉重建材料及胸壁软组织重建,是达到手术根治性及保证远期生存的关键。     

A Primer on Interstitial Lung Disease and Thoracic Radiation

Interstitial lung disease (ILD) is a term used to describe a heterogeneous group of lung disorders with characteristic clinical and imaging features. Patients with ILD are at an increased risk of developing NSCLC, which is frequently medically comorbid, often precluding operative management. In this scenario, radiotherapy (RT) is generally recommended; however, ILD is known to increase the risk of RT-related toxicity. Recommendations for treatment with appropriately individualized risks and benefits are thus dependent on integration of patient-, ILD-, and cancer-specific factors. We aim to provide an overview of ILD for the thoracic oncologist, an assessment of risk of thoracic RT in patients with ILD, and evidence-based recommendations for treatment in a variety of clinical scenarios.