Alveolar Soft Part Sarcoma of Urinary Bladder Occurring as a Second Primary Malignancy: A Case Report and Literature Re

[D.ap]

I question..

We report a case of bladder alveolar soft part sarcoma in an 18-year-old Thai male patient who had been treated with testicular radiation and systemic chemotherapy for acute lymphoblastic leukemia with testicular relapse. He presented with recurrent dysuria and gross hematuria. Cystoscopy revealed a 2-centimeter irregular sessile mass at the bladder base adjacent to left ureteral orifice. Transurethral resection of the tumor was performed. The histopathological diagnosis was alveolar soft part sarcoma. Chest and abdominal computed tomography showed no evidence of metastasis. He was treated with partial cystectomy and left ureteral reimplantation with negative surgical margin. No evidence of recurrence was found during a 28-month follow-up period with surveillance cystoscopy and computed tomography of the chest and abdomen.




https://www.researchgate.net/publicatio ... ure_Review

[D.ap]

Abstract
Alveolar soft part sarcoma (ASPS) represents <1% of all soft tissue sarcomas and harbors the ASPSCR1-TFE3 translocation, which is found in pediatric renal cell carcinomas arising after chemotherapy. We present the case of a female patient, treated for metastatic retinoblastoma (Rb) with surgery, radiation, and chemotherapy at age 21 months, who was diagnosed with ASPS of the bladder 5 years later when imaging revealed a polypoid mass arising from the left bladder wall. Endoscopic biopsy and tumor resection were performed. After histopathologic confirmation of ASPSCR1-TFE3 fusion-positive ASPS, negative margins were achieved with wide local excision. At 18 months post-surgery, she remains recurrence-free.



https://www.sciencedirect.com/science/a ... 6617302282

[D.ap]

What’s quite interesting is that both cases above where discovered after chemo/radiation treatments from 2 other cancers . ALL ( acute lymphoblastic leukemia) and retinoblastoma .

I believe we have a patient on immune therapy who had breast cancer then was discovered to have ASPS as well.
: /

http://www.cureasps.org/forum/viewtopic ... 322#p10108

[D.ap]

Also remembered a article talking of an ALL patient also devoloping ASPS..?


http://www.cureasps.org/forum/viewtopic ... mia#p10710

Oncologist report

Dear Editor,

DNA mismatch repair (MMR) is a system for recognizing and repairing errors, which occur during DNA replication and recombination. Biallelic deleterious germline mutations in MMR genes (MLH1, MSH2, MSH6, and PMS2) lead to constitutional MMR deficiency syndrome (CMMR-D).[1]

We are reporting two cases of CMMR-D in siblings of a family, who developed acute lymphoblastic leukemia (ALL) with glioblastoma multiforme and ALL with alveolar soft part sarcoma (ASPS) (first case in CMMR-D spectrum) respectively.[2]

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5379899/

[D.ap]

We all know statistically how likely we are to have ASPS.
It looks like the other 2 cancers plus breast cancer , are more much more likely to develope in patients .

The million dollar question is do they (the other cancers, genetics ) push the odds up for ASPS to result , sooner than later or in Pams case later than sooner .

[D.ap]

Any thoughts all?

[Olga]

You can not make any stats from few cases, and clinical medicine is a statistical based subject So basically no thoughts. Interesting, but inconclusive.