Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Alveolar soft part sarcoma: the new primary intracranial malignancy


Abstract

The purpose of this paper is to serve as a reference to aid in the management of this poorly understood intracranial malignancy. The authors report their experience treating the eighth ostensible case of a primary intracranial alveolar soft part sarcoma (ASPS). A 21-year-old man presented to hospital after collapsing. He gave a 1-year history of headache, a 2-month history of reduced visual acuity and on examination had left facial paraesthesia with left-sided incoordination. MRI of the brain revealed a large left posterior fossa mass. The patient underwent resection of the tumour with good recovery in function. Immunohistochemical analysis of the tumour specimen confirmed an ASPS, and multimodal imaging in search of an extra-cranial disease primary was negative. A review of the literature yielded only seven other cases of primary intracranial ASPS. A variety of diagnostic imaging modalities were employed in search of a disease primary, as were various combinations of surgical resection, chemotherapy and radiotherapy as treatment. Half of the cases documented delayed disease recurrence. The authors discuss the following: the unique radiological and immunohistological characteristics of this disease including the potential for its misdiagnosis; the investigations required to diagnose a primary intracranial ASPS; the efficacy of current medical and surgical treatment options and the factors that will aid in prognostication. This is the first review of this new primary intracranial malignancy. From our analysis, we offer a joint radiological and immunohistochemical algorithm for the diagnosis of primary intracranial ASPS and specific operative considerations prior to resection.




https://link.springer.com/article/10.10 ... 017-0874-4

Extra-cranial disease primary might have had a spontaneous regression, there were cases like this in ASPS

Hi Olga
What’s your understanding , to the regression of known cases ?
Maybe as a result of the primary having been removed prior? And no known removal from a patients knowledge ?
So consequently there was a prior primary ??

I truly am at a loss..

I am not saying that the primary in a brain is not possible, but rather that since there are known (rare) cases of the spontaneous regression of the primary in cancers and sarcomas that are immunogenic (able to attract the immune system to attack it under certain conditions), it is hard to say if this is a true primary versus the late metastasis from the dormant cells that were spread from the primary before it has regressed. Dormant cells might have stayed unnoticed by the immune system? We already know that ASPS is immunogenic and the immune system is able to recognize and attack it and even form the long term memory about it.
Cases of the spontaneous regression of the primary were the reason Coley created his concept of the cancer vaccine. His works started the process that in our life time came to the immunotherapy result for our kids.
Some reading:
Immunity over inability: The spontaneous regression of cancer
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3312698/

Olga
Thanks for the clarification 😊
I absolutely agree that the folks in the study above could of indeed had prior primaries that either were resected and misdiagnosed as something other than ASPS
AND or the primary resolved but not without having sent out metastasis.
Thanks for the article
I’ll read it later .

Olga wrote: ↑Thu Oct 01, 2020 9:14 pm I am not saying that the primary in a brain is not possible, but rather that since there are known (rare) cases of the spontaneous regression of the primary in cancers and sarcomas that are immunogenic (able to attract the immune system to attack it under certain conditions), it is hard to say if this is a true primary versus the late metastasis from the dormant cells that were spread from the primary before it has regressed. Dormant cells might have stayed unnoticed by the immune system? We already know that ASPS is immunogenic and the immune system is able to recognize and attack it and even form the long term memory about it.
Cases of the spontaneous regression of the primary were the reason Coley created his concept of the cancer vaccine. His works started the process that in our life time came to the immunotherapy result for our kids.
Some reading:
Immunity over inability: The spontaneous regression of cancer
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3312698/

Olga had brought up the immune response having been studied as early as Coleys era and supplied an article to read .
Within that article there was made mention of an even earlier report and documention of spontaneous tumor regression by a St Peregrine.

So consequently it’s been termed St Peregrine , when a regression is seen in a tumor , after in this case infection boosted the immune response to immune edit the immune system to attack the tumor verses ignore the tumor and let it proliferate.


The biography of the immune system and the control of cancer: from St Peregrine to contemporary vaccination strategies.


https://bmccancer.biomedcentral.com/art ... 407-14-595

https://bmccancer.biomedcentral.com/art ... 407-14-595

The biography of the immune system and the control of cancer: from St Peregrine to contemporary vaccination strategies.


Abstract

Background

The historical basis and contemporary evidence for the use of immune strategies for prevention of malignancies are reviewed. Emphasis is focussed on the Febrile Infections and Melanoma (FEBIM) study on melanoma and on malignancies that seem to be related to an overexpression of human endogenous retrovirus K (HERV-K).

Discussion

It is claimed that, as a result of recent observational studies, measures for prevention of some malignancies such as melanoma and certain forms of leukaemia are already at hand: vaccination with Bacille Calmette-Guérin (BCG) of new-borns and vaccination with the yellow fever 17D (YFV) vaccine of adults. While the evidence of their benefit for prevention of malignancies requires substantiation, the observations that vaccinations with BCG and/or vaccinia early in life improved the outcome of patients after surgical therapy of melanoma are of practical relevance as the survival advantage conferred by prior vaccination is greater than any contemporary adjuvant therapy.

Summary

The reviewed findings open a debate as to whether controlled vaccination studies should be conducted in patients and/or regions for whom/where they are needed most urgently. A study proposal is made and discussed. If protection is confirmed, the development of novel recombinant vaccines with wider ranges of protection based, most likely, on BCG, YFV or vaccinia, could be attempted.