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.

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.

Comparison of Laparoscopic Versus Abdominal Radical Hysterectomy for FIGO Stage IB and IIA Cervical Cancer With Tumor Diameter of 3 cm or Greater

2014 ◽  
Vol 24 (2) ◽  
pp. 280-288 ◽  
Author(s):  
Tae Wook Kong ◽  
Suk-Joon Chang ◽  
Jisun Lee ◽  
Jiheum Paek ◽  
Hee-Sug Ryu
Keyword(s):  
Cervical Cancer ◽  
Radical Hysterectomy ◽  
Operating Time ◽  
Figo Stage ◽  
Tumor Diameter ◽  
Pelvic Magnetic Resonance Imaging ◽  
Laparoscopic Radical Hysterectomy ◽  
Stage Ib ◽  
Estimated Blood Loss ◽  
The Mean

ObjectiveThere have been many comparative reports on laparoscopic radical hysterectomy (LRH) versus abdominal radical hysterectomy (ARH) for early-stage cervical cancer. However, most of these studies included patients with International Federation of Gynecology and Obstetrics (FIGO) stage IA2 and small (tumor diameter ≤2 or 3 cm) IB1 disease. The purpose of this study was to compare the feasibility, morbidity, and recurrence rate of LRH and ARH for FIGO stage IB and IIA cervical cancer with tumor diameter of 3 cm or greater.Materials and MethodsWe conducted a retrospective analysis of 88 patients with FIGO stage IB and IIA cervical cancer with tumor diameter of 3 cm or greater. All patients had no evidence of parametrial invasion and lymph node metastasis in preoperative gynecologic examination, pelvic magnetic resonance imaging, and positron emission tomography–computed tomography, and they all underwent LRH or ARH between February 2006 and March 2013.ResultsAmong 88 patients, 40 patients received LRH whereas 48 underwent ARH. The mean estimated blood loss was 588.0 mL for the ARH group compared with 449.1 mL for the LRH group (P< 0.001). The mean operating time was similar in both groups (246.0 minutes in the ARH vs 254.5 minutes in the LRH group,P= 0.589). Return of bowel motility was observed earlier after LRH (1.8 vs 2.2 days,P= 0.042). The mean hospital stay was significantly shorter for the LRH group (14.8 vs 18.0 days,P= 0.044). There were no differences in histopathologic characteristics between the 2 groups. The mean tumor diameter was 44.4 mm in the LRH and 45.3 mm in the ARH group. Disease-free survival rates were 97.9% in the ARH and 97.5% in the LRH group (P= 0.818).ConclusionsLaparoscopic radical hysterectomy might be a feasible therapeutic procedure for the management of FIGO stage IB and IIA cervical cancer with tumor diameter of 3 cm or greater. Further randomized studies that could support this approach are necessary to evaluate long-term clinical outcome.

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 Recurrence of cancer cervix in patients treated by radical hysterectomy followed by adjuvant external beam radiotherapy

1970 ◽  
Vol 36 (2) ◽  
pp. 52-56 ◽  
Author(s):  
Fauzia Sobhan ◽  
Farzana Sobhan ◽  
Arif Sobhan
Keyword(s):  
Squamous Cell Carcinoma ◽  
Local Recurrence ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Radical Hysterectomy ◽  
External Beam Radiotherapy ◽  
Late Recurrence ◽  
Distant Recurrence ◽  
External Beam ◽  
Distant Failure

One hundred and twenty patients with FIGO stage Ib-IIa cervical cancer who had radical hysterectomy prior to January 2003 in different hospitals of Bangladesh and thereafter received external beam pelvic radiotherapy (RT) at National Institute of Cancer Research and Hospital, Dhaka were studied. Until December 2007, 50 (42%) patients developed recurrence. Thirty-four (70%) patients experienced local recurrence, 13 (26%) distant recurrence and 2 (4%) both local and distant recurrence. Of 15 patients with distant metastasis, 6 (40%) experienced it in extra-pelvic lymph nodes. The median time to recurrence was 19 months (range 6-120 months) for local failure, 33 months (range 12-108 months) for distant failure and 25 months (range 13-36 months) for those with both local and distant failure. Eighty percent recurrences occurred within 36 months. There was significant correlation between lymph node metastasis and recurrence. All 5 (100%) patients with adenocarcinoma who had positive nodes experienced local recurrence compared with 18 of 49 (37%) squamous cell carcinoma patients with positive nodes. Recurrence more than 5 years after treatment developed in 5 (4%) patients. Mentionable, 4 (80%) out of 5 patients with late recurrence had squamous cell carcinoma with negative pelvic nodes. DOI: 10.3329/bmrcb.v36i2.6987Bangladesh Med Res Counc Bull 2010; 36: 52-56

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.

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