LOCALLY ADVANCED BLADDER EWINGS SARCOMA: AN EXCEPTIONAL CASE REPORT

Extra skeletal Ewings sarcoma is a rare entity. The primary bladder localization is exceptional, their diagnosis is difficult because it is extremely rare in this place and because of diagnostic difficulties posed by a group of very heterogeneous tumors. Its clinical presentation is similar to that of transitional cell carcinoma, however the two entities must be distinguished because the protocols for treatment are different. The diagnosis is dependent on the pathological examination with the immunohistochemical and cytogenetic study. We report the case of a 36-year-old man with locally advanced Ewing sarcoma of the bladder.

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Transitional Cell Carcinoma of the Kidney Graft: An Extremely Uncommon Presentation of Tumor in Renal Transplant Recipients

Case Reports in Transplantation ◽

10.1155/2013/196528 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Vital Hevia ◽

Victoria Gómez ◽

Sara Álvarez ◽

Víctor Díez Nicolás ◽

Carmen Gómez del Cañizo ◽

...

Keyword(s):

Transitional Cell Carcinoma ◽

Cell Carcinoma ◽

Clinical Presentation ◽

Locally Advanced ◽

Preoperative Staging ◽

Transitional Cell ◽

Pathological Examination ◽

Renal Transplant Recipients ◽

Kidney Graft ◽

High Grade

Purpose. Transitional cell carcinoma (TCC) affecting the graft after renal transplantation is a very infrequent way of presentation of this tumor. Our aim is to present our single institution experience with 2 cases, as well as to perform a review of the literature about this tumor after the transplant.Materials and Methods. TCC of the graft developed in 2 of 1365 patients from 1977 to 2010, both cases in women. Data were analyzed for incidence, clinical presentation, treatment, and outcomes.Results. Both cases occurred in 2 mid-age women and resulted to be high grade and locally advanced TCCs, representing an incidence of 0,14% (2/1365). Clinical presentation was urinary obstruction for the first case and incidental ultrasound finding for the second. Preoperative staging was made with CT, cytology, pyelography, ureterorenoscopy, and biopsy. Treatment performed was nephroureterectomy of the graft with bladder cuff and regional lymphadenectomy. Pathological examination showed in both cases a locally advanced and high grade urothelial carcinoma of the pelvis allograft. After 24 and 14 months of followup, both patients are disease free.Conclusions. TCC of the kidney graft is an infrequent tumor that has only been reported in a few cases in the literature. It usually appears at a lower age, more often locally advanced, and with poor differentiation. A multidisciplinary approach to treatment should be required in these cases.

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Neuroendocrine Small-Cell Carcinoma of the Gallbladder: Case Report

Surgical Case Reports ◽

10.31487/j.scr.2020.01.03 ◽

2020 ◽

pp. 1-2

Author(s):

Carolina V. Canhoto ◽

Rui Oliveira ◽

Carolina V. Canhoto ◽

Fernando Azevedo ◽

José Guilherme Tralhão ◽

...

Keyword(s):

Neuroendocrine Carcinoma ◽

Neuroendocrine Tumours ◽

Clinical Presentation ◽

Locally Advanced ◽

Rare Condition ◽

Small Cell ◽

Rare Entity ◽

Incidental Diagnosis ◽

Neuroendocrine Small Cell Carcinoma ◽

Disseminated Disease

Gallbladder neuroendocrine carcinoma is a very rare entity, accounting for <0.2% of all neuroendocrine tumours [1]. Of the cases described in the literature, the initial presentation is usually locally advanced or even with disseminated disease [2]. In this paper we report the case of a 43-year-old female patient with an incidental diagnosis of gallbladder neuroendocrine carcinoma. Although clinical presentation is the most common, it is a very rare condition that poses a therapeutic and prognostic challenge.

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Delayed diagnosis of Peutz–Jeghers syndrome due to pathological information loss or mistake in family/personal history

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01900-7 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Yu-Liang Jiang ◽

Xiao-Dong Xu ◽

Bai-Rong Li ◽

En-Da Yu ◽

Zi-Ye Zhao ◽

...

Keyword(s):

Clinical Presentation ◽

Novel Mutation ◽

Delayed Diagnosis ◽

Personal History ◽

Pathological Examination ◽

The Novel ◽

Novel Mutations ◽

The Family ◽

Genetic Level ◽

Peutz Jeghers Syndrome

Abstract Objective To report Peutz–Jeghers syndrome (PJS) cases with non-definitive clues in the family or personal history and finally diagnosed through pathological examination and STK11 gene mutation test. Clinical presentation and intervention PJS was suspected in 3 families with tortuous medical courses. Two of them had relatives departed due to polyposis or colon cancer without pathological results, and the other one had been diagnosed as hyperplastic polyposis before. Diagnosis of PJS was confirmed by endoscopy and repeated pathological examinations, and the STK11 mutation test finally confirmed the diagnosis at genetic level, during which 3 novel mutation were detected (536C > A, 373_374insA, 454_455insGGAGAAGCGTTTCCCAGTGTGCC). Conclusion Early diagnosis of PJS is important and may be based on a family history with selective features among family members, and the pathological information is the key. The novel mutations also expand the STK11 variant spectrum.

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A Rare Entity of Benign Recurring Mesenchymal Tumor of the Female Urethra

Case Reports in Urology ◽

10.1155/2013/698456 ◽

2013 ◽

Vol 2013 ◽

pp. 1-2

Author(s):

Onur Telli ◽

Haşmet Sarıcı ◽

Berat Cem Özgür ◽

Cem Nedim Yücetürk ◽

Mehmet Ali Karagöz

Keyword(s):

Lower Urinary Tract ◽

Soft Tissue Tumor ◽

Benign Tumor ◽

Pathological Examination ◽

Rare Entity ◽

Female Urethra ◽

Mesenchymal Tumors ◽

Urinary Tract Symptoms ◽

Myxoid Matrix ◽

Lower Urinary

This is a case report of a 51-year-old female patient with benign mesenchymal tumors of paraurethral region which caused lower urinary tract symptoms. The pathological examination of the lesion was reported as angiomyxoma which is a distinct soft tissue tumor characterized by the presence of prominent myxoid matrix and numerous thin-walled blood vessels. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. It is a benign tumor, and total excision is curative. Recurrence is rare except for aggressive angiomyxomas.

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Distal Humerus as Delayed Site of Metastasis from Small Cell Carcinoma of Gallbladder

Case Reports in Gastrointestinal Medicine ◽

10.1155/2013/946835 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Mutahir A. Tunio ◽

Mushabbab AlAsiri ◽

Asma Mohammed F. Ali ◽

Eyad Fawzi AlSaeed ◽

Muhammad Shuja ◽

...

Keyword(s):

Cell Carcinoma ◽

Small Cell Carcinoma ◽

World Literature ◽

Case Reports ◽

Locally Advanced ◽

Distal Humerus ◽

Small Cell ◽

Rare Entity ◽

Dismal Prognosis ◽

Advanced Stages

Background. Small cell carcinoma (SCC) of the gallbladder is a rare entity and is often seen in elderly women. SCC of gallbladder is typically a nonsecretory carcinoid tumor without overt clinical symptoms and is often discovered at advanced stages. SCC of gallbladder carries a dismal prognosis as compared to SCC of lung and adenocarcinoma of gallbladder. To date, only 73 case reports have been published in the world literature.Case Presentation. Herein, we report a case of a 73-year-old Saudi woman who presented with one week history of right upper quadrant abdominal pain and obstructive jaundice and was found to be a case of locally advanced, metastatic SCC of gallbladder cT4N1M1 (liver, para-aortic lymph nodes, and bone). The patient was treated with neoadjuvant etoposide and cisplatin (EP) chemotherapy three cycles after biliary stenting followed by radical cholecystectomy, lymphadenectomy, and adjuvant EP chemotherapy and then one year later developed distal humerus osseous metastasis.Conclusion. SCC of the gallbladder is very rare entity and is often seen at advanced stages. Osseous metastases of peripheral skeleton from SCC gallbladder are rarely reported. Surgery is curative option but only for early stage tumors. Incorporation of chemotherapy along with radical resection increases the survival.

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Primary Angiosarcoma of the Bladder

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-1543-paotb ◽

2006 ◽

Vol 130 (10) ◽

pp. 1543-1547 ◽

Cited By ~ 5

Author(s):

Raja R. Seethala ◽

Jose A. Gomez ◽

Funda Vakar-Lopez

Keyword(s):

Data Extraction ◽

Locally Advanced ◽

Female Ratio ◽

Primary Angiosarcoma ◽

Study Selection ◽

Pathologic Features ◽

Male Female Ratio ◽

Bibliographic Search ◽

Primary Bladder ◽

Microscopic Pathology

Abstract Context.—Primary bladder angiosarcomas are extremely rare, and their clinical and pathologic features are not well described. Objective.—To further refine the clinical features of primary bladder angiosarcomas and define their pathologic spectra. Data Sources.—Relevant sources were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews. We also searched the M. D. Anderson pathology archives. Study Selection.—After excluding 4 cases that likely secondarily involved the bladder, we identified 9 true primary bladder angiosarcomas. Data Extraction.—Data were extracted on the following: demographics, clinical presentation, predisposing factors, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes. Data Synthesis.—Primary bladder angiosarcomas were found at a mean age of 64.2 years, with a male-female ratio of 8:1. Two cases arose in a postirradiation setting. Primary bladder angiosarcomas typically presented with hematuria and were grossly hemorrhagic, raised masses (mean size, 6.7 cm) of the trigone and/or dome. Histologically, most showed classic anastomosing channels lined by plump hyperchromatic cells, though many showed variant histology such as solid growth and epithelioid cytology. Three (43%) of 7 patients died within a year, but only 1 patient died with evidence of disease. The remaining patients were alive at the time of publication of their respective cases (mean, 22 months). Conclusions.—Primary angiosarcomas of the bladder are typically rare tumors of middle-aged and elderly men that present with locally advanced disease and show a wide histologic spectrum. However, their prognosis may be better than previously thought.

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Repeated Intravesical Instillations of an Adenoviral Vector in Patients With Locally Advanced Bladder Cancer: A Phase I Study of p53 Gene Therapy

Journal of Clinical Oncology ◽

10.1200/jco.2003.09.138 ◽

2003 ◽

Vol 21 (12) ◽

pp. 2247-2253 ◽

Cited By ~ 99

Author(s):

Lance C. Pagliaro ◽

Afsaneh Keyhani ◽

Dallas Williams ◽

Denise Woods ◽

Baoshun Liu ◽

...

Keyword(s):

Gene Therapy ◽

Bladder Cancer ◽

Gene Transfer ◽

Transitional Cell Carcinoma ◽

Cell Carcinoma ◽

Locally Advanced ◽

Transitional Cell ◽

P53 Gene ◽

Advanced Bladder Cancer ◽

Intravesical Instillations

Purpose: We investigated the feasibility, safety, and biologic activity of adenovirus-mediated p53 gene transfer in patients with locally advanced bladder cancer. Patients and Methods: Patients with measurable, locally advanced transitional-cell carcinoma of the bladder who were not candidates for cystectomy were eligible. On a 28-day cycle, intravesical instillations of INGN 201 (Ad5CMV-p53) were administered on days 1 and 4 at three dose levels (1010 particles to 1012 particles) or on either 4 or 8 consecutive days at a single dose level (1012 particles). Results: Thirteen patients received a total of 22 courses without dose-limiting toxicity. Specific transgene expression was detected by reverse transcriptase polymerase chain reaction in bladder biopsy tissue from two of seven assessable patients. There were no changes in p53, p21waf1/cip1, or bax protein levels in bladder epithelium evident from immunohistochemical analysis of 11 assessable patients. Outpatient administration of multiple courses was feasible and well tolerated. A patient with advanced superficial bladder cancer showed evidence of tumor response. Conclusion: Intravesical instillation of Ad5CMV-p53 is safe, feasible, and biologically active when administered in multiple doses to patients with bladder cancer. Observations from this study indicate that this treatment has an antitumor effect in superficial transitional-cell carcinoma. Improvements in the efficiency of gene transfer and the levels of gene expression are required to develop more effective gene therapy for bladder cancer.

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