Ewing Sarcoma of Urinary Bladder Showing EWSR1 Rearrangement on FISH Analysis and Unique Response to Chemotherapy

Vallonthaiel, Archana George; Kaur, Kavneet; Jain, Deepali; Singh, Geetika; Tiwari, Deviprasad; Pramanik, Raja; Singh, Prabhjot; Sharma, Mehar Chand

doi:10.1016/j.clgc.2015.11.001

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…[ 18 , 19 ] Previously, the presence of EWS/FLI-1 fusion gene was detected in 92% of the cases using new assistive technologies. [ 13 ] In the patient discussed in the present study, a strong and diffuse immunoreactivity to the CD99 antigen was observed, especially for vimentin, synaptophysin, neuron-specific enolase, and CD56 antigen. A series of immunohistochemical results may be more helpful in the diagnosis of PNET of the bladder.…”

Section: Literature Review and Discussionmentioning

confidence: 48%

Primitive neuroectodermal tumor of urinary bladder

Gao

Xie

et al. 2020

Medicine

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Rationale: Primitive neuroectodermal tumor (PNET) of the urinary bladder is a highly aggressive tumor with high local recurrence and distant metastasis rates in cases of incomplete excision. We report a case of a young female patient, in whom early laparoscopic radical cystectomy combined with standard lymph node dissection and a modified vincristine, doxorubicin hydrochloride, and cyclophosphamide (VAC) chemotherapy regimen was controversial. Because PNET of the urinary bladder is a rare malignancy, the standard treatment regimen has not yet been established. It is not clear whether surgery combined with postoperative chemotherapy for PNET patients may be superior to surgery alone on long term survival. Patient concerns: The patient was a 45-year-old Chinese woman who complained of lower urinary tract symptoms, including urgency, frequency, and difficulty in urination, for 2 months. Diagnoses: PNET. Interventions: The patient underwent laparoscopic radical cystectomy and standard lymph node dissection, combined with modified VAC chemotherapy regimens. Outcomes: After undergoing radical surgery in 2018, the patient completed 6 courses of adjuvant chemotherapy. Abdominal and thorax computed tomography scanning was performed 3, 6, 9, and 12 months after the surgery was completely free of tumor. The patient is still alive with no signs of recurrent disease 2 years after diagnosis. Lessons: Radical surgery and standard lymphadenectomy combined with adjuvant chemotherapy may be essential to improve the prognosis of PNET of the urinary bladder.

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Section: Literature Review and Discussionmentioning

confidence: 48%

Primitive neuroectodermal tumor of urinary bladder

Gao

Xie

et al. 2020

Medicine

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“…Of the total of 346 suspected ESFT, 44 (20), leg (4), popliteal fossa (2), gluteal region (5), cheek (2), orbit (2), nose and nasopharynx (2), scalp (1), neck (7), parapharyngeal (2), chest wall (29), mediastinum and thorax (5), paraspinal (20), abdomen and retroperitoneum (14). Visceral tumours were noted in 34 (22.22%) patients and the sites included ovary (4), cervix (3), vagina (1), kidney (7), bladder (2), prostate (1), lung (4), breast (5), lymph node (3), intestine (2), and brain (2).…”

Section: Resultsmentioning

confidence: 99%

“…Further ESFTs in bladder and prostate noted in this cohort were rarely reported. [42][43][44][45][46][47] Metastatic disease involving lung, lymph nodes, and several organs has been described 12,48,49 ; however, primary ESFTs of breast, [50][51][52] lung, [53][54][55] intestine, 56,57 and brain 22,[58][59][60][61][62] were rarely reported.…”

Section: Clinical Pathologymentioning

confidence: 99%

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Murthy

Challa

Raju

et al. 2020

Clin Med�Insights�Pathol

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Introduction: The diagnosis of Ewing sarcoma family of tumours (ESFT) is challenging, especially in adults and in extra-skeletal or visceral location. Several morphologic mimics with varied treatment options and prognosis confer diagnostic dilemmas. Application of ancillary diagnostic modalities in surgical pathology in clinical routine has enabled accurate diagnosis of ESFT in bone, soft tissues, and viscera. Aim: The study aims to assess the clinicopathological features including molecular test results of ESFT with emphasis on sex, age, and location, especially extra-skeletal soft tissue and visceral location. Material and Methods: Data of clinicopathological, molecular tests (wherever performed), diagnosis rendered in 302 ESFT over a decade from our centre were reviewed. Statistical comparison of skeletal and extra-skeletal tumours with reference to age and sex was done using SPSS package. The P value of <.05 was considered significant. Results: The cohort included 302 ESFTs with 49% skeletal and 51% extra-skeletal tumours. Thigh was most common site among skeletal tumours; chest wall, paraspinal location, and retroperitoneum among soft tissues (39.4%); and kidney, ovary, and cervix among visceral tumours (11.3%). Fluorescence in situ hybridisation for EWSR1 gene rearrangement was positive in 54 patients and reverse-transcriptase polymerase chain reaction in 19 patients. Predominance of male sex, younger age and location in extremities among skeletal tumours and lack of gender predilection, higher age and axial location in extra-skeletal tumours were noted, which were statistically significant. Molecular tests were performed more frequently in extra-skeletal tumours, especially in visceral tumours to establish the diagnosis. Conclusions: The study showed statistically significant differences in the age, sex, and location between skeletal and extra-skeletal ESFT. The increased percentage of extra-skeletal tumours especially in viscera was attributed to the increased awareness and availability of ancillary techniques.

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